Case Studies in Pediatric Dysphagia 1635503973, 9781635503975

Table of contents :
Introduction
Acknowledgments
Interviewees
Reviewers
Chapter 1. Differential Diagnosis in Pediatric Dysphagia
Chapter 2. Genetic Syndromes
Chapter 3. Aerodigestive Management of Swallowing Disorders
Chapter 4. Gastrointestinal Diagnoses
Chapter 5. Trauma-Informed Care
Chapter 6. Pulmonary Diagnoses
Chapter 7. Traumatic Brain Injury
Chapter 8. Ethical Considerations
Conclusion
Index

Citation preview

Wilson

Through clinical cases and interviews with working professionals, Case Studies in Pediatric Dysphagia takes an original approach to understanding pediatric dysphagia. This text, designed to address the increased need for clinical education, covers topics such as differential diagnosis, medical terminology, and goal setting. Written with group interaction and collaboration in mind, the book provides role-play scenarios, suggestions on how to delve deeper into the case studies, integrating interprofessional practice, and evidencebased treatment approaches. Each chapter includes a case study and a detailed interview with a pediatric dysphagia professional, providing a complete and thorough view of the topic in a reflective and practical way.

Key Features:

• Gives guidance for integrating interprofessional practice as it relates to pediatric dysphagia • Written to prompt the reader to shift toward implementation science, merging research and clinical practice to better serve patients with pediatric dysphagia • Informative interviews with specialists working and conducting research in the field • Offers additional resources at the end of each chapter for the reader to continue their learning on specific case areas of focus • Provides a deeper understanding of cultural considerations and how to incorporate them into practice patterns • Allows students and professionals to integrate both diagnosis and treatment choices and explores current evidence-based approaches to treatment Both speech-language pathologists and clinical educators will benefit from having this valuable resource on their bookshelves.

Jennifer J. Wilson, MA, CCC-SLP, BCS-S, is a Clinical Assistant Professor in Speech Pathology at the University of Tennessee Health Science Center. She has been practicing for 30 years, providing services in children’s hospitals, private practice, and home-based services. She enjoys teaching graduate students and young professionals about pediatric dysphagia through use of case studies to develop skills in clinical problem solving.

www.pluralpublishing.com

CASE STUDIES in Pediatric Dysphagia

• Provides increased opportunities for students and young professionals to gain experience with complex clients before engaging in intervention

CASE STUDIES in

Pediatric Dysphagia Jennifer J. Wilson

CASE STUDIES in

Pediatric Dysphagia

CASE STUDIES in

Pediatric Dysphagia Jennifer J. Wilson, MA, CCC-SLP, BCS-S

9177 Aero Drive, Suite B San Diego, CA 92123 email: [email protected] website: https://www.pluralpublishing.com Copyright ©2023 by Plural Publishing, Inc. Typeset in 11/13 Garamond by Flanagan’s Publishing Services, Inc. Printed in the United States of America by Integrated Books International All rights, including that of translation, reserved. No part of this publication may be reproduced, stored in a retrieval system, or transmitted in any form or by any means, electronic, mechanical, recording, or otherwise, including photocopying, recording, taping, web distribution, or information storage and retrieval systems without the prior written consent of the publisher. For permission to use material from this text, contact us by Telephone: (866) 758-7251 Fax: (888) 758-7255 email: [email protected] Every attempt has been made to contact the copyright holders for material originally printed in another source. If any have been inadvertently overlooked, the publisher will gladly make the necessary arrangements at the first opportunity. Library of Congress Cataloging-in-Publication Data: Names: Wilson, Jennifer J. (Speech-language pathologist), author. Title: Case studies in pediatric dysphagia / Jennifer J. Wilson. Description: San Diego, CA : Plural Publishing, Inc., [2023] | Includes bibliographical references and index. Identifiers: LCCN 2022056083 (print) | LCCN 2022056084 (ebook) | ISBN 9781635503975 (paperback) | ISBN 1635503973 (paperback) | ISBN 9781635503982 (ebook) Subjects: MESH: Deglutition Disorders--diagnosis | Deglutition Disorders--therapy | Deglutition--physiology | Diagnosis, Differential | Infant | Child | Case Reports | Interview Classification: LCC RC815.2 (print) | LCC RC815.2 (ebook) | NLM WI 258 | DDC 616.3/23--dc23/eng/20230130 LC record available at https://lccn.loc.gov/2022056083 LC ebook record available at https://lccn.loc.gov/2022056084

Contents Introduction vii Acknowledgments xv Interviewees xvii Reviewers xix

Chapter 1. Differential Diagnosis in Pediatric Dysphagia

1

Chapter 2. Genetic Syndromes

27

Chapter 3. Aerodigestive Management of Swallowing Disorders

57

Chapter 4. Gastrointestinal Diagnoses

79

Chapter 5. Trauma-Informed Care

105

Chapter 6. Pulmonary Diagnoses

135

Chapter 7. Traumatic Brain Injury

161

Chapter 8. Ethical Considerations

179

Conclusion 199 Index 201

v

Introduction It’s March 2020. The week before there was speculation, but I received the email notifying me that the University of Tennessee was closed due to COVID-19. Suddenly, it was time to get “up to speed” on how to do clinical simulations and teletherapy. I contacted Plural Publishing and asked for permission to use the case studies in Pediatric Swallowing and Feeding by Arvedson, Brodsky, and Lefton-Greif. I had read these case studies during my lunch break every day as I was preparing for the American Board of Swallowing and Swallowing Disorders exam. They were challenging, but the thing I loved about them was the stories that they told. The journey began. As clinical faculty, we were learning the clinical simulation process quickly. Through Simucase, we were introduced to new terms, like debrief. I wrote up a protocol for the case studies as simulation tools and the meetings began. First, the students were sent case studies to read independently. Next, either one-on-one or small group meetings began. It was a front-porch clinical simulation at its finest. Students read the case studies out loud, learning how to attack the new terms. After each paragraph, we stopped and rehashed what had been read. How old was the child? What would we expect developmentally at this age? As the cases unfolded, the layers of the story emerged. Students were able to imagine parental perspectives, role-play relaying results of modified barium swallow studies, and discuss oral sensorimotor findings. As we began doing the cases, previous cases came to mind. I shared anecdotal stories. Connections were made through bringing these cases to life that were different from any supervision experience I had provided. We spent time talking about what we had learned and what therapeutic approaches might have been good to try if you had seen that client for therapy. The takeaways from each case helped provide a sense that others’ perspectives were taken, new terms were learned, and skills in professional discussion were developing in these students. A few months later, the university clinic reopened. The in-person cases slowly started again. However, I knew that I had been changed as a supervisor. I was inspired to open a dialogue about differential diagnosis, vii

viii  CASE STUDIES IN PEDIATRIC DYSPHAGIA

assessments, and referrals, considering that every case in pediatric dysphagia was unique. I was also ready to have conversations about intervention. There is so little empirical evidence to support pediatric dysphagia intervention. This population is so difficult to research because each case is so unique. I wanted to begin to “talk about the elephant in the room.” We are always talking about how there is so little research, but the bottom line is these children can’t wait for the research. We must do the best that we can for them with deep learning regarding oral and pharyngeal anatomy and physiology and rely on our medical counterparts to support our intervention. We need to spend some time talking with each other and listening carefully. We need to encourage a culture of wisdom and experience through mentorship. Each chapter culminates with an expert interview. Some were researchers, and some were experienced clinicians. The strides they have made for our profession through research and intervention are incredible. The perspectives shared were so varied and provided so much more depth than I would have ever imagined. I am so grateful to the professionals who helped us understand research, unique training, and future directions of our profession. The book content won’t be timeless. Studies will be completed so research findings can be applied. Technology will improve the way that we evaluate and treat. Yet, discussions sharpening our ability to provide the best care for our patients can move us forward as clinicians.

Introductory Interview Rebecca Smith, MS, CCC-SLP, CNT, is a clinical educator/adjunct instructor. She is a Certified Neonatal Therapist, Voice and Dysphagia at Appalachian State University. She is also pursuing her clinical doctorate. Jennifer Wilson ( J): Tell us about your current work in pediatric dysphagia. Rebecca Smith (R): I work in a medical focus university-based clinic in a rural community. I see a lot of variety throughout my day. I’m working toward developing an infant and pediatric feeding and swallowing program in the clinic. To do that, I have been focused on making sure that I am myself up to date in evidence-based practice patterns. I want to make sure that I am using the best patient-reported outcome measures, that I’m making connections with providers in the area, and that I’m also

INTRODUCTION   ix

supplying resources to students. A lot of them receive little pediatric dysphagia-focused coursework in their graduate programs. Our university is different in that we do have an elective pediatric dysphagia course that they can take. I’m planning on teaching in the fall. I’m developing that coursework and planning. It’s tying together nicely for my interest. J: You are also working on developing your dissertation, right? R: In this process of developing my research interests, I began realizing how teaching and research were coming together as I was developing this program in the clinic. I talked to mentors in the fields who have done similar things at their university-based clinic, people in the field of pediatric dysphagia I really respect. They all commonly were telling me about the infant child feeding questionnaire tool as developed by Feeding Matters. They expressed the importance of making sure that I’m capturing the four domains of pediatric feeding disorder in my intake paperwork for patients and then also in my evaluations and making sure that I’m educating students about them. Getting that consistency in talking to people, this sparked an interest in looking more at, “Do providers understand the four domains of pediatric feeding disorder as established by Goday et al. consensus paper, with support from Feeding Matters. What are providers currently doing now to show patients with disordered feeding? If they’re given education on the four domains and introduced to the six-item screening tool that was developed by Feeding Matters and colleagues, where if there’s two or more positives on the screening tool, then it is indicative of a need for a possible referral to feeding specialists, will that result in early identification of pediatric feeding disorder? Will it result in a change of practice? How will increased awareness be reflected in their clinical practice patterns?” I think just really marinating on those thoughts helped me focus my research interests and as I’m developing my dissertation. I know that I’m going to have to narrow that significantly just for the purposes of getting my PhD finished but I see myself continuing in this line of research. J: Talk a little bit about your earlier work leading up to your transition now into the academic world. R: Most of my clinical background was in acute care, specifically in the neonatal intensive care unit. I also did objective swallow studies for outpatient pediatrics. Patients would come to the hospital to get a study and then they would go back to their clinic. They were treated by whoever referred them for this study. I really enjoyed the hospital

x  CASE STUDIES IN PEDIATRIC DYSPHAGIA

setting. I enjoyed the variety of my day and the pace, but I felt that in my next position, I wanted to make a different kind of difference. J: Talk about how you made that transition from a community hospital to the university-based clinic. R: I was at a point in my career where my interest was moving toward investigating best practice and how to best educate students in my area of clinical interest. That led me to start a PhD program. I was thinking, I’m in this program, I want to go somewhere that would support me and continuing this academic journey, a place that’s going to have the resources for me to do the research that I want to do and be able to measure change in students’ learning. The hospital certainly was supportive of me getting my PhD, but it wasn’t really something that I would have used there in the clinic and then clinical setting at the hospital because it wasn’t a university-based hospital or it was a community hospital and, in that setting, I was able to make connections in the community. It gave me the opportunity to educate more generic providers on specialty services like talking a lot about trauma-informed care in the NICU (neonatal intensive care unit) and how to provide counseling and communicating with patients and families that are in a vulnerable stage in their disease journey. They taught me a lot of important things but ultimately when I was interested in continuing with my career, I was ready to make a change. J: Tell me about your academic training in pediatric dysphagia. R: My graduate program I thought was a great program, but I left it with little knowledge of pediatric dysphagia and even less knowledge of infant feeding. I think that was partially because of the rurality of our program. Also, it was several years ago, and I feel like pediatric dysphagia was just covered as a part of the regular dysphagia coursework, which truthfully is how it is in a lot of places still now. I think that there is more of an emerging recognition of the need for more education, and we need to talk about it more. I think that now courses often include more sessions on pediatric dysphagia. J: Post graduate school, what have been some strategies for you that you have used in getting skills in pediatric dysphagia? R: For neonatal practice, I went to work at a large university-based hospital and that was a requirement of mine to work there, to be trained in the NICU. I didn’t know anything about it. I was willing to learn, and they had a set of competencies that I was expected to achieve through continuing education and reading of research and then completion of skills check-offs. When you’re fortunate enough

INTRODUCTION   xi

to be in a situation like that where someone is handing you what you’re supposed to know, it makes your learning journey a lot easier. J: Have you talked to other speech pathologists about similar experiences? R: I can tell you that most people are not in that situation. They take over a caseload in their CFY (clinical fellowship year) and a lot of times their CFY supervisor is not well versed in that subject. I know several former students who are experiencing that in their pediatric feeding disorder caseload. J: Talk about how we can continue to grow and learn as feeding and swallowing specialists. R: Even here at this university-based clinic, I’m really the first person that has had these research and clinical interests. I’ve had to really take responsibility for my own learning in a way that a lot of other people have many times before me. I am currently doing that through reading research, setting up mentors in the field (that was huge for me), and then taking sound continuing education courses. I think this is most challenging in pediatric dysphagia because there are so many types of courses that aren’t necessarily grounded in evidence. You really must be a consumer of literature and surround yourself with people you trust to further your own education. I feel like I’ve done that where I’m now because I had a lot of diagnostic background but maybe not as much treatment background. I find myself taking coursework and really reading literature, speaking to occupational therapists, speaking to other people in the field who have a lot of knowledge about growth and fine motor development, neural development. In pediatric feeding disorder, you must look at everything, not just one piece of the puzzle. J: Talk a little bit about challenges that feeding and swallowing specialists face in rural areas. R: Well, I think there are a lot of challenges. I talk to my peers about that a lot. I think one of the biggest challenges that I face now is that there are a lot of pediatricians but not as many pediatrics specialties services such as GI (gastrointestinal), pediatric ENT (ear, nose, and throat). There are a lot of providers in town who are generalists. They must see a lot of different things because they just don’t have the ability to specialize as much as they might like. There is a lack of provider presence. Oftentimes, when we need our patient to see pediatric GI, we must refer them to a place that’s a couple of hours away. Transportation can often be an issue for these patients with

xii  CASE STUDIES IN PEDIATRIC DYSPHAGIA

weather-related issues and travel expenses. Getting an appointment is a problem sometimes. Some of these patients just can’t afford to wait. That is a huge barrier. Access to instrumentation can be a problem. It’s not a problem for me. I’m fortunate about that but it is for some places that are rural, and I think that the lack of communication between specialty providers and the clinician that ends up seeing the patient in their rural hometowns can be a challenge. They don’t always get all the report information. They don’t really know what happened because they didn’t go with the patient to the hospital and watch the instrumental study. Oftentimes, people in small private practices who are treating patients for pediatric feeding disorder have funding issues. There are ways to circumvent that if you educate yourself. They just must work a little bit harder in providing an evidence-based practice that is also possible and financially helpful. We have a good amount of access to supplies. I feel fortunate. Not all home health practitioners have everything they need. Sometimes parents are the ones that must advocate for their children’s medical supplies, but they don’t even know where to begin. You can feel really disconnected I think in rural areas. J: How do you feel that we can bridge the gap between clinical work and research in pediatric dysphagia? R: A wonderful way to bridge the gap would be for there to be more individuals interested in pediatric dysphagia in academia. I think that would be a fantastic way to start bridging that gap. Also, for there to be more of a specific course and clinical rotation-based focus in pediatric dysphagia for those individuals who are interested in pursuing that after graduation. Involving the students in research because they’re often excited to have opportunities to be involved in projects like that. That would be a wonderful way. Even those in academia who do not specialize in pediatric dysphagia could still create working relationships and research relationships with clinicians who also work in the field and who are not necessarily connected to an academic institution who have a research interest. I know of several. They don’t have an academic background or they’re not currently pursuing a terminal degree, but they’re super interested in contributing to research. I think to get that going, they often need a connection to someone at an academic institution. R: There are some who don’t work directly for a specific university, but they work in teaching-based hospitals and are given research time but that is the exception not the rule. Most people, like myself, if I was going to be contributing to research in infant child feeding

INTRODUCTION   xiii

and swallowing when I was still at my last hospital, that would have been something I would have had to do on my own time. I wouldn’t have had time in my day to really do that. It’s simply hard for people to have a research interest, a full clinical load, and then keep any kind of work-life balance. I struggle with it. I’m doing a little better here just because I have more flexibility in my schedule than I did when I was in the hospital. It’s a problem that we talk about a lot, but there doesn’t seem to be a lot of solutions for it, at least one that’s easy. I think that pediatric dysphagia specialists who are in academia need to step up in their contributions to research. So many clinicians are waiting for some of the methods they use to be validated. There’s just such a lag in generating evidence-based creation of treatment practices and evaluation methods in this patient population and a lack of actual validation of those methods. Because of that, I think it creates a lot of polarization in choosing specific modalities and it’s very confusing for new graduates where to go. I think a lot of it starts with people in academia asking questions and getting the data that is needed for clinicians to feel like they’re choosing the best evaluation and treatment approaches. That can’t really happen until people in academia step up and do more studies. J: I love the way you were so transparent there about how it can feel uncomfortable as a recent graduate when you’re wanting to follow evidence-based practice and you really must rely so heavily on clinical judgment. In that triad, that’s really where we are in pediatric dysphagia and put that pressure on young clinicians that recently graduated who don’t have a lot of clinical experiences is a precarious position, isn’t it? R: It is and they’re more likely to get a job in pediatric and feeding and swallowing-based programs than they are in adult dysphagia, truthfully. Most home health, EI (early intervention), and private practice, places like that are what’s going to hire these new clinicians, because there’s such a shortage. They don’t have a knowledge base and it’s very scary for them. I also think that clinicians must challenge themselves. Researchers must rise to the occasion of doing more research in this area if they are able to do so. Clinicians must step up and read what little research on specific methods and find mentors and they must do things like that to achieve the best outcomes for their patients. They should never really be in a place where they’re doing the same thing as 10 years ago that they’re doing today.

Acknowledgments I am deeply appreciative of the many mentors throughout my academic and professional training who directed me progressively toward pediatric dysphagia. It began with education. Here are some educators whose words shaped my clinical practice: Dr. Marilyn Newhoff, Dr. Patricia McCarthy, Dr. Carol Swindoll, Dr. Joel Kahane, Ms. Alicia Ashcraft, and Ms. Sallie Hillard. As a clinical fellow, I continued to find mentors in Dr. Jeri Logeman, Dr. Monica Wojick, Mr. Robert Beecher, Dr. Joan Arvedson, and Dr. Miriam Weinstein. There are countless others who continue to shape my practice. Thank you to so many who provided advice and direction during this project. Dr. Devin Casenhiser helped shape the text format through his suggestion about interviews with leaders in the field. Susan Tucker provided insight into the best way to edit the interviews to improve their readability. My children and grandchildren, Wyeth, Eli, Ella, and Jackson, provided me with better insight into pediatric dysphagia than any other professional experience. Most important, my husband Danny Wilson spent countless hours providing illustration/design and an ear to bend throughout the editing process.

xv

Interviewees Denise Chapman, MS, CCC-SLP Speech-Language Pathologist Monroe Carell Jr. Children’s Hospital at Vanderbilt Nashville, Tennessee Memorie Gosa, PhD, CCC-SLP, BCS-S Associate Professor and Chair Communicative Disorders The University of Alabama Tuscaloosa, Alabama Katlyn Elizabeth McGrattan, PhD Assistant Professor Department of Speech-Language-Hearing Sciences University of Minnesota Minneapolis, Minnesota Heidi Liefer Moreland, MS, CCC-SLP, BCS-S, CLC Clinical Coordinator Thrive Tube-Weaning Program Alexandria, Virginia Rebecca F. Smith, MS, CCC-SLP, CNT Clinical Educator/Adjunct Instructor Department of Communication Sciences and Disorders Appalachian State University Boone, North Carolina Miriam Weinstein, MD Physical Medicine and Rehabilitation Specialist University of Tennessee Health Science Center Memphis, Tennessee

xvii

xviii  CASE STUDIES IN PEDIATRIC DYSPHAGIA

Emily Wojtowicz, MS, RD, CSP, LD, IBCLC Doctoral Candidate, Community Nutrition University of Tennessee Knoxville, Tennessee Robert Yost, CRT Certified Respiratory Therapist East Tennessee Children’s Hospital Knoxville, Tennessee Emily Zimmerman, PhD Associate Professor Communication Sciences & Disorders Northeastern University Boston, Massachusetts

Reviewers Chelsea Bell, MA, CCC-SLP Pediatric Feeding Specialist Chelsea Bell SLP, LLC Denver, Colorado Christopher Bolinger, PhD, CCC-SLP Assistant Professor Department of Communication Sciences and Disorders Texas Woman’s University Denton, Texas Allison Tidwell Brown, MS, CCC-SLP, BCS-S Speech-Language Pathologist Children’s Health Medical Center Dallas, Texas Lori Caplan-Colon, MS, CCC-SLP Speech-Language Pathologist Founder of Montclair Speech Therapy Montclair, New Jersey Melissa Carrier-Damon, MA, CCC-SLP, BCS Program Development Manager/Speech-Language Pathologist American Board of Swallowing and Swallowing Disorders Jamie Fisher, PhD, CCC-SLP Adjunct Professor Western Kentucky University Bowling Green, Kentucky Laura H. Loveless, MS, CCC-SLP, BCS-S, CLC Speech-Language Pathologist Texas Children’s Hospital Houston, Texas xix

xx  CASE STUDIES IN PEDIATRIC DYSPHAGIA

Jenan Maaz, MS, CCC-SLP, CBIS Acute Care Speech-Language Pathologist Doctor of Speech-Language Pathology Candidate at Northwestern University Towino Paramby, CScD, CCC-SLP, BCS-S Associate Professor Department of Communication Sciences and Disorders University of Central Arkansas Conway, Arkansas Julie Raplee, MS, CCC-SLP Assistant Professor and Chair St. Joseph’s University Brooklyn, New York Shannon W. Salley, SLPD, CCC-SLP Assistant Professor Department of Communication Sciences and Disorders Longwood University Farmville, Virginia Bonnie K. Slavych, PhD, CCC-SLP, ACUE Assistant Professor Missouri State University Springfield, Missouri Karin Thomas, SLPD, CCC-SLP Assistant Professor Saint Mary’s College Notre Dame, Indiana Jan Traughber, EdD, CCC-SLP Associate Professor Department of Communication Sciences and Disorders Harding University Searcy, Arkansas

1 Differential Diagnosis in Pediatric Dysphagia INTRODUCTION Pediatric feeding disorder (PFD) has been defined as impaired oral intake that is not age appropriate and is associated with medical, nutritional, feeding skill, and/or psychosocial dysfunction (Goday et al., 2019) (Figure 1–1). Recent incidence data provide an estimate of the annual incidence of PFD in the United States to be between 1 in 23 and 1 and 37 for children under 5 years of age. The incidence for children under 5 years of age with chronic diseases was between 1 in 3 and 1 in 5 (Kovacic et al., 2021). As PFD becomes more clearly defined and there is more clarity about an increased incidence, organizations like Feeding Matters and Dysphagia Research Society have helped provide networking and resources to professionals engaged in research, education, and intervention of pediatric

Medical

Nutrition

Feeding skill

Psychosocial

Figure 1–1.  Four key domains associated with pediatric feeding disorder. 1

2  CASE STUDIES IN PEDIATRIC DYSPHAGIA

dysphagia (Dysphagia Research Society, 2022; Feeding Matters, 2022). Evaluation tools to better screen and evaluate skills have been introduced and assessed for test-retest reliability (Silverman et al., 2020; Thoyre et al., 2018). However, the literature to support feeding and swallowing intervention has not emerged in alignment with these other advancements. In their 2016 article, “Pediatric Feeding/Swallowing: Yesterday, Today, and Tomorrow,” Lefton-Greif and Arvedson (2016) postulate that the oralmotor and sensory-based intervention strategies used in treatment lack evidence-based support in the literature. As the literature is combed, this point has resonated repeatedly. Oral and pharyngeal treatment studies are characterized by limited sample sizes, diverse populations, and differing intervention techniques. The need for further research was also a common point of discussion throughout the expert interviews of this text. While the framework is laid to better guide intervention, a scoping review revealed gaps in research of pediatric feeding disorder. Another gap mentioned was limited to first authorship in the domains of feeding skill and nutrition. Authors also cited that though research is occurring, it is not well represented in non-Western countries (Estrem et al., 2022). Before grappling with the lack of evidence to support intervention, feeding and swallowing specialists should be trained in clinical reasoning. Researchers cite that the use of treatment strategies should be supported through applying scientific therapy practice (Gosa & Dodrill, 2017). Another good place to start is by having a firm foundation of knowledge in normal feeding and swallowing (Figure 1–2). As more studies emerge regarding normal feeding and swallowing skills at specific ranges of development (Delaney et al., 2021), further support will be in place to guide intervention practices with this heterogeneous population. More information about cultural differences must be considered (Hall & Johnson, 2020). Gaining more data about parent and caregiver roles in development and intervention must also be researched. Evie’s case study provides a window into the complexities of diagnosing feeding skill issues, managing a multifactorial case (e.g., potential gastrointestinal issues, lack of caregiver consensus), and understanding

DEVELOPMENTAL STAGES IN INFANT AND TODDLER FEEDING https://infantandtoddlerforum.org/media/upload/pdf-downloads/3.5_ Developmental_Stages_in_Infant_and_Toddler_Feeding_NEW.pdf FEED YOUR BABY & TODDLER RIGHT: EARLY EATING AND DRINKING SKILLS ENCOURAGE THE BEST DEVELOPMENT Author: Diane Bahr, MS, CCC-SLP, CIMI Figure 1–2. Supplemental resources to learn more about normal feeding and swallowing skills.

1.  DIFFERENTIAL DIAGNOSIS IN PEDIATRIC DYSPHAGIA   3

the importance of caregiver education and communication in managing acute issues (e.g., spitting out food, refusing food). Evie lived in two different housing environments during treatment. She lived with both her foster parents and had increased visitation with her biological mom. The potential for lack of health equity for Evie was considerable given the differences between the two environments. Throughout the intervention, there was increased time spent with her biological mom. These transitions and the lack of reporting from her biological mom make it difficult to determine her response to intervention. Following the case study, Dr. Katlyn McGrattan discussed the importance of considering swallowing physiology in determining our course of intervention. She drove home the point that we must use the best evidence available through a thorough understanding of anatomy and physiology. She also stressed the importance of relying on a team of professionals.

CASE STUDY:  EVIE Case Study Medical History Episode 1:  Evie was referred for outpatient services at 9 months of age adjusted due to difficulty transitioning to solid foods. (See Figures 1–3 and 1–4 for images of a 9-month-old and 3-month-old.) Both her foster mother and biological mother were present and provided relevant information.

Figure 1–3. Nine-month-old.

Figure 1–4. Three-month-old.

4  CASE STUDIES IN PEDIATRIC DYSPHAGIA

She was the product of a 27-week gestational pregnancy with a birthweight of 2 pounds and 12 ounces. Prenatally, she was exposed to methamphetamine, heroin, oxycodone, marijuana, and tobacco use. Her birth mother was also positive for hepatitis C. At birth, Evie was diagnosed with jaundice. She was later diagnosed with bronchopulmonary dysplasia. One minute after birth, Evie’s APGAR score was 6, and 5 minutes after birth, her score was 8. She passed her hearing screening bilaterally. A follow-up was needed at 9 months, which she also passed. She remained in the neonatal intensive care unit (NICU) for 4 months. In terms of developmental milestones, Evie was reported to sit unsupported at 8 months adjusted and crawled at 9 months adjusted. During the evaluation, she stood and walked while holding onto supports (e.g., table, caregiver’s hand). Speech and language milestones did not occur as expected. Babbling and the emergence of jargoning had not been observed. Evie made few sounds during the evaluation other than open vowel productions. Feeding and Swallowing History:  Evie was not breastfed and required formula changes. Her first bottle was given at approximately 3 to 4 months old unadjusted in the NICU. She was given cereal at 6 months adjusted, but she frequently spit it back up. Foster mom stated that “Evie has issues swallowing and controlling her tongue.” (See Figure 1–5 for information Age Range

Oral Motor Action

4 to 6 months

Introduction of cup

6 to 8 months

Suckling pattern for liquid intake from cup; wide jaw excursions; liquid loss

8 to 12 months

Sucking pattern for liquid intake; up-and-down jaw excursions; tongue may protrude underneath cup to provide stability for sucking; liquid loss during intake

12 to 18 months

Sucking pattern for liquid intake; may bite down on cup to gain jaw stabilization; upper lip closes on edge of cup for seal while drinking; less jaw excursion while drinking

18 to 24 months

Use of a more mature up-and-down sucking pattern; cup is held between the lips; internal jaw stabilization is emerging

24 months

Use of a sucking pattern for liquid intake; may hold edge of cup with teeth; eventual development of internal jaw stabilization without biting on edge of cup

Figure 1–5.  Development of cup drinking skills. Source: Pediatric Dysphagia: Etiologies, Diagnosis, and Management (p. 75) by Willging, J. P., Miller, C. K., and Cohen, A. P. Copyright © 2020 Plural Publishing, Inc. All rights reserved. Used with permission.

1.  DIFFERENTIAL DIAGNOSIS IN PEDIATRIC DYSPHAGIA   5

about the development of cup drinking skills.) Evie did not use a cup for drinking and “has tended to lose liquid when she tries. She does not chew while eating.” (See Figure 1–6 for a timeline of Evie’s medical history.) Evie was taking famotidine 40 mg/5 mL (8 mg/mL) oral suspension. This medication was prescribed to manage gastroesophageal reflux.

Evaluation Lips:  Appeared symmetrical. Pursing was evident. However, anterior liquid loss was noted throughout the evaluation. No drooling was observed. Tongue:  Appearance unremarkable. Tongue lateralization was not observed when presented with soft solid textures. Bolus organization, containment, and propulsion appeared to be inconsistent, and there was decreased evidence of each throughout the evaluation. Jaw:  Appeared symmetrical. Bite-through consistencies were not presented to adequately evaluate emergence of rotary chew. Hard palate/soft palate:  Both hard and soft palate were observed. Hard palate appeared high and arched, and soft palate appeared normal. Speech/language and communication:  There was one vocalization observed during the evaluation. Evie vocalized “uh,” which appeared to be in response to her mom stating, “Uh-oh.”

Evie’s Case Study Timeline • Born at 27 weeks’ gestation, maternal history positive for drug use and hepatitis C • 4-months in the NICU (Neonatal Intensive Care Unit) • Discharged to foster care at 3 months old, adjusted age • At the time of the 11-month evaluation, Evie lived with the foster family. Weekly visitation with biological Mom • Current diagnoses: gastroesophageal reflux, bronchopulmonary dysplasia, neonatal abstinence syndrome Figure 1–6.  Evie’s case study timeline.

6  CASE STUDIES IN PEDIATRIC DYSPHAGIA

Evie was placed in a Rifton chair. Foster mom fed her Stage 2 baby food: mango and juice. Evie was prompted to accept bites from mom. She did not readily open her mouth for each bite. She demonstrated limited oral containment and propulsion but did not seem distressed in initiating a swallow. Dissolvable puffs were presented one at a time. Evie was able to contain the puffs but demonstrated limited tongue movement in propelling them to the base of her tongue. When Evie was offered more than one puff, she retained puffs in her cheeks. Foster mom reported similar behavior at home and a need to pace Evie to avoid her mouth-stuffing behavior. Evie was also observed to express approximately 1/2 to 1 oz of juice via a soft spout sip cup. There were no overt signs of physiological distress as she expressed the juice independently. (See Figure 1–7 for information on the development of jaw skills for feeding and swallowing.) The primary issues were poor oral containment and propulsion with Stage 2 textures and lack of progression with more complex solids (e.g., Stage 3 baby foods, soft solids).

Diagnosis and Treatment Planning Within the PDF definition, feeding and swallowing specialists should focus on determining whether there are problems with oral skills, pharyngeal skills, and/or esophageal skills. Often, this can be difficult to determine during an initial evaluation. In Evie’s case, it was important to begin referrals and work toward a differential diagnosis. At first, it was questionable whether there were feeding skill issues for her. Interdisciplinary team members were necessary. Her foster mother was also very diligent in completing a feeding log so that her skills could be more thoroughly assessed outside of the outpatient therapy session. What was an appropriate initial diagnosis for Evie? Consider the International Classification of Disorders, 11th Revision (ICD-11) diagnostic codes. The potential codes were oral dysphagia, oral pharyngeal dysphagia, pediatric feeding disorder–acute, and pediatric feeding disorder–chronic. (See Figure 1–8 for more information on differential diagnosis in the feeding skill domain of pediatric feeding and swallowing disorders.) Who were the professionals currently involved in Evie’s care? (Evie had a history of gastroesophageal reflux and was placed on a histamine-2 blocker by her pediatrician [Nguyen et al., 2022]). Did referrals need to be made to further determine concerns regarding skill? What were potential obstacles to progression of skills in feeding and swallowing? (See Figure 1–9 to see a list of team members for Evie.) Feeding and swallowing therapy was recommended and a speech and language evaluation within the next 3 to 6 months. The Pediatric

Age Range

Lip, Tongue, and Jaw Motion in Response to Solids

5 to 6 months

Phasic biting predominates

6 to 9 months

Upper or lower lip may draw inward with presentation of food Cheek and lip tense with side placement of food to hold in place for chewing. Vertical jaw movements emerge during attempts at mastication Intermittent phasic biting occurs Diagonal jaw movement occurs in response to food placed on the surface of the gum Lateral tongue movements begin to emerge

9 to 12 months

Upper and lower lips pull in with presentation of food to the lip Begin to see active lip motion in conjunction with jaw motion Lips make contact in the center or the side as the jaw moves up and down during chewing Upper lip may move forward and downward during chewing Vertical jaw movement occurs with intermittent diagonal jaw motion Tongue moves food from the center to the side of the mouth during chewing

12 to 15 months

Lips become active during chewing Upper incisors or gums are used to clear food from the lower lip There is occasional loss of food or saliva while chewing Diagonal rotary jaw movements increase

15 to 24 months

Upper and lower lips are active during chewing Ability to chew with the lips closed develops Ability to control food intraorally without anterior loss when lips are open emerges Corner of lip and cheek draw inward to assist with control of food placement Jaw movements range between vertical, diagonal, and rotary Circular rotary chewing occurs when transferring food across the midline of the tongue from one side of the mouth to the other

24 months and beyond

Basic set of skills is in place for chewing; movements are refined as the child continues to develop strength and efficiency of chewing

Figure 1–7.  Development of jaw skills. Source: Pediatric Dysphagia: Etiologies, Diagnosis, and Management (p. 72) by Willging, J. P., Miller, C. K., and Cohen, A. P. Copyright © 2020 Plural Publishing, Inc. All rights reserved. Used with permission.

7

8  CASE STUDIES IN PEDIATRIC DYSPHAGIA International Classification of Disorders, 10th Revision (ICD-10) Both feeding and swallowing diagnoses can be applied. Feeding diagnoses Pediatric feeding disorder — acute (present less than 3 months) (ICD-10 63.31) Pediatric feeding disorder — chronic (present for 3 months or more) (ICD-10 63.32) Swallowing diagnoses Dysphagia, oral phase (ICD-10 13.11) Dysphagia, oropharyngeal phase (ICD-10 13.12) Dysphagia, pharyngeal phase (ICD-10 13.13) Dysphagia pharyngoesophageal phase (ICD-10 13.14) Figure 1–8.  Differential diagnosis in the feeding skill domain for pediatric feeding and swallowing disorders. Adapted from World Health Organization. (2022, August 31). International Classification of Diseases (ICD). https:// www.who.int/standards/classifications/classification-of-diseases

Biological parent

Occupational therapist

Early interventionist

Pediatrician

Foster parent

Registered dietician

Gastroenterologist

Social worker

Figure 1–9.  Team members for Evie.

Eating Assessment Tool (Pedi EAT), a parent-report instrument developed to assess symptoms of feeding problems in children aged 6 months to 7 years (Thoyre et al., 2018), provided information to guide treatment. Initially, there were concerns about lack of food exposure. Evie’s foster mom expressed concern about offering Stage 3 and soft solids that Evie was not ready to handle. Another concern was whether there were physiological reasons for food refusal behaviors. There were no signs and symptoms that would suggest pharyngeal difficulty (e.g., frequent upper respiratory infections). Work on feeding skills was initiated (Morris et al., 2000) and a referral to gastroenterology was made. (See Figure 1–10 for a listing of assessment protocols available for pediatric feeding and swallowing.)

Assessment Protocols

Comments

About Your Child’s Eating (AYCE)

• Measure of child-parent feeding relationships for children ages 8 to 16 years with chronic illness

American SpeechLanguage-Hearing Association Sample Templates for Evaluation http://www.asha.org

• Variety of consensus-based templates for infant, toddler, and adolescent feeding assessment

Behavioral Pediatric Feeding Assessment Scale (BPFAS)

• 35-item scale • Identification of feeding issues in children with a range of conditions • Includes caregiver perceptions

Bristol Tongue Assessment Tool

• Assessment of tongue appearance and function in infants with tongue tie • Rates severity of tongue tie

Children’s Eating Behavior Inventory (CEBI)

• Assessment of eating and mealtime problems in children ages 2 to 12 years • Parent report form

Dysphagia Disorder Survey

• Survey designed to identify and describe swallowing and feeding disorders in children and adults with developmental disability • Tested for reliability and validity

Dysphagia Management Staging Scale (DMSS)

• Five-level scale for rating severity of dysphagia based on management needs and health-related outcomes • Requires certification for use

Early Feeding Skills (EFS)

• 36-item checklist for assessing infant oral feeding readiness, oral feeding skill, and oral feeding recovery

Functional Oral Intake Scale (FOIS)

• Rates degree of oral intake on a 7-point ordinal scale, beginning with tube dependency and ending with total oral intake with no restriction

LATCH

• Breastfeeding charting system that provides a systematic method for gathering information about individual breastfeeding sessions

Marshalla Oral Sensorimotor Test (MOST)™

• Assessment of oral movements, oral-tactile sensitivity, oral and facial tone, resonance, and respiration and phonation skills • Criterion referenced scores for ages 4 years to 7 years, 11 months

Mealtime Behavior Questionnaire

• 33-item questionnaire in which parents are asked to rate mealtime or feeding behaviors over the past week

Figure 1–10.  Examples of assessment protocols. Source: Pediatric Dysphagia: Etiologies, Diagnosis, and Management (p. 315) by Willging, J. P., Miller, C. K., and Cohen, A. P. Copyright © 2020 Plural Publishing, Inc. All rights reserved. Used with permission.  continues 9

10  CASE STUDIES IN PEDIATRIC DYSPHAGIA Assessment Protocols

Comments

Montreal Children’s Hospital Feeding Scale

• 14-item parent report tool designed to identify feeding problems in children 6 months to 6 years of age

Neonatal Oral-Motor Assessment Scale (NOMAS)®

• Observation of normal, dysfunctional, and disorganized tongue and jaw movements in infants up to 8 weeks of age • Requires specific training for administration

Oral Motor Feeding Rating Scale

• Screens and categorizes oral motor movements

Parent Mealtime Action Scale (PMAS)

• Measure of child feeding practices

Pediatric Assessment Scale for Severe Feeding Problems (PASSFP)

• Parent-report measure for progress in development of oral feeding skills in children who require prolonged tube feeding

Premature Oral Feeding Readiness Assessment Scale (POFRAS)

• Objective criteria to assess preterm infant readiness to breastfeed

Preterm Infant Breastfeeding Behavior Scale (PIBBS)

• 10-item scale administered to assess feeding readiness

Pediatric Eating Assessment Tool (Pedi-EAT)

• Parent-report measure of problematic feeding behaviors

Schedule for Oral Motor Assessment (SOMA)

• Assessment of oral motor skills in infants and toddlers between 8 months and 24 months of age

• Age 1 to adults • Identifies specific parent practices included in “permissive feeding”

• Rates gestational age, color, activity, state, cues, and tone

• Content validated

• Tested for reliability and validity Screening Tool of Feeding Problems (STEP) applied to Children (STEP-CHILD)

• Measures a range of feeding problems in children (both normal and with special needs) between ages 24 months and 18 years

Systematic Assessment of the Infant at the Breast (SAIB)

• Observations of latch, compression, and audible swallowing during breastfeeding in full-term infants

Source: Adapted from (1) Heckathorn, D. E., Speyer, R., Taylor, J., & Cordier, R. (2016) Systematic review: Non-instrumental swallowing and feeding assessment. Dysphagia, 31, 1–23. (2) Pados, B., Park, J., Estrem, H., & Awotwi, A. (2016). Assessment tools for evaluation of oral feeding in infants less than 6 months old. Advances in Neonatal Care, 16, 143–150.

Figure 1–10.  continued

1.  DIFFERENTIAL DIAGNOSIS IN PEDIATRIC DYSPHAGIA   11

Case Study Intervention (See Figure 1–11 for areas of concern for Evie identified through the evaluation process.) The following goals were addressed: (a) skills in oral containment and propulsion with more complex textures, (b) ability to identify and label oral structures and functions (e.g., lips, tongue, teeth), (c) thin liquid expression through various modalities, and (d) emergence of typical meal consumption. Treatment approaches utilized: sequential oral sensory (SOS), food chaining, tools (e.g., seating system and specialized utensils), modeling, and shaping. As therapy was initiated, the caregiver and clinician were concerned about Evie’s heavy reliance on formula to meet nutritional needs. Evie’s lack of sustained attention during mealtimes and challenges in getting her to remain seated were also obstacles. Grazing could easily be a pattern of feeding for Evie given her resistance to remaining seated for more than a few minutes. More overt food refusal behaviors were also an immediate concern. Spitting out and turning her head were observed at different times of day. Intermittent reflux and emesis were also reported. Consider important questions to ask as these behaviors and the emesis have emerged. The following questions could be posed: (a) Can you tell me about an incident when Evie threw up during the past week? (b) Do

Oral Preparatory Phase Oral Transit Phase

Evie’s Potential Areas of Concern

Pharyngeal Phase Esophageal Phase Figure 1–11.  Evie’s potential areas of concern.

12  CASE STUDIES IN PEDIATRIC DYSPHAGIA

you remember the food that she ate that day? (c) Tell me about her food refusal. How does her food refusal this week compare to the previous week? Opposition to remaining seated can inhibit progression of skills in many ways. Mealtimes can be reduced. Sensory exposure to less familiar foods can be lost. Caregivers and family members are unable to model mealtime behaviors. Ultimately, encouraging a child to comply through timers and a more restrictive seating system may be beneficial. However, reasons for elopement from a seated mealtime should be considered. Was there any pain or discomfort causing Evie’s opposition to remaining seated during mealtimes? It can be helpful to guide parents through the referral process — for example, letting Evie’s parent know that reporting the emesis and food refusal behaviors might help her get the testing needed to rule out pain or discomfort related to oral intake. The gastroenterology referral could be followed up with a phone call. Opening that line of communication by stating exactly what the concerns were leading to the referral could lead to the most efficient medical care. We wanted to determine if there was discomfort caused by gastric motility issues. Given Evie’s continued use of acid-reducing medication for gastroesophageal reflux and drug exposure, there was enough information to suggest a gastrointestinal obstacle to skill progression. Evie’s gastroenterologist performed an esophagogastroduodenoscopy (EGD). Results of this procedure suggested some irritation at the level of the lower esophageal sphincter.

Evie’s Feeding Journal The following detailed feeding journal was completed by Evie’s foster mother and provided a window into one of her three feeding environments. Evie also spent time with her biological mother during the week and was in full-time daycare. This wasn’t the entire story, but it was a wonderful guide. Changes in feeding and swallowing skills occur so quickly and causal factors vary (e.g., illness, schedule or caregiver differences). This journal provided important insight into Evie’s sequence of feeding, a stronger understanding of food refusal behaviors, and guided how to best intervene during subsequent therapy sessions. Week 1:  Ate a few bites of the ravioli but definitely not as excited about it as she was on at feeding therapy. Choked on the fourth and fifth bites and then she took the bites off the spoon, moved them around in her mouth, and then spit all of them back out. Same with the pepperoni pieces and bread pieces. “Chewed” on them but would not actually swallow them, just spit them back out on her bib. Made some of the oatmeal and barley apple

1.  DIFFERENTIAL DIAGNOSIS IN PEDIATRIC DYSPHAGIA   13

and cinnamon toddler meal and she was excited, leaning forward for bites, opening mouth wide and kicking her legs in excitement just like she did the raviolis at feeding therapy. Week 2:  6 a.m.: 4-ounce bottle. 9 a.m.: No formula. Ate one jar of mango baby food. Eagerly ate the first 3/4, started getting fussy at the last quarter of a jar, started spray spitting the last two bites swatting with the last four or five bites. 12 p.m.: 4 ounces of formula. Did not nap very well at all. 3 p.m.: Handful of star puffs and another jar of mango baby food. Once again, eagerly ate the first 3/4, started getting fussy at the last quarter. Started spitting at the last two or three bites. About 3 ounces of mixed fruit flavor Revitalyte in sippy cup after snack. Barbecue chicken and potatoes for dinner at 6 p.m. She ate a few bites of chicken, sometimes spitting it out when I put it back in. She would usually chew it up a little bit more and swallow it. After about 8 to 10 bites, she would chew it, and spit it out. She would then refuse to swallow any of it. I gave her some potatoes, sometimes with a little bit of barbecue sauce on them and she ate those very well. Sometimes I slipped a small amount of chicken in with it, and she would eat it and swallow it. Eventually she just started spitting the potatoes back out. Gave her a small amount of juice between bites, trying to help her get some of the food down. Sometimes it helped, sometimes it didn’t. 4 ounces of formula at 6:30 p.m. before bed. Next day:  7 a.m.: 4-ounce bottle with vitamins and medicine. 8:45 a.m.: tried a couple of bites of maple and brown sugar instant oatmeal but wouldn’t eat it. I made a small bowl of rice cereal mixed with formula. She ate all of it and about 2 ounces of formula before naptime. I had difficulty getting her to go to sleep. Wi-Fi was not working so there was no music, just lullaby music from her vibrating puppy dog. She fell asleep for about 20 minutes, and she’s acted sleepy since she woke up at 7 a.m. 3 p.m.: Snack was blueberries, apple baby food, and star puffs. She had star puffs around 5:30 p.m. and some apple cider while at the fall festival. We came back to the house to eat dinner. She would not eat potatoes this time. Maybe they were too hot or not soft enough, although most of them were soft. She ate a bowl of rice cereal with baby formula mixed in and a few more puffs with some Pedialyte. 4 ounces of formula before bed. Next day:  6 a.m.: 4 ounces with vitamins and meds. Tried some rice cereal mixed with formula at the 9 a.m. feeding but she only took a few bites. She gagged once, and then started spitting it back out, “spraying” it back out. She stopped taking it

14  CASE STUDIES IN PEDIATRIC DYSPHAGIA

altogether. I gave her 3 ounces of formula at 9 a.m. 12 p.m.: 4-ounce bottle. 3 p.m.: Most of a pouch of puree squash/chai, and apples. 5:45 p.m.: She had potatoes. She ate about 3 to 4 small potatoes and about 3 to 4 bites of puree turkey and then started spray spitting it out at me again (both). I gave her some star puffs between a few bites of potato, and she would take a few more bites. But then, she started spitting it out again. I gave her a few more stars and she put them in her mouth but then spit most out. Evie progressed in feeding and swallowing skills. She improved in oral propulsion and containment. She was eating a variety of tastes and textures without evidence of food refusal for consecutive weeks. She improved in thin liquid expression skills through a variety of modalities. Three months postintervention, Evie was no longer using a bottle. Problems with elopement and grazing were also shaped. Gastrointestinal follow-up appointments were necessary with less frequency. The frequency of feeding and swallowing therapy decreased, and eventually Evie was discharged.

Discussion This case study provides an example of how caregiver involvement can improve treatment outcomes. Evie’s biological and foster mom both attended treatment sessions. A weekly feeding log was completed by her foster parent, giving us an increased window into the mealtime routines and an increased understanding of food refusal behaviors. Her caregivers asked questions, collaborated in goal creation, and complied with provider recommendations. Increased exposure to a variety and tastes and textures was important. There were challenges in team communication to gain adequate information about Evie’s skill progression in both environments. The biological parent had a history of drug use throughout the pregnancy, which most likely affected her ability to integrate and function as a valued member of the team. Different feeding practices in each household may have been related to differing socioeconomic status or eating practices. Careful navigation was important to provide the best care. See Figure 1–12 for information on sharpening clinical skills in cultural humility to begin examining how critical self-reflection of one’s biases and privilege can facilitate better treatment team building. A few key areas that began to emerge were limited variability in food presentation (always presenting Stage 2 mangoes) and that food refusal behaviors may be Evie’s communication (spitting out food as a sign of fullness or physiological discomfort). Habitual presentation of the same

1.  DIFFERENTIAL DIAGNOSIS IN PEDIATRIC DYSPHAGIA   15

•  Place value on the client as the expert on their own culture. • Be aware of the client’s cultural background in the moment with critical selfreflection of one’s biases and privilege. privilege • See yourself as an active collaborator with the client during both the assessment and treatment processes when figuring out how to be aware of their cultural needs. • Build a genuine relationship in therapy with the client that is based on mutual goals, tasks, and a collaborative process. From My Perspective/Opinion: Moving Forward as a Profession in a Time of Uncertainty Cultural competence and cultural humility are the gold standard in communication sciences and disorders. By Kyomi Gregory, August 4, 2020 Figure 1–12.  Sharpening clinical skills in cultural humility. Adapted from Gregory, K. (2020, August 4). From My Perspective/Opinion: Moving Forward as a Profession in a Time of Uncertainty. ASHAWIRE. https://leader.pubs.asha.org/do/10.1044/ leader.FMP.25082020.8/full/

tastes and textures had occurred when a lack of textural progression was observed. Instrumental testing and observation of structure and function helped provide evidence that Evie was capable of handling textures. Communicating these skills to the caregiver helped in their comfort with transition to textures beyond puree. A gastroenterologist referral was important. Instrumental evaluation of structures provided information that structure and function were areas of concern that required medical management (e.g., increased dosage of gastroesophageal medication). Evie’s diagnosis of pediatric feeding disorder that was chronic in nature was accurate. Initiating a few different processes simultaneously proved a successful approach for Evie. The success of her case was due to an interdisciplinary approach, caregiver involvement in the treatment process, and a facilitated progression of skills. While Evie’s case does represent a period of disordered feeding and swallowing skills, this is an example of progression through short-term intervention. The intervention led to parental/caregiver education and empowerment. Evie was able to transition to an age-appropriate diet as team-based problem solving occurred. Evie’s case study provides an example of a complex medical history. She was the product of a premature pregnancy, low birthweight, and exposure throughout pregnancy to a variety of drugs. It is difficult to determine the long-term effects of neonatal abstinence syndrome and how they

16  CASE STUDIES IN PEDIATRIC DYSPHAGIA

play into this case. Digestive motility did appear to be a factor in feeding skill progression. As Evie develops, there may be a need to pursue food sensitivity and allergy testing.

INTERVIEW WITH DR. KATLYN MCGRATTAN, PhD, MA, CCC-SLP Dr. Katlyn McGrattan talks about the importance of understanding normal infant feeding behavior. She has begun researching normal infant feeding behavior through various projects and provides a framework for a provider when determining normal versus disordered feeding and swallowing skills. Dr. McGrattan also discusses the practical way of integrating reasoning and clinical judgment with current research. Jennifer Wilson ( J):  I’m really excited about our profession’s emerging research agenda in normal feeding and swallowing. I thought it might be nice just to do an overview of some things that we need to learn about normal feeding and swallowing. I know that’s a very open-ended question. Dr. Katlyn McGrattan (K):  Everything. I think that we need to know so much more starting from just looking at sucking physiology and moving on to pharyngeal physiology, the interaction between the two, and how those interchange with respiration. That’s a very vague answer. Generally, we have the most data on what’s normal for sucking physiology because it’s the least invasive to measure. It’s relatively easier to measure than the other components. Even looking at that, it’s striking how little we have. The data that we have mostly looked at sucking physiology with bottle-fed babies, because that’s easier to measure. So that’s a limitation. We don’t really know much about differences in sucking physiology between bottle and breast with good data behind it. Most of the studies that people have done, looking at bottle feeding, don’t control the nipple flow rate and other variables. We know from research that we have the strong potential to influence sucking physiology, but we don’t have norms or anything like that. So, I think from a sucking physiology standpoint, we know the basics, but we really need to get more correlates of how that is influenced by different flow rates on a clinical level. Then moving on from a swallowing level, we know very little about swallowing physiology in normals (normally developing children). That, to me, is a huge focus. That is where I see the void inhibiting clinical care

1.  DIFFERENTIAL DIAGNOSIS IN PEDIATRIC DYSPHAGIA   17

the most. We’re doing swallow studies and we’re making conclusions about deficits, but we really don’t know what normal is. Until we have a better understanding of normal, whether we get that from a swallow study or some other type of instrumental assessment, then I think we’re always going to be held back in understanding what we’re aiming for. So, those would be the main things from a big picture perspective. J:  There’s also the different types of formula and versus breast milk variable. K:  Yeah, there’s so many variables. We’re doing a study right now measuring sucking physiology in babies in their house. People know that state changes sucking to an extent, and we all have experienced clinically that the feeder might change feeding. For example, a baby might come into my office, and I’ll look at them. As soon as they see me watching them, they start feeding weird because they’re distracted. There are all these things that we think make a difference, but we don’t really know exactly what influence that is yet. We may be artificially setting our kids up to look better or worse than they typically do. So, I think really getting at that more is important and relatively easy from a research standpoint, getting healthy babies for research is much easier. They are more abundant than getting the sick ones in the hospital. J:  How do you measure state changes in the normally developing babies? K:  Yeah, so I know they have scales that can be used. I’ve used some of those scales in the past, which is good, but there are so many variables that sometimes it’s overwhelming. In the hospital setting, because like you said, there are so many comorbidities and different variables you can’t control for. Some clean studies are needed to weed out some of those things. And even just looking at variability. In some of the research we’ve been doing in looking at healthies, we are seeing that kids are so variable. We always appreciate that medically fragile kids are variable, especially our preemies, but I don’t know that I fully appreciated, until doing the study, how variable healthy term babies are in how they feed. Some of our studies look at how often they cough and some days they cough a ton and others, they don’t. This is observed when they’re fed from the same bottle. It doesn’t seem like it makes sense that they would change that much, but sometimes it can be messy data, so we’ve got to sort through that, so we know what to make of it clinically.

18  CASE STUDIES IN PEDIATRIC DYSPHAGIA

J:  How has your work with disordered feeding and swallowing driven your research on normal feeding and swallowing skills? K:  Looking at normals has always been kind of a step one goal for me. To me it seems like it makes most sense to figure out what we are trying to get at. What are we aiming for? And then work backwards, to see how some of these babies have functional impairments. What are they doing that is making it so that they can’t feed well relative to their healthy peers? Whether I’m seeing patients clinically or in a research study, it always comes back to what the healthies are doing, because we need the comparison. The other topic is that healthy babies don’t feed perfectly. Sometimes, we look for perfection. I think, the more we know about the imperfections in a healthy, functional, term baby’s feeding, it can shed light on what type of imperfections in an impaired baby are OK versus not OK. It’s a circle, but those healthy full-term babies are the foundation. J:  Many parents are constantly questioning that, aren’t they? K:  I wonder how much of that is Internet and different in our culture currently compared to maybe like 20 years ago or 30 years ago. We are much more into expecting perfect everything. A lot of millennials are starting to have babies, right? And that is a different trait. I think parent reports are always important to any interview and taking that is a huge part of what’s going on as far as functional problems. It always makes me wonder, how much should I put on that? Sometimes, parents will describe things to me that don’t sound that bad, like it doesn’t sound that abnormal or concerning. They are coming in concerned about it so it’s hard to weed out. Are they not expressing to me how bad it is in a way that I can quantify? Are they kind of getting more upset about something that is normal? You know, I don’t know. It’s kind of like the reflux thing, like all babies have reflux. People keep putting their babies on medicine to make it stop, but that’s a normal part of infancy. J:  That’s another tough one, reflux management. You’ve made a good point in appreciating when you’re serving this population that so much of the work is with parents and caregivers. Validating what they’re saying, appreciating what you’re hearing from them is so important. I try to appreciate that it’s the most challenging thing at that moment. We may see it as more of a molehill maybe, and it’s to them, it’s a mountain. K:  At the end of the day, regardless of whether what their babies are doing is normal, it’s causing a lot of stress. I think that’s a key thing.

1.  DIFFERENTIAL DIAGNOSIS IN PEDIATRIC DYSPHAGIA   19

The first thing is realizing this parent is feeling a lot of stress based on how this baby is feeding. Now let’s determine if the baby truly does have a problem or they don’t. That will be like the next ideal thing to figure out, how to move forward. If what you find and what you’re seeing is that, well, this seems consistent with normal neuromotor immaturity and this isn’t something that truly should be classified as an impairment or warrants an intervention that would potentially cause more harm than good, right? Like thickening or something then? That doesn’t mean that you just say, “OK, no, they’re fine. See you later.” I think, in part, helping the parents to understand what is normal and what we would expect is important because that gives them peace of mind at least that, “OK, yes, this is not an ideal situation, but that is expected. It’s not like there’s anything crazy wrong with your kid based on what we’re seeing if that is the case.” And then, continuing to support them. And I think that again comes back to the variability issue. Maybe that parent’s complaint and concern that they come in with, you don’t see it in that clinical evaluation. Well, babies are so variable, so I don’t think you should stop at that point and send them out the door. Have them come up for a followup or two to see if you can better understand what their concern is or see if they can do a video or something like that to help you understand what’s happening when their babies are out of the office with you. J:  There can be so much inconsistency in performance. And it can be difficult to fully appreciate what the challenge is right away, and so I think like you’re saying, follow up in parent education. What are some suggestions that you might have for students and young professionals who want to gain skills in pediatric dysphagia? K:  My biggest recommendation I have for my students is to “calibrate yourself to normal.” Many young clinicians going into the field haven’t had kids of their own. They probably haven’t had that many young infants, or if they have, they haven’t tried to evaluate different parameters that we evaluate in a clinical exam. J:  What are some good ways to “calibrate to normal?” K:  Finding a daycare center and seeing if you can get involved there. A volunteer internship that allows you to feed the babies when they are due to eat. Find a Facebook group. If you have families, there’s often new moms, families, and there you explain what you’re doing. I’ve been amazed at how many different opportunities I get through Facebook from research. Families are usually very eager if it’s not invasive. Most parents are happy for you to feed their babies so they

20  CASE STUDIES IN PEDIATRIC DYSPHAGIA

can take a shower. But another way to gain experience is if you go to any of the pediatric wards in your hospital that are seeing babies for things other than feeding problems. Maybe they had ear tubes put in or you know X, Y, or Z. Trying to set something up there where you can help the nurses feed the babies for them so that you can better evaluate how do healthy babies without functional problems feed? I think the other general recommendation is to keep in mind that just because you have a “hammer” doesn’t mean “everything is a nail” phenomenon. We become biased into looking for problems and we’re seeing kids with problems. For example, we get a referral, then we’re immediately like, “Oh yes, this is a problem.” I think that’s very common. You’re consulting because there’s a problem. You are looking for deficits versus knowing that there is some normal variability, imperfections that we’re starting to uncover. Don’t go in expecting a baby to feed normally. That’s easier said than done because we don’t have great data on what is normal yet. Hopefully we’ll have that for you soon, but I think that’s just important to keep in mind. Looking at functional outcomes, not just physiology. I love physiology. I love measuring things. That is what I love to think about. But, at the end of the day, what matters most is function and so if you have slight impairments in physiology, but you still have function, then that’s fine, right? I talk to my students about looking first at what are the functional outcomes? For a baby it’s, “Can they meet nutritional needs with cardio, pulmonary stability? Can they take full nutrition orally without having any bradys, apnea, desats, respiratory infections.” And then likewise, without stress from the baby or the parent. (See Figure  1–13 for more information about these terms related to an infant’s stability for oral feeding.) Those are the primary functional outcomes. Look at those in an infant. Is one of those impaired, if any, and how’s physiology related to that? That’s how I weed out what is important and what’s not. That’s how I determine how conservative I should be in treatment.

Bradycardia — slower than expected heart rate Desaturation — low blood oxygen concentration Apnea — cessation of breathing for more than 20 seconds or a shorter respiratory pause associated with oxygen desaturation and/or bradycardia in infants who are younger than 37 weeks’ postmenstrual age (PMA) at birth Figure 1–13.  Some terms related to infant stability to feed orally.

1.  DIFFERENTIAL DIAGNOSIS IN PEDIATRIC DYSPHAGIA   21

J:  Any other thoughts about what you’re seeing therapeutically in pediatric dysphagia? What’s going well in terms of treatment approaches? K:  We’re getting more research in the area, so I think that’s encouraging. People are looking at the mechanism and bringing more answers, which is good. It seems like most clinicians understand that these feeding disorders are multifaceted. You need an interdisciplinary approach to them. Along those lines, it is important to realize the scope of what your expertise is and engage those other team members. Don’t necessarily do so in a black and white way. In my clinical practice, most feeding problems are very much in a gray area. There’s very few that are black and white. I very rarely give a black and white answer to families or providers. For example, I’ll get a swallow study and I might say the conservative option based on what we saw in this exam is to do A and then a more liberal approach would be to do option B. So, for example, if you have a baby that had trace aspiration of thin liquids, but they haven’t had any respiratory problems, I would, in that case, 100% divert to the medical team that knows that baby far better than I do from a systemic health standpoint, and I’ll say, “Well, this baby had trace aspiration, but overall, their physiology looks good.” Given that they haven’t been having any respiratory problems and they’re healthy, they’re having breast milk, then one could say, “Let’s continue doing breast milk, thin liquids. We will slow the flowrate a little bit.” Hopefully, they reduce how frequently that happens and then we will monitor them very closely and make sure that they are maintaining a good standing. If we start to see that they are having any type of problem, respiratory wise or with discomfort, then we can look at a more conservative approach, which would be thickening. We may have to take them off breast milk XYZ. Ideally, I don’t really take babies off breast milk if we feel like they can be safe at all on the way that they’re feeding. We know the benefits of human milk are so great. Likewise, I would potentially also say, “That being said, if you’re concerned that this baby can’t tolerate any aspiration (maybe it was a hypoplastic status post repair Stage 1 palliation), if you feel like they can’t tolerate any aspiration, then the best method would be thickening liquid.” I will typically provide a couple of options. If it’s not a straightforward case, which most aren’t, then I go to the team, and I’ll talk to them about it and see where they stand on it. They typically appreciate that, and I think it makes sense because again, I don’t know how much aspiration that baby can take, if any. I think it’s good to always get their feedback instead of treating it like this is the only potential route to go. J:  I love the way you present different options.

22  CASE STUDIES IN PEDIATRIC DYSPHAGIA

K:  I always tell them there’s two paths and then I will feel like some are so overwhelmed by the problem that they want to be told what to do, they want to rely on your expertise. What do you recommend? I completely appreciate that, but I think it’s important for them to know that it is not black and white. This seems like the best option right now, but we can reevaluate it and see. Other families want to be really engaged and they know their child well. They may have a background in a medical field, or, at this point, their child has had so many procedures that they essentially have a background in the medical field. I think it’s just good to give options and not treat it like this is the only route. Really, I don’t think that’s typically the case. J:  Cases in pediatric dysphagia are difficult to research given their multifactorial nature. How can we put our best foot forward considering the evidence is not always available to support our treatment? K:  We don’t have enough research to know how much aspiration is too much. How much of a stress cue is too much? We’re doing this normal analysis on healthy, functional babies without reports of feeding problems and in looking at how they feed, they show stress cues. A lot of them show stress cues throughout the feed on occasion, like it’s not the entire feed. So, I think the bottom line is just acknowledging that we don’t know it all, and that’s OK. No profession knows it all. But I doubt that any profession feels like they’ve completely figured it out. There probably will always be questions that we don’t have answered yet. But I think while we’re working to gather that, just accepting that we don’t know it all. I tell patients and providers that, “This is a new science, and that we don’t have a ton of data on.” We can apply what we do understand, and it can guide us exactly on the best way to move forward given this situation. I think people appreciate that honesty as you move forward. Everyone is like, “Don’t hesitate to say that you don’t know, and the profession doesn’t know.” I think generally people can appreciate that you’re honest and you bring it from an evidence-based perspective. There’s no evidence here yet, but based on theory, that’s what I typically say. There’s no evidence for this. There’s no research I’ve seen in this area but based on theory. Therefore, I’m moving forward that way, and so, giving a rationale for that. But that kind of piggybacks into my second reason, my second thing is: We have so little hard data and research to guide in many areas of our practice that I think in order to perform best practice and best evidence-based practice, you as a clinician need to make sure you have a strong foundation in physiology and the

1.  DIFFERENTIAL DIAGNOSIS IN PEDIATRIC DYSPHAGIA   23

evidence that surrounds that. If you understand physiology and how different variables affect physiology, then I think that you can be your own scientist from a clinical perspective. For example, If I’m doing a study, my hypothesis is this and the reason for that is that previous research has shown X, Y, and Z and therefore we would anticipate that this would influence it this way. Clinically, I do the same thing. I’ll say, we don’t have research to indicate what we should do here, but we know based on how physiology works that if we move the baby into this position, it might influence their vital capacity and because that position has better vital capacity. No one’s ever looked at it while feeding. I think if you have a strong foundation of physiology and how different potential treatments that you have applied you know relate to that, then you have at least the theory for why you would do something in the absence of science, I think that’s a good place to be. That’s all you can really ask for. J:  Let’s talk about some other obstacles to treatment. You’ve got all those other professionals at your fingertips in a hospital setting. How does that look different when you’re in more of a rural setting? Any thoughts about that? K:  Yeah, I think it’s hard. There’s a reason I don’t practice in that setting. That’s hard, you know. I admire everyone in that. I think I feel for people in that area because your hands are kind of tied to some extent. You don’t have access to a lot of times, the equipment as much as would be ideal, right? I’ve worked in both rural and urban settings. I would not say rural, but I’ve worked in more outpatient clinics not associated with a hospital setting before. I can appreciate those pain points where the medical team is kind of like, “Well, this is what you’re doing.” But then, I’m like, “But I’m seeing this patient weekly and you’re not. This is what I’m seeing.” So, my biggest recommendation from being on both sides is if you can pair up with a clinician at a medical setting, that is so valuable for both of you. We would be constantly discharging patients from the hospital and to find a well-trained clinician in more of an outpatient setting sometimes felt like an act of God for these medically complex kids. We have a list of certain ones we refer children to for outpatient services. We want them to go to this person because we know that you will take care of them and we would communicate with them well because we had that relationship with them, and we knew they were well trained so we could speak the same language. If you’re feeling like you are “on a little island,” what I would recommend is just seeing if you can reach out to some of those clinicians at the hospitals. Maybe set up a time

24  CASE STUDIES IN PEDIATRIC DYSPHAGIA

to get coffee and just talk about what their needs are from discharging and getting continuity of care from the other side of things. See how you can collaborate more closely so that you can get more of that team science and communicate for optimally better care. J:  That’s wonderful and it really sets up a mentoring kind of relationship in some ways too potentially. There may be age and experience difference or just maybe some different skills that the acute care specialist is bringing to the table versus that outpatient specialist, and vice versa. K:  Typically, levels of care are so completely different, and I think that’s so great to share those varied perspectives. I had an outpatient clinician that was seeing that kid and the baby was coming in for swallow study. I knew that I would get an email from them saying, “This is what we’ve been doing. This is what we specifically have concerns about.” That was awesome because I can do a swallow study, but if I’m seeing or hearing what’s happening clinically, it is better. If I can cater to what’s happening for that patient and I can easily send you the results that way, then I don’t have to try to hunt down the child’s therapist and send it through the parent. It’s better for families, it’s better for everyone. J:  Let’s talk about the research that you’re doing more specifically. You touched on it just a little bit, but can you talk about the work that you’re doing to gain more information about normal oral and pharyngeal processes? K:  We’re doing two big studies right now. The first one is a home monitoring study where we’re trying to better understand how many times healthy term babies cough when they feed. Small outcome. Pretty simple outcome. We’re looking at other things, including how much they eat and drink, how frequently they eat and drink, how long it takes them. Things like that. We’re trying to get a better understanding of whether signs of aspiration are normal on occasion. Does this change throughout the course of development? We’re looking at this over the first 6 months of life. And then, are there differences in how frequently babies show signs of aspiration in bottle-fed babies versus breastfed feeds? We are hopefully going to be consenting our last participant probably tomorrow or Friday. She was in labor earlier this week and was texting me from the delivery room. I feel like I’ve been in the delivery room with lots of people from the study. We will have 65 individuals in the study, and they’ve been longitudinally studied for 6 months. We have all that information on them, and the

1.  DIFFERENTIAL DIAGNOSIS IN PEDIATRIC DYSPHAGIA   25

results are fascinating. And it’s honestly been a fun project to get to know the community a lot, because many speech pathologists are in the study. We have posted it on Facebook, speech, Facebook groups. We have a lot of clinicians involved. It’s been a really great opportunity to get to meet different clinicians from all over the world via Zoom. That is one big investigation we’ve been working on that one for 2 years, and we’re wrapping it up this week for recruitment. We still have 6 months of monitoring of these babies and will have that out soon. Hopefully the first manuscript will be written up in the next couple months. So, that is one of the studies. The other study that I’m really excited about involves technology. Our team has been working on developing this “smart bottle,” for lack of better words, that can better identify kids who are having sucking and swallowing problems and aspirating without the use of videofluoroscopy. Our goal is not necessarily to replace the swallow study. I think the swallow study will, as far as I can see, as far as technology is going, will have a place for the long term. This is more as an adjunct as another noninvasive objective measuring technique that clinicians can use because so much of what we do is subjective and obviously the ones that we have that are more objective, our instrumentals are invasive. With this study we have two arms. One is a preemie arm, and one is the term arm. For the term healthy babies, we feed the baby with the smart bottle, and we go into their homes. A couple of kids came to the lab but most of them were in their house fed by their parents using the same flow-rate bottle nipple that they typically use. All the babies in this had to be fed by Dr. Brown’s just because that is the way we design the bottle nipple for the smart bottle. We use whatever flow rate parents typically used within Dr. Brown’s flows. And as part of this, we measure how quickly they eat and how long it takes. We video record all these things. So, we are analyzing their feeding performance. We are using feeding scales and looking at what is normal from that perspective. In addition to that, we are analyzing what is going on from a sucking standpoint. What’s normal in that regard? We have the last participant scheduled to run next Friday. We are having a big lab party after that. It’s been a drive to say the least and we are all ready for these two to be done with and recruitment to end so that we can start to look at these results and close some of these open bookends as we move forward. J:  That’s neat that you’re ending with both at the same time. K:  Yes exactly. It’s the best job ever!

26  CASE STUDIES IN PEDIATRIC DYSPHAGIA

REFERENCES Delaney, A. L., Van Hoorn, M., Staskiewicz, S., Feuling, M. B., Pladies, S., Bansal, N. K., & Goday, P. S. (2021). Texture consumption patterns of 8- to 12-month-old infants: A reflection of typical feeding development. American Journal of Speech-Language Pathology, 30(6), 2643–2652. https://doi​ .org/10.1044/2021_AJSLP-21-00048 Dysphagia Research Society. (2022). About. https://www.dysphagiaresearch​ .org/​page/Mission Estrem, H. H., Park, J., Thoyre, S., McComish, C., & McGlothen-Bell, K. (2022). Mapping the gaps: A scoping review of research on pediatric feeding disorder. Clinical Nutrition ESPEN, 48, 45–55. https://doi.org/10.1016/j.clnesp​ .2021.12.028 Feeding Matters. (2022). Who we are. https://www.feedingmatters.org/whowe-are/ Goday, P. S., Huh, S. Y., Silverman, A., Lukens, C. T., Dodrill, P., Cohen, S. S., . . . Phalen, J. A. (2019). Pediatric feeding disorder: Consensus definition and conceptual framework. Journal of Pediatric Gastroenterology and Nutrition, 68(1), 124–129. https://doi.org/10.1097/MPG.0000000000002188 Gosa, M., & Dodrill, P. (2017). Pediatric dysphagia rehabilitation: Considering the evidence to support common strategies. Perspectives of the ASHA Special Interest Groups, 2(13), 27–35. https://doi.org/10.1044/persp2.SIG13.27 Hall, K. D., & Johnson, L. W. (2020). The three CCCs of dysphagia management: Culturally competent care. Perspectives of the ASHA Special Interest Groups, 5(4), 1000–1005. https://doi.org/10.1044/2020_PERSP-19-00112 Kovacic, K., Rein, L. E., Szabo, A., Kommareddy, S., Bhagavatula, P., & Goday, P. S. (2021). Pediatric feeding disorder: A nationwide prevalence study. The Journal of Pediatrics, 228, 126–131. https://doi.org/10.1016/j.jpeds​ .2020.07.047 Lefton-Greif, M. A., & Arvedson, J. C. (2016). Pediatric feeding/swallowing: Yesterday, today, and tomorrow. Seminars in Speech and Language, 37(4), 298–309. https://doi.org/10.1055/s-0036-1587702 Morris, S. E., Klein, M. D., & Sater, E. (2000). Pre-feeding skills: A comprehensive resource for mealtime development (2nd ed.). Pro-Ed. Nguyen, K., Dersnah, G. D., & Ahlawat, R. (2022). Famotidine. In StatPearls [Internet]. StatPearls Publishing. Silverman, A. H., Kristoffer, S. B., Linn, C., Pederson, J., Schiedermayer, B., & Barkmeier-Kraemer, J. (2020). Psychometric properties of the Infant and Child Feeding Questionnaire. Journal of Pediatrics, 223(2), 81–86. https:// doi.org/10.1016/j.jpeds.2020.04.040. Thoyre, S. M., Pados, B. F., Park, J., Estrem, H., McComish, C., & Hodges, E. A. (2018). The Pediatric Eating Assessment Tool: Factor structure and psychometric properties. Journal of Pediatric Gastroenterology and Nutrition, 66(2), 299–305. https://doi.org/10.1097/MPG.0000000000001765

2 Genetic Syndromes

INTRODUCTION Genetic syndromes are often listed among the diagnoses when receiving a referral in pediatric dysphagia. Feeding and swallowing specialists who see babies and young children should be knowledgeable about syndrome characteristics and communicate with professionals who specialize in these areas (Medline Plus, 2022). Regional specialty clinics provide care and can assist local providers (National Down Syndrome Congress, 2022). Establishing strong networks of communication is important to optimize care. Some examples of syndromes associated with dysphagia include Down syndrome and 22q11.2 deletion syndrome. In a retrospective study of children diagnosed with Down syndrome, over 50% had a pharyngeal phase of swallow problems and over 60% had an oral phase of swallow problems. Oral and pharyngeal dysphagia have also been well documented in 22q11.2 deletion syndrome, also called DiGeorge syndrome ( Jackson et al., 2016; Wong et al., 2020). The case below will highlight a less documented chromosomal difference and the medical obstacles involved in managing care. (See Figure 2–1 for some resources to sharpen clinical skills in genetics.) The following case study provides an example of chromosomal differences. Although understanding trisomy 12 with mosaicism doesn’t tell the whole story in the case study presented, it provides an important foundation to understanding Eli. With a rare genetic syndrome like trisomy 12 27

28  CASE STUDIES IN PEDIATRIC DYSPHAGIA Some resources to learn more about genetics Morgan, A. (2013). Speech-language pathology insights into genetics and neuroscience: Beyond surface behaviour. International Journal of Speech-Language Pathology, 15(3), 245–254. https:// doi.org/10.3109/17549507.2013.777786 Websites Online Mendelian Inheritance in Man (OMIM), sponsored by the National Center for Biotechnology Information (NCBI), features information about all known Mendelian disorders and 12,000 genes. Included are descriptions of phenotypes, gene locations, modes of inheritance, how a gene was discovered, and how it interacts with other genes. GeneTests, also sponsored by NCBI, provides current information on genetic disorders, genetic tests, and contact information for laboratories. Hearing loss and deafness are covered extensively, as are secondary causes of speech and language disorders (for example, autism). Primary speech and language disorders are covered sparsely. Linked to GeneTests is GeneReviews GeneReviews, a searchable database with peer-reviewed information on disorders of genetic origin. The National Coalition for Health Professional Education in Genetics offers guidelines with recommended professional competencies in genetics for all health care professionals. Specific guidelines for SLPs and audiologists are also included. Peter, B. (2012). The future of genetics at our doorstep. The ASHA Leader, 17(11). https://leader.pubs.asha.org/doi/10.1044/leader. FTR3.17112012.16 Figure 2–1.  Sharpening clinical skills in genetics.

with mosaicism, a broader level of retrospective analysis was not available. Therefore, increased interdisciplinary guidance was important for Eli’s level of medical management during more acute points in the treatment process (Brady & Roe, 2020; Cooper-Brown et al., 2008; Desai & Lim, 2019; Fuller et al., 2022; Madhoun & Jadcherla, 2016; Muldoon et al., 2021). The case study is followed by an interview with Dr. Emily Zimmerman. She shared genetic research with Dr. Jan Maron, a neonatologist. They were able to gain information about FOXP2 gene expression and feeding skills in infants. Dr. Zimmerman also described research partnerships with a variety of colleagues. As you will discover, the lines of research Zimmerman and colleagues are pursuing are broad and will help us gain a deeper understanding of pediatric dysphagia through some more recent international work.

2.  GENETIC SYNDROMES   29

CASE STUDY Referral for outpatient feeding/swallowing: Eli — 7 months old (unadjusted) (see Figure 2–2 image of Eli).

Case Study Medical History Birth and medical history: Eli was the 1-lb, 12-oz product of a pregnancy with a 31-week gestation. During the pregnancy, his mother experienced intrauterine growth restriction (IGR). He was delivered via C-section. Following his birth, he spent 4 months in the NICU. (See image of Eli and mom in the NICU in Figure 2–3.) When discharged from the NICU, Eli was diagnosed with the following: dysphagia, patent foreman ovale (PFO), trisomy 12, mosaicism, chromosome X duplication, chromosome 16 duplication, hypospadias, chronic lung disease, and hypothyroidism. Surgeries: During his hospitalization, he had a right inguinal hernia repair at 1 month of age (adjusted), a G-tube placement at 2 months of age (adjusted), and a GJ-tube placement at 4 months of age (adjusted). Eli had his first videofluoroscopic swallow study (VFSS) during his stay in the NICU. Mom reported that this study suggested no penetration

Figure 2–2. Eli—7 months old unadjusted.

Figure 2–3.  Eli and mom in the NICU.

30  CASE STUDIES IN PEDIATRIC DYSPHAGIA

or aspiration of thin liquids. A detailed report was not available. Following the study, he had his first bottle. He had a poor latch and poor suck and incidences of gagging and vomiting. He stopped taking bottles 3 weeks prior to this evaluation. The pulmonologist recommended that his mother stop feeding Eli bottles and try baby food. Eli began to throw up all the food, which resulted in the switch to a GJ-tube. G-tube feeding schedule: Eli was on a 16-hour continuous feeding schedule with two 1-oz feeds daily. Mom administered the 1-oz feedings at 8 a.m. and 11 a.m. and initiated the 16-hour continuous feed at 2 p.m. Eli received 48 mL per hour, but his gastroenterologist was hoping to work toward his ability to tolerate 50 mL per hour. He received EleCare formula. Last month at his primary care visit, mom reported his weight was 13 lb, 9 oz and length of 22.5 in. His weight and height were reported at less than the second percentile on growth charts. In order to adequately determine treatment goals, thorough information should be included regarding pharyngeal skills on the VFSS. (See Figure 2–4 for information that should be obtained from a VFSS.) Eli was referred to the following physicians: pulmonology, gastroenterology, and neurology. He was taking the following medications: Synthroid, Prevacid, Reglan, VSL #3, and Carafate. Previous and current therapy: Following Eli’s stay in the NICU, he was seen by the local hospital home health for feeding/swallowing therapy. At the time of this evaluation, he was transitioning to outpatient services. He has recently been evaluated at a local private practice for occupational therapy and physical therapy. (See Eli’s medical timeline in Figure 2–5.)

Evaluation Oral Structure and Function: Lips:  Lips appeared symmetrical. Slight retraction was observed. No rounding or tightening was observed. •  Initiation of the swallow was normal/delayed •  Epiglottic inversion was normal/partial/absent •  Laryngeal elevation was normal/partial/absent •  Pharyngeal stripping was normal/diminished/absent •  Cricopharyngeal relaxation was normal/abnormal/luminal narrowing •  Nasopharyngeal reflux was observed/not observed •  Pharyngeal residue was none/trace/small/moderate/large • Laryngeal penetration was observed/consistency/volume/transient, deep, plus/minus cough reflex/not observed Figure 2–4.  Information that should be obtained from a VFSS.

2.  GENETIC SYNDROMES   31

Eli’s Case Study Timeline

• Born at 31 weeks’ gestation, intrauterine growth restriction (IGR) • 4 months in the NICU • G-tube placement at 1.5 months (all ages unadjusted) • Videofluoroscopic swallow study (VFSS) suggested no penetration or aspiration • First bottle at 2 months • Inguinal hernia repair 3 months • G-J tube placed at 4 months • 2nd VFSS scheduled at 7.5 months • Diagnoses: mosaic trisomy 12, chromosome X duplication, chromosome 16 duplication, chronic lung disease, patent foramen ovale, hypospadias, dysphagia Figure 2–5.  Eli’s case study timeline.

Tongue:  Surface appeared within normal limits. Retraction was observed when presented with a gloved finger with some medial tactile input. Eli had a suckle response. Jaw:  Jaw appeared symmetrical; no functional deviations were apparent. He seemed to enjoy tactile input to the jaw. He did not present with a reflexive tonic bite. Hard and soft palate:  Hard and soft palate should be further observed as treatment is initiated. Vocal quality/respiratory function:  Eli was observed to have a raspy vocal quality and evidence of crackles during supported sitting. Feeding skills:  Eli was placed in a Tumble form seat. Mom presented Stage 2 baby food via a standard spoon. Eli did not open mouth spontaneously in response to spoon presentation. When prompted to accept presentations, he demonstrated poor bolus containment and poor bolus transit. Eli was diagnosed with oral dysphagia with concerns regarding his ability to protect his airway. The referring physician was contacted to discuss concerns. Before initiating feeding/swallowing therapy, medical concerns needed to be resolved. See questions in Figure 2–6.

32  CASE STUDIES IN PEDIATRIC DYSPHAGIA Immediate questions to pose during the evaluation: There was enough information reported and observed to suggest that Eli needed medical management. There were some immediate questions to help support Eli’s care. Gastrointestinal Tell me more about the food refusal behavior. Has it improved or gotten worse lately? How often is he throwing up? Pulmonary How often does he see the pulmonologist? Have there been recent changes in his breath sounds when you are holding him? Depending on which physician has provided the referral for the feeding and swallowing evaluation, they should be contacted with a description of the concerns (e.g., rales felt and heard when holding him, emesis, lack of spoon acceptance) Figure 2–6.  Eli’s evaluation questions.

Pulmonary and Gastrointestinal Issues Were Resolved With pulmonary concerns, it was important to contact Eli’s pulmonologist. A swallow study soon after treatment began suggested no penetration and aspiration. The following month, an esophageal pH probe study showed moderate reflux and a fundoplication was recommended. Cultures were taken from the lungs and possible aspiration was noted. See examples of instrumental studies for pediatric dysphagia in Figure 2–7. Tracheomalacia was also diagnosed by his pulmonologist, and chronic inflammation was noted at the bottom one third of the trachea. A fundoplication was performed the following month. Following the procedure, mom reported considerable improvement. Specifically, she reported no vomiting and increased contentment. A week after the procedure, mom began reporting that he was spitting up a lot of mucous and expressed concern that he could not handle his secretions. See an image depicting a fundoplication in Figure 2–8. This pattern of severe reflux of stomach contents and poor airway protection needed to be fully resolved before oral feeding skills could be addressed therapeutically. Once Eli’s system had healed, it was time to determine treatment goals.

2.  GENETIC SYNDROMES   33

Videofluoroscopic swallow study (VFSS) — also known as a modified barium swallow study (MBSS) Fiberoptic endoscopic evaluation of swallowing (FEES) High-resolution pharyngeal manometry Esophageal pH study Figure 2–7.  Examples of instrumental studies for pediatric dysphagia.

Figure 2–8. Fundoplication. Source: Comprehensive Management of Swallowing Disorders, Second Edition (p. 461) by Carrau, R. L., Murry, T., and Howell, R. Copyright © 2017 Plural Publishing, Inc. All rights reserved. Used with permission.

Setting Treatment Goals With Eli’s Family The following are some open-ended questions to ask parents and caregivers to welcome their participation in goal formation: What are some important priorities for Eli? What are your goals for Eli over the next several months? What do you feel the biggest obstacles are in achieving those goals? Consider the goals that other team members are working on in treatment. Involving physical therapy and occupational therapy in optimal positioning (e.g., adequate trunk and head support) and tool selection (e.g., flat bowled spoons) will be beneficial. See resources for goal writing in Figure 2–9.

34  CASE STUDIES IN PEDIATRIC DYSPHAGIA SMART goal writing The acronym SMART stands for Specific, Measurable, Achievable, Relevant, and Time-Bound. • Specific — Goals need to be specific so that the team knows exactly what the patient or client will be working on in treatment. They need to be well defined with a clear outcome. • Measurable — The goal must include some type of measurement. Is it a percentage or so many times out of a total number of trials? There are many ways to measure a goal or objective. There is no set way, and it is largely dependent on how the goal is written. • Relevant — The goal must be relevant to their daily living skills. • Time-Bound — Goals are time-bound based on the terms of their treatment period (e.g., 3 months, 6 months). Adapted from Tricks to Take the Pain Out of Writing Treatment Goals by Irene Torres ASHA Leader Live, September 9, 2013. Patient-Centered Focus on Function: Pediatric Feeding and Swallowing https://www.asha.org/siteassets/uploadedFiles/ICF-Pediatric-FeedingSwallowing.pdf Figure 2–9.  Resources for goal writing.

Treatment Goals The following treatment goals were established following increased medical stability: Decreased tongue retraction in response to multisensory input Optimal positioning and use of tools to facilitate optimal lingual, labial, and jaw use Caregiver education regarding instrumental findings and recommendations. See areas of concern identified during the evaluation process in Figure 2–10. Communication from mom a few weeks after the fundoplication: Mom:  For some reason I’m having a meltdown about Eli ever eating again. I just read this article and think it’s interesting. How do you feel about this approach, and have you ever done this before? The article was entitled, “Ideas for Transitioning Off a Feeding Tube” (Brackett, 2017).

2.  GENETIC SYNDROMES   35

Eli’s Potential Areas of Concern

Oral Preparatory Phase Oral Transit Phase

Pharyngeal Phase Esophageal Phase Figure 2–10.  Eli’s potential areas of concern.

Mom:  I’m not so worried about the fundo. He’s done well with it and rarely retches. I don’t feel like he’s bloated either. I’m more worried that I’m giving him 30 oz a day of a 24-calorie formula, which is far more than I even gave my other son. And I’m worried he’s going to forget how to eat because he never experiences hunger. Communication from mom 1 month later: Mom:  We tried spaghetti today. He at least played with it and I put a little marinara sauce in his mouth and he didn’t hate it. Eli started sucking on a pacifier today a little bit! He is still very afraid and hesitant when I try and feed him, so I think I’m going to back off a little and try and let him just have fun with food. How do you feel about a Jell-O dig Wednesday for therapy? I will make some Jell-O and bury some of his teething toys and we can just let him play in it and have fun touching it? And maybe he’ll put some in his mouth? The doctor switched Eli to PediaSure today, so hopefully he starts drinking because it tastes better. One month after the fundoplication: Out of nowhere Eli just drank 20 mL (via bottle)!!!! He sucked and swallowed!!!!!

36  CASE STUDIES IN PEDIATRIC DYSPHAGIA

Three months after the fundoplication: Mom:  Eli hasn’t thrown up in 2 days and today he ate 1 yogurt melt and 7 syringes of Jell-O without gagging. Therapist:  Do you think erythromycin is helping? Mom: Maybe. A few days later, Eli gained 4 oz and the dietician said he is a perfect candidate for food blends. A consultation at 4 months after fundoplication with an interdisciplinary feeding team: body mass index, 16.65 (53rd percentile); weight, 17 lbs, 15.8 oz (3rd percentile); height, 27.56 in. (below 1st percentile); and head circumference, 45 cm (11th percentile). (Percentiles calculated through World Health Organization [WHO] boys 0–2 growth chart) (Kiserud et al., 2018). From the consulting dietician:  Eli looks adequately nourished and hydrated, and while he is small for his age, he is adequately proportional in his length compared to his weight. Try the samples of Compleat Pediatric Organic blends. Occupational therapy and speech therapy recommendations:  Continue local therapy services — he is making some nice progress! Continue to supervise and monitor safety with all oral presentations. Continue to position him upright in supported seating with oral presentations. Use a blanket or towel for side support as we want him to be well supported as he learns his oral motor skills. Offer the puree pouches and easily dissolvable solids as you have been doing. Some additional items/ideas as he gains skills may include ranch-flavored veggie sticks, apple cinnamon sticks, graham cracker sticks, Ritz or club crackers, and Lorna Doone cookies. Work toward helping bite the items on the side. May try dipping the dissolvable as well (yogurts, sauces, spreads, humus, etc.). Continue trials of liquid with the honey bear straw, a cut-out cup, a small medicine cup, Dixie cups, and so on. Could try some liquids that are just a bit thicker, so the flow is slower as he gains experience (e.g., drinkable yogurts, juices mixed with puree fruit). See Eli at 11 months old (adjusted) in Figure 2–11. A message from mom 1 month after the interdisciplinary appointment: Eli ate pancakes for breakfast and pizza for dinner! Still having trouble getting him to drink anything but he’s exploring different foods. At this point, medical problems arose. Eli was admitted to the hospital and diagnosed with rotavirus. Another challenge was a drop in blood sugar. An endocrinologist was consulted. A couple of weeks later: Eli was hospitalized again and nil per os (NPO). Doctors ran tests to assess immune function. Blood sugar levels also continued to be a concern. Cortisol levels were low. One month later:

2.  GENETIC SYNDROMES   37

Figure 2–11.  Eli —11 months old unadjusted.

Eli was really congested from his ear infection and began gagging on everything. Another reevaluation of skills occurred after Eli was discharged from the hospital. He returned home with some medication changes and resolution of the acute problems resulting in his hospitalization.

Reevaluation:  Eli — 14 Months of Age Over the 7 months of treatment, Eli demonstrated considerable improvement. Weight gain began, which led to a transition away from continuous feeding throughout the night. This change alone may have led to increased appetite and decreased food refusals. Pulmonary function was also improving. There was also progression of gross motor skills (improved independent sitting stability and emerging crawling). The following goals were addressed during this treatment period: Organization/propulsion of puree textures presented via spoon

38  CASE STUDIES IN PEDIATRIC DYSPHAGIA

Expression of 3 to 5 teaspoons of thin liquids through a variety of modes (bottle, straw, soft spout cup) with maximal support Questions for the parent/caregiver: What were some positive changes that you see in Eli? Are there occasions when you have seen oral intake enjoyment? Follow-Up on Eli At 4 years old, Eli leads an active healthy life, attending preschool, playing soccer, and swimming. He orally consumes 600 to 800 calories per day. His mom has begun talking with him about how many calories he needs to eat daily to be weaned from his G-tube.

Discussion Eli’s case study highlights the importance of understanding medical history, relying on interdisciplinary consultation, and the extreme changes that can quickly occur medically alongside intervention. Eli’s case also is an example of a child who did not develop a strong motor memory for suck-swallow-breathe synchrony as reflexes were in place to support learning. As physiological function improved to facilitate a stronger, more coordinated pharyngeal swallow, he had challenges in motor planning for oral intake (e.g., retracted lingual pattern). Addressing improved oral motor awareness and function was important. Not only were muscles underutilized (e.g., limited development of intrinsic and extrinsic tongue muscles), but they were also often fixed in place. The fixing may have been due to numerous negative experiences with chronic reflux and pulmonary sequelae (e.g., increased secretions, bilateral pneumonia). There were many important team members. Eli’s mother was a highly educated participant through her professional career as a pediatric nurse. She was very proactive in engaging with his specialists and reporting their findings. She also sought out an interdisciplinary feeding team and brought the information back to her local providers. Further evidence through messaging provides a window into rapid, acute changes with Eli’s medical status and feeding performance. It also provides the reader with a look at how parents process feeding challenges. Eli’s mom transparently expressed her fears that he may never eat orally and that the volume of tube feedings recommended made her uncomfortable. While some parents express these concerns, others may not openly express them. She both expressed them and shared the information she found online through her Internet search.

2.  GENETIC SYNDROMES   39

Mom Reflecting on Eli’s Treatment What are some of the most difficult experiences that you can recall during Eli’s medical journey? Mom: The word “difficult” has taken on a different meaning throughout the many stages of Eli’s journey. When he was born, the NICU was difficult. It was hard to want to be so hands-on in caring for my child but having to take a back seat and trust others to do it for me. There were so many unknowns during my pregnancy that carried over to when Eli was born. We didn’t learn of his genetic disorder until he was several weeks old. Up until that point, there really was no explanation for why he wasn’t growing. Learning of his genetic condition gave us that answer but also opened a whole new realm of unknowns. His condition was so rare, no one gave us a clear answer as to what to expect for his future. That’s hard to hear as a parent. I remember one day specifically getting a feeling from Eli’s care team that he wasn’t making the progress they had hoped he would make. I finally asked Eli’s primary nurse practitioner for an honest answer. She admitted Eli wasn’t progressing with his feeding or oxygen. It was at that point we had a serious conversation regarding when it would be safe to discharge him. I was given an option: discharge with home health and I would be his nurse at home or keep him in the NICU until he shows more progress. I chose to take him home. That brings me to the next set of “difficult” experiences. I did not factor in how physically, mentally, and emotionally exhausting it would be to bring my child home and be the primary nurse. In hindsight, that was one of the things I would change regarding his care. I would have stayed in the NICU longer because of the resources available being inpatient. The next several months consisted of almost daily appointments, between several specialists and all his therapies. He had several surgeries as well as several hospitalizations due to illness in his first year of life. He has slowly been discharged from his specialists (i.e., cardiology, pulmonology, physiatry, immunology, endocrinology) and no longer requires any therapies. It took several years to get to this point, but he is no longer developmentally delayed and is progressing (slowly) with oral feeding and weight gain. What currently is most “difficult” is weaning Eli from his feeding tube. He has made tremendous progress over the past year, but he is still about 50% tube fed. It has taken years to get him to even smell, touch, or lick certain foods. Feeding therapy has been extremely helpful in encouraging Eli to try new things, as well as teaching me how to encourage those things at home. I often think that I learned more than Eli through therapy, and that is why we’ve been able to make progress at home. He has recently tried several new foods, and being in preschool has been helpful for him to see his peers eat orally. My husband and I are very encouraged by the progress Eli has made but still struggle with the unknown of when/

40  CASE STUDIES IN PEDIATRIC DYSPHAGIA

if he’ll ever eat 100% orally and how his eating and small size will affect his future. What kinds of resources were the most helpful to you as you managed his feeding and swallowing issues? Mom:  There were several helpful resources throughout Eli’s journey, all starting in the NICU. There was a social worker who got us set up with home health to begin feeding therapy as soon as we were discharged. With Eli being on oxygen, having someone come to the house each week was extremely helpful. I never realized what a learned skill eating was until I had a child who couldn’t eat. I always thought it was an innate skill that everyone automatically knew when they were born. I was very wrong. Watching Eli struggle to coordinate how to suck, swallow, and breathe every day was very eye opening. Once Eli was officially weaned from his oxygen, we began going to therapy outpatient, and we got connected with the early intervention system who helped us coordinate those therapies. In addition to therapy, I joined groups on Facebook for parents of tube-fed children and read several books regarding feeding and swallowing difficulties. A lot of Eli’s journey was trial and error. I would try offering a certain food or texture that may have worked for another parent but didn’t work for us. I tried at least 10 different cups with Eli before finding one that he could drink from. It ultimately came down to reading his cues and doing what he was comfortable with using. How did your professional training as a pediatric nurse help you navigate his feeding and swallowing intervention? Mom:  Being a pediatric nurse was helpful in navigating Eli’s feeding difficulties. When Eli was discharged from the NICU, he had a nasogastric (NG) tube. These tubes are meant to be temporary, and very few patients are usually discharged with this type of tube, particularly premature babies. If a child does go home with one, the parent usually has to take them to the emergency room (ER) to have it replaced if it is pulled out. I was able to replace Eli’s NG tube at home (which happened A LOT) and avoid an ER visit because of my experience as a nurse. I also feel that my knowledge as a nurse helped me in my decision-making to ultimately have a gastrostomy tube (GT) placed. Prior to Eli’s G-tube placement, I had cared for several patients with G-tubes. This allowed me to be very comfortable with replacing his tube and navigating his feeding pump at home.

INTERVIEW WITH DR. EMILY ZIMMERMAN Dr. Emily Zimmerman is an Associate Professor in Communication Sciences & Disorders at Northeastern University and has done research with

2.  GENETIC SYNDROMES   41

a neonatologist, Dr. Jan Maron. Below, she shared how she became interested in pediatric dysphagia and how her research interests have evolved through some fortuitous collaborations. Jennifer Wilson ( J):  Tell me about how you got into the profession. Dr. Emily Zimmerman (E):  I started my journey in research during undergrad at the University of Kansas working with Dr. Steven Barlow in his lab and doing random things and I had worked in other labs at the University of Kansas too. What I realized is that research really can connect with your passion. Once I started working with Dr. Barlow, particularly his work in the neonatal intensive care unit with preterm infants, I really found a passion toward research and toward this population. I tend to be a very people-oriented person and I really love working clinically. I wasn’t sure if I could envision a research path for myself, but I kept both areas going throughout and toward the last semester of my grad program. I decided to start taking a few PhD courses because I had a little bit of time in my schedule, [and at the same time] I ended up working in the sixth grade as an SLP. That was really the only part-time placement for somebody like me who was doing my PhD during the day. Then after school, I would do home health early intervention (EI). Once I finished my CF (clinical fellowship), I continued to work a little bit. But then, I really switched gears to focus on research. My dissertation looked at different types of sensory stimulation and how they can work on improving sucking and feeding outcomes. When I graduated from the University of Kansas, I went to Brigham and Women’s Hospital and looked beyond feeding at other short-term and important clinical outcomes like weight gain, growth trajectories, other neurodevelopmental outcomes. From there, I transitioned into a faculty position at Northeastern University where I’ve been 8 years. I direct the speech neurodevelopment lab and, in this lab, we’re really looking at typical development as well as various populations of interest. But really, trying to see how early sucking and feeding and oral motor movements relate to subsequent neurodevelopment, but also how these early oral motor behaviors and movements are impacted by things like genetics, their physiology, how it differs, maternal psychosocial factors, and the environment. So, trying to look at all these different variables that maybe we wouldn’t consider in our typical training, to learn more about how these impact sucking and feeding. So that’s the Emily story. J:  Your work is valuable. Sensory information is valuable. The normal development element is very valuable as well.

42  CASE STUDIES IN PEDIATRIC DYSPHAGIA

E:  Yeah, I completely agree, and I think as we talk about our topic today, that’s something to consider. Until we understand what is typical, it’s really challenging to understand what is different or how these different genetic implications can affect sucking and feeding, so I think that’s something we’re really trying to understand; how does sucking or feeding change within the first year of life? Who are the key players? I spent so much of my early career focused on the baby and my more recent career focused on the mom and realizing that she is the volitional person of the two and can make the decisions. While we have these underlying genetics and these underlying anatomical features and neural maturation patterns we’re working with, my research helps to inform what are the other layers that play a big role into these pieces that make up the feeding puzzle. J:  Tell us about your work at Northeastern University. E:  I direct the speech neurodevelopment lab and, in this lab, we’re looking at typical development as well as various populations of interest. But really, I am trying to see how early sucking and feeding and oral motor movements relate to subsequent neurodevelopment. Also, how these early oral motor behaviors and movements are impacted by things like genetics, physiology, how it differs, maternal psychosocial factors, and the environment. Trying to look at all these different variables that maybe we wouldn’t consider in our typical training, to learn more about how these impact sucking and feeding. J:  Can you tell us about your work with Dr. Maron? E:  I first started connecting with Dr. Maron during my postdoc at Brigham Women’s. I was learning about her oral feeding work, and she was doing something called salivary transcriptome. At the time I did not know what that was. I was a recent graduate with my PhD, but not with any specific courses in genetics. Salivary transcriptome is a somewhat recent discovery that a large panel of human ribonucleic acid (RNA) can be readily detected in saliva and gives rise to a novel clinical approach of collecting this genetic information. I was on maternity leave when the underlying idea for my work with Dr. Maron came about and it was a news ticker at the bottom of like, the Today show, that said we finally know why women are so chatty. It is because they have higher forkhead box protein P2 (FOXP2) gene expression levels or gene amounts. At this time, I was thinking about it, and I remembered learning that FOXP2 is the speech and language gene. So, then I wanted to learn more. I learned that there was this KE family (Lai et al., 2000). I had some recognition of learning about this at some point in one of my

2.  GENETIC SYNDROMES   43

classes. Fifteen members of this family had mutations to the FOXP2 gene, so a mutation changes in the genetic sequence. Their FOXP2 had a different genetic sequence and they suffered from speech and language delays. They underwent many speech and language assessments and these tests indicated that one core deficit in the affected family members was higher-order orofacial motor impairment that was best exemplified in speech. These studies together really showed the phenotype. This is something that’s used a lot to describe genetics. These are characteristics: cognitive, personality, behavioral, psychiatric patterns that typify a disorder. We know that speech requires precise selection and coordination of timing sequences of rapid orofacial movements. We also know that, so does feeding. I wanted to learn more about this FOXP2 gene and, again, totally new to this area. I had to do my own research. FOXP2 is located on chromosome 7 and it encodes a multidomain transcription factor that belongs to a large class of deoxyribonucleic acid (DNA) binding proteins known as winged helix or forkhead proteins. These play an important role in regulating the expression of genes involved in cell growth, proliferation, differentiation, etc. They also interact and regulate downstream gene targets that control expression levels, with those genes that the FOXP2 talks to. And what’s important here is that the FOXP2 regulates many downstream target genes associated with common forms of language impairment. It may not be the FOXP2 gene itself, but what it communicates to downstream. FOXP2 is expressed in the brain, the lungs, the heart, and the gut. I kept considering, OK, so what does this have to do with feeding? FOXP2 exists also in many vertebrates, showing comparable neural expression patterns in humans, monkeys, rats, birds, crocodiles, and zebra finch. So why do we care about this? Well, if you can find out more about the gene that you’re interested in looking at through animal studies, you can see what happens if there’s a deletion or if something goes wrong in the chain. There have been notable studies that have shown in young zebra finch functional knockdown studies. This is an experimental technique where the expression of one or more of the genes is reduced. So, you take a zebra finch, you knock off one of the FOXP2, and they found incomplete, inaccurate vocal imitations during song learning. Knockout mice, and now we’re working with the mice model, who had mutations with two copies, and this is called homozygous mutations of FOXP2. They had impaired vocalizations, lung and brain development. And when they compare that to mutations in just one copy, there were just reduced vocalizations.

44  CASE STUDIES IN PEDIATRIC DYSPHAGIA

What we’re learning from these animal models is that if one or two of the FOXP2 are mutated or damaged, we’re going to see different outcomes. We’re going to see either reduced vocalizations or a more in-depth impairment. So, getting back to the news ticker that said, “Do you know why are women so chatty? Because they have this higher FOXP2 expression.” I went and found that article. It is by Bowers and colleagues and showed sex-mediated differences in children. In this study, they examined the cortex of children postmortem. They found that the left hemisphere in 4-year-old boys, essentially Brodmann’s area 44 or Broca’s area, had significantly lower FOXP2 expression than their peers (Bowers et al., 2014). Now we’re showing this finding that FOXP2 is differentially expressed based on sex. I’m taking this journey of learning more about FOXP2. We also know that there are speech and language and feeding differences that we’ve seen clinically between males and females as they are developing. So, these prior studies also pointed to the notion that FOXP2 could be differentially expressed among populations. The first study that I did with Dr. Maron aimed to determine whether salivary FOXP2 gene expression levels at the start of oral feeding attempts were predictive of oral feeding success in preterm newborns. In this study, we sampled gene expression from sampling the saliva in infants. We looked at 21 infants. They were about 30 to 34 weeks, birth gestational age. We collected this saliva at the onset of oral feeding. They were in the NICU. They had received their orders for per os (PO) or feeding by mouth. At this point, we did a slight suction, got some saliva, and tried to capture the FOXP2. Total RNA was extracted from the saliva and underwent reverse transcriptome quantitative polymerase chain reaction (PCR). This is a polymerase chain reaction test (PCR). We all know PCR now that we’ve lived during a pandemic. We do this PCR test, and we see if FOXP2 is amplified. Something that I didn’t realize is important when you’re analyzing a gene is that you need to have reference genes. We used Dr. Maron’s Scalable and Accurate Targeted (SAT) reference genes that she typically uses. But it’s crucial to produce reliable PCR results, so the expression of the reference genes is used to correct for fluctuations in the target gene expression levels. These are maybe technical variations or to account for RNA you have attained. We took FOXP2 and ran them against the reference genes and we had some interesting findings. But to go back to what we looked at when we looked at oral feeding success. It was the days required to attain full oral feeds. We have a FOXP2 number, and the days it takes for a baby to take 100% PO. Our first finding from this paper that was

2.  GENETIC SYNDROMES   45

relatively novel was that FOXP2 is indeed amplified in neonatal saliva, if we were to go up to infants and were able to see their expression level of FOXP2. Some genes are amplified later in development and we’re going to touch on that in a minute. But we found in these young infants in the NICU that we were able to test the FOXP2. That was the first step, “Can we even detect it?” Then, in our model, we controlled for infants’ sex because other studies have shown that to be an important variable, the birth gestational age and the weight at the time of the saliva collection. We found that FOXP2 expression was significantly associated with oral feeding success. The higher the expression level of the FOXP2, the shorter the duration to be on full oral feeds. Salivary FOXP2 expression levels were significantly associated with oral feeding success in the preterm newborn (Zimmerman et al., 2016). While we were writing this manuscript, I received an email from a mother whose son was in the NICU and they found that their son had a FOXP2 deletion. She searched the web and could not really find anything on this topic. This baby was really struggling with feeding, and she came upon my name and emailed me. This mother was very, very kind and agreed to let us do a case study of her son. To summarize that case study, her son was a nondysmorphic appropriately and symmetrically grown male infant born at 35 weeks’ gestational age. He had a prolonged NICU stay because of persistent oral feeding incoordination. He eventually required a gastrostomy tube placement because of his feeding challenges. Cardiac and neurological imaging were within normal limits (Zimmerman & Maron, 2016). At this point, the neonatologist was starting to wonder what is happening as far as the feeding progression. Why isn’t this baby moving forward? And at some point, somewhere in the process, because of the uniqueness of this case, they decided to go ahead and get some genetic testing done. A microarray analysis found a ∼9-kb loss within chromosome band 7q3.1 that contains exon 2 of FOXP2 demonstrating a single copy of this region instead of the normal two copies. So, this case study really expands our current understanding of the role FOXP2 exerts on motor planning and coordination and really is important in looking at the consequences for future diagnosis and treatment of infants with FOXP2 deletions, mutations, and varying levels of gene expression. If somebody had not ordered that genetic test, we would not see through that microarray that this child had a FOXP2 deletion. We continued to want to learn more about FOXP2, but also other speech and language genes. A lot of my work is focused on feeding

46  CASE STUDIES IN PEDIATRIC DYSPHAGIA

and particularly speech having a lot of shared connections. We know that kids with speech and language difficulties tend to also have a history of feeding delay, and it depends on which part of the scale we’re looking at. Are we working with 8-year-olds and asking them retrospectively if they’ve had feeding delays? What is lacking in our field is and what my lab is starting a large project to do, to collect these feeding data along the way and then measure it in relation to important speech motor control outcomes and language outcomes as well as cognition. We know that human infants are born with a very small capacity to do a lot, they’re called altricial, meaning they need nourishment from a caregiver. This is very much unlike a giraffe who gets up and walks around. They need us to take care of them, but one behavior and reflex that’s kind of on target, right when the baby is born, unless there are delays, is sucking and feeding. In this way, we can use this as a window into the infant’s developing brain to try to understand. These are the same pathways that speech and language are going to be using too to deal with the sensory motor environment, so we wanted to look more about speech and language genes in relation to oral feeding outcomes. In this study, we aimed to determine if the speech-associated genes FOXP2 contact an associated protein, like CNTNAP2 (cat nap), which is directly downregulated from that FOXP2 and tends to be more implicated in language delay. Glutamate receptor ionotropic N-methyl-D-aspirate 2A or GRIN2A and neurexin 1 were also considered. And we wanted to see if these genes were detectable in neonatal saliva and could predict feeding outcomes. Like other methods I described, we collected saliva from 51 preterm infants. This time, they were between 30 and 34 weeks’ gestational age at birth, and we looked at different stages along their oral feeding development for when we collected their gene expression. Here, we looked at binary expression profiles. So, did they have the gene, yes or no, in relation to the days it took to attain full oral feeds? We found that GRIN2A and neurexin 1 were rarely amplified in neonatal saliva and therefore were not informative. That’s important to say because sometimes when we consider these different research paths, we want everything to be significant, we want everything to be connected, but as we’re exploring these genetic profiles, it means that these genes come on target later, and that would make sense if they were speech and language genes. Infants who amplified the FOXP2 gene but not cat nap 2 at the start of oral feeds achieved oral feeding success 3 days sooner than other different combinations. So interesting, FOXP2 and cat nap 2

2.  GENETIC SYNDROMES   47

may be informative in predicting oral feeding outcomes in newborns if we look at these gene expression levels compared to each other. It also warrants that a salivary analysis at the start of the oral feed trial may inform feeding outcomes. I think it’s important to look at the timing of when you’re collecting the salivary samples. The takeaway from these studies is that, with something that’s unknown and unexplored as far as genetics goes, you should work with a collaborator who is an expert as I did with Dr. Maron, who really had this novel and informative transcriptome analysis from saliva. We did a lot of educating each other on the speech and language aspects. She’s also a feeding expert and a neonatologist, so she didn’t need much information from me there, but together our expertise complemented each other to learn more about this gene. Even when there were situations where we didn’t have expected findings, that’s OK because it was still informative as to, at what time point are these genes amplified? And then I think, the takeaway is that feeding is extremely complex and that genetics and gene expression levels are one piece of the puzzle that I think we often don’t think of on a regular basis. I think it’s something to consider and to know that these genes do and can affect our feeding development. J:  What do we know about amplification and timeframe? Is there a lot of variability there regarding when gene expression is amplified like you were talking about with FOXP2? E:  We don’t know much. Dr. Maron’s work she’s doing looking at a host of different genes and different sensory factors and proteins to try to learn more about how expression of different platforms of facial development or olfaction. She’s looking at sensory information through saliva. I think we’re at the point of knowing, OK, so now we know FOXP2 is amplified. Yes, cat nap 2 is consistently amplified. These other kinds of further downregulated genes are not. We’re trying to determine at what point of the feeding process, likely at the beginning of the oral feed, will help us to understand what interventions could be useful and to see if, for example, we just need more time, maybe the amplification will happen a week later. Or maybe there’s populations like those for preterm where the amplification might occur at a different time compared to infants born full term. We’re trying to understand the nuances of this because it’s relatively new. I think the future would be to eventually, try to learn, do I have, because I’m a chatty person, more expression of the FOXP2? Consider some clients maybe don’t have FOXP2 gene expression. They might be more anxious about communication or are delayed in

48  CASE STUDIES IN PEDIATRIC DYSPHAGIA

their speech, are any of the clients you are seeing to feed who aren’t progressing? Maybe they have a mutation that we’ve never examined. But we won’t know this until more future studies are done across patient populations, across at-risk populations to truly understand, like we talked about earlier, what is typical of this type of gene expression? What happens when there’s a mutation and things go in a different way for that gene? J:  Fascinating. That’s exciting for our field. It is exciting to think how much that could affect intervention and improve intervention to have that information and particularly the timing element of it too. E:  Absolutely. J:  Talk a little bit about graduate students and young clinicians. What kinds of things are important to understand in terms of gene expression and syndromes? E:  I think, for a beginning clinician, it can seem overwhelming to navigate this space. When I shared my journey with this one gene, it was really to show that I too did not know anything about this area. I had to seek out the resources, the collaborators, the experts to learn more. And it ended up really being a fun and cool study. I think there are key aspects and when clinicians are working in any specialty area or with any specialty population, that education is key. I would really encourage clinicians to spend time understanding the traits or the phenotypes of the syndrome that you’re working with or certain genes. A phenotype trait is an obvious observable and measurable trait. I think that if you know the specific behavioral phenotypes in these phenotypes, in general, you’re also able to see strengths and limitations within certain syndromes. J:  What kinds of questions should we be asking when we get a new referral and see an unfamiliar genetic syndrome in the case history? E:  Are your assessments or therapies potentially drawing to the strengths of certain syndromes strengths compared to a limitation? I think that it’s important to be mindful of. Are there specific anatomical or structural differences that you should be aware of, and how these differences are affecting feeding? J:  Talk about the differences within the diagnosis and how comorbidities can play a role. E:  While it’s very important to know the underlying phenotypes and the traits, there’s also a lot of heterogeneity within a syndrome, so just because you saw one individual with fragile X (FXS) feed in a

2.  GENETIC SYNDROMES   49

certain way, that doesn’t mean all individuals with FXS will feed in that certain way. So, you need to make sure you’re being careful about assessments and criteria, and really keep that individual piece of the puzzle, which I think is so important for feeding. In general, we learn all these important pieces of the puzzle, but we need to understand that this is one client that we need to also look at on an individual basis and not miss some of those specific important aspects for that client. And then I also think it’s important to be mindful of co-occurrences of disorders. Many individuals, for example with FXS, may also have ADHD (attention-deficit/hyperactivity disorder) or may also have autism or a seizure disorder. All of these can affect feeding, swallowing, and communication outcomes. One other key point to keep in mind is where the family that you’re working with is going to be along the course of discovery for that syndrome. J:  Talk about how that discovery phase can affect treatment. E:  You might be working with a family of an infant with Down syndrome and trying to understand, “At what point did they know that the child had Down syndrome?” Other syndromes, like FXS, may not be diagnosed until later. So you might be, at the very onset, where you’re going to see someone at a Down syndrome clinic or a Down syndrome program and it’s very clear to you the syndrome that you’re working with. If you’re working with sucking and feeding, and there’s challenges and you’re wondering, “Could it be something else?” And maybe this individual then goes on to have FXS diagnosis, just being aware that you may not know the full story. And, to help the family with the resources depending on where they’re at, but also just be mindful of that process for the family. And then, I think, keep looking at research in this area. J:  It is also important to keep track of research and keep learning, right? E:  I think with more and more technology, we can learn and understand more about genetics and the various syndromes that we work with. But I think that much more is needed. So, as we discussed, we don’t know what is typical of gene expression. Let alone, when it’s amplified, when it’s not, which are the key feeding and swallowing genes? I think that those data are really emerging, so I would continue to tell clinicians to keep searching. J:  What are some ways to keep breaking down genetic information? E:  Other ways to work with genetic differences, which was another question along the same path, is to really know the terms. I tried to

50  CASE STUDIES IN PEDIATRIC DYSPHAGIA

define terms as I presented them today, but I think it’s hard to communicate with the genetic specialist or even the physician that you might be working with to say, “I’d like a genetic test because this feeding challenge seems above and beyond what we might expect.” I think both in research as in clinic, as you work with different professions, trying to get an underlying foundation of the words: mutation and knockout and what these words mean. Sometimes it doesn’t take going back to graduate school, just taking a little bit of time in your day to try to understand these keywords so you can really digest the research when it is available. A lot of the studies that I share with different animal models are cumbersome to read, but once you have that understanding, they’re easier to get through. J:  Thank you for breaking down this information so well for the reader. Do you have any resources that you would recommend for engaging with those terms and really understanding them in a foundational way? E:  There’s not a specific genetics book that I read. There’s a database of genomic variants that people can look up. When a case study is done, when you are the first to report on some genetic deviation or deletion with a set phenotype, you must report it. If you were to ask for a genetic test on a patient and then you get the report and you have no idea what you are reading, I would just try to take time like a dictionary is great, like what are these words. A simple Google search. I’m sure there are simple genetic books. And there’s more research always being done in this field. I think that will be a great resource. J:  After reading this interview, hopefully readers will find some of the studies you have done with Dr. Maron. E:  One of the articles with Dr. Maron was in American Journal of Speech-Language Pathology that we wrote together. I think that it has more clinically applicable information (Bartolome et al., 2020). J:  Give us some of your thoughts regarding genetic testing. E:  Something else that’s important is when to consider genetic testing: I have a friend who is a sociologist, and she has all these little zebras in her office, and it’s from a pharmaceutical company. The zebras are all a different color, and it’s like “expect the unknown.” The implied message is trying to encourage everyone to do genetic testing for everything. And I wouldn’t want to go that far, but I think that if the case is unusual and maybe in conjunction with family history, you might start to wonder whether the feeding challenges have an underlying genetic etiology. And I think that is something to talk through

2.  GENETIC SYNDROMES   51

as a team, the speech pathologist, the physician, or, potentially, the genetic expert on the team. J:  I was reading a little bit about your work in Puerto Rico, and I really wanted to learn more about the project that you are involved in there. Talk a little bit about how that project began and what you are working on in Puerto Rico. E:  Yeah so, I think it’s kind of interesting. A common thing that most of these studies share is sending random emails on random data to people. I think that feeding is so complex that if we think of it siloed, we’re never going to get anywhere. I reached out to Dr. Maron, she is a feeding expert, but we look at different things and come from different backgrounds and how we can inform each other. J:  How did you learn about the Puerto Rico project? E:  My work in Puerto Rico involves environmental health. At Northeastern every day, we get a “News at Northeastern” email that comes up and I always take the 30 seconds to skim through. There was this story about why the preterm birth rate was so high in Puerto Rico. A decade ago, it was much higher than the mainland United States. The person leading the study, Dr. Akram Alshawabkeh, does environmental health at Northeastern but looks at water and air quality. He is a civil engineer, totally different than my areas of interest, but I emailed and said, “How are you following these preterm infants? Are you looking at their feeding development?” That was the email that started this collaboration. They were really asking the question, “Is there something in the water? Is there something in the air that’s causing this preterm birth rate to be so high?” J:  Tell us about the first study. E:  The first study was the Puerto Rico test site for exploring contamination, threats or protection. This study looks at pregnant moms during their entire pregnancy, but we collect data at the first, second, and third trimester. These are a lot of exposure data, so you might look at maternal urine to try to determine how many phthalates the mom has been exposed to. Phthalates are like plastics. So, what is the water like that the mom is drinking? What are the other chemical toxins that mothers put in their body? What is the mother eating? How stressed is the mother? This group of studies started with the moms and the pregnancy without that outcome. After considering, “Are these higher chemicals resulting in higher preterm birth?” when I came along there was no follow-up for the children. Together with a large group of interprofessional researchers and pediatricians and other

52  CASE STUDIES IN PEDIATRIC DYSPHAGIA

developmental specialists, we created CRECER, which is Spanish for grow and it’s a child environmental health center program. And this is looking at air pollution multiple chemicals and trying to see what this prenatal exposure as far as chemicals does to the child’s development. When we first started meeting about this project, my job, as a speech pathologist, was to come up with the kind of sequence of neurodevelopmental tests that we would run so we are administering the Battelle Developmental Inventory (BDI) (Battelle Developmental Inventory, 1988), the Ages and Stages Questionnaire (ASQ) (Squires et al., 2015), and the Child Behavior Checklist (CBCL) (Achenbach, 1999). We utilize a handful of neurodevelopmental tests across domains from birth to 4 originally, now it is 6. Something that’s kind of unique was that I mentioned to the group that in my lab, I have a quantitative way to analyze non-nutritive suck in infants. I have a pressure transducer system that attaches to a pacifier, and we can see the baby suck pattern in real time. They’re like “What?” So, I proposed, what if we did that right when the babies were born almost as that test is a real index of brain integrity. Sucking is the thing that the child is doing from a motor standpoint soon after birth. Could we learn more about the central nervous system integrity at this time point, and potentially could we learn about the exposures? We have about 250 babies that we’ve done this on so far, and we have data showing that increased maternal stress during pregnancy alters infant suck patterning. Environmental exposures like phthalates exposure, plastics, higher air pollution can alter infants’ suck patterning soon after birth. This is the first time that, to my knowledge, anyone has looked at exposures or the environments from this kind of toxicant level. When I was in the NICU, I was looking at the environment, for example, let’s dim the lights, let’s modify those types of environmental factors, not the environment itself. The other thing I’ve learned along the way is that these toxic exposures, when they happen at a critical period, like pregnancy or certain time points during pregnancy, result in even worse outcomes for the baby. So, there’s critical periods where you know you want to protect yourself from exposure. But in general, the work in Puerto Rico has been extremely impactful because we do report back for our participants, and our pediatrician when she does her various measures and might even be the first one to diagnose a child with autism. We have been the first to really find a lot of differences or challenges in children that maybe their pediatricians didn’t pick up because their visits are extremely short and their early intervention services are really limited, so it’s been impactful to not only be able to provide them with this assessment information where they would not have gotten this otherwise. But then, to give them

2.  GENETIC SYNDROMES   53

the report to then give to their pediatrician to be like, “See, my child is three standard deviations below the mean in communication, so I need early intervention.” It’s really been nice to see your pediatrician really advocate for the children we see as they grow and then we can also measure that development over time (Morton et al., 2021; Zimmerman et al., 2021). J:  What a far-reaching study. Yeah, I’m glad you guys were able to expand it to age 6. That’s wonderful. E:  Yeah, and now it’s still ongoing. Now, we’re a part of this program called ECHO, which is the Environmental Influences on Child Health Program, and this is a program that’s run by the National Institutes of Health (NIH). Basically, what it does is collect data from all these child centers that are child environmental health centers that are all around the United States and pulls the data together. It might be hard to make a good case with 200 infants. If we can pull the data together and do more advanced modeling, we’re able to talk more about exposure and child development. It’s been interesting. I presented a few years ago at ASHA with my colleague who is an environmental toxicologist. It was informative for her too. I was like, nobody in our fields considers the environment at all, but we’re working with many people who likely have challenges or delays because of environmental exposures. We don’t realize it and we go into the home where we’re seeing the exposure. How could we be providing resources when this is part, I  would argue, of some of our standard of care while we’re seeing pesticide or tons of plastics. Can we provide this as part of the resources that we’re providing to parents because we have this unique view of the home and the environment that pediatricians really don’t? J:  We have unique insight when doing home visits, don’t we? E:  Right, and even in the NICU, they’ve done a lot of testing on the number of plastics that the babies are exposed to during their NICU stay. We have not looked at how that relates to feeding or their subsequent development. There’s been steps to reduce that exposure, but it’s all a lot and it’s complex. I would encourage grad students and SLPs in general to really consider these other fields and their influence on the sucking and feeding and communication challenges we see with our various populations. J:  It’s very complicated, isn’t it? I had so much fun learning more about you and you are a busy woman. E:  I am a busy woman.

54  CASE STUDIES IN PEDIATRIC DYSPHAGIA

J:  You are doing a lot of great research in a lot of different areas, and you probably could have spoken about just about any of these chapters. But it sure was great that we got to sit and talk about gene expression in your work with Dr. Maron.

REFERENCES Achenbach, T. M. (1999). The Child Behavior Checklist and related instruments. In M. E. Maruish (Ed.), The use of psychological testing for treatment planning and outcomes assessment (pp. 429–466). Lawrence Erlbaum. Bartolome, R., Kaneko-Tarui, T., Maron, J. L., & Zimmerman, E. (2020). The utility of speech-language biomarkers to predict oral feeding outcomes in the premature newborn. American Journal of Speech-Language Pathology, 29, 1022–1029. Battelle Developmental Inventory. (1988). DLM Teaching Resources. Bowers, J. M., Perez-Pouchoulen, M., Roby, C. R., Ryan, T. E., & McCarthy, M. M. (2014). Androgen modulation of FOXP1 and FOXP2 in the developing rat brain: Impact on sex specific vocalization. Endocrinology, 155(12), 4881–4894. https://doi.org/10.1210/en.2014-1486 Brackett, K. (2017). Ideas for transitioning off a feeding tube. http://pediatric​ feedingnews.com/ideas-for-transitioning-off-of-a-feeding-tube/ Brady, G. C., & Roe, J. W. G. (2020). Whose service is it anyway? Patients as co-designers to improve dysphagia care pathways. Perspectives of the ASHA Special Interest Groups, 5(4), 1011–1014. https://doi.org/10.1044/2020_ PERSP-19-00177 Cooper-Brown, L., Copeland, S., Dailey, S., Downey, D., Petersen, M. C., Stimson, C., & Van Dyke, D. C. (2008). Feeding and swallowing dysfunction in genetic syndromes. Developmental Disabilities Research Reviews, 14(2), 147–157. https://doi.org/10.1002/ddrr.19 Desai, H., & Lim, A. N. (2019). Neurodevelopmental intervention strategies to improve oral feeding skills in infants with congenital heart defects. Perspectives of the ASHA Special Interest Groups, 4(6), 1492–1497. https://doi​ .org/10.1044/2019_PERS-SIG13-2019-0017 Fuller, L., Miles, A., Dharmarathna, I., & Allen, J. (2022). Variability in swallowing biomechanics in infants with feeding difficulties: A videofluoroscopic analysis. Dysphagia, 37(6), 1740–1747. https://doi.org/10.1007/s00455-02210436-2 Jackson, A., Maybee, J., Moran, M. K., Wolter-Warmerdam, K., & Hickey, F. (2016). Clinical characteristics of dysphagia in children with Down syndrome. Dysphagia, 31(5), 663–671. https://doi.org/10.1007/s00455-016-9725-7 Kiserud, T., Benachi, A., Hecher, K., Perez, R. G., Carvalho, J., Piaggio, G., & Platt, L. D. (2018). The World Health Organization fetal growth charts:

2.  GENETIC SYNDROMES   55

Concept, findings, interpretation, and application. American Journal of Obstetrics and Gynecology, 218(2S), S619–S629. https://doi.org/10.1016/j​ .ajog.2017.12.010 Lai, C. S., Fisher, S. E., Hurst, J. A., Levy, E. R., Hodgson, S., Fox, M., . . . Monaco, A. P. (2000). The SPCH1 region on human 7q31: Genomic characterization of the critical interval and localization of translocations associated with speech and language disorder. American Journal of Human Genetics, 67(2), 357–368. https://doi.org/10.1086/303011 Madhoun, L. L., & Jadcherla, S. R. (2016). The complex relationship between dehydration and dysphagia in neonates. Perspectives of the ASHA Special Interest Groups, 1(13), 57–65. https://doi.org/10.1044/persp1.SIG13.57 Medline Plus. (2022, April 14). Genetics. National Library of Medicine. https:// medlineplus.gov/genetics/ Morton, S., Honda, T., Zimmerman, E., Kirwa, K., Huerta-Montanez, G., Martens, A., . . . Suh, H. H. (2021). Non-nutritive suck and airborne metal exposures among Puerto Rican infants. The Science of the Total Environment, 789, 148008. https://doi.org/10.1016/j.scitotenv.2021.148008 Muldoon, D., Meyer, L., Cortese, J., & Zaleski, R. (2021). A literature review: Evidence base in speech-language pathology for the management of pediatric oral phase dysphagia. Perspectives of the ASHA Special Interest Groups, 6, 444–453. https://doi.org/10.1044/2021_PERSP-19-00080 National Down Syndrome Congress. (2022). Down syndrome clinic listings. https://www.ndsccenter.org/programs-resources/health-care/down-syn​ drome-clinics/1 Squires, J., Bricker, D., & Twombly, E. (2015). Ages & Stages Questionnaires ®: Social-Emotional, Second edition (ASQ ®:SE-2): A parent-completed child monitoring system for social-emotional behaviors. Paul H. Brookes. Wong, N. S., Feng, Z., Rappazzo, C., Turk, C., Randall, C., & Ongkasuwan, J. (2020). Patterns of dysphagia and airway protection in infants with 22q11.2-deletion syndrome. Laryngoscope, 130(11), 2532–2536. https:// doi.org/10.1002/lary.28317 Zimmerman, E., Maki, M., & Maron, J. L. (2016). Salivary FOXP2 gene expression and oral feeding success in premature infants. Cold Spring Harbor Molecular Case Studies, 2, a000554. Zimmerman, E., & Maron, J. L. (2016). FOXP2 gene deletion and infant feeding difficulties: A case report. Cold Spring Harbor Molecular Case Studies, 2(1), a000547. https://doi.org/10.1101/mcs.a000547 Zimmerman, E., Watkins, D. J., Huerta-Montanez, G., Rosario Pabon, Z., Feric, Z., Manjourides, J., . . . Meeker, J. D. (2021). Associations of gestational phthalate exposure and non-nutritive suck among infants from the Puerto Rico Test site for Exploring Contamination Threats (PROTECT) birth cohort study. Environment International, 152, 106480. https://doi.org/10.1016/j​ .envint.2021.106480

3 Aerodigestive Management of Swallowing Disorders INTRODUCTION Children with pediatric dysphagia often experience issues with multiple organ systems and require care from multiple providers. Currently, there are over 50 aerodigestive centers across the United States in 32 states. As more aerodigestive centers are established, increased access to comprehensive services is possible for more children (Boesch et al., 2018; Wootten et al., 2019). A study by Collaco et al. (2015) cited an average cost savings per family per preoperative assessment clinic (PAC) visit of $182 (Yibrehu et al., 2020). A consensus definition of a pediatric aerodigestive patient is a child with a combination of multiple and interrelated congenital and/or acquired conditions affecting airway, breathing, feeding, swallowing, or growth that require a coordinated interdisciplinary diagnostic and therapeutic approach to achieve best outcomes (Collaco et al., 2015). See Figure 3–1 for sharpening clinical skills in aerodigestive disorders. In the following case study, Sam provides an example of the importance of managing a complex system, considering structural and functional challenges. He also represents a child with a dynamic system. Oral dysphagia was present, as evidenced by decreased oral strength and coordination (e.g., 57

58  CASE STUDIES IN PEDIATRIC DYSPHAGIA • Person-Centered Focus on Function: Pediatric Feeding and Swallowing [PDF] https://www.asha.org/siteassets/uploadedfiles/icf-pediatric-feedingswallowing.pdf • Aerodigestive Disorders of the Airway (Pharynx and Larynx) [PDF] https://www.asha.org/siteassets/practice-portal/aerodigestive-disorders/ aerodigestive-disorders-of-the-airway.pdf • Aerodigestive Disorders of the Pulmonary Tract (Trachea, Bronchi, and Lungs) [PDF] https://www.asha.org/siteassets/practice-portal/aerodigestive-disorders/ aerodigestive-disorders-of-the-pulmonary-tract.pdf • Aerodigestive Disorders of the Upper Digestive Tract (Esophagus) [PDF] https://www.asha.org/siteassets/practice-portal/aerodigestive-disorders/ aerodigestive-disorders-of-the-upper-digestive-tract.pdf • American Speech-Language-Hearing Association (2022, June 18). ASHA Practice Portal —Aerodigestive Disorders https://www.asha.org/practice-portal/clinical-topics/aerodigestive-disorders/​ #collapse_7 Figure 3–1.  Sharpening clinical skills in aerodigestive disorders.

lip closure, tongue lateralization, jaw use). Neuromuscular (cerebral palsy) and structural issues (jaw micrognathia) affected oral skill. Pharyngeal issues were also both structural and functional (e.g., tracheomalacia, silent aspiration). Surgical intervention, growth, and development contributed to structural and functional improvements (Evans, 2018; Sidman et al., 2001). Sam was diagnosed with cerebral palsy, tracheomalacia, and micrognathia. As treatment was under way, he received a diagnosis of Sanfilippo syndrome (Fedele, 2015). Research intervention from both a neuromuscular and a structural perspective will improve the quality of evidence-based treatment (Benfer et al., 2013; Boesch et al., 2006; Clark, 2003; Morgan et al., 2012; Sheppard, 2005, 2008). Though Sam was seen in an outpatient setting, the interview for this chapter gives the reader an opportunity to appreciate a different setting of care. Through her years of experience, acute care speech language pathologist Denise Chapman provides practical advice in the management of pediatric dysphagia and how collaborative work in their cardiac unit has led to better patient outcomes.

Initial Information About Diagnoses and Medical and Developmental History (See an image of Sam at 2 years, 5 months in Figure 3–2.) Prior to his initial outpatient evaluation, Sam received a videofluoroscopic swallow study

3.  AERODIGESTIVE MANAGEMENT OF SWALLOWING DISORDERS   59

Figure 3–2.  Sam at 2 years, 5 months.

(VFSS) at a neighboring acute care facility. The following VFSS report was reviewed prior to his outpatient evaluation: He has had feeding difficulties since birth and has been primarily nil per os (NPO) for all his life. He has had several VFSSs that showed aspiration in all consistencies. He received Alfamino bolus feeds 210 mL (about 7.1 oz) six times per day. He has participated in small per os (PO) trials (primarily water via syringe and tastes of puree via fingers and toys). Parents reported difficulty with secretion management (substantial drooling and suctioning him about once a day). They indicated that breathing and drooling have improved following jaw distraction. His parents also reported that he has a strong cough and has never been diagnosed with pneumonia. For the procedure, he was seated upright in a Tumbleform chair. His father fed him. He was awake/alert interactive. He was unsure of PO trials and intermittently accepting but was mostly accepting liquid trials with time encouragement and reinforcement. He expectorated most puree/ thickened liquid trials. Presentation: Syringe Oral:  Anterior spillage of the bolus, pooling in anterior sulcus, reduced base of tongue retraction, decreased anterior-posterior

60  CASE STUDIES IN PEDIATRIC DYSPHAGIA

lingual movement, premature spillage of the bolus to the hypopharynx, passive leak over base of tongue, poor bolus formation/movement, prolonged oral transit time, tongue residue Pharyngeal:  Reduced palatal movement, failure to initiate a swallow, unable to complete a swallow, pyriform sinus residue, vallecular residue, cricopharyngeal dysfunction, delayed swallow, laryngeal penetration, silent aspiration Honey liquid type:  Minimal swallows view of honey-thick liquid Puree food type:  Minimal puree swallows viewed Assessment Sam exhibited a severe to profound oropharyngeal stage dysphagia characterized most prominently by difficulty initiating and completing a swallow. He also showed poor oral control with anterior loss, passive leak of the bolus over the base of the tongue, and premature spillage of the bolus to the hypopharynx; reduced lingual movement and reduced palatal movement; cricopharyngeal dysfunction; and a delayed swallow. He was first presented with a thin liquid via syringe and several boluses. He was inconsistently able to initiate a swallow, and even when he was able to initiate and complete a swallow, residue remained in his hypopharynx (valleculae and pyriform sinuses). He was noted to aspirate liquid from the pyriform sinuses into the airway both before and after swallows several times (of note, aspiration was not directly viewed during a swallow). No cough response was present with aspiration events. Numerous attempts were made to help Sam accept puree, thin puree, pudding, and thickened liquid consistencies, but almost all contrasts of these consistencies were lost anteriorly, and minimal swallows were viewed. Trials were eventually stopped secondary to extensive fluoroscopy time. Of note, tonsils and adenoids appeared large. (See Figures 3–3 and 3–4 for lateral views of the aerodigestive tract.) Recommendations (a) Based on the VFSS and severity of dysphagia, Sam should continue to receive all nutrition via a feeding tube. (b) Pending Sam’s pulmonologist approval, it appears reasonable for Sam to continue PO tastes for oral stimulation with aggressive oral care and close monitoring of respiratory status. (c) Follow up with ENT about results/recommendations. Of note, tonsils and adenoids (particularly adenoids) appeared large. It is hypothesized that reduced palatal and lingual movement may be partially related to adenoid hypertrophy. (d) Continue outpatient feeding therapy to address oral motor feeding skills. (e) May consider repeating VFSS in the future if management of secretions, respiration, and small PO trials/tastes

INFANT Hard Palate

Soft Palate

Vallecula

Maxilla

Nasopharynx Tongue

Mandible

Hypopharynx Epiglottis

Hyoid Larynx

Esophagus

Trachea

Figure 3–3.  Lateral view of the aerodigestive tract. Source: Pediatric Swallowing and Feeding: Assessment and Management, Third Edition (p. 12) by Arvedson, J. C., Brodsky, L., and Lefton-Greif, M. A. Copyright © 2020 Plural Publishing, Inc. All rights reserved. Used with permission.

OLDER CHILD

Nasopharynx Tongue

Soft palate

Oropharynx

Vallecula

Hypopharynx Hyoid

Larynx

Epiglottis Trachea Esophagus

Figure 3–4.  Lateral view of the older child’s upper aerodigestive tract. Note the wide distance between the soft palate and the larynx. The elongated pharynx is unique to humans and has allowed the development of human speech production. 61

62  CASE STUDIES IN PEDIATRIC DYSPHAGIA

improve. (See Figure 3–5 for a comparison of two instrumental measures used in pediatric dysphagia, VFSS vs. fiberoptic endoscopic evaluation of swallow [FEES].) (f) Follow up with gastrointestinal (GI) regarding results/ recommendations.

Initial Evaluation At 2 years, 5 months, Sam was referred for outpatient feeding and swallowing evaluation following a mandibular distraction procedure performed a few months ago. Sam’s father and nurse also attended the evaluation. Outpatient feeding and swallowing evaluation:  Sam was born at 33 weeks’ gestation (about 7 and a half months) due to placental abruption and trauma during delivery. His birthweight was 5.5 lb. Following birth, he was hospitalized in the neonatal intensive care unit (NICU) for 2 months. During his NICU stay, he was diagnosed with dysphagia, mild cerebral palsy, and tracheomalacia. He received a G-tube at 1 month of age (unadjusted) due to feeding difficulties. He also received pressure equalization tubes at approximately 7 months old. The tubes were replaced around a year ago due to failure of hearing tests from fluid in the ear. Around that same time, he also had a mandibular distraction. A few

FEES

VFSS

Focus limited to pharyngeal phase, contraction of pharynx prevents direct visualization during the swallow

Evaluates all phases of swallowing with no loss of view

Direct visualization of anatomy and function, may test laryngeal sensation

Limited evaluation of anatomy, no sensory testing

Minimal patient participation required, allows for evaluation of nonoral feeders

Requires patient participation

Invasive. Discomfort or presence of endoscope may affect swallowing physiology

Noninvasive

No radiation exposure. Multiple consistencies and compensatory strategies may be evaluated

Radiation exposure limits duration and frequency of study

May be performed at bedside, does not require special positioning

Must be performed in fluoroscopy suite

Figure 3–5.  FEES vs. VFSS. Source: Comprehensive Management of Swallowing Disorders, Second Edition (p. 53) by Carrau, R. L., Murry, T., and Howell, R. Copyright © 2017 Plural Publishing, Inc. All rights reserved. Used with permission.

3.  AERODIGESTIVE MANAGEMENT OF SWALLOWING DISORDERS   63

months ago, he had respiratory distress and was also recently diagnosed with rotavirus. In terms of respiratory issues, Sam’s breathing was noisy, and he used an inhaler once daily. He seemed congested and has always been a mouth breather. He was also suctioned daily approximately one to five times through the mouth. In terms of gastrointestinal problems, Sam has experienced reflux and diarrhea. He was diagnosed with allergies. High-resolution pharyngeal manometry for swallowing is a tool used to evaluate esophageal motility disorders. It was not used with Sam, but may have been beneficial in his care. See Figure 3–6 for an example of high-resolution pharyngeal manometry. It was also reported that gross motor skills were 4 to 6 months behind. His father reported that Sam had received occupational therapy in the past but was not currently receiving services. Sam is the youngest child and has two older siblings. His family is active and enjoys spending time together during mealtimes. Dad is a chef and mom is a teacher at the local elementary school.

Figure 3–6.  High-resolution pharyngeal manometry. Source: Wikimedia Commons: http://commons.wikimedia.org/wiki/File:HRM.jpg#/media/File:HRM.jpg. Used with permission from Creative Commons CCC BY-SA 3.0.

64  CASE STUDIES IN PEDIATRIC DYSPHAGIA

Current medications:  Sam was prescribed Augmentin for his allergies and used an inhaler once a day. He received three bolus feeds a day. He took omeprazole through his G-tube once a day and Flovent and Proventil through his inhaler. Sam was followed by the following physicians: a gastroenterologist, pulmonologist, neurologist, and physiatrist. Sam had another VFSS scheduled in one month. Oral mechanism:  Lips: symmetrical. Lip closure and retraction were observed. Lip rounding was not observed. Excessive drooling was also noted throughout the session. Tongue: Tongue appearance was unremarkable. Some protrusion was noted after significant prompting and modeling. Tongue tip elevation and lateralization were not observed. Teeth:  An overbite was apparent. Otherwise, the appearance of teeth was noted to be age appropriate. Jaw:  Jaw appeared to be symmetrical. Bilateral mandibular distraction scarring was clear. Hard and soft palate:  Hard palate was observed to be high. Soft palate was not adequately observed. Receptive and expressive language:  Gestural communication was clear through pointing, commenting, and requesting. Sam answered yes/no questions through head nods. Vocal language was limited to open vowels. Feeding/swallowing:  Sam had never eaten by mouth, as noted by his father. He has been tube fed since birth. Dad noted that he has had several VFSSs performed, which have all suggested aspiration. His dad expressed that Sam’s performance on VFSS has made it difficult to safely expose him to varied tastes and textures. Sam’s dad reported that he receives three bolus feedings a day of 210 mL over a 40- to 50-minute period. He reported that the time can be sped up. On occasion, his parents let Sam chew pieces of food and then take them out of his mouth before he could swallow. It was noted by the father that Sam likes the taste of strong-flavored things. “How will the muscles get stronger if we don’t let him practice using them?” asked Sam’s dad. Perceptions about oral intake: (Sam’s pulmonologist was consulted before this evaluation and cleared therapeutic trials of water with suction-

3.  AERODIGESTIVE MANAGEMENT OF SWALLOWING DISORDERS   65

ing). During the observation, Sam reached for water and brought the cup to his lips. Liquid expression was characterized by poor oral containment, no evidence of propulsion, and significant liquid loss. Throughout the evaluation, he seemed interested in mouthing items presented, immediately bringing items to his mouth (e.g., chewy tubes, empty cups, spoons). Using a straw, he blew bubbles into a small bottle of water. When a maroon spoon was introduced, Sam put it on his tongue to touch it with maximal prompting and visual support. He did not show signs of stress and seemed interested in the items put in front of him in relation to his mouth. Sam did not show any overt signs of physiological distress when putting 1 to 2 mL of water in his mouth (about 0.07-oz boluses). He actively engaged in oral suctioning with his nurse, shaking his head yes that he wanted to be suctioned when asked and signing “more” when he wanted to be suctioned again. She orally suctioned Sam on two occasions. See Figure 3–7 for Sam’s medical history timeline.

An Initial Discussion to Ease the Evaluation Process The parent interview process will help you understand their perspective and their motivation for intervention. Role-playing can be a helpful way to understand others’ perspectives on the feeding/swallowing team. What are some things that are important to the family? Sam’s family expressed the importance of family meals. They also cited the importance of him being able to eat and enjoy food.

Sam’s Case Study Timeline

• Outpatient referral at 2 years • 2 months in the NICU • Several VFSS suggesting poor airway protection with all consistencies assessed • NPO with G-tube placement at 1-month unadjusted • Mandibular distraction surgery at 2 years, 1 month • Current diagnoses: cerebral palsy, Sanfillipo syndrome, tracheomalacia, oropharyngeal dysphagia, gastroesophogeal reflux Figure 3–7.  Sam’s case study timeline.

66  CASE STUDIES IN PEDIATRIC DYSPHAGIA

What are some strengths that are already clear in putting together Sam’s treatment plan? Organizing information can be challenging. It can be beneficial to consider categorization by systems involved. For example, consider the swallow systematically. The oral phase of swallowing problems began due to structural problems (micrognathia) (Mabry, 2016; Rutter, 2006). Difficulties were further affected by upper respiratory congestion (nasal and middle ear) and limited oral experiences due to early G-tube placement and NPO status. Next, begin considering Sam’s pharyngeal phase structurally and functionally. What are some pharyngeal phase issues? (e.g., tracheomalacia, lack of airway protection). Finally, begin to consider the esophageal phase. What are the diagnoses and symptoms related to his gastrointestinal system (e.g., G-tube placement, history of reflux)? Considering the entire system can help in goal creation. As treatment goals were formulated, it became clear that more oral structure awareness and understanding would be a critical part of Sam’s treatment process. Multisensory goals needed to be created to target identification and labeling of structures. Discussion began with the family about augmentative communication as receptive language skills seemed sharply ahead of expressive language/communication skills. Sam received speech/language therapy through another provider and was progressing through a total communication approach. See Figure 3–8 for Sam’s areas of concern identified through the evaluation process.

Oral Preparatory Phase Oral Transit Phase

Sam’s Potential Areas of Concern

Pharyngeal Phase Esophageal Phase Figure 3–8.  Sam’s potential areas of concern.

3.  AERODIGESTIVE MANAGEMENT OF SWALLOWING DISORDERS   67

The following treatment goals were formulated for Sam: Identify oral body parts (teeth, tongue, and lips) through a proximal point. This goal was quickly expanded to understanding and imitative use of mouth part function (e.g., licking with tongue, smacking lips). Increase awareness and management of oral secretions by wiping his mouth × 3 with a verbal prompt.. This goal was also expanded to increase understanding of lip closure and swallowing through multisensory input (e.g., visual and tactile exposure to muscle movement in swallowing). Increase regulation during feeding and secretion management using total communication (e.g., singing “more” and “all done”) to express requesting more and all done. This goal was expanded to address improved ways of expressing physiological distress through social stories (e.g., “My tummy hurts,” “I don’t like that”). See an image of Sam at 3 years old in Figure 3–9. Conversation During Treatment Two years, 6 months — A message for Sam’s dad: We have gotten a new diagnosis for Sam since the last time we saw you. He has an ultra-rare

Figure 3–9.  Sam at 3 years old.

68  CASE STUDIES IN PEDIATRIC DYSPHAGIA

genetic condition called Sanfilippo syndrome Type B. I can bring you information on it next week. It won’t change his short-term care, but it will long term. Sam will have another MRI to get a baseline for moving forward. Further Information Regarding the Patient and Their Trajectory Following another swallow study scheduled through the aerodigestive clinic, Sam continued to demonstrate aspiration. There were some improvements in his base of tongue response, and overall skills had improved. Sam’s pulmonologist approved water trials during therapy, with Sam’s nurse present and immediate suctioning. The following treatment goals were set: Use of oral structures involved in swallowing (tongue — lick, lips — open/close, teeth — bite, nose — smell) with maximal cuing. Awareness of jaw function and use by using incisors to achieve a vertical bite pattern on a nonfood object or a food item (e.g., straw, raw carrot) with an imitative model. Self-pacing strategies (e.g., coughing, pausing) during a sip of thin liquid/puree with verbal prompts two times during a session. An open therapeutic cup was used to control bolus size and allow Sam a more natural oral transit time (e.g., versus use of a syringe requiring a more immediate base of tongue response). Complete two facilitated pharyngeal upper respiratory exercises two times a week after being modeled in session.

Discussion There was a progression of skills throughout Sam’s intervention. Therapy started following mandibular distraction surgery. This increased intraoral space provided him with much-needed lingual space to use intrinsic and extrinsic muscles. The decreased oral experience due to an early NPO status, G-tube placement, and diagnosis of mixed receptive and expressive language disorder were also contributing factors. Fortunately, Sam was interested in oral experiences. He seemed curious about varied tastes and textures. He was able to progress in tongue and jaw use through direct modeling and real-time video (e.g., selfie mode video modeling). Aggressive oral suctioning was another important strategy in Sam’s care plan. To provide him with safe oral experiences, working with his nurse and pulmonologist was necessary. As pharyngeal skills improved, oral suctioning decreased, and Sam gained skills in coughing and swallowing through intervention.

3.  AERODIGESTIVE MANAGEMENT OF SWALLOWING DISORDERS   69

The Sanfilippo syndrome Type B diagnosis was also important in informing long-term treatment. Sam’s skill were continuing to progress during treatment, but the probable regression meant goal modification. As functional skills were attained, discharge planning began. A VFSS was done a couple of years into his treatment with no signs of penetration and aspiration. He continued to use a G-tube for nutritional support but was able to take part in family meals and enjoy a wide variety of foods. See an image of Sam at 5 years old in Figure 3–10.

INTERVIEW WITH DENISE CHAPMAN, MA, CCC-SLP Denise Chapman, MA, CCC-SLP, is an acute care speech-language pathologist. She has worked at Monroe Carell Jr. Children’s Hospital at Vanderbilt since 2004. She manages a variety of pediatric disorders with a focus on

Figure 3–10.  Sam at 5 years old.

70  CASE STUDIES IN PEDIATRIC DYSPHAGIA

pediatric feeding and swallowing in the inpatient and outpatient settings, including the administration of VFSSs. She also provides services in the Level I to IV NICU, pediatric intensive care unit, pediatric cardiac intensive care unit, and general pediatric units. She is the lead speech-language pathologist on the cardiology inpatient team and contributor and helped with development of a cardiac feeding protocol that has been published in the Journal of Pediatrics (O’Neal Maynord et al., 2021).

Jennifer Wilson ( J):  How did you get started working in the acute care setting? Denise Chapman (D):  I had a friend who worked in the acute pediatric setting during graduate school. I went and observed her work with this acute care population, and I fell in love. J:  Tell me about your work on aerodigestive issues. The term that’s really taken hold in the last several years. I know this is a very multifactorial population and this is a loaded question, but what are some warning signs that there are aerodigestive issues or aerodigestive changes in a client that call for further instrumentals and/or a shift in treatment? D:  I am a periphery support to the Complex Aerodigestive Evaluation Team (CADET). We consider referring children to the CADET program for follow-up if they have failed an earlier swallow study. Kids come in who have failed earlier swallow studies at outside hospitals. A recommendation to start thickened liquid may be recommended in these cases. J:  It sounds like candidates for the CADET program often have had unresolved, long-term swallowing problems? D:  Yes, we hear those histories that there was a failed swallow study that we’ve not done anything since then. At that point, we find that further assessment of their swallow needs to be completed. J:  What are some other things that you see in their history that would result in a referral? D:  Any kind of history of pneumonia. These patients are so multi­ factorial. You could have a patient who is aspirating reflux, but you’re not seeing aspiration on a swallow study. Our kids who have pneumonia could be refluxing at night, aspirating the reflux material. If there is any history of pneumonia, we would like to do a swallow study, or if there is any coughing or choking with thin liquid.

3.  AERODIGESTIVE MANAGEMENT OF SWALLOWING DISORDERS   71

D:  We had a little one come in who has a history of stridor, reflux, and no pneumonia, but sounded congested, so we just did a swallow study outpatient. She had never been admitted. She was seen for outpatient feeding therapy, but she had never been admitted. She was aspirating all consistencies. Any time our parents say they are coughing or choking when they’re drinking, whether it be a bottle, a sippy cup, an open cup, there is a need to get a baseline instrumental study. When our younger ones cough and choke, I think that might be a more severe problem that we probably need to investigate. J:  What is another factor that would cause concern? D:  We will also do a swallow study or further investigation for any of our babies who have poor weight gain. These are the failure to thrive babies. With some of those babies, we are consulted when they come into the hospital. The doctors want us to look at their feeding skills. J:  What are some questions that you would ask when you see this diagnosis? D:  How long does it take them to feed? Are they having anterior loss of liquid during feeding? Gulping? Coordination difficulties? J:  What are some solutions you sometimes find helpful for these failure to thrive babies? D:  Sometimes it may just be that the formula is not being fortified, or they’re having a tough time gaining weight because of how much they’re taking in or how much reflux they have. Our role is to assess what is happening with their oral feeding skills. We also want to consider their skills if they are gagging and choking on solids. J:  What are some other diagnoses that you could see related to gagging and choking? D:  Sometimes we get gagging and choking on solids because they have a sensory issue, and they don’t like certain textures. With the aerodigestive kids, we have been seeing large tonsils and adenoids. J:  What is the course of action when you see enlarged tonsils and adenoids? D:  Our ENT colleagues and sometimes GI will typically say, “Let’s do a swallow study first. Let’s look at anatomy to make sure.” This is especially important for gaining more information in understanding problems with solids. What is the cause of the gagging and that

72  CASE STUDIES IN PEDIATRIC DYSPHAGIA

choking on the solids? Looking at the anatomy and making sure that nothing is impeding that swallow with consistencies. J:  What are some challenges in communicating with providers in the community? D:  Our team just gave a presentation at the National Hospitalist Organization about feeding and swallowing issues. They don’t have access to all the interdisciplinary team. We often get referrals that are not necessary. They could be eliminated with just a speech pathologist in an outpatient setting rural or not, to put eyes on them and decide “there’s no swallowing problem, they gag every time that they have this bite of Stage 3 baby food in their mouth.” In many cases, it is important to do a noninstrumental evaluation before referring for an instrumental evaluation. J:  What are some things that are helpful to you when you get a new referral from out of town? D:  I love personally to be able to read an outside speech pathologist’s report explaining what they observed. It just gives us an idea of what is happening. If an outside speech pathologist evaluated them first, we could go from there, we could narrow down what we’re looking for. This is still fluoro time, it is still radiation. We can only get a minimum amount, and we’re giving them barium, so sometimes we don’t get a great look at what is really going on. Having very specific information is helpful for an optimal swallow study. J:  Tell us a little bit about your experiences in working with children with reflux management issues. D:  I think a lot of education needs to go into our interdisciplinary work in reflux management. A swallow study is not going to assess reflux. So, if you’re concerned that this child is aspirating from reflux, whether it be during the day, whether it be after they feed and they are aspirating, it is very unlikely that we’re going to see reflux on a swallow study. We’re not seeing a 6-ounce bottle in a swallow study, so typically we’re not going to see reflux. Education would be good. I work on education even in the inpatient setting with my cardiology babies. For example, a baby might not be taking anything by mouth. Nothing by mouth, but a swallow study was ordered. This is inappropriate. We will have parents come in concerned that their baby is vomiting and choking at night. That is outside of my scope because what I have shown you is we did a swallow study when they’re drinking the

3.  AERODIGESTIVE MANAGEMENT OF SWALLOWING DISORDERS   73

bottle. There’s no aspiration. There should be a better referral process in place for reflux management. It’s great for speech pathologists to know the purpose of these different tests. Why would you do an upper GI? Why would you do an esophagram compared to a video swallow study? J:  How has increased telehealth affected your practice patterns in acute care? D:  With COVID, a lot of telehealth has been introduced. I think that on a first evaluation, if our speech pathologists have a consultation, I think the first evaluation should absolutely be in person. You cannot get the full picture. For example, I can see you right now, but I don’t know how you sound. If you were drinking, I can’t hear what I need to hear, I can’t feel, I can’t see what’s happening with a multifactorial issue. For our rural areas with a medically complex child, a “hands-on, eyes-on” first evaluation would be the most important. Do I need to see them every week in person? Maybe not? But I think that initial evaluation would be crucial. J:  It’s been really challenging during this time with these multihandicapped kids during the pandemic. J:  Tell us a little bit about the cardiac feeding program protocol in place at your institution. D:  It all came about because we have a cardiac patient who, before they came in for cardiac surgery, were often full oral feeders. But after surgery we found some incredibly significant issues. J:  What kinds of issues were you seeing? D:  There was intolerance. There was blood in the stool after surgery. There was vocal paralysis after surgery. There was upper airway inflammation due to intubation. D:  A surgeon came over and said, “We are doing too many G-tubes. What is happening? We’re sending kids out with G-tubes, left and right.” And I said, “Well, we are doing these swallow studies. It shows aspiration for whatever reason, and once our cardiologist sees aspiration, they just go immediately to G-tube.” They want to keep this heart repair functioning as well as possible and so we want to keep the lungs as clean as possible, so G-tubes placement had become the standard protocol. J:  They began to realize they did not need to move to G-tube placement so quickly?

74  CASE STUDIES IN PEDIATRIC DYSPHAGIA

D:  We started to discuss what can we do differently postop? We could hopefully get this child to a place where we do not need a G-tube. The team consisted of a cardiologist, one intensivist, a dietitian, I was the speech pathologist and a nurse practitioner. We worked in the acute care setting and the step-down setting together. Over 3 or 4 years, we established a more efficient procedure rather than immediate G-tube placement. It has changed the way that we approach our patients (O’Neal Maynord et al., 2021). J:  Can you provide us with an example? D:  After surgery, the patients are placed on an NPO status. Then we will start some trickle feeds through naso-jejunal tube (NJ) in the jejunum and then we pull back to NG. Once they tolerate that, then we go to higher calories. We adhere to this gradual feeding regimen. It sets our patients up for success rather than long-term nonoral feeds. Even our patients who have a mild cardiac defect and had a noninvasive surgery. It was still cardiac surgery. Instead of expecting them to fully oral feed when they come into surgery within 24 hours after being extubated, they may have vomiting or gagging or choking. We have set our patients up for success. Meanwhile, we are not sending them out any later. Discharge is not prolonged because we are doing this step by step. Our discharges have been timelier. The patients are now tolerating a stable feeding regimen that can get the patient home so that then they can work on further transition back to 100% PO feeding as they were before, or back to their baseline feeding as they were before. It is fantastic! J:  Then you can do an instrumental procedure? D:  We must decide whether to do a swallow study or FEES (flexible endoscopic evaluation of swallowing). If we are doing a FEES, we must coordinate with the ENT. Do we do both? J:  And now you can train others in doing this procedure. D:  I have worked on this for so long that it is second nature to me. There is a process that we have that has been so fantastic for me to learn that I’ve been able to share with other professionals, other speech pathologists at other hospitals. J:  What are some challenges in training graduate students in pediatric feeding and swallowing? D:  Sometimes we have graduate students observe. I feel like it is just so much information and these patients are so sick, and they are so fragile. With some of these patients, I begin thinking, this is one of

3.  AERODIGESTIVE MANAGEMENT OF SWALLOWING DISORDERS   75

the sickest babies in the hospital we have right now, right here, right in front of the students. It is important to have that respect for this medical complexity. This is real life. You may have to tell a mom that her baby can no longer feed because they have vocal cord paralysis after cardiac surgery, a mom that is devastated because she cannot breastfeed her baby. J:  Tell us more about teaching and mentoring young professionals. D:  We have two therapists that started. They have never worked with this population before. You can take those skills that they have learned and those interpersonal skills and things like that. But it takes some time. It takes some training too. J:  What’s your best advice to graduate students and young professionals? D:  Instead of just sending a report, that interpersonal relationship is everything. Let physicians and other providers know what we do and why we are doing it, and these are the results. Communication can help problem solving for the best care of our patients. Instead of sending a fax or providing a report, calling a referral provider and saying, “I’m worried about this patient or these other results. This is what I am seeing.” I have been as honest to say, “I do not know why this is happening, but this is where I am concerned.” Opening that discussion is important. You never forget those conversations. That builds a relationship that maximizes our taking care of our patients. J:  We need to be talking within our profession too, don’t we? D:  It is important that we do not knock down what other facilities are doing, but problem solve together instead. For example, “OK, you had thickening, you add rice cereal in your NICU. We are not allowed to. Do you have any other ideas of what we could do?” That just builds up our profession. They are doing this; they are doing this, or this doctor does not like this. We are fortunate enough to be able to talk face to face in our setting. Even if you work in a school setting or if you work in a clinic, just to be able to speak to the other therapists on board and say, “This is what I’m thinking.” You are part of a team, even if you are in private practice, you are still part of the team where you are part of this family now, you are part of their treatment plan. Just as you would sit down with your mom and dad or sit down with your kid and have a conversation. That needs to be part of our profession that somehow sometimes gets lost, and it becomes more of a procedure and just another evaluation rather than considering

76  CASE STUDIES IN PEDIATRIC DYSPHAGIA

that you are affecting someone’s life. That is what we need to keep it in the foreground. J:  What can a speech pathologist do to improve their skills in interdisciplinary practice? D:  As a CF (clinical fellow), to be in a setting where you can learn from other people and you are mentored, that is the most important. I did my CFY with a group of speech pathologists. They could guide me and direct me with their ideas. It was so beneficial for me as a therapist. It really shaped me to where I felt more well rounded. Students have better access to research. They are seeing what other facilities are doing. They may say to me, “Hey, so I know what Texas Children’s is doing.” I am like, “OK, do you know why? And let us problem solve that?” That helps us as professionals that as speech pathologists, not get stuck in a rut because of the way we did it for 30 years, but because this might be new, and this might benefit our patients. American Speech-Language-Hearing Association (ASHA) has done a fantastic job providing information about dysphasia management. I love getting other people’s ideas and asking for help with these certain patients through the ASHA Special Interest Group (SIG) 13 Swallowing and Swallowing Disorders. That has really helped us as a profession. We read them all the time. I do not want to sound haughty but when you get to a level that I am at when working with this type of patient in this setting and these are my roadblocks, you can go to ASHA Special Interest Group (SIG) 13 Swallowing and Swallowing Disorders and say, “OK, I have a cardiac patient who has vocal cord paralysis. We are going to do an injection and they might be able to unthicken liquids. Would you do unthickened liquid with a 3-week-old?” Those are the kinds of questions that not a whole lot of people can answer. It is so beneficial, hearing what other people are doing.

REFERENCES Benfer, K. A., Weir, K. A., Bell, K. L., Ware, R. S., Davies, P. S., & Boyd, R. N. (2013). Oropharyngeal dysphagia and gross motor skills in children with cerebral palsy. Pediatrics, 131(5), e1553–e1562. https://doi.org/10.1542/ peds.2012-3093 Boesch, R. P., Balakrishnan, K., Acra, S., Benscoter, D. T., Cofer, S. A., Collaco, J. M., . . . Wood, R. E. (2018). Structure and functions of pediatric aerodigestive

3.  AERODIGESTIVE MANAGEMENT OF SWALLOWING DISORDERS   77

programs: A consensus statement. Pediatrics, 141(3), e20171701. https:// doi.org/10.1542/peds.2017-1701. Boesch, R. P., Daines, C., Willging, J. P., Kaul, A., Cohen, A. P., Wood, R. E., & Amin, R. S. (2006). Advances in the diagnosis and management of chronic pulmonary aspiration in children. The European Respiratory Journal, 28(4), 847–861. https://doi.org/10.1183/09031936.06.00138305 Clark, H. M. (2003). Neuromuscular treatments for speech and swallowing: A tutorial. American Journal of Speech-Language Pathology, 12(4), 400–415. https://doi.org/10.1044/1058-0360(2003/086) Collaco, J. M., Aherrera, A. D., Au Yeung, K. J., Lefton-Greif, M. A., Hoch, J., & Skinner, M. L. (2015). Interdisciplinary pediatric aerodigestive care and reduction in health care costs and burden. JAMA Otolaryngology-Head and Neck Surgery, 141(2), 101–105. https://doi.org/10.1001/jamaoto.2014.3057 Evans, A. K. (2018). Development of laryngeal structure and function: An otorhinolaryngology perspective. Perspectives of the ASHA Special Interest Groups, 3(13), 18–27. https://doi.org/10.1044/persp3.SIG13.18 Fedele, A. O. (2015). Sanfilippo syndrome: Causes, consequences, and treatments. The Application of Clinical Genetics, 8, 269–281. https://doi.org/​ 10.2147/TACG.S57672 Mabry, K. (2016). Pediatric feeding in infants with Pierre Robin sequence following mandibular distraction osteogenesis. Perspectives of the ASHA Special Interest Groups, 1(13), 5–9. https://doi.org/10.1044/persp1.SIG13.5 Morgan, A. T., Dodrill, P., & Ward, E. C. (2012). Interventions for oropharyngeal dysphagia in children with neurological impairment. The Cochrane Database of Systematic Reviews, 10, CD009456. https://doi.org/10.1002/14651858​ .CD009456.pub2 O’Neal Maynord, P., Johnson, M., Xu, M., Slaughter, J. C., & Killen, S. (2021). A multi-interventional nutrition program for newborns with congenital heart disease. The Journal of Pediatrics, 228, 66–73. https://doi.org/10.1016/j​ .jpeds.2020.08.039 Rutter, M. J. (2006). Evaluation and management of upper airway disorders in children. Seminars in Pediatric Surgery, 15(2), 116–123. https://doi.org/​ 10.1053/j.sempedsurg.2006.02.009 Sheppard, J. J. (2005). The role of oral sensorimotor therapy in the treatment of pediatric dysphagia. SIG 13 Perspectives on Swallowing and Swallowing Disorders (Dysphagia), 14(2), 6–10. Sheppard, J. J. (2008). Using motor learning approaches for treating swallowing and feeding disorders: A review. Language, Speech, and Hearing Services in Schools, 39(2), 227–236. https://doi.org/10.1044/0161-1461(2008/022) Sidman, J. D., Sampson, D., & Templeton, B. (2001). Distraction osteogenesis of the mandible for air-way obstruction in children. Laryngoscope, 111(7), 1137–1146. Wootten, C. T., Belcher, R., Francom, C. R., & Prager, J. D. (2019). Aerodigestive programs enhance outcomes in pediatric patients. Otolaryngologic Clinics of North America, 52(5), 937–948. https://doi.org/10.1016/j.otc.2019.06.007

78  CASE STUDIES IN PEDIATRIC DYSPHAGIA

Yibrehu, B. A., Krakovsky, G. M., Rana, M. S., Pillai, D. K., Sehgal, S., Collins, M. E., . . . Bauman, N. M. (2020). Pediatric quality-of-life scores following a multidisciplinary aerodigestive team approach to manage chronic cough. The Annals of Otology, Rhinology, and Laryngology, 129(11), 1088–1094. https://doi.org/10.1177/0003489420931558

4 Gastrointestinal Diagnoses

INTRODUCTION Gastrointestinal function and nutritional status are closely linked to pediatric dysphagia. Food refusal behaviors or unexplained weight loss can signal gastrointestinal issues as young children often lack communication to clearly report pain or discomfort. A closer look at structures and functions as well as growth is often necessary to determine whether there are medical problems that might impede therapeutic intervention. Research has been emerging over the past several years regarding the gut microbiome and its complex relationship to immune function development and metabolic function. From birth, the gut microbiome provides a barrier of protection against pathogenic organisms, plays a key role in metabolic function, and influences growth of intestinal lining and immune function. The gut microbiome has been found to vary during infancy due to various factors, including mode of delivery, oral food source, and medication usage. For example, specific bacteria are ingested through vaginal delivery (Nuriel-Ohayon et al., 2016). Prematurity and the type of infant diet (e.g., human milk vs. formula) also affect the diversity of gut microbiota. Medication use may also alter the gut microbiome. Specifically, the use of proton pump inhibitors, prescribed to treat gastroesophageal reflux, was shown to decrease microbial diversity (Ihekweazu & Versalovic, 2018). 79

80  CASE STUDIES IN PEDIATRIC DYSPHAGIA

Further research is needed to understand how these microbiome differences affect development (Tanaka & Nakayama, 2017). As the research comes in, it seems increasingly apparent that feeding and swallowing specialists work with groups who are vulnerable for gastrointestinal issues. Whether children are born prematurely or diagnosed with neonatal abstinence syndrome, autism, or neuromuscular issues, the research and the guidance of a team-based approach should be employed to effectively unpack information and provide evidence-based care. Our case studies, two sisters, provide an example of ongoing gastrointestinal issues that impact feeding and swallow. As with many cases in the text, these cases could have easily fallen into another chapter given their diagnosis of cystic fibrosis and a strong case for applying a traumainformed care perspective. Though there are multiple systems affected (e.g., pulmonary, gastrointestinal), these cases represent how poor prenatal and postnatal care can cause a rapid domino effect of amplified comorbidities (e.g., neglect leading to failure to thrive and poor early management of cystic fibrosis) (Duff et al., 2003; Hammons & Fiese, 2010; Hart et al., 2019 ). The case studies are followed by an interview with a registered dietician and lactation consultant who is also a doctoral student in community nutrition, Emily Wojtowicz. She provides the reader with a window into the challenges of working with parents/caregivers on breastfeeding through her work at Women, Infants, and Children (WIC) and in private practice as a lactation consultant. As she is pursuing a PhD, she further discusses how her clinical work is driving her research.

CASE STUDIES — ANNIE AND LEXI Annie and her sister Lexi were referred for a feeding and swallowing evaluation. They lived in a rural area and had been receiving outpatient services prior to the referral. Annie was 3 years, 5 months at the time of the referral and Lexi was 2 years, 6 months. Both girls were served through the early intervention system, although Annie had recently aged out of services when she had her third birthday.

Case Study Medical and Developmental Information — Annie (See image of Annie at 4 years old in Figure 4–1.) Reason for referral: Feeding evaluation due to limited food repertoire and difficulty with oral preparation and propulsion. Annie was born at 35 weeks, weighing 4 pounds. She subsequently lost weight and received a diagnosis of cystic fibrosis (CF). Due to her CF,

4.  GASTROINTESTINAL DIAGNOSES   81

Figure 4–1.  Annie — 4 years old.

she also had pancreatic insufficiency for which she was prescribed enzyme medications. Another issue related to CF was thick mucus in her throat. She had frequent hospitalizations. Annie initially received a nasogastric (NG) feeding tube. This was switched to a gastrostomy (G) tube at 4 months old. She received most of her nutritive intake via G-tube. Throughout the case study, her adoptive parents will be referred to as her parents. There was evidence of neglect for Annie before being adopted by her parents, at 18 months of age. At that time, she weighed 15.9 lbs and wore clothing intended for a 3- to 6-month-old. Annie tested positive for methamphetamines twice in her first 18 months of life but did not test positive at birth. When her parents received custody of Annie, it was discovered that she was allergic to her formula and was experiencing emesis with every feed. She was put on a different formula and gained 4 lbs very quickly.

82  CASE STUDIES IN PEDIATRIC DYSPHAGIA

Previously, she was more open to trying more food than she was at the time of the evaluation. Her food repertoire decreased over time, and she didn’t eat the variety of foods that she used to eat. Her mother reported that Annie “has no interest in food.” She required 1,100 mL (about 37.2 oz) of water per day and preferred to take it via syringe. Her mother reported that Annie gagged textures thicker than nectar and responded best to thinner textures. She explained that she overstuffed her mouth with food, and she did not understand instructions to chew her food. Her current food repertoire consisted of mango puree baby food, Neocate splash, thin pudding, butter, water, and ranch dressing. Annie had the following daily tube-feeding schedule: She began a night feed of a bolus of Neocate splash and 240 mL of water around 8:30 p.m., which ended around 12:30 a.m. Her mother got up around 2:00 a.m. and provided another feed of 16 oz (about 473.18 mL). This feed finished around 4:00 a.m. Annie received another feed around 6:00 or 7:00 a.m., which ended around 8:00 or 9:00 a.m. She received another 16-oz feed around 2:00 p.m. (See Annie’s medical history timeline in Figure 4–2.) Her mother reported that Annie displayed both hypersensitivity and undersensitivity characteristics in response to feeding. For example, she was reported to lack skills in retaining food, sometimes failing to contain and propel foods without seeming to notice. Her mother reported that with syringe food presentation, Annie occasionally closed her lips around a spoonful and syringe full of food without noticing and the food spilled on her. She sometimes overfilled her mouth with food (often associated with undersensitivity) and avoided certain textures (a characteristic associ-

Annie’s Case Study Timeline • Born at 35 weeks’ gestation, maternal drug use • Nasogastric tube placed soon after birth due to poor oral intake and weight gain • Diagnosed with cystic fibrosis and pancreatic insufficiency • G-tube placement at 4-months unadjusted age • Placed in foster care at 18 months of age due to neglect and failure to thrive • Current diagnoses: cystic fibrosis Figure 4–2.  Annie’s case study timeline.

4.  GASTROINTESTINAL DIAGNOSES   83

ated with oral hypersensitivity). She preferred water intake, and her only oral acceptance of formula was through a syringe. Annie could self-feed with a spoon, straw, and syringe. She took Prevacid at bedtime. Typically, emesis was immediate after feeding and occurred several times per week. In earlier feeding therapy, Annie showed improved motivation and acceptance of food when eating next to her brother, who was close in age. Annie was seen by an interdisciplinary team that included specialized care for her cystic fibrosis diagnosis.

Evaluation — Annie Feeding/Swallowing Observations Preferred foods were provided. She was observed to drink water from a cup with a straw independently. Annie swallowed the enzyme with a syringe of water and was noted to cough. She also tried a ranch dressing taste from her mother’s finger without any overt signs of distress. Annie’s mother fed her mango puree baby food with a spoon. Facial grimacing and gagging were noted. Annie fed herself a small amount of puree with a chewy spoon. When asked if she was full, Annie said “yes.” However, her mother said she didn’t think that was possible since she hadn’t eaten much that day. She got some ranch dressing on her hand and showed it to her mother to wipe it off. Annie was observed to hold boluses in the anterior portion of her tongue or between her lower teeth and her tongue prior to swallowing. Her mother presented another bite. She was observed to accept the initial spoonful of a puree bolus and refuse the second. Questions to facilitate treatment planning: (a) Are there positive oral experiences for Annie? This can provide a window into tastes and textures that feel “safe” for Annie. (b) How is Annie doing with brushing her teeth? Aggressive dental care is recommended for children who are tube fed (Dyment & Casas, 1999). See the areas of concern identified during the evaluation process in Figure 4–3.

Treatment — Annie Goal recommendations following the evaluation: (a) expanded food repertoire, (b) increased awareness and use of oral structures, (c) increased working vocabulary for foods and food categories, (d) increased ability to express pain and discomfort during/after mealtimes, and (e) increased parent and caregiver education.

84  CASE STUDIES IN PEDIATRIC DYSPHAGIA

Oral Preparatory Phase Oral Transit Phase

Annie’s Potential Areas of Concern

Pharyngeal Phase Esophageal Phase Figure 4–3.  Annie’s potential areas of concern.

Obstacles to Treatment Treatment progression was difficult due to frequent emesis and repeated adverse feeding experiences. Often, Annie had just been given her morning bolus feeding and medications. Her mother was fearful that food exposure would result in emesis and that there would be calorie loss. Treatment was child focused, following a sensory hierarchy. Parent education was important for increased understanding of how a hypersensitive system appeared to be affecting Annie’s ability to orally feed. Use of an explanatory model provided evidence of understanding. What is the teach-back method? How could using the teach-back method ensure health literacy for Annie and her parents (Shersher et al., 2021)? The following information was shared by Annie’s mother throughout the treatment process: Two months after the initial evaluation (the message was sent after an out-of-town medical appointment):  Annie has had a rough time. We traveled, which made her stomach upset and then yesterday the pharmacy couldn’t get her enzyme in on time, so we had to adjust her whole schedule, which did not agree with her at all, so she gagged and gagged some more. Won’t even drink anything except water out of a syringe. Four months after the evaluation:  Annie just ate 10 pieces of macaroni. I don’t think she chewed them. Swallowed them

4.  GASTROINTESTINAL DIAGNOSES   85

whole. On the last one, she threw up. Luckily her belly was empty, so she only lost one noodle. Five months after the initial evaluation:  She is swallowing the pieces whole. Right after she ate, she got sick when she vomited all three pieces of cheesy bread up she had eaten, and they were still whole. Six months after the initial evaluation:  Annie vomited yesterday when she should not have and had been incredibly gassy. Maybe it was a stomach bug? She refused food this morning. Hasn’t even wanted her water cup, which is unusual. I think that is some of their issues, a fear of vomiting. It’s traumatic for them. Six and a half months after the initial evaluation:  Annie is starting antibiotics for a sinus infection, but I think the appetite stimulant (Sant’Anna et al., 2014) has helped with some of her nausea because ever since starting it, she’s been able to tolerate more volume of food but I’ve also been letting her do the plunger herself so she can feel it going in instead of me doing it, which seems to be helping. She used to not be able to do more than 10 oz before she started gagging and yesterday, she did 16 and we are right now at 14, getting ready to push another 2. That was always our goal. Seven months after the initial evaluation:  The ENT said that there is absolutely nothing wrong with her. Her sinuses look good and as far as her vomiting, every time somebody touches her nose and her crying that it hurts, he thinks it’s all behavioral. He said she has an aversion to it, and she doesn’t like it and that’s why she does it, so I don’t know if we need to focus more on helping her overcome her aversion to stuff touching her face because I think it’s all related to her eating. She doesn’t like stuff touching her, but I don’t know how to fix that. Eight months after the initial evaluation:  They referred Annie to a neurobehaviorist. They want her tested for autism since everything seems to be sensory. Eight and a half months after the initial evaluation:  Annie is the same. She has taken some pills orally. Asked for food once or twice and swallowed it whole. No real progress. I’m going to try and focus on getting her to drink something now that she seems a little more comfortable communicating. I just haven’t made the leap yet. Something other than water. Nine months after the initial evaluation:  Annie decided she didn’t want to feed and wanted to eat. Good decision. It did not go well and now she’s decided no more food.

86  CASE STUDIES IN PEDIATRIC DYSPHAGIA

Ten months after the initial evaluation:  The clinic visit was good. Annie remained the same and gained some. She is doing four packs of real food blends with 8 oz of water. Total volume of 64 oz. Daily. Weight was 16 kg.

Case Study Medical History — Lexi (See an image of Lexi at 3 years old in Figure 4–4.) Lexi was born at 36 weeks and was diagnosed with cystic fibrosis (CF). Like her sister, Lexi experienced neglectful parents before being adopted at 8 months old. At 2 days old, she underwent surgery to remove part of her bowel and required a colostomy bag. The colostomy bag was in place for 6 weeks before it was removed. Lexi did not see a GI doctor after her bowel surgery at 6 weeks old and did not receive the correct enzymes needed for her CF until she was 8 months of age. Her biological parents reported feeding her multiple bottles per day, but her adoptive mother believes that there was limited oral intake under their care. At 8 months old, Lexi was 13.1 lbs. and severely undernourished with a failure to thrive diagnosis. She began being monitored by a GI doctor and pulmonologist. She also began working with a dietician. Lexi previously had food allergies

Figure 4–4.  Lexi — 3 years old.

4.  GASTROINTESTINAL DIAGNOSES   87

including casein and milk protein and was fed formula having dairy until the allergies were found after 8 months old. Lexi grew out of these allergies and began consuming dairy daily. Lexi was seen by an interdisciplinary team that included specialized care for her cystic fibrosis diagnosis. More recently, Lexi underwent surgery to remove her tonsils and adenoids to reduce mucus impact and bacterial infections in her sinuses. Her tastes and food preferences have reportedly changed significantly after this surgery. Her mother reported that some foods Lexi enjoyed before her tonsil and adenoid removal surgery now make her gag (e.g., applesauce). Her mother also reported that it has been difficult to encourage her to chew a wider variety of foods because she takes a long time to chew and swallow them and must be constantly reminded to drink from a cup independently. Her recent changes in medications included discontinuing antibiotics prescribed for her sinus bacterial culture and adding cyproheptadine (an appetite stimulant). She took azithromycin, albuterol through a nebulizer (to ease airway issues), Orkambi (cystic fibrosis modulator), and allergy medications including Zyrtec. Current food repertoire: Neocate, Fruit Loops nutritional drink, pudding (sometimes with added heavy cream, DuoCal, or coconut oil for additional calories and thinner texture), smooth yogurt, and occasionally chips, crackers, and chicken nuggets. She also drank olive brine and water. Her dietician recommended that Lexi consume 1,200 calories per day. The following was a daily schedule for Lexi: Around 9:00 to 10:00, Lexi was woken up, was given her medications, and had one meal of Neocate. She then did her breathing treatments and played for about an hour after that. She got a second bottle of Neocate after this and a third bottle after around 2 hours. Lexi took a nap and then received her fourth bottle of Neocate. She then had tablet time and consumed her fifth bottle of Neocate before going to bed. Her mother reported that Lexi has always slept through the night and does not have difficulties with sleep patterns. See Lexi’s medical history timeline in Figure 4–5.

Evaluation — Lexi Oral Peripheral Examination: Lips appeared symmetrical. Lip closure was observed. Drawing the corners of the lips was observed but the rounding of the lips was not. The tongue surface and frenulum appeared within normal limits. Forward movement and protrusion of the tongue was not observed. Jaw appeared symmetrical. No functional deviations were apparent. The hard and soft palate were not adequately observed.

88  CASE STUDIES IN PEDIATRIC DYSPHAGIA

Lexi’s Case Study Timeline • Outpatient referral at 2 years, 7 months • Born at 36 weeks’ gestation • Immediate cystic fibrosis diagnosis, but poorly managed medically until about 8 months of age • Bowel resection and colostomy bag placement at 2 days old • Foster care placement at 8 months of age due to neglect and failure to thrive diagnosis • Current diagnoses: cystic fibrosis,pancreatic insufficiency, failure to thrive Figure 4–5.  Lexi’s case study timeline.

Lexi’s mother provided some foods and drinks representative of her intake patterns. She was observed to drink water from a cup with a straw. She also consumed water from a small syringe independently. She was fed noodles and chewed them for an extended period, until her mother gave her more water to drink and she swallowed them. Lexi did not utilize a rotary chew but munched on the food with only her anterior teeth. Her mother noted that she pockets food under her tongue and in her cheeks until it is soft, and she is ready to swallow it. Her mother also offered her chips and crackers, and some facial grimacing was noted with oral intake. Lexi was observed to consume a 60-mL syringe of Fruit Loops–flavored nutritional drink with sequential swallows. See areas of concern for Lexi identified through the evaluation process in Figure 4–6.

Treatment — Lexi The following goals were initiated for Lexi: (a) Improved oral phase sequencing. Oral phase sequencing appeared challenging for Lexi. This was addressed through increased education in oral structures and functions, demonstrating more efficient oral sequencing/propulsion. (b)  Increased ability to state discomfort or physiological distress related to feeding and swallowing. Questions to consider in intervention: (a) What are some multisensory ways of teaching oral containment and propulsion efficiency? (b) Who are the other important team players in Lexi’s treatment? See an image of Lexi at 3 years old in Figure 4–7.

Lexi’s Potential Areas of Concern

Oral Preparatory Phase Oral Transit Phase

Pharyngeal Phase Esophageal Phase Figure 4–6.  Lexi’s potential areas of concern.

Figure 4–7.  Lexi — 3 years, 5 months old. 89

90  CASE STUDIES IN PEDIATRIC DYSPHAGIA

The following information was provided by the mom throughout the treatment process. Two months after the initial evaluation:  Lexi should be fine from her endoscopy, but I’ve noticed it takes her an extra minute to bounce back from stuff. She’s been refusing all food. I can still get the PediaSure in her but only half of what I was and more frequently in order to get her calories in and only in a syringe. She’s a little gaggy and shakes her head when offered anything but water. Four months after the initial evaluation:  Lexi ate about five pieces but per usual she pocketed them until mushy and then swallowed at random when it suited her. Six months after the initial evaluation:  The stomach bug caused a major setback for Lexi. She didn’t even want pudding. I fed her less than half a pudding cup and she gagged on every bit and refused any food orally. She still is good with PediaSure but no food. Seven months after the initial evaluation:  Sending a video of Lexi swallowing several pills: I wanted you to see how effortlessly she swallowed all those pills. That’s a mouthful. She also ate four big bites of chicken noodle soup. Eight months after the initial evaluation:  Lexi has been doing well asking for food and eating it. However, she does not eat during regular mealtimes. She eats when she wants to; otherwise, she holds it in her mouth. I do think she has either some sensory issue or some sort of mental block. I guess that she just cannot grasp chew, then swallow. She can do both of those things individually but rarely in succession without coaching. Nine months after the initial evaluation:  Lexi has made progress! She eats cheese and she’s currently munching on chicken nuggets. It’s taken her almost an hour to eat it but she’s doing it. Ten months after the initial evaluation:  The clinic visit was good. Lexi hasn’t gained so they increased enzymes and PediaSure (ideally food but if she doesn’t want to eat, then PediaSure). Weight was 15.1 kg. Eleven months after the initial evaluation:  New idea for Lexi: I let her chew until the sand runs down (an hourglass timer). Then she has to swallow. I have a 10-minute, 5-minute, 2-minute, 1-minute, and 30-second timer. We are using 2 minutes tonight. Trying to work our way down. So far, she is doing well. She ate half a bowl of cheesy rice with taco meat and this morning she

4.  GASTROINTESTINAL DIAGNOSES   91

ate a whole sausage patty in about 15 minutes. (See Figure 4–8 for factors that signal considerations to supplement nutritive intake through nonoral feeding.) Lexi continued to increase food repertoire and transition from a grazing feeding schedule to small, less frequent meals. She continued to require cuing to decrease oral holding. External reinforcers and direct modeling were effective in shaping this behavior. Consider how to support parents with parent education and modeling positive feeding and swallowing experiences. Consider how, as a feeding and swallowing specialist, there can be a need to reeducate when a child has so many negative feeding and swallowing experiences. Consider the other professionals who should be consulted in providing the optimal care for Annie and Lexi.

Discussion These cases highlight how several factors can impact gastrointestinal skills. The sisters’ care was improved through a stable home environment and appropriate medical care for their cystic fibrosis. However, they had many other obstacles to overcome. First, they both were diagnosed with failure to thrive in early infancy. There were multiple factors that led to this diagnosis. The dangerous home environment and questionable feeding practices most likely had lasting effects that cannot be easily measured or understood.

Nutritional/ Hydration Status

Consider overall health of patient, caloric intake, and hydration

Current diet

What types of food is the patient eating?

Coughing/choking

How often, and with what consistencies?

Fatigue

Do mealtimes exceed 30 minutes? Is the patient able to finish a meal?

Secretions

Is the patient managing his or her own secretions?

Anxiety

Is the patient anxious before every meal? Scared to eat?

Patient autonomy

What does the patient want?

Figure 4–8.  Considerations for nonoral feeding. Source: Comprehensive Management of Swallowing Disorders, Second Edition (p. 192) by Carrau, R. L., Murry, T., and Howell, R. Copyright © 2017 Plural Publishing, Inc. All rights reserved. Used with permission.

92  CASE STUDIES IN PEDIATRIC DYSPHAGIA

Other barriers include living in a rural community with decreased resources. Community and medical resources required considerable travel. A global pandemic further magnified the limited resources, given their increased need to social distance due to the cystic fibrosis diagnosis. The profound lack of health equity was evident in these cases. Fortunately, the sisters continue to be on a positive trajectory. The following interview with Emily Wojtowicz will provide a window into the early care that Annie and Lexi missed as she relates experiences providing care to infants through Women, Infants, and Children (WIC). Consider how Annie and Lexi had alterations to their gut microbiome due to their medical comorbidities and early home environment. Emily will discuss barriers that parents often experience in the initial stages of breastfeeding. She provides insight into how often parents and caregivers receive conflicting, confusing advice from professionals on the team (e.g., pediatricians, lactation consultants, other parents). Through her work with WIC and private practice as a lactation consultant, she has observed teams that don’t communicate well and teams that work better together to help guide parents and caregivers more effectively.

INTERVIEW WITH EMILY WOJTOWICZ, MS, RD, CSP, LD, IBCLC Emily Wojtowicz received her Bachelor of Science degree in Nutrition and Dietetics from the University of Arizona. Then, she went on to complete a combined dietetic internship and Master of Science program at Case Western Reserve University (CWRU) in Cleveland, Ohio. Emily’s master’s degree is in Community Nutrition with an emphasis on child and maternal health. After completing her dietetic internship, she worked with the WIC program as a high-risk dietician, lactation consultant, and Breastfeeding Peer Counselor Program (BFPC) coordinator. Currently, Emily is a doctoral student at the University of Tennessee and a funded Maternal and Child Health (MCH) trainee. Her research interests include barriers to breastfeeding, infant feeding, and human lactation. Jennifer Wilson ( J):  What are some signs that you may see as an International Board Certified Lactation Consultant (IBCLC) and registered dietician (RD) that nutritional needs are not being met? Emily Wojtowicz (E):  I have worked with patients in a few different settings. If I were working with them in the clinical setting, usually there’s other things going on that have already been red flagged. If they come in after in-patient care, then usually there’s labs done. In that case, it is obvious what I need to focus on. A lot of times they

4.  GASTROINTESTINAL DIAGNOSES   93

are failure to thrive, so we see poor weight gain, poor growth if it has been ongoing and nobody has caught it. I would have caught it in WIC if it had not already been caught in a pediatrician’s office. That would be the first big red flag for me. So almost always parents have an idea. Mom’s intuition is usually pretty good, especially if they are breastfeeding. They spend a lot of time close to the baby. They know when the baby is not eating enough. Diapers are not sufficient, or diapers look weird. That kind of stuff is usually obvious to mom. The babies are just fussy. Mom might come in and just be really concerned. And it is early on. Those are early red flags. Sometimes, they can manage to get past that and then we see them later. But a lot of the stuff I feel like is obvious right out of the gate, that something is off. The babies are not eating and do not want to eat. Stools are not normal. That is often an indicator. They are struggling to gain weight and keep their growth curve. Those are the important things and usually we can catch those things before there are nutrient deficiencies. They may be back with big red flags and then we would see them in the hospital. (See Figure 4–9 for further information about WIC.) J:  What would be your next step if there were red flags? E:  We would like to know findings from specialists seeing the child. I’m looking at it from a WIC perspective because that is where the bulk of my work has been. Usually, when they are coming in if they haven’t had a referral yet, then I would make an immediate referral to a pediatrician. I would want to know how swallowing is going if they had a swallowing eval. The first few days of breastfeeding, babies are getting colostrum. The colostrum is so much thicker that it’s easy to not notice there’s a problem right away and families get discharged and in their home on their own.

The Special Supplemental Nutrition Program for Women, Infants, and Children (WIC) provides federal grants to states for supplemental foods, health care referrals, and nutrition education for low-income pregnant, breastfeeding, and nonbreastfeeding women and to infants and children up to age 5 who are at nutritional risk. Figure 4–9.  Explanation about WIC. Source: Pediatric Dysphagia: Etiologies, Diagnosis, and Management (p. 273) by Willging, J. P., Miller, C. K., and Cohen, A. P. Copyright © 2020 Plural Publishing, Inc. All rights reserved. Used with permission.

94  CASE STUDIES IN PEDIATRIC DYSPHAGIA

J:  What kinds of problems do you see after they are discharged? E:  If I’m seeing them just for breastfeeding, that’s when all sorts of chaos start to hit once the milk starts to thin out, and they’re getting more mature milk. It’s a touchy time. They may come into WIC just once, and then they would have their next pediatrician’s visit. They might come back to us and say, “Oh the doctor told me, my baby doesn’t want to breastfeed.” The doctor might not catch it because nothing is super obvious at this point. And then, I might see a mom with a puffy baby who’s struggling to breastfeed. The baby may not seem to want to eat. Parents may tell me they’re not doing well with the bottle either. The pediatrician told them to switch and begin making bottles. There is this whole moment of chaos for everybody before it’s clear what’s going on, or that a speech-language pathologist needs to be involved in doing an evaluation. J:  That shift in texture from colostrum to the thinner breast milk consistency must really be difficult. E:  The baby may be struggling with colostrum and then they’re struggling a lot with milk. The doctor might put them on either a bottle or formula or something to try to make sure they’re getting enough, not realizing that something else is wrong. And then, I would see them again when they still want to breastfeed. J:  How do you work with them in encouraging them to continue to breastfeed? E:  They can’t quite make it work and the bottles aren’t being tolerated either. A lot of times if the parent still wants to breastfeed, they are referred now to somebody with speech for swallowing or an ENT. Making sure they’re working directly with the lactation consultant, and they have open communication is important. To continue to successfully breastfeed, their messages must align and a lot of times they don’t. A lot of time I would end up running defense. For example, the doctor told me this and this is what the ENT told me, then the nurse told me this when I was leaving and they’re all different recommendations, and none of them are super supportive of breastfeeding. What do I do? Why isn’t anybody telling me the same thing? Members of the team need to be supportive of what the family has heard from other providers, even if the provider doesn’t agree. J:  What would you say some of the greatest challenges are for you as a lactation consultant? E:  Having to confirm other things that parents have heard from other places, especially if it’s parents or caregivers with fragile infants or

4.  GASTROINTESTINAL DIAGNOSES   95

infants that have swallowing problems. They’ve heard information from all these different providers, and it might not make sense. J:  How do you help them sort through the information that they have been given? E:  What I first need to do is know what they’ve heard. That’s a really challenging thing because there’s not a way to share those notes all the time. If I’m seeing them either with private practice or with WIC, I don’t have access to their medical records. I can’t see what the doctors have documented. All I can go by is what the parents are telling me. That can be a huge barrier. And of course, whatever they’re telling me is what they have internalized from the provider. That’s not going to be exactly what those providers have said. It’s what they felt in that moment when the provider said this is a diagnosis, or this is what I wanted to do, and then the mom sits with it for a week with all the emotions of having a baby that might not be well or it not being the experience that she expected and then she comes back and tells me after she has internalized all of it. So that can be a big struggle. J:  The process you are describing seems challenging. E:  Another parent or caregiver may just be dealing with all the recommendations from nonbreastfeeding professionals. Everybody has an idea of what they think the mom should do and a lot of people are not lactation professionals that are giving that advice and it might be just based on their own personal experience and so there’s going to be a handful of people who say don’t feel bad to stop breastfeeding if it’s stressing you out, which is valid, but it’s coming from somebody who may have chosen not to breastfeed and the advice has nothing to do with the parent’s situation. It is a bias that the person has from their own experience. Or they can meet with a lactation consultant in the hospital before they come to see me and that person was just like, “You must breastfeed, that is the only choice.” There are all these strong opinions. A lot of times they come from personal bias and personal experience, and they don’t always meet the client where they are. The opinions may not be meeting their needs. It is important to ask them what they want, what they care about. I must weed through all of that to figure out what it is that you want, what’s important to them? How can I help you? How can I confirm all these things they have heard from people that may or may not be relevant to your situation? That “emotional tie-in” to giving practical advice is hard, and sometimes they’ll just tell you what you want to hear, which makes it even harder to help them. You need to know what they want and care about. Otherwise, your advice is just another pamphlet they’re

96  CASE STUDIES IN PEDIATRIC DYSPHAGIA

going to throw into the stack when they get home, overwhelmed by all the visits they have had. J:  What are some gastrointestinal issues that affect breastfeeding success? E:  The biggest one is this one; we’ll hear moms come in and they’ll say, “My baby is allergic to my milk.” I have mixed feelings about it because a lot of the time, it is not real. It’s not diagnosed. It’s just a medical opinion but there was never any test. They might say, “My pediatrician told me the baby is allergic to my milk.” This is so rare it is not even worth mentioning. Moms will say it so regularly. For a baby to be truly allergic to mom’s milk is exceedingly rare. Sometimes they might have a mild intolerance to something in the mom’s diet. And that’s not uncommon, but even that’s not super common. Babies might be fussier with moms having certain things in their diet. There are so many wives’ tales around it that people will do all kinds of crazy things to try to eliminate what they perceive to be allergies to mom’s milk. Sometimes there are allergies related to the mom’s diet, but it’s not actually an allergy to mom’s milk, if that makes sense. For example, a baby could come in who has gone through lab work with the doctor and they’ve met with an allergist. There might be a little bit of blood in the stool, or mucousy stools or something like that is a sign that there’s something in mom’s diet that’s not working for the baby. It’s almost always a protein food or protein from a certain food. Dairy is one of the big culprits. Eggs are common. Those types of things, so that’s probably the biggest GI issue we see with breastfeeding. Occasionally there are other issues, like if moms have a whole lot of milk like they just have a large milk supply and they have a strong letdown. Babies can get overloaded with the first milk that comes out, which is higher in lactose and lower in fat. It is lower in fat and protein but mostly lower in fat. That can translate into some weird stools, and it can have some GI effects. It’s not an issue with babies; it’s just a compatibility issue in that mom is producing a lot of milk and the baby’s body is just not handling the volume right away. Usually, they grow out of it. Those are the most common gastrointestinal (GI) issues. There can be other oral issues, like tongue tie and high palate depending on how you look at the GI tract. The mouth is the first part of that, though, and those issues can be related too. Generally, mother’s milk is perfectly suited for the baby. It takes severe issues for that to not be the case. Very rarely is there an allergy or something like phenylketonuria (PKU), but those situations are rare.

4.  GASTROINTESTINAL DIAGNOSES   97

J:  That’s interesting. Babies and breastfeeding moms adjust to one another, don’t they? E:  Yes, if a mom has breastfed four kids already, her body is ready to produce milk. She may produce a lot, and so they do have to adjust to each other. It can be tricky, and there are ways a lactation consultant can absolutely help a mom. There are tricks to get through that without having to stop breastfeeding and without really having to complicate things too much. But it’s a matter of sitting with someone and figuring out what their goals are and what they’re comfortable with and how to accommodate that. J:  Now you are working on research for your PhD. Could you talk a little bit about your research and what is driving your research, particularly how clinical experiences drive your research? E:  I think my clinical experiences have had a significant impact. There’s this unfortunate disconnect between research and clinical practice. I’m sure you’ve probably seen this. There are so many questions that people have that they ask, and they research without having had their “hands in the mud” so to speak. They must work directly with moms. They don’t know the questions moms are asking or how lactation results might address a certain issue, but there’s a philosophical question that is related to lactation. There might be an answer in practice that would address that or would change the questions they might ask. The two are so far disconnected that the research and the practice don’t always align. I struggle with this a lot as a practitioner from the research perspective, having all these questions. There are already some answers out there that might guide what those questions would look like. I struggle with both sides, because I’ll read research articles to try to inform my practice, but then the more you learn as a PhD student, how research is done, you realize, this practitioner didn’t think about this part, or this researcher left this question out or why didn’t they ask the participants to do this? It just seems like you can see that they don’t live in the same world, that has been a big struggle for me, the disconnect. I would like to bring a lot of people together. There’s a lot of work we do in practice that doesn’t have evidence to support it. For example, what I’m planning to study for my dissertation. There’s no evidence to back it up even though it is used across the board. If you go into any WIC program or most lactation consultants, they are recommending paced bottle feedings and there is almost no literature to show what the benefits of it are and or why it should

98  CASE STUDIES IN PEDIATRIC DYSPHAGIA

be done. Who should do it? And I use it in practice. I have had great outcomes with my clients, but there is nothing to say that’s the right approach, but the handouts and everything teaching people how to do it are abundant. It’s just that balance for me of backing it up with research on what we’re doing. For example, I can now go to the pediatrician and say, here’s the evidence to say why this is good. You should also be recommending this, because right now it’s only really used by lactation consultants and WIC clinics. So, it’s tricky. I want to have the evidence to go to pediatricians and say you need to be doing this. Also, it’s not just for lactation consultants that teach one another. We should have that open dialogue. This is something everybody should be doing and to really support that more with, requiring pediatricians and obstetricians (OBs) to have lactation training. The Academy of Nutrition and Dietetics does more to support and teach about breastfeeding. Dieticians should really know more about breastfeeding than we’re required to know right now. The combination of bridging that gap between practitioners and researchers. Also, having a little bit more say in the community and pushing forward that more people must know about lactation. Those are my two driving forces. J:  Talk about some areas of research and community nutrition that should currently be informing our practice in pediatric dysphagia. E:  I know there’s a little bit of research on this on feeding therapies with whole foods versus like formulas. I’m waiting to see more research come out about that because I know it’s something that a lot of people are talking about. People have taken to social media and they’re spreading their word that way, and they’re not doing research, but then, setting fires really. Some of them good, some of them bad, depends on your stance. But there’s a lot of talk about moving away from these commercially made formulas and moving in the direction of whole foods. And when we talk about health equity and all of that, we know that the formulas aren’t sustainable for a lot of families. But whole food blenderized options are not always the safest choice, so it’s a tricky balance. But it’s one of those things where I think if somebody is afraid of liability, they might not teach about one choice versus the other. If a family can’t afford formula and it only is covered so much by their insurance, they’re going to have to find other options. If we’re not comfortable enough to talk about what they might be doing when we’re not around, we’re doing a disservice to our patients. For example, it’s not uncommon to see 1- to 2-year-olds that might be getting their medical formula from WIC. WIC is a common supplier of formula for that age group so

4.  GASTROINTESTINAL DIAGNOSES   99 .

they might not get it through their insurance because WIC provides it. They’re not getting it through their state insurance and then the dietician might not be comfortable talking to them about blenderized foods like formulas. They are making it themselves at home because they’re afraid or worried about contamination or other issues that might occur, and then the family just kind of makes it up on their own or does what they see on the Internet or something like that. I think there needs to be a lot broader thought and recommendations when we think about health equity and feeding these clients. Not all of them are going to have the money to buy what we’re recommending. We should make sure that the RDs (registered dieticians), lactation consultants, or the pediatricians are comfortable acknowledging the situation those clients are in and support them. They should not make assumptions when giving them a recommendation. This is the safest, healthiest choice. We should recognize that they’re going to do other things and make sure they have informed support and guidance. That is a big one for me. It’s not just the whole food aspect of exclusively formula-fed toddlers and that age group. They could still be receiving breast milk at that point and that would be great. But it’s also the health equity piece, and I think there’s a lot of research coming out on that right now, not just with financial disparities, but race and ethnicity. We’re talking more about disparities and how they’re being supported by their providers. That’s a big body of research that is going to come out. We should be sure that we’re recognizing what our clients need, and we’re addressing those things. That’s the public health side of me coming out. J:  It’s an amazing time in terms of health equity and as health care professionals considering the way we’re doing things. That’s exciting, but it’s challenging, though, isn’t it? E:  Yes, but when I think about the hot topic areas and research I expect and hope to see, that is some of it. There are a lot of people pointing out that if we enable people to eat whole real foods, we don’t have to worry about not getting them the right formula. When people are eating real foods, their bodies can take and use the nutrients they need a lot easier than when everything is manmade. So, yes, I think there’s a lot of interesting things happening. J:  What are the ways that dieticians and feeding and swallowing specialists can better work together? E:  Open dialogue for sure. That’s one of the big issues is just recognizing that we need to be a team. I think that’s the first step, and it’s not often done. For example, we may be functioning with this

100  CASE STUDIES IN PEDIATRIC DYSPHAGIA

mindset: “You send me the swallow eval and that’s as much as we need to talk.” I worked in the field during my internship and a couple of situations here and there in feeding clinics where professionals worked well together. There’s one specifically in Tucson, Arizona, that I got some great materials from and learned a lot from. At this facility, the dieticians, speech-language pathologists, and/or ENT, the team all worked together. They’re a legit care team and they work together through the entire process. That open dialogue is the biggest thing. Team members should not just saying, “I work with them and I kind of skim their notes after they see the patient.” Instead, team members might do rounds together or have conversations where they’ve all worked with a patient. They talked together and they formulated plans together. Team members should not be working independently in those situations where the patient needs to be working with all the different professions in the care team. They need to all be working together. J:  Can you tell us a little bit about your dissertation work? E:  I am in the planning stage. Essentially, what I want to look at is if we can teach families to do this infant-led bottle feeding where essentially you work on the position of the baby, their position at the bottle, so milk is not able to flow out on its own. That all has to do with position, and that’s a part of paced bottle feeding. For healthy full-term infants, we don’t have to regulate what they’re able to take. We don’t have to pace them by tilting the bottle back and stopping them from eating. We just must know how to supply the bottle and position the baby in a way that sets them up for control. My hope is that we can mimic breastfeeding a lot more closely. I think if we can make bottle feeding that much more like breastfeeding, the disparities related to bottle feeding can potentially be eliminated or reduced? We know babies who drink bottles tend to drink a lot more in one sitting than breastfed babies. They tend to eat more overall than breastfed babies. They tend to gain weight faster than breastfed babies. There are other things that are associated with bottle feeding like incidences of coughing and wheezing or sudden infant death syndrome (SIDS) or all these other things that haven’t been researched. These problems may be associated with bottle feeding, but they haven’t been teased out. What is it about bottle feeding that’s the problem? Is it the formula? Is it the amount that they’re consuming? What exactly is it? Is it how fast the baby is consuming that amount? If we can make bottle feeding look a little bit more like breastfeeding through giving the baby control of how much they’re taking and how often they’re eating, maybe we can address or at least reduce some of those other issues that are related. I want to focus on volume of intake, weight

4.  GASTROINTESTINAL DIAGNOSES   101

gain, and weight gain trajectory. Once we figure that out, we can look at some of those other things that have been shown to be associated with bottle feeding and determine if it does improve intake and weight gain trajectory, does it improve sudden infant death syndrome (SIDS)? Did it reduce the risk of SIDS if babies are being fed this way? Does it reduce reflux? All those things. When we talk to parents about it anecdotally, even though professionals may tell mom that it does reduce reflux, there’s no evidence to support that. There’s none. Eventually, I want to look at all those pieces. J:  You want to alter the bottle-feeding process to make it look more like breastfeeding? E:  The idea is that the way the bottle is held allows the tip of the nipple to never be completely full. Milk only goes up to where the hole is. When that’s the case, it doesn’t flow out without the baby creating suction. That has an impact on how much they get at any given time. I always keep a bottle in my office and use it as a teaching tool. The tip of the bottle is where the milk will flow and where I would fill it up. I hold it and show the different angles that you can hold the bottle and at what point milk stops dripping out of the bottle. This shows them the angle to hold the bottle. At any given fullness, the very tip should have milk just to the hole and then beyond that, anything above that milk you can show the client that it will drip out on its own in the baby’s mouth. If you hold it lower, so that’s just as the baby sucks milk will come out, they don’t suck, they don’t drip on their own. The baby then has control of the feeding like with breastfeeding. Obviously that milk is not going to really flow out of mom if the babies do not stimulate it to flow. J:  Have you seen similar bottle designs? E:  Yes, a zero-pressure bottle has been engineered, which is like Dr. Brown’s bottles. Part of me is a little nervous about measuring the feeding practices. We have a lot to consider. We will teach them how to do it. They learn effectively how to do it, but they might be worried about how they need to get that bottle in that baby. The time and training will be challenging. The type of bottle that is used will also be a factor, and there may be barriers affecting the type of bottles study participants use. J:  Thanks for sharing those plans for your dissertation research. See Figure 4–10 for further information about the content and metabolic processing for breast milk versus infant formula.

102  CASE STUDIES IN PEDIATRIC DYSPHAGIA Human Milk Versus Formula* 1. Human milk is almost entirely whey and gradually shifts to contain a nearly equal ratio of whey to casein after breastfeeding/chestfeeding is well established (closer to 6 months). 2. Infant formula composition is static, though some improvements have been made in recent decades to increase the whey to casein ratio to be more like human milk, usually around 50:50. 3. The forms of casein and whey have been found to be different in human milk versus formula, with formula digestion of casein typically resulting in formation of curds that are harder to digest. 4. Infants fed human milk exclusively have been shown to have significantly higher body fat and less lean mass compared to formula-fed infants (Andreas et al., 2015). *For the purposes of this text, formula refers to standard commercial cow’s milk formula.

Figure 4–10. Supplemental information about human milk versus formula. Source: Andreas, N. J., Kampmann, B., and Mehring Le-Doare, K. (2015). Human breast milk: A review on its composition and bioactivity. Early Human Development, 91(11), 629–635. https://doi.org/10.1016/j.earlhumdev.2015.08.013

REFERENCES Duff, A. J., Wolfe, S. P., Dickson, C., Conway, S. P., & Brownlee, K. G. (2003). Feeding behavior problems in children with cystic fibrosis in the UK: Prevalence and comparison with healthy controls. Journal of Pediatric Gastroenterology and Nutrition, 36(4), 443–447. https://doi.org/10.1097/​ 00005176-200304000-00004 Dyment, H. A., & Casas, M. J. (1999). Dental care for children fed by tube: A critical review. Special Care in Dentistry, 19(5), 220–224. https://doi.org/​ 10.1111/j.1754-4505.1999.tb01389.x Gulati, I. K., & Jadcherla, S. R. (2019). Gastroesophageal reflux disease in the neonatal intensive care unit infant: Who needs to be treated and what approach is beneficial? Pediatric Clinics of North America, 66(2), 461–473. https://doi.org/10.1016/j.pcl.2018.12.012 Hammons, A. J., & Fiese, B. (2010). Mealtime interactions in families of a child with cystic fibrosis: A meta-analysis. Journal of Cystic Fibrosis, 9(6), 377–384. https://doi.org/10.1016/j.jcf.2010.07.002 Hart, B. J., Viswanathan, S., & Jadcherla, S. R. (2019). Persistent feeding difficulties among infants with fetal opioid exposure: Mechanisms and clinical reasoning. The Journal of Maternal-Fetal & Neonatal Medicine, 32(21), 3633–3639. https://doi.org/10.1080/14767058.2018.1469614

4.  GASTROINTESTINAL DIAGNOSES   103

Ihekweazu, F. D., & Versalovic, J. (2018). Development of the pediatric gut microbiome: Impact on health and disease. The American Journal of the Medical Sciences, 356(5), 413–423. https://doi.org/10.1016/j.amjms.2018.08.005 Nuriel-Ohayon, M., Neuman, H., & Koren, O. (2016). Microbial changes during pregnancy, birth, and infancy. Frontiers in Microbiology, 7, 1031. https:// doi.org/10.3389/fmicb.2016.01031 Sant’Anna, A. M., Hammes, P. S., Porporino, M., Martel, C., Zygmuntowicz, C., & Ramsay, M. (2014). Use of cyproheptadine in young children with feeding difficulties and poor growth in a pediatric feeding program. Journal of Pediatric Gastroenterology and Nutrition, 59(5), 674–678. https://doi.org/​ 10.1097/MPG.0000000000000467 Shersher, V., Haines, T. P., Sturgiss, L., Weller, C., & Williams, C. (2021). Definitions and use of the teach-back method in healthcare consultations with patients: A systematic review and thematic synthesis. Patient Education and Counseling, 104(1), 118–129. https://doi.org/10.1016/j.pec.2020.07.026 Tanaka, M., & Nakayama, J. (2017). Development of the gut microbiota in infancy and its impact on health in later life. Allergology International, 66(4), 515–522. https://doi.org/10.1016/j.alit.2017.07.010

5 Trauma-Informed Care

INTRODUCTION In the United States, it is estimated that 60% of children have been exposed to a potentially traumatic event (PTE) within the past year (Marsac, 2016). A trauma-informed approach incorporates four key elements: (a) realizing the widespread impact of trauma; (b) recognizing how trauma may affect clients, staff, and others in the program, organization, or system; (c) responding by applying knowledge about trauma into practice; and (d) preventing retraumatization (Marsac, 2016; Oral et al., 2016). The following case study provides an example of a child who has experienced a long-term hospitalization following birth, recurrent medical procedures, and the demise of a twin. There are some factors to consider as we address pediatric dysphagia through a trauma-informed care lens. First, trauma and post trauma should be considered not only for the client but also for the parents/caregivers. Second, the trauma may become clear at various stages of the grieving process. For example, an unrelated event may cause memories to resurface. Additionally, the trauma may be clear in various behaviors that may change over time (e.g., overt avoidance, fawning). See an image of Leah in the NICU in Figure 5–1. There are also important medical diagnoses to consider in reading and discussing Leah’s case. Gastrointestinal issues should be considered as well as neuromuscular issues. Other factors to consider include a diagnosis of Chiari malformation and pulmonary comorbidities given her extreme prematurity. Throughout her treatment, medical diagnoses needed to be 105

106  CASE STUDIES IN PEDIATRIC DYSPHAGIA

Figure 5–1.  Leah in the NICU.

managed so that they were not obstacles to treatment. Utilizing a medical model with an interdisciplinary team is important when there are so many medical comorbidities. Following the case, Heidi Liefer Moreland, MA, CCC-SLP, BCS-S, CLC, talks about how trauma can be an obstacle to treatment. She provides some examples of parental perspectives and child behaviors. She also gives some practical strategies for clinicians to use in sparking an open dialogue and helping caregivers move toward a resilient, problem-solving perspective.

CASE STUDY Initial Evaluation for Outpatient Therapy Leah at 5 years, 9 months of age was referred for feeding and swallowing evaluation and treatment due to concerns about limited quantity and quality of food intake. See an image of Leah at 4 years old in Figure 5–2. Leah was born prematurely at 25 weeks (about 5 and a half months) with a birthweight of 1.9 lbs. She was the product of a twin pregnancy characterized by fetal demise at 18 weeks’ gestation (about 4 months). She had a patent ductus arteriosus (PDA) ligation at 10 days old and a brain bleed at 10 days old, and she has a history of aspiration for 3 to 4 months, requiring a ventilator. She spent 7 months in the NICU. Leah’s

5.  TRAUMA-INFORMED CARE   107

Figure 5–2.  Leah — 4 years old.

history also included inguinal hernia surgery and Clostridium difficile (C-diff). Leah had the following diagnoses: bronchopulmonary dysplasia, gastroesophageal reflux, cerebral palsy, apraxia, anxiety, failure to thrive, and Chiari malformation. She was taking the following medications at the time of her referral: Singular (respiratory), probiotic Zantac (GI), Prozac (anxiety), Zyrtec (allergies), and vitamins with iron. Leah showed the ability to sit unsupported at 1 and a half years old, crawl at 1 and a half years old, and walk at 2 and a half years old. She was receiving weekly occupational and physical therapy at the time of the evaluation. She also had weekly group sessions with a child psychologist. Leah’s mom reported that the most significant difficulties with feeding and swallowing were chewing more complex textures that needed multiple chews. She also reported a fear of swallowing. More current issues include complaints about her stomach hurting.

108  CASE STUDIES IN PEDIATRIC DYSPHAGIA

What are some questions to ask to gain more information? Are there certain times of day that she complains about her stomach hurting? Which professionals are following her? (See Leah’s medical history timeline in Figure 5–3.)

Evaluation Leah was observed during the evaluation session eating popcorn (two to three pieces), string cheese (four to five bites), pancakes (six to seven bites), and a strawberry cookie (one bite). She often seemed hesitant to take bites. Bite sizes were small (approximately 1/2 teaspoon), and rate of intake was slow. She was also observed to take a few bites of some food and discontinue (e.g., pancakes). Leah was observed during the evaluation session drinking 1 to 2 oz (about 59.15 mL) of water from an open cup. She also drank approximately 8 oz (about 236.59 mL) of PediaSure from a bottle. Some excerpts from Leah’s developmental journey will help us further understand the journey. The following quote is from her mom: “On this

Leah’s Case Study Timeline

• Referred for outpatient therapy at 5 years, 9 months • Born at 25 weeks’ gestation, twin pregnancy with fetal demise at 18 weeks’ gestation • 7-months in the NICU • Brain bleed at 10 days old • Aspiration evident at 3–4 months, ventilator dependent • Inguinal hernia surgery, c-diff • Current diagnoses: bronchopulmonary dysplasia, Chiari malformation, cerebral palsy, apraxia, failure to thrive, global developmental delays, pyloric stenosis detected during early stages of treatment, febrile seizure occurred in early stages of treatment, autism spectrum diagnosis later in treatment Figure 5–3.  Leah’s case study timeline.

5.  TRAUMA-INFORMED CARE   109

day several years ago I got to hold our daughter for the first time. Their only reason they let us do so was because she was going in for open heart surgery that morning. They always want parents to have the opportunity to hold their baby in case they don’t make it through.” Here is what one of her moms posted at the time: “Our baby girl. She’s resting well. Bless her little heart. Her heart surgery scar goes from her right chicken wing across her back, around her left side, across her left tummy and to her belly button.” Based on information gathered during the feeding observations and caregiver report, Leah was diagnosed with oropharyngeal dysphagia characterized by challenges in motor planning of bolus organization/propulsion, delays in initiating a swallow, and a limited repertoire of foods. Leah seemed anxious during the evaluation, so oral structures were not closely observed, but drooling was not noted, and symmetry was noted for easily visible areas (lips, teeth, jaw). Skills in the areas of speech, language, hearing, voice, fluency, and behavior were judged to be within normal limits. Based on observations and information gathered from caregiver report, evaluators determined the following treatment goals for Leah: (a)  increased intake of proteins, (b) increased efficiency in thin liquid intake through varied modalities, (c) increased efficiency in bolus organization and propulsion, and (d) increased mealtime initiation skills. In preparing for treatment, it is important to consider Leah’s experiences in the NICU (Madhoun & Dempster, 2019). Evidence should also be gathered about oral phase dysphagia (Muldoon et al., 2021) and cognitive/ behavioral approaches to treatment (Huston et al., 2019). Treatment approaches applied: Multisensory cuing, modeling, visual supports, intermittent reinforcement for thin liquid attempts. Information requested from parents: Explain a typical feeding day for Leah. What are some changes you would like to see? Can you identify pleasurable eating experiences for Leah? (See areas of concern identified during the evaluation process in Figure 5–4.)

Intervention The following goals were targeted: Consume an entire piece of food (e.g., single chicken nugget, French fry) during the therapy session with maximal cueing through a transition to age-appropriate bolus sizes and an

110  CASE STUDIES IN PEDIATRIC DYSPHAGIA

Oral Preparatory Phase Oral Transit Phase

Leah’s Potential Areas of Concern

Pharyngeal Phase Esophageal Phase Figure 5–4.  Leah’s potential areas of concern.

improved oral propulsive sequence. Leah was observed to move away from foods following one bite. She seemed upset when she was prompted to complete food that required two to five bites to complete. Increase skills in identifying and labeling a variety of foods, including proteins, fruits, and vegetables. Leah was observed to have a limited working expressive vocabulary for food labels. Drink and swallow water while simultaneously consuming food with minimal cueing from clinician or caregivers. Leah seemed to be anxious in alternating between food textural differences (e.g., liquid vs. solid) to help propulsion. Increase her daily intake of food to five to six different food items per day by caregiver initiation. Leah lacked exposure to a variety of tastes and textures, which appeared to be due to previous adverse experiences and habitual feeding routines (e.g., child-driven meals, limited exposure to varied tastes and textures). Therapy could begin with more passive food exposure (e.g., going grocery shopping together) but should evolve into more varied food exposure (e.g., visually accepting less familiar foods). Independent initiation of the following sequence: Improve use of medial jaw, organize and propel bolus with her tongue, and

5.  TRAUMA-INFORMED CARE   111

swallow with/without a visual model from the clinician. Skill issues were evident in the oral and pharyngeal phases. As sessions began, increased challenges with both initiating and completing the oral phase emerged (e.g., running to the bathroom to spit out foods) as well as issues in cup drinking her formula rather than drinking it from a bottle. Transitioning from bottle feeding was a concern for her parents because she was only able to get medications through her morning bottle feeding. Though Leah’s school day was structured mealwise, feeding time at home was often more of a grazing schedule based on Leah’s interest or disinterest. Questions that we needed to decide in planning new goals were the approaches to use to (a) help Leah transition away from bottle feeding, (b) increase her oral organization/propulsion, and (c) increase her exposure to varied tastes and textures.

Notes From Mom Throughout the Treatment Process Leah had pyloric stenosis. We didn’t know it, so they found it when they did an endoscopy. She didn’t have any symptoms. Do you think that’s why she thinks it will hurt your tummy? Regarding problems spitting out food: We are working hard at home too. She’s super quick to do it at home. But we are trying to catch her. Progression of skills: She is eating the heck out of some scrambled eggs so I told her I would send you a pic. She tried steak tonight. I made steak. It’s very thinly sliced. She tried one bite. She had three bites of this taco that I made tonight. She chewed the ground beef until it was mush and spit it out but in her mouth it is closer. The doctor mentioned a stimulant before I did. He wants to hold off on the scopes until we see how the stimulant works. He said it’s highly unlikely the pylorus would happen again, especially given her age. So, we try this and see what happens. We’ve also been talking about how to express the fact that she feels hungry. We will see what happens. She is taking it twice a day. It never really dawned on me that she has felt hunger but might not know what it is. The thought of her being hungry and not expressing it makes me sad. Leah had what we think is a febrile seizure. She stopped breathing and had an ambulance ride to the hospital, where we have been for a couple of days.

112  CASE STUDIES IN PEDIATRIC DYSPHAGIA

Leah’s school eating schedule: At around 12, we are going to give her a little PediaSure. The nurse suggested we decorate a cup and mark it. She will drink 4 oz, however many oz in about 8 minutes. This will at least give her a little more. She is with her SPED teacher who is encouraging her to eat a snack while she’s in there. That is at around 1:30 to the end of the day. It’s not perfect but I think it will help her. Leah just drank 4 oz of milk from a cup! A lot of coaxing but she did it! We got an assessment report from P’s auditory processing testing. I am sending a copy of the report via email. She was eating meat spaghetti sauce last night. Kept asking for more. J You might have seen we got an autism diagnosis. I’m still struggling with it. What I see at home is so different than what I see in public. I just have a hard time believing it’s not attributed to anxiety. She just ate a cashew. She is taking melatonin at night. It’s chewable but it’s grape. She doesn’t like it, so she swallows it with water. I think it bothers her getting the milk on her lips. We need to know how much to push her. She took the tiniest bite last night and it was a fight (not willing). Her appetite is bigger, but she only wants the same stuff. How much should we push her to eat new things? We have always tried not to make it upsetting for her. She drank 4 oz of milk today. It took an “act of Congress” and a milk drinking race with me, but she did it! She talks about the dentist when she is nervous. Complications when having a virtual therapy session: Leah deleted Zoom off her iPad. I asked her why she had done that. She said, “I’m done with Zoom. I don’t need it anymore.” I think she is over the virtual stuff. She craves interaction. Also, I think it’s hard because she has not met the clinicians working with her in person. After the pulmonologist gave permission, Leah was able to resume in-person therapy.

5.  TRAUMA-INFORMED CARE   113

The ADD med makes her a little more anxious too. It’s a doubleedged sword. We are also noticing her processing is much slower on the meds. It has slowed her brain down and she’s not sure what to do with it. Her doctor says it will get better. She’s so used to her brain going so fast all the time. The first couple of days that she was on it, she could feel her heart racing. What do you think is triggering her? Do you think she is just overwhelmed? I feel hopeless with her eating. The ADD med is messing with her appetite. She went to the dentist yesterday and did awesome!!!!! She didn’t eat at (school) lunch today. She hasn’t eaten at lunch since last Tuesday. She’s starving every day. She is exhausting right now. She just tried tomato soup. She tried celery. Couldn’t bite it but tasted it. I’m not sure Leah knows how to describe foods because she doesn’t have a lot of foods she eats with different tastes. What if we did a tasting day with different foods with drastic tastes. Just a thought. The notes from mom throughout the treatment process can provide a window into how her parents perceived and celebrated attempts with nonpreferred foods. (See resources for sharpening skills in trauma-informed care in Figure 5–5.)

114  CASE STUDIES IN PEDIATRIC DYSPHAGIA The Body Keeps the Score by Bessel A. van der Kolk The Childhood Trauma and Attachment Gap in Speech-Language Pathology: Practitioner’s Knowledge, Practice, and Needs https://doi.org/10.1044/2021_AJSLP-21-00110 Stress Effect on the Body https://www.apa.org/topics/stress/body • Acute trauma:  Results from exposure to a single overwhelming event/ experiences (car accident, natural disaster, single event of abuse or assault, sudden loss, or witnessing violence) • Repetitive trauma:  Results from exposure to multiple, chronic, and/or prolonged overwhelming traumatic events (i.e., receiving regular treatment for an illness) • Complex trauma:  Results from multiple, chronic, and prolonged overwhelming traumatic events/experiences that are compromising and most often within the context of an interpersonal relationship (i.e., family violence) • Developmental trauma:  Results from early onset exposure to ongoing or repetitive trauma (as infant, child, or youth) and includes neglect, abandonment, physical abuse or assault, sexual abuse or assault, emotional abuse witnessing violence or death, and/or coercion or betrayal. This often occurs within the child’s caregiving system and interferes with healthy attachment and development. • Vicarious trauma:  Creates a change in the service provider resulting from empathetic engagement with a client’s/patient’s traumatic background. It occurs when an individual who was not an immediate witness to the trauma absorbs and integrates disturbing aspects of the traumatic experience into his or her own functioning. • Historical trauma:  Is a cumulative emotional and psychological wounding over the life span and across generations emanating from massive group trauma. Examples of historical trauma include genocide, colonialism (i.e., residential schools), slavery, and war. • Intergenerational trauma:  Describes the psychological or emotional effects that can be experienced by people who live with people who have experienced trauma. Coping and adaptation patterns developed in response to trauma can be passed from one generation to the next. Figure 5–5.  Sharpening clinical skills in trauma-informed care.

5.  TRAUMA-INFORMED CARE   115

Test results for language testing completed by her school SLP just prior to her reevaluation of feeding and swallowing skills: Standard score:  69 Narrative: Success on the Pragmatic Language Test indicates the ability to use language in a social way that is recognized and approved of by society. The ability is measured across three major areas of communication: (a) using language for different purposes or intentions, (b) changing language according to the listener or situation, and (c) following rules for conversation or storytelling. Standard scores 77 and below are considered delayed, between 78 and 84 borderline or at risk, and between 85 and 115 average. Leah’s performance was as follows: Leah’s performance on the Comprehensive Assessment of Spoken Language Second Edition (CASL®-2) fell in the delayed range as compared with her peers. She was able to interpret and respond to scenarios by responding to gratitude with a peer, greeting a teacher, asking to join friends in play, asking a parent for permission or information, and asking a sibling for help. Leah did not demonstrate an ability to politely refuse an unwanted item, express regret to a parent for an accident, express regret to a friend for a disappointment, give a compliment to a teacher, respond to an introduction, evaluate nonverbal cues of sadness in order to express concern, or express sympathy to a peer. She frequently described the scenario presented rather than giving the requested response. Pragmatic language skills should be integrated as much as possible throughout the school day in a variety of settings with a variety of listeners. Standard score:  79 Narrative: Leah’s second grade teacher completed a Pragmatic Language Skills Inventory (PLSI) to rate her pragmatic language skills exhibited in the classroom setting. Her results were as follows: Classroom interaction:  poor) Social interaction:  below average Personal interaction:  below average The results of Leah’s pragmatic language testing indicate that she presents with deficits with her social language knowledge and skills, particularly with participating in verbal exchanges with peers, appropriately maintaining a topic of conversation, and participating in peer-directed routines.

116  CASE STUDIES IN PEDIATRIC DYSPHAGIA

Reevaluation of Feeding and Swallowing Skills At age 7 years, 3 months, Leah had improved in oral pharyngeal skills. She was not reported to spit out food before initiating a swallow. Bolus sizes also had increased. There were still challenges with her rate of intake during school lunch periods. A nurse was able to sit with her for snack time so that she could have supplemental feeding times during the school day. She continued to rely on PediaSure and the use of a bottle to ensure the proper number of calories per day but was less reliant on the bottle for nutrition/medication intake needs. Intervention focus was increasing, becoming more age appropriate. She was showing an increased improvement in food repertoire. Issues that were a continued challenge were rate of intake, intermittent sequencing/ propulsion issues, and some level of continued reliance on bottle use to meet nutritional and medical needs. Consider what the goals would be and how to formulate goals in a functional way. Goals should communicate parent/caregiver involvement in the treatment process and a demonstration of skills. During this treatment period, as Leah was also diagnosed with autism, language therapy goals were added at her parents’ request. Treatment goals were written to address verbal problem solving, responses to questions, and a perceived challenge in stating discomfort and physiological distress (e.g., running to the bathroom to spit out food instead of stating lack of hunger). See an image of Leah at 9 years old in Figure 5–6.

Discussion Considering Leah’s birth and medical history was an important part of her intervention. Her moms had spent so many of her early years managing medical procedures and developmental concerns. There were moments that they felt grief and pain as well as triumph over milestones reached. After years of an atypical journey, feeding and swallowing problems had persisted. Intervention focus had to be medically based. When therapy was initiated, there were oral skills that needed to be addressed (Muldoon et al., 2021). As oral sequencing improved, considering cognitive behavior principles was helpful (Huston et al., 2019). Both integrating use of positive words and active participation in the goal-setting process provided Leah with novel feeding experiences. Slowly, some of the black and white perceptions that she had toward eating and drinking were altered. A trauma-informed care lens helped to provide the support that Leah and her moms needed throughout the therapeutic process. Through her wealth of experience, the following interview with Heidi Liefer Moreland provides further insight into trauma-informed care.

5.  TRAUMA-INFORMED CARE   117

Figure 5–6.  Leah — 9 years old.

INTERVIEW WITH HEIDI LIEFER MORELAND, MS, CCC-SLP, BCS-S Heidi has over 25 years of experience working with children with feeding challenges. She supports families virtually and in person through family mealtime coaching, remote responsive feeding, and the Thrive by Spectrum Pediatrics Feeding Tube Weaning Program. She provides insight into how trauma-informed care has shaped her treatment and practical suggestions for feeding/swallowing intervention.

Jennifer Wilson ( J):  Can you talk through a trauma-informed approach to care for pediatric dysphagia? Heidi Liefer Moreland (H):  I think one of the things that occurred to me after we talked last week is that I can’t imagine a case involving dysphagia, whether pediatric or adult, that does not involve a traumatic experience.

118  CASE STUDIES IN PEDIATRIC DYSPHAGIA

J:  How does that trauma affect our patients with pediatric dysphagia? H:  There was a difficulty that caused physical harm. The trauma is over, it’s time to move ahead. For example, a baby or young child may have had difficulties with thin liquid. Following that experience, every time an infant or child with dysphagia drinks, there is a likelihood that they feel compromised with every swallow. I can’t imagine how traumatizing it would be at an extremely basic level to feel as if you were drowning with every swallow. J:  How does that infant or child’s response affect caregivers? H:  It is also traumatizing for the caregiver. This is most likely not what they expected when they were planning their child’s birth and childhood. Frequently there is unwelcome news with every single nurse’s comment or doctor’s visit. Earlier diagnoses may have been life-threatening. Even for children who were protected from the results of aspiration due to early diagnosis, there are likely altered circumstances that can result in the feeling of compromise and danger at a basic level. Although some children and/or caregivers seem to be more resilient than others, there is often a lack of safety at a basic physical and emotional level for both. J:  I was thinking of us as providers when doing intervention. It seems important to think about how the caregiver, the parent, and the child has “framed it.” H:  It’s important to realize that trauma is personal. Studies tell us about the impact of perceived trauma (van der Kolk, 2013). Meaning, it doesn’t matter if one child aspirated everything that ever went in their mouth and another child that may have only penetrated food or liquid presented. If that child perceived it as traumatic, it counts as trauma. If that mother perceived that as traumatic, it counts as trauma. Family perspectives can also vary widely. For example, another child may have aspirated everything, but they didn’t die from their heart disease. The family may process the more significant aspiration differently, because they were so relieved that their child is alive. J:  How does feeding and swallowing rank compared to other medical problems? H:  Food, eating, and feeding your child are so emotionally meaningful that an inability to eat safely might feel more traumatic to that family than another significant medical problem. Everyone perceives things differently. I forgot that when I was an early clinician. I probably felt like clients and caregivers were overly concerned about some aspects of treatment, but that’s not how trauma works. If you look at some of

5.  TRAUMA-INFORMED CARE   119

the data, put out by the National Child Traumatic Stress Network, 15% to 20% of kids who have had medical trauma have lasting traumatic stress. Twenty percent to 30% of parents or caregivers present with trauma significant enough that it can affect their ability to carry out treatment (The National Child Traumatic Stress Network, 2022). That’s a high percentage, and I think that’s helpful for families to know that they are not alone. This is a result of what happened. This is not your fault. It comes across in diverse ways. I remember a family bringing in a picture of their baby covered with bruises. I was trying to talk to them about their 1-year-old, about putting some boundaries in place around eating, like to not let him eat all day long and you know check the consistencies. I don’t remember what the recommendations were but the next week she came in with a picture of him at birth covered with bruises because he’d been stuck up under her rib cage at birth and they had to suction him out. It was a traumatic birth, and he was fine. And I kept saying, “But look, he’s fine.” But what she was seeing in her head was that baby covered with bruises and what she was saying by showing that picture was, “I can’t say no to him.” If I were doing it now, we would have talked about it differently. At the time I was like, “OK!?!” I did not know how to respond to that because I didn’t understand the impact that had. But it comes across in so many ways and there’s a couple of big ones that we can talk about. I think as medical providers, a lot of us still don’t understand it, but I think trauma-informed care is an up-and-coming thing that we’re getting better at. There are words for it now. But I think as dysphagia specialists, if we haven’t been trained in that we’re missing the boat, because we see these kids and families regularly. More than the doctors do. We’re the people that they trust. We’re the ones who are the speech therapists working on it. We’re the ones who give them communication skills. We’re the ones who can read signs and talk about routines and we’re well positioned to be the ones who spot that and help them deal with it. We’ve been undereducated or at least I was in trauma, especially with dysphagia, because like I said, I can’t even imagine a situation involving dysphagia where there isn’t an opportunity. Not everybody is traumatized but everybody has had an experience that could result in traumatic stress. J:  That story that you just told just highlights the importance of active listening, doesn’t it? H:  The baby healed from the bruises, and everything was fine. They weren’t even affecting her airway, but there were other unseen obstacles.

120  CASE STUDIES IN PEDIATRIC DYSPHAGIA

J:  Tell us about the kinds of trauma that you see in children, parents, and caregivers that they’re “bringing to the table” when you’re working on feeding and swallowing therapy. H:  I think it’s probably easiest to start with the idea that we all already understand, the fight or flight response, the response someone has to a situation that is frightening to them. For a lot of these kids, it’s food or eating. This is hard for parents to get their head around. We’ve done a decent job as a profession, educating people on where that learned fear comes in. A lot of people understand that. That definition has probably been expanded or the explanation has been expanded a bit. There’s fight or flight but there’s also freeze and sometimes there’s “fawning” or pleasing the people who are doing that to you. We also see extreme independence in kids. They don’t want to have anything to do with it. They’re going to grab the spoon away from you and do it themselves. They don’t trust anybody around them to do anything about it. They completely refuse. They bite. They push it away. A lot of these kids get a diagnosis of sensory problems, but what’s interesting is that many types of sensory problems don’t expand anywhere beyond food. Is that a sensory system problem if it’s only revolving around food? We all have sensory differences. There can be some sensory things that go along with it. How we react to that is a different situation. We get the fight response or the refusal. A child may say, “Don’t do it, don’t do it,” and push it away. I think the more subtle ones that we see are kids who just sort of acquiesce. They melt a little bit. They just sit down, and they give in and let you do it. But they don’t really engage. They don’t really want to be a part of it. I think we use the word a lot of times “will accept.” They’ll “accept” this. Those are all reactions that can result from a negative experience or a traumatic experience around food. It looks so different for every kid. It does tend to come down to a refusal, a push away, a turn the head away or an extreme independence with it. Toddlers have extreme independence anyway. But even more so. For example, “No one can put a spoon in my mouth. I am the only one who can put the spoon my mouth.” I see a lot of guarded responses, taking the spoon. So, taking the spoon and guiding it in and fighting their teeth down on the edge of it very carefully so that it’s not going to be pushed in any further. Then, they want to please the feeder. But they’re going to control it to make sure that it feels safe. All those things can be a result of traumatic experiences with food in the past. Those are all reactions to consider depending on how your body responds to trauma. J:  Talk more about caregivers and parents and their responses to trauma.

5.  TRAUMA-INFORMED CARE   121

H:  Some of them will name the trauma. There are some people who are good at talking about it. When we’ve given them the words, when we’ve talked about the fact that there is possibly some trauma, there are some parents who will say, “I’m still traumatized.” There are some people who experience avoidance. Avoidance or overreacting is another way that a lot of people can deal with it, like the mom I talked about with the little guy who had such a traumatic birth. She was saying, “There was trauma for me.” She was saying, “I can’t do that because of this.” I don’t know if she used the word trauma. That was a long time ago so I don’t think that would have been the word that was around in that way. Some people are avoidant. Some of them have a tough time with follow-through, or difficulty with setting boundaries. Some of them just can’t focus, depending on how active they are, how recent it was, and how their body reacts to things. Sometimes they just have a tough time focusing, not sleeping, reliving experiences over and over. When you look at PTSD, which is a little bit different than posttraumatic stress, those are all things that would show. You must be trauma informed to pick it up. It’s not like they’re coming in and losing control of their emotions over and over. A lot of them might look scattered. Let’s face it, having a baby, and particularly a baby with a lot of special needs, is going to make you more scattered anyway. These are all things we can certainly be present in but what we see a lot of the time is real difficulty in following through with some of those things and real difficulty in processing. It does make it much more important for us as communication specialists to communicate in a way that they understand. We already know that people in trauma can’t take in the same information in the same way. Even when I deal with family members who are medical providers, they will tell me every time, “Pretend I’m not a medical provider.” It’s different when it’s your own and so you do need to say it in a unique way. Some of that’s just personalization. It doesn’t mean it’s trauma. A good strategy in supplying information is to give short, brief bursts of information. I had someone describe it one time as, “it’s like listening underwater.” There’s this big wall that you’re trying to listen through that when you’re super-duper anxious about anything, you can’t take it in. “Do this, turn that corner, do this thing,” but you can’t take it in. You can’t take in “if this, then that.” Those are all things that are harder to do when you’re in the middle of something that’s a traumatic event like that. It looks different for parents and kids, for everybody. J:  Describe that hyperreactive response that you sometimes see.

122  CASE STUDIES IN PEDIATRIC DYSPHAGIA

H:  A lot of children get diagnosed with sensory processing disorder. One of the things that we know about trauma is that it can make you hyperreactive. For example, picture someone who was mugged. How would you feel if you were walking down a dark area in a scary part of town where you’ve never been, and someone touched you on the shoulder? You’re already heightened and anxious and so someone touching you on the shoulder when you’re in that state is going to be different. We wouldn’t diagnose them with a sensory disorder because they overreacted in a scary, unfamiliar area to touch. But I think that’s what happens to a lot of our kids. They’ve been in the hospital. They’ve been sick. They’ve been poked and prodded with needles every day and breathing tubes. If you think about a Nuk brush and a suction catheter, they look remarkably similar. It probably is a little bit similar going in. We’re diagnosing all these kids with sensory problems and hyperreactions, but that’s how the brain is designed to react to protect us from negative experiences in the future. Our brain is designed to predict situations, so we don’t get ourselves in the same loop repeatedly. When these kids are reacting appropriately to an experience like one that caused them trauma in the past, they are being diagnosed as hypersensitive. Sometimes they get treatment to try and desensitize what they’re doing, when what they really need is some space and some rest to heal from those experiences in the past, instead of repeating it over and over. We also know that physical trauma, like a lot of our kids in the NICU experience, or significant pain can have the opposite effect. It can make it easier to disassociate from your body. It can make it easier for you to shut down those painful feelings and not attend to them anymore, because it was too much. We picture our kids in the NICU with the noise and the light. We’ve gotten better over the years, and we are trying but there’s going to be painful experiences. They have had scars and surgeries. They had needle pricks every day. You can also have the opposite effect of disassociation. So now, we’re saying, “Why don’t they feel hungry? Why don’t they know the food in their mouth?” and it’s also a protective response. Both are right protective responses to disassociate from those feelings. We just need to keep in mind that when we’re reconnecting those things, because the brain can always learn right, it’s plastic, and so when we’re trying to reconnect those sensations, we need to remember that we’re not “pummeling” harder. We should be saying, “How do we reconnect those sensations for them? How do we reconnect them in a positive way that makes the child feel good that makes the child want to recognize those feelings and not further disassociate from what caused them to begin with?”

5.  TRAUMA-INFORMED CARE   123

J:  What are some ways that a feeding and swallowing specialist could differentiate between sensory-based issues versus trauma-based issues? How would you recommend for them to obtain information to be able to do a differential diagnosis between those two things? H:  The easy answer is, does it occur with just food and swallowing or is it overall? Some of the kids have had significant trauma. Kids who’ve been in the NICU for extended periods of time have a significant amount of trauma. They may seem hyperreactive or sensory avoidant. You can always talk to an occupational therapist who is trauma informed as well, because to some degree, the fact that it exists doesn’t change, right? That’s still the way their sensory system reacts to those stimuli. The difference is how reroutable is the brain? How adaptive is it? How do we help them overcome that? Do we feel like we need to desensitize, and so we’re continually providing them with that same stimulus over and over in the hopes that they get used to it? Or do we have a more trauma-informed lens for the situation? How do we help them approach this stimulus in a way that makes them feel empowered, comfortable, and able to reroute their brain in a way that makes sense to them so that they can interact with that stimulus in a functional way? This should be considered regardless of the reason for that reaction. Just looking at where it occurs, why it occurs. I have things that I don’t like. I would much rather have my purees with the crunchy thing. I put nuts in my ice cream. I put tortilla chips in my soup. I like the mix of both crunchy and smooth, but I haven’t gotten a diagnosis of anything! J:  In your experiences with parents and caregivers, how do you explain that the trauma they may be experiencing is affecting their ability to take part in treatment? H:  I think it’s probably rarer that we had someone who self-identifies trauma and why that makes it difficult. There has been progress in self-identification and public awareness. The word “trauma” is much more prevalent in the past couple of years. I’m part of a lot of diverse groups and again, social media extremes kind of spirals things to go with what you’re looking at, but it seems to me that I’m hearing a whole lot more about trauma. I’m hearing a whole lot more people who are informed about trauma. As clinicians, we’ve asked more questions so that’s probably coming up a whole lot more. Most of it is discussions that we have had where people are able to say that it’s affecting or making it difficult is with a discussion with us. We’ve had to have some coaching discussions where we say, “This is a challenge. Do you know why this is hard for you? What are some reasons

124  CASE STUDIES IN PEDIATRIC DYSPHAGIA

why this might be hard for you?” This is well within our scope of practice to ask coaching questions like that. Coaching is a method of interacting with families that early intervention uses a lot. So just saying, “Why do you think this is difficult for you? Do you have any ideas that might be easier or harder for you? What are some ways that we can do that?” I think one of the things you and I both mentioned was resilience in the beginning of really building in instead of hammering in and going, “Man, you look really traumatized.” It’s more helpful and research shows us that as well. We’re not being unsympathetic but we’re building the reserves for resilience if we’re saying, “Wow, you have really overcome a lot.” It’s one of the reasons why survivors of trauma in other arenas have started saying, “I’m a survivor, not a victim.” Using some of those words for kids and families and looking forward to overcoming them. “Oh my gosh, he was sick. He was aspirating everything and you’re still working with him. You guys have really got a good relationship.” Or like, building resilience in them, building on their strengths instead of every time they go to the doctor, “Well, he’s not doing that either,” or saying, “She missed that milestone.” Just building resilience. That’s one of the things that we’ve done for a lot of families too, not dismissing the difficulties the kids have had but of just saying, “Wow, look how far he has come. Did you ever think you would be that big and run around like that? We’ve still got some swallowing work to get done but look how far he’s come.” Continuing to point these things out over and over and over. We spent a lot of time talking too much about worry and anxiety and trauma. I think that heightened it. We really wanted to be trauma informed so we spend a lot of time saying, “Oh man, you must be traumatized,” and they were like, “You’re right I am.” And then, there we all were. Stuck in trauma, worry, anxiety, or whatever. We have learned a lot about saying “Wow, that must have been hard. I can see you have worked hard to overcome that.” One thing we talk to our families about is, “Who are some people who are likely to help you with this?” Line up some folks as we’re going through tube weaning to follow up with the swallow study. Who is the most likely to bring you down? We can help them with those resources. It doesn’t have to be a professional. Remember that statistic I told you about earlier that 20% to 30% of some caregivers have trauma significant enough to affect with treatment. Probably in our practice, which is almost all medically complex kids, there’s probably 20% to 30% that I wish could get some professional resources for their family. As a therapist, I think we can go a long way towards healing the trauma with the kids by the way that we interact with them. We’re building new experiences in the

5.  TRAUMA-INFORMED CARE   125

brain every single day and they’re in such a learning mode. If we’re careful and we’re not therapists for the parents by any stretch of the imagination, there’s not a lot of resources out there for them. When we feel like families would receive help from that, we usually have to say, “I know you would do anything for your child. I know you don’t want to take time out for yourself to do this, but it is important for him that you do get some help with this.” So, they usually do it for their kids, but they won’t do it for themselves, because “We’re strong.” We say “I can do it. I can soldier through.” Some of those things that do seem traumatic do improve as the situation improves. So, as the kids improve and heal, they can be healthy along with their kids. I remember that I did a talk on the traumatic effects of having a kiddo having all these medical complications and there was a woman in the audience who came up afterwards and said, “My son is in high school now. He plays football and there’s still a couple of times a year when I am pulled back to that time when he was in the NICU and I’m so worried about him that I can’t breathe.” She said, “You need to keep this in your talk all the time because there are still times when you get pulled back into that little baby and he’s a high school football player now. It’s strong, it’s really, strong.” Sometimes it gets more faint as time goes on, but it comes back. That’s a different layer. Some people are extremely resilient. We’re not saying 100% of the people have this, like 20% to 30%. So that means, 70% to 80% of the people have the reserves or the resources mentally, emotionally, physically, financially even. They’ve got some reserves to deal with the emotional piece of it, but I think as a rehab specialist, we are probably more likely to see it because when you are in the hospital, when you’ve got a heart problem, there’s a doctor who is going to fix it. It’s really traumatizing for people to be helpless and watch. That’s hard to feel like you can’t do anything, but there’s expectation and hope and belief that someone is going to take care of it, and it doesn’t have to be you figuring it out. But then you get home and there’s still dysphagia, there’s still a feeding tube, there’s still a breathing tube. All the things that are still there, and now you’re the one who must do it. You’re the one who is coping with the effects as a caregiver. Sometimes you see it a lot more after they come home, not in the crisis. Because in the crisis, you don’t have time. You can’t think about it now. You’re dealing, dealing, dealing and there’s an expert, expert, expert. Then, as therapists that end up seeing them once a week, we can see that in a unique way than some of the other people. It is important for us to keep this in mind.

126  CASE STUDIES IN PEDIATRIC DYSPHAGIA

J:  Yeah, absolutely, that’s a good point that parents and caregivers may be managing those challenges a little bit differently when they are in an acute care setting versus when they are in a less acute setting, like home health or outpatient therapy, removed from as many medical professionals right there at their fingertips. H:  Right, and it’s subtle. By that point, the biggest crisis is over, so the child may be receiving services in an outpatient setting, early intervention or school. In some ways, it might be a little more subtle; in some ways, it’s not subtle. For example, I have worked with intense families. I remember saying to myself, “This mom’s coming in. And she’s always set off at the littlest thing.” And I used to be worried and dread it a little bit. We know who they are when they come in, as clinicians. As clinicians we tend to become desensitized to that and we have a bigger picture and we can say yes but, you know the aspiration is way better. He’s going to get better. We have a different lens, and our emotions are not tied up in past events that happened. We walk in for a period and leave. We weren’t there. In some rare cases, we are there for the whole thing, but for the most part, we’re going to step in and step out for a period, and sometimes we bring fresh eyes and can see something they did not, that would not have been clear for someone who knew them very well. But we are there for a time to care for this unit of people and if we are just worried about the babies swallowing or the 3-year-old chewing wherever they are, in that continuum of progress at that time. If we’re not caring about the whole unit and their reaction to what happened in the past, we’re going to miss some of the important parts of the solution. J:  How can we measure the level of trauma that the children and families are experiencing? Are there any assessments that can be part of the treatment that we’re doing? So much of what we talked about today is so intuitive, and that may just be where we are right now. H:  There’s a resource called the National Child Traumatic Stress Network that has been developed by a team of hospitals that are looking at trauma. It’s not just medical trauma. It’s also other kinds of trauma, child abuse and events, floods, storms, things like that. They have information. There is a toolkit there specifically for medical providers in trauma-informed care. It’s a great place to start for us to continue to learn more. And that lets you tweak it towards your population a little bit. For example, if you’re a clinician who works in early intervention in an area that has a lot of refugees, they’re going to have a different kind of trauma around food. Their trauma around food might be scarcity and not having enough. Whereas if you work

5.  TRAUMA-INFORMED CARE   127

next to an acute care hospital, you’re going to have a lot more kids in the NICU with true dysphagia. It just depends a little bit on the population that you’re working with and how their various kinds of experiences in the past are going to have an impact. J:  The point is that in various levels of care, the trauma is going to feel different, look different. The way that you’ve talked about how trauma may be waxing and waning over time is important for us to consider just in all areas of feeding and swallowing work, language, and communication. H:  Good listening and good tools are key. It’s easy also to say, “Oh, they’re traumatized, but maybe they don’t have resources.” It’s easy to then overfind trauma everywhere you see it too and I don’t think you can go wrong with treating someone as if they had some negative experiences in their past. I think that kind of approach can be helpful in lots of ways, but I think we need to focus on how we get them out of that. Once we’ve found that, what do we do that can help them move past that? As communication specialists, as people who see people weekly, as people who can talk about communication and read body cues and read all those verbal cues. As speech therapists and then as occupational therapists, there are different tools to do those things too as all kinds of providers. We have some of those tools and so I don’t want to get stuck in the idea that they must be referred to someplace else because that’s where I would have started when I was younger by saying, oh, they need psychology. That’s not me, but just knowing that it is the responsibility of everybody who interacts with them to become educated in the best way to do some of those things. And yes, we can’t take on that job if it’s a bigger job than we can handle. But using that lens of interaction is going to go a lot longer than just passing them on to somebody else. J:  To capture what you’re saying, you are really talking about the fact that if you’re hitting a wall in the treatment that you’re doing, that this is the kind of warning sign that we need to know that may be stifling your ability to intervene. When all things that you can see as a professional would lead you to believe that intervention should be working. Perhaps that’s really when you need to start exploring what to do? H:  Exploring what their experiences were in the past is a good place to start. For kids who are 100% tube dependent, certainly being hungry is a part of it. Is the child still running away from the food? Are they still clamping down on the bottle nipple, even though it seems like their swallowing is safe now? Do some more digging and

128  CASE STUDIES IN PEDIATRIC DYSPHAGIA

see. Talking to the parents a little bit more can be helpful. There’s a lot of value in well-placed questions and working with the family as a team rather than professionally, just imparting information. “Why do you think that is? Are there situations when this is better? What do you think made that situation better?” Using good questioning to work together to get to the heart of the problem can lead you to the solution.

ADDITIONAL RESOURCES FOR TRAUMA-INFORMED CARE Balluffi, A., Kassam-Adams, N., Kazak, A., Tucker, M., Dominguez, T., & Helfaer, M. (2004). Traumatic stress in parents of children admitted to the pediatric intensive care unit. Pediatric Critical Care Medicine, 5(6), 547–553. n Of the 272 parents completing the initial assessment, 87 (32%)

met symptom criteria for ASD. Of the 161 parents completing follow-up, 33 (21%) met symptom criteria for PTSD. PTSD symptoms at follow-up were associated with ASD symptoms assessed in the PICU, unexpected admission, parent’s degree of worry that the child might die, and the occurrence of another hospital admission or other traumatic event subsequent to the index admission. Neither ASD nor PTSD responses were associated with objective measures of a child’s severity of illness (PRISM III score). n Conclusion:  Traumatic stress symptoms are common among parents in the PICU and may persist long after discharge. There is strong support from these data for continued attention to supporting parents both during and after a child’s PICU admission. Critchley, H. D., & Nagai, Y. (2012). How emotions are shaped by bodily states. Emotion Review, 4(2), 163–168. n How emotions are affected by physiological states (hunger).

Didehbani, N., Kelly, K., Austin, L., & Wiechmann, A. (2011). Role of parental stress on pediatric feeding disorders. Children’s Health Care, 40(2), 85–100. n Stress increased when parent transitioned from observer to

feeder. n Increase in negative behaviors with parents — children picking up on stress.

5.  TRAUMA-INFORMED CARE   129

n Monitor parent stress: Clinical implication is that we need to

find a way to reduce it. National Scientific Council on the Developing Child (2010). Persistent Fear and Anxiety Can Affect Young Children’s Learning and Development: Working Paper No. 9. http://developingchild.harvard.edu Fox, N. A., & Shonkoff, J. P. (2012, February). How persistent fear and anxiety can affect young children’s learning, behaviour and health. In Social and economic costs of violence: Workshop summary (p. 69). National Academies Press. Center for Early Childhood Mental Health Consultation Modules: https:// www.ecmhc.org/tutorials/trauma/mod2_3.html Greer, A. J., Gulotta, C. S., Masler, E. A., & Laud, R. B. (2008). Caregiver stress and outcomes of children with pediatric feeding disorders treated in an intensive interdisciplinary program. Journal of Pediatric Psychology, 33(6), 612–620. n Negative mealtimes cycle. n In addition to the medical issues associated with feeding

disorders, there are some unique aspects of caring for a child with a feeding disorder that may be particularly stressful. (Craig, Scambler, & Spitz et al., 2003) reported that caregivers of children with feeding disorders feel personally responsible for their child’s disorder. Because providing physical nourishment to a child is one of a caregiver’s most fundamental responsibilities, the inability to do so can have profound effects on one’s feelings of self-esteem, self-efficacy, and confidence in parenting (Craig et al., 2003; Crist et al., 1994; Powers et al., 2002). The caregivers’ increased stress and anxiety over not being able to feed their child can also lead to increased stress in the child from the repeated failed meals. Moreover, the caregiver’s visible signs of anger and frustration can lead to even more avoidance of the meal by the child. This negative meal cycle can repeat itself without some direct intervention to help the caregiver overcome the child’s desire to avoid food. Hartman, D. M., & Medoff-Cooper, B. (2012). Transition to home after neonatal surgery for congenital heart disease. The American Journal of Maternal Child Nursing, 37(2), 95. n Great description of difficulties, parent stress, lack of hunger

cues.

130  CASE STUDIES IN PEDIATRIC DYSPHAGIA

Jones, J., & Passey, J. (2004). Family adaptation, coping and resources: Parents of children with developmental disabilities and behaviour problems. Journal on Developmental Disabilities, 11(1), 31–46. n Different types of disabilities (feeding tubes not specific). n Coping patterns — what helped and what was stressful. n The importance of developing strategies to counter family

stress. Kazak, A. E., Alderfer, M., Rourke, M. T., Simms, S., Streisand, R., & Grossman, J. R. (2004). Posttraumatic stress disorder (PTSD) and posttraumatic stress symptoms (PTSS) in families of adolescent childhood cancer survivors. Journal of Pediatric Psychology, 29(3), 211–219. Kazak, A. E., Kassam-Adams, N., Schneider, S., Zelikovsky, N., Alderfer, M. A., & Rourke, M. (2006). An integrative model of pediatric medical traumatic stress. Journal of Pediatric Psychology, 31(4), 343–355. n Different stages to acute and chronic stages. n Similarities cut across injury and illness groups. n Remember that different families had different preexisting

coping abilities. Lutz, K. F. (2012). Feeding problems of neonatal intensive care unit and pediatric intensive care unit graduates: Perceptions of parents and providers. Newborn and Infant Nursing Reviews, 12(4), 207–213. Melnyk, B. M., Alpert-Gillis, L., Feinstein, N. F., Crean, H. F., Johnson, J., Fairbanks, E., . . . Corbo-Richert, B. (2004). Creating opportunities for parent empowerment: Program effects on the mental health/coping outcomes of critically ill young children and their mothers. Pediatrics, 113(6), e597–e607. n Parents were given more knowledge about children’s ability

to cope and knowledge about their conditions, as well as activities to help their child cope with stress. n ~ Two percent of the control group exhibited behavioral symptoms at 6 months, and ~26% of the group without intervention exhibited behavioral stress symptoms. Follow-up data were included in article with similar spread between the groups. National Child Traumatic Stress Network. (n.d.). Types of traumatic stress. https://www.nctsn.org/what-is-child-trauma/trauma-types/medical-trauma n Medical trauma is stress.

5.  TRAUMA-INFORMED CARE   131

n Information on ICU stay and stress. n Includes information for family coping. n Up to 80% of ill or injured children will experience some

traumatic stress following life-threatening injury or illness. n Twenty percent to 30% of parents and 15% to 25% of children and siblings may experience persistent traumatic stress reactions that can impact treatment and recovery. National Child Traumatic Stress Network. (n.d.). Medical events & traumatic stress in children and families. http://www.ncts.org/sites/default/ files/assests/dfs/MedicalTraumaticTress.pdf n Explanation of how medical events can lead to traumatic stress

for child and family. n Symptoms of traumatic stress in child and family. n Risk factors for child and family. n Specific data for children with cancer, illness, and injury included. National Child Traumatic Stress Network. (n.d.). Pediatric injury and traumatic stress: The physical injury is healing, but how is your patient coping? https://www.aftertheinjury.org/sites/ati/files/prov_broch.pdf n Information for pediatricians and caregivers.

National Scientific Council on the Developing Child. (2010). Early Experiences Can Alter Gene Expression and Affect Long-Term Development: Working Paper No. 10. http://developingchild.harvard.edu n Long term effect of early childhood stress (and malnutrition). n Biochemical and structural changes.

Southwick, S. M., Bonanno, G. A., Masten, A. S., Panter-Brick, C., & Yehuda, R. (2014). Resilience definitions, theory, and challenges: Interdisciplinary perspectives. European Journal of Psychotraumatology, 5(1), 25338. Streisand, R., Braniecki, S., Tercyak, K. P., & Kazak, A. E. (2001). Childhood illness-related parenting stress: The Pediatric Inventory for Parents. Journal of Pediatric Psychology, 26(3), 155–162. n Parents of kids with physical limitations are at risk for poor

mental health outcomes. n Stress can impact information learning, processing, and recall, which could compromise parental comprehension of, and adherence to, the child’s prescribed treatment.

132  CASE STUDIES IN PEDIATRIC DYSPHAGIA n Stress on the part of parents may be directly related to stress

subsequently experienced by children facing health threats. May lead to systemic family modeling of stress. n Elevated stress levels among caretakers could negatively influence children’s own medical regimen adherence behaviors.

REFERENCES Craig, G. M., Scambler, G., & Spitz, L. (2003). Why parents of children with neurodevelopmental disabilities requiring gastrostomy feeding need more support. Developmental Medicine & Child Neurology, 45(3), 183–188. https://doi.org/10.1111/j.1469-8749.2003.tb00928.x Crist, W. D., McDonnell, P., Beck, M., Gillespie, C. T., Barrett, P., & Mathews, J. R. (1994). Behavior at mealtimes and the young child with cystic fibrosis. Journal of Developmental & Behavioral Pediatrics, 15, 157–161. Huston, P. L., Dempster, R., & Garbacz, L. (2019). Cognitive and behavioral strategies for the treatment of feeding disorders in adolescents. Perspectives of the ASHA Special Interest Groups, 4(6), 1498–1506. https://doi.org/​ 10.1044/2019_PERS-SIG13-2019-0011 Madhoun, L. L., & Dempster, R. (2019). The psychosocial aspects of feeding in the neonatal intensive care unit and beyond. Perspectives of the ASHA Special Interest Groups, 4(6), 1507–1515. https://doi.org/10.1044/2019_ PERSP-19-00097 Marsac, M. L., Kassam-Adams, N., Hildenbrand, A. K., Nicholls, E., Winston, F. K., Leff, S. S., & Fein, J. (2016). Implementing a trauma-informed approach in pediatric health care networks. JAMA Pediatrics, 170(1), 70–77. https:// doi.org/10.1001/jamapediatrics.2015.2206 Muldoon, D., Meyer, L., Cortese, J., & Zaleski, R. (2021). A literature review: Evidence base in speech-language pathology for the management of pediatric oral phase dysphagia. Perspectives of the ASHA Special Interest Groups, 6(2), 1–10. https://doi.org/10.1044/2021_PERSP-19-00080 Oral, R., Ramirez, M., Coohey, C., Nakada, S., Walz, A., Kuntz, A., . . . Peek-Asa, C. (2016). Adverse childhood experiences and trauma informed care: The future of health care. Pediatric Research, 79(1–2), 227–233. https://doi.org/​ 10.1038/pr.2015.197 Powers, S. W., Patton, S. R., Byars, K. C., Mitchell, M. J., Jelalian, E., Mulvihill, M. M., . . . Stark, L. J. (2002). Caloric intake and eating behavior in infants and toddlers with cystic fibrosis. Pediatrics, 109(5), e75. https:// doi.org/10.1542/peds.109.5.e75 The National Child Traumatic Stress Network. (2022). Pediatric medical traumatic stress: A comprehensive guide [Brochure]. https://www.nctsn.org/

5.  TRAUMA-INFORMED CARE   133

sites/default/files/resources/pediatric_toolkit_for_health_care_providers​ .pdf van der Kolk, B. (2013). The body keeps score: Integration of mind, brain, and body in the treatment of trauma. Milton H. Erickson Foundation.

6 Pulmonary Diagnoses

INTRODUCTION Most newborn babies begin acquiring the skills necessary for liquid expression orally in their mother’s womb. If anatomical structures are intact, neural pathways are following the expected sequence, and intrauterine space is adequate, an in vitro baby initiates the process of the suck-swallow sequence with amniotic fluid as early as 13 weeks. If the full gestation period of 36 to 40 weeks’ gestation is achieved, they have many weeks of practicing their suck-swallow)process. (Lau C., 2016).A photograph of a baby in vitro at 33 weeks’ gestation is shown in Figure 6–1. At birth, the respiration component is added to the sequence and a suck-swallow-breath sequence must begin for oral nutritive intake. As this motor learning process is initiated, there are reflexes in place to facilitate the process. A rooting reflex and suck reflex give the newborn the ability to locate the food source and an initial motor plan (suckling). Premature infants are born with two major disadvantages. They lack motor learning in vitro because of abbreviated gestation, and they also lack elements of lung development that occur during the final weeks of gestation. Further factors affecting them may be caregiver interruptions (e.g., a prolonged time away from primary caregivers) and neuromuscular issues (e.g., intraventricular hemorrhage, cerebral spinal fluid accumulation). Figure 6–2 depicts a full-term infant with an active sucking reflex a few hours after birth. 135

Figure 6–1.  Ultrasound at 33 weeks’ gestation.

Figure 6–2.  Newborn in the NICU. 136

6.  PULMONARY DIAGNOSES   137

CASE STUDY The following case study provides an example of how multiple factors can affect feeding and swallowing progression. Jane was not only affected by extreme prematurity, poor prenatal care and maternal drug use were also part of her feeding and swallowing developmental challenges. She experienced further problems due to neuromuscular issues and pulmonary underdevelopment. Discussion of her case followed by a conversation with a respiratory therapist will give the reader an appreciation for the respiratory challenges that many patients with pediatric dysphagia face.

Case Study Medical History Jane was referred for outpatient feeding and swallowing therapy at 12 months of age. She had been seen through home health services for the past 3 to 4 months, with discharge reporting provided by physical and speech therapy. Prior to that, she received feeding and swallowing therapy through inpatient services while in the neonatal intensive care unit (NICU). Jane was born at 26 weeks’ gestation due to placental abruption. Some of the details were unavailable regarding prenatal care and birth since her birth parents relinquished their parental rights. Her adoptive mother will be the parent mentioned throughout this case when referring to Jane’s mom. She began caring for Jane at 3 months of age. Near birth, Jane presented with bilateral grade 3 brain bleeds and hydrocephalus. She remained in the NICU for 6 months after birth. At the time of her referral for outpatient feeding/swallowing therapy, Jane’s mom reported the following medical information: She had multiple surgeries, including two shunt surgeries, G-tube placement, and patent ductus arteriosus (PDA) ligation. She has had multiple hospital stays, most recently due to a need for a shunt revision and a staphylococcal (staph) infection in her stomach. Due to concerns for neurological and gastrointestinal functioning, medical tests performed included magnetic resonance imaging (MRI), computed tomography (CT), a gastric emptying study, a swallow study, an electroencephalogram (EEG), and a sleep study. A laryngoscope was scheduled for 2 months from the time of this 12-month feeding and swallowing evaluation. Current diagnoses were hydrocephalus, chronic lung disease, hypothyroidism, prematurity, seizures, and sleep apnea. She had a tracheostomy and began using a Passy Muir valve (PMV) 4 months prior to our evaluation. She required the use of a ventilator at night and when sick. See an image of a tracheostomy in Figure 6–3.

138  CASE STUDIES IN PEDIATRIC DYSPHAGIA

Figure 6–3. Tracheostomy.

Consider reasons and possible findings of her laryngoscope. What types of issues could occur 6 months after a tracheostomy? The respiratory therapist interviewed below will mention the importance of these findings in tracheostomy care and the decannulation process. Mom also reported that Jane was teething, and it had affected her desire to eat and drink. She was reported to vomit when jostled right after a feed. In the last few months, she had decreased seizure activity. Mom was hopeful that Jane would be weaned off seizure meds. Limited information was provided about vision and hearing during the initial evaluation.

Digging deeper: Discuss signs of seizure activity in nonverbal children with a neuromuscular disorder. What are some signs that suggest a child is having a seizure?

The providers (physical therapy and speech-language pathology) who were seeing Jane through home health from 6 to 14 months of age reported the following: “Progress with her rehab goals has been slow. She

6.  PULMONARY DIAGNOSES   139

has been able to wean from mechanical ventilation throughout the day and only uses the ventilator when sleeping due to central apnea. She is now tolerating family and community outings and would benefit in transitioning to an outpatient setting.”

What are some resources that would help the family with this transition? Who are some important professionals to have on the team to help with equipment and transportation needs?

Her speech-language pathologist reported that she “had a loose latch on the nipple and was able to extract liquid without signs of distress or aspiration accepting 1½ ounces at a time. She was able to tolerate the PMV.” She had an extensive medication list, including clonidine, Keppra, Synthroid, omeprazole, an iron supplement, Flovent, and MiraLAX. Consider how the medications might affect her feeding and swallowing development. What were the purposes of the medications? Which medical specialists should be linked to which medications? See Jane’s medical history timeline in Figure 6–4.

Jane’s Case Study Timeline

• Outpatient referral for feeding/swallowing evaluation and treatment at 12 months unadjusted age • Born at 26 weeks’ gestation with complication of placental abruption • 6 months in the NICU • Limited history available prior to foster/adoptive placement • Bilateral grade 3 brain bleeds, hydrocephalus near birth • Placed in foster, and eventual adoptive care, at 3 months • Current diagnoses: hydrocephalus, chronic lung disease, hypothyroidism, seizures, sleep apnea, global developmental delay, oropharyngeal dysphagia, gastroesophageal reflux Figure 6–4.  Jane’s case study timeline.

140  CASE STUDIES IN PEDIATRIC DYSPHAGIA

Evaluation and Recommendations During the evaluation, Jane was first presented with a slow-flow bottle nipple filled with 3 oz of formula given in a feeding position chosen by the mother that was typical during bottle feedings. She was positioned in an upright, supported posture with a slight distance from her mother near the edge of her lap. She demonstrated limited response to the bottle — specifically, there was no response through movement (e.g., kicking legs) or turning head toward the bottle. When it was presented orally, there was limited tightening/latching around the bottle nipple. Suck strength and coordination or non-nutritive suck were assessed using a gloved finger. Tongue movement was evident, but strength was decreased. Tongue cupping around the gloved finger was also decreased. Facial grimacing was observed, and a slight head turn, away from the stimulus, suggesting aversion. Jane was also presented with formula on a small narrow, shallow bowl spoon. During feeding attempts, she exhibited slight facial grimacing and an inconsistent lingual response. For example, in one presentation, tongue pumping was observed with a 1- to 3-cc bolus presentation. Lack of lingual response was observed in another presentation. Jane demonstrated limited motoric ability (e.g., volitional reaching, involuntary trunk support) and seemed to have difficulty attending to a visual stimulus. She demonstrated an expression change suggesting physiological stress upon being repositioned from the floor to the beanbag chair. She vocalized, smiled, and attended to her brothers and sister during a play routine (tickling, razzing her). Vocalizations were noted when she was using a PMV. The PMV was removed after approximately 15 to 20 minutes since Jane seemed to be experiencing more labored breathing. There was no information at this point in treatment regarding a diagnosis of cortical vision impairment (CVI). This became evident during the evaluation, and we were able to work with a vision therapist through our early intervention system. Consider how treatment should be altered in response to information about CVI. Though Jane was followed by a neurologist, she had also not been diagnosed with cerebral palsy. Jane’s parents had been told that she would not be able to walk and talk due to her extensive neurological damage. During this period, we began focusing on increased ability to express thin liquid from varied modalities, including a therapeutic open cup, increased positioning, and support to facilitate puree propulsion. Tongue propulsion was addressed through use of tools, positioning, and jaw/ cheek support. Therapeutic focus continued to shift from quality versus quantity of intake and increased positive experiences with oral intake. Optimal mouth care was also addressed given that Jane was not eating daily by mouth. Jane was at risk for bacterial growth due to lack of oral use. There was also a concern that Jane would develop increased oral sensory hypersensitivity without passive and active oral motor stimulation.

6.  PULMONARY DIAGNOSES   141

Questions for the Family Are there oral experiences that are enjoyable for Jane? How often does Jane see the pulmonologist? What are his current recommendations? What is Jane’s current feeding tube delivery schedule and oral feeding schedule? What are some ways that Jane currently participates in family meals or that you would like to see her participate in meals? What types of instrumental testing and findings have you gotten regarding swallowing skills? (See areas of concern identified through the evaluation process in Figure 6–5.)

Respiratory Changes Jane was diagnosed with COVID-19 and double pneumonia at 2 years, 6  months of age. She demonstrated increased pulmonary issues and began requiring supplemental oxygen. Initially, she was cared for at home but required intermittent hospitalizations over the next several months. Seizure activity also began increasing. Feeding and swallowing therapy was through telepractice. This transition was due to Jane’s more fragile

Oral Preparatory Phase Oral Transit Phase

Jane’s Potential Areas of Concern Pharyngeal Phase Esophageal Phase

Figure 6–5.  Jane’s potential areas of concern.

142  CASE STUDIES IN PEDIATRIC DYSPHAGIA

system and the onset of a global pandemic. Some challenges at this point in treatment were changes in respiratory status, decreased access to adequate instrumental tools (e.g., less scheduling of modified barium swallow studies because of the global pandemic), and a challenging intervention platform given Jane’s medical comorbidities. Consider the best ways to support Jane during this episode of treatment (Marpole et al., 2020; Schwartz, 2021). When providing treatment to a medically fragile child, it is important to have up-to-date medical releases. This will give you permission to talk with their medical providers and share concerns. Following the COVID-19 diagnosis, respiratory status was altered. Jane’s parents were asked to report factors such as PMV use or capping, ventilator dependency during the day, and medication changes. Tremendous responsibility was placed on the parent and/or caregiver when working with a telepractice platform. Mom was guided to use therapeutic techniques. The telepractice platform served as a wonderful way of encouraging carryover of skills but was also a lot of responsibility for Jane’s caregivers. Pulmonary issues were not as evident using a virtual platform. Fortunately, the family was able to provide information about crackles, a need for supplemental oxygen, and seizure activity. Unfortunately, increased seizure activity and increased need for supplemental oxygen seemed to be hindering intervention during this period of Jane’s treatment. Improvements were not happening as anticipated. Jane’s mom reported that everyone else had recovered from COVID-19 but that’s Jane’s breathing difficulties were not resolved. It became increasingly evident that the changes in her pulmonary status were affecting her skills in airway protection. Obtaining a modified barium swallow study would have been difficult at this point in the pandemic. Modified barium swallow studies can be helpful with respiratory changes. In the interview with respiratory therapist, Bob Yost, he will discuss cervical auscultation as a tool when respiratory changes occur. During this therapeutic episode, Jane’s mom was asked about the nutritional supplement that she was given through the feeding tube. Interviewer:  What type of formula or blend is Jane receiving via G-tube? Jane’s mom:  We began giving her real food blends at 14 months of age. She did horrible with formula. She would throw up every feed. Her gastroenterologist talked about doing a Nissen fundoplication, but I asked if we could try blended food first. A fundoplication is a surgical procedure in which the top part of the stomach is folded and sewn around the lower esophageal sphincter.

6.  PULMONARY DIAGNOSES   143

Three months after the COVID-19 diagnosis, more seizures were reported. Five months later, another seizure was reported, and she was diagnosed with streptococcus (strep). A significant seizure was again reported at 6 months requiring hospitalization. One year after the COVID-19 diagnosis, extra secretions were reported requiring emergency room care. Jane was placed back on a ventilator and moved to homebound status. When respiratory concerns arise, it is important to consult a pulmonologist. In Jane’s case, ordering instrumental measures was not an option at this point in treatment due to the pandemic. The pulmonary symptoms were increased crackles, increased need for supplemental oxygen, and increased occurrence of respiratory infections following COVID-19. Contacting the pulmonologist’s office and sharing very specific information regarding observations can help patient management. For example, “Jane was observed to seem responsive today and mom reported hearing and feeling crackles throughout the day. Would you recommend any changes in her PO status?” See Figure 6–6 for further understanding of respiratory terminology. Her mother was interviewed 6 months after Jane’s COVID-19 diagnosis. Here is how she felt about Jane’s feeding and swallowing skills at this point: Interviewer:  Tell us a little about the journey that you have had with Jane in terms of feeding and swallowing and the challenges that you have faced. Jane’s mother:  Jane was born as a 26-week-old preemie and had suffered brain damage because of bleeding in her brain and immediately when they tried to feed her by bottle started aspirating the formula into her lungs. It became very evident early on that she would need a G-tube. When she was about 5 months old, they did surgery to put a G-tube into her stomach and that has been the primary way that she has been fed. She had a swallow study done, probably at about 8 months old or so, and she did pass it and they said that we could try to start offering her food and drinks. But because of her brain damage along with the fact that she had never had food, it has been hard

Medically Fragile Patients:  Fitting Dysphagia Into the Bigger Clinical Picture https://doi.org/10.1044/leader.FTR3.07182002.1 Treatment for Cough:  Two Sides to the Story https://pubs.asha.org/doi/10.1044/persp2.SIG3.113 Figure 6–6.  Sharpening clinical skills in pulmonary diagnoses.

144  CASE STUDIES IN PEDIATRIC DYSPHAGIA

for her to learn to eat food but also to drink things. She has a very strong gag reflex and reflux, and she often gags and will throw up sometimes. So, it has been a journey trying to get her comfortable and she is still not completely there as far as having any kind of food or liquids in her mouth. She still is only taking small bites at a time even though we have been doing therapy since before she was a year old to try to get her used to eating. Jane also does not speak, and they have told us that she will be nonverbal, but we are going to continue to work with her to find a way to communicate. Interviewer:  What is important for Jane to be able to do in terms of feeding and swallowing? Jane’s mother:  As far as feeding goes, I know that she may never get one day to where we can get rid of her feeding tube. Obviously, we would love for her to take everything in her mouth, but if that does not happen, my biggest thing is I want to see her having enjoyment for the time that she does get to have food or drinks orally. I want her to be able to enjoy things. Tracheostomy and ventilator needs increased during Jane’s episodes of treatment. She became a ventilator-dependent patient after a case of bilateral pneumonia that did not easily resolve with medication. She was discharged from feeding and swallowing therapy but continued to receive therapy to improve expressive communication using augmentative and alternative communication.

Discussion Jane’s case provides an example of a dynamic system. A progression of skills often cannot be the trajectory of treatment when working with medically fragile patients. Providers must closely monitor changes in medical diagnoses, consult medical professionals, and keenly observe through virtual delivery of service lenses. As we ask questions and get feedback, parents and caregivers can provide valuable insight. We must learn to ask the right questions and provide the highest quality of care. In Jane’s case, her parents were placed in a demanding situation caring for a medically fragile child during a pandemic. They were also placed in a position where they were the sole reporters of respiratory changes. They had previously opted out of nursing care during their first home health cycle of treatment (post-NICU). A medically fragile system resulted in a need to transition back to a homebound status.

6.  PULMONARY DIAGNOSES   145

Jane’s case reflects the importance of parents and caregivers being given the tools that they need to make the best decisions as medical care needs increase. Jane’s case also represents an example of COVID-19 occurring in a child with neuromuscular involvement and an underdeveloped, fragile pulmonary system. When therapy was initiated at 12 months of age, discussions regarding decannulation were under way. Prior to the COVID-19 pandemic, nocturnal apnea was identified. Unfortunately, 12 months after COVID-19, pulmonary function decreased, and she became ventilator dependent.

INTERVIEW WITH BOB YOST, RESPIRATORY THERAPIST Bob served as the Education Coordinator for Respiratory Care at East Tennessee Children’s Hospital (ETCH). As coordinator, he developed an asthma education initiative for the ETCH Respiratory Care Department, in which therapists educated all admitted asthma patients/families, and a therapist performed status call-back for all asthma patients discharged after a visit to the emergency department. He had a leading role in ETCH’s implementation of comprehensive electronic medical records; the creation of standardized, best-practice care maps for various pulmonary disease processes; and improvements in our departmental employee selection and orientation processes.

Jennifer Wilson ( J):  I became inspired to interview a respiratory therapist (RT) recently in my work with medically fragile kids with tracheostomies (trachs). There have been times I thought patients that I have been working with needed to be suctioned. I have always wanted a better understanding of what to look for and how to explain respiratory issues. Bob Yost (B):  I appreciate the questioning approach that you included in your statement just now. A fundamental clinical question that we as SLPs and RTs should always be asking is “What am I really dealing with in this patient? Is there any clinical element here that might have gone unappreciated?” In that way we are much alike in what we do. If I may use an analogy here, one single recognition truly revolutionized asthma care in the 1990s. It was the increased awareness that severe asthma exacerbations left significant tissue-level disruptions behind when the

146  CASE STUDIES IN PEDIATRIC DYSPHAGIA

initial episode appeared to be resolved after a few days. A child who was back to “normal” was usually not back to normal. Children can, and will, play through almost any distressing situation because play is so important to them. J:  Tell us a little bit about asthma exacerbation and the lung recovery process. B:  Micrographs of airway tissues from a serious asthma exacerbation can be quite striking and tell a totally different story. There can be significant deposits of cellular debris and extra fluid and edema in airway tissue spaces where those elements should not be present. The key clinical realization for asthma care and control was that after 3 to 5 days of oral steroid therapy, essential tissue repair had not yet happened and that complete repair often takes 2 or 3 months of daily inhaled corticosteroid therapy (ICS). Especially in the past decade as the importance of extended ICS therapy has been standardized, we have witnessed fewer children experiencing second and perhaps third asthma exacerbations within a period of months because that therapy is now routinely ordered and hopefully administered to completion. J:  How do direct service providers like respiratory therapists and feeding and swallowing therapists have a front-row seat with many of these patients? B:  Back to the idea of unappreciated clinical elements, both of our professions, because of the time and familiarity we often gain from time spent with a patient and family, have many opportunities to recognize things other providers may block out for one reason or another — things that are not always immediately obvious. J:  Tell us about the instrumental studies that provide us with information about the respiratory system? B:  Chest x-rays (CXRs) are what everyone thinks of as the diagnostic gold standard, but they are not always a necessary or right answer, especially for children. There are ongoing national initiatives seeking to minimize the number of CXRs ordered for children who appear to have uncomplicated cases of bronchiolitis or asthma. When there is suspected lung consolidation due to excessive retained airway secretions that promote bacterial or viral growth, then a CXR will rule out other complications and help point you in the right therapeutic direction. Lung consolidation occurs when the air that usually fills the small airways in your lungs is replaced with fluid. J:  What are some things that don’t show up on a CXR?

6.  PULMONARY DIAGNOSES   147

B:  When we’re just talking about the kind of small airway edema and swelling characteristic of bronchiolitis or asthma, those do not show up on a plain CXR. You’re not going to do an MRI or a CT scan looking for that sort of thing either without a lot more reason. So, if you can’t get a picture of a given problem, you are thrown back on old-school clinical judgment and respiratory assessment tools and techniques — techniques that all RTs have a lot of practice using. J:  You mentioned that as SLPs or an occupational therapist (OT), we’re normally going to encounter RTs as inpatient hospital clinicians, usually as we work on trach vocalization, feeding, and/or swallowing. Could you advise outpatient therapists who work with medically fragile patients following acute events? What are some general respiratory warning signs to look for in the pediatric population in the outpatient setting? B:  OK, you are talking about physical clues that something is not right in the pulmonary system. Bluish, gray, or extremely pale lips or nail beds are all obviously bad and need to be assessed and treated immediately. J:  What are some general numeric guidelines? B:  An increased respiratory rate is kind of tough to assess in pediatrics because kids come in all shapes and sizes and normal respiratory rates change with age. I try to keep just a couple of easy numbers in my head for quick assessments. For infants 6 months to a year of age, any sustained respiratory rate greater than 40 breaths per minute (bpm) is above normal. For children 12 years and older, rates above 20 bpm are higher than normal. Between 1 year and 12 years, the normal rate obviously falls somewhere between the 20/40 bpm points. J:  Tell us about some more observable signs of respiratory distress. B:  Some of the respiratory signs can’t simply be counted. Respiratory effort comes first. Any extra work effort you witness for a child on either inspiration or exhalation deserves to be further assessed. Stiff lungs from fluid overload or an upper airway obstruction can cause retractions of the soft tissues above the clavicles, between the ribs, etc. upon inspiration. If the situation is bad enough, you might also witness the flaring of the patient’s nares. Expiration, as we all know it, is normally a passive process. The classic cause of patients having to actively work to exhale is an asthma attack. Spasm of smooth muscles of the medium and small airways will be the cause. Administration of a bronchodilator such as albuterol or Xopenex is the usual fix. You might also note a

148  CASE STUDIES IN PEDIATRIC DYSPHAGIA

persistent cough for a certain patient indicating that there is irritation of the airways within the lung. If this is something you witness repeatedly, some questions might be appropriate. (See an example of a tracheostomy tube and the anatomic placement in Figure 6–7.) J:  What types of questions should be asked to investigate the nature of the cough? B:  The cough might simply be from a recent cold, or you might be witnessing an undiagnosed case of asthma. A cough that a parent notes frequently at night or first thing in the morning can be one of the early signs of undetected asthma, or the first sign of an oncoming attack for a known asthmatic. Those are the major signs and symptoms that you might encounter. Lung sounds are tougher to assess without a stethoscope to auscultate the chest. J:  I’d love you to expand on auscultation a bit. High resonance cervical auscultation (HRCA) has been shown potential as a dysphagia

Figure 6–7. Tracheostomy tube. Source: Center for Devices and Radiological Health. (2022, October 31). Reuse Tracheostomy Tubes or Switch to Appropriate Alternatives During Shortage: FDA Safety Communication. U.S. Food And Drug Administration. https://www.fda.gov/medical-devices/safetycommunications/reuse-tracheostomy-tubes-or-switch-appropriate-alternatives-during-shortage-fda-safety-communication.

6.  PULMONARY DIAGNOSES   149

monitoring tool for adults with neurogenerative diseases (Donohue, Khalifa, Mao, Perera, Sejdic´, & Coyle, 2021). B:  Yeah, if you’re not used to doing it, you might be surprised at what you hear. For infection control purposes, every child gets their own stethoscope at my children’s hospital and it’s a single use, disposable item. One thing I encourage all our therapists to do is practice auscultation with a parent and tell them that the stethoscope can go home with them if they don’t have one already. With COVID, it seems that everybody has jumped to monitoring respiratory status with a pulse oximeter. There’s lots of value in oxygen saturation readings, of course, but the stethoscope is kind of a forgotten tool, and it really shouldn’t be. Any parent that uses a stethoscope with their chronically ill child is, in my opinion, ahead of the game. J:  It seems like feeding and swallowing specialists should build their skills in stethoscope use. Do you have some suggestions? B:  Find yourself a friendly respiratory therapist. Spend some time with them and a couple of their patients. Have them talk to you about what you hear on auscultation and what might be going on respiratory-wise. You might also want to become familiar with basic breath sound terminology so you can use it at the appropriate time. SLPs are probably like RTs in that a conversation with a provider is at times short and quick. Busy docs want to hear it all in 10 seconds. You have that 10-second space then to get their attention and prove you are telling them something of value. J:  Give us some information about some terminology that we should learn. B:  Using the right terminology can show that you know what you’re talking about. Regarding specific breath sounds, “crackles” is a commonly used term. It is a “Rice Krispies in milk” sort of popping sound upon inspiration. It is caused by collapsed or fluid-filled small airways and alveoli in the peripheral segments of the lungs popping open. Crackles are often appreciated with fluid overload, pneumonia, or bronchiolitis. An older term was for the sound was “rales” (pronounced either as short-a “rawls” or as long-a “rails”). That term has fallen into disuse across the board in respiratory and nursing schools and for resident physicians coming out of training. Crackles is a more descriptive and much less ambiguous term. Regarding treatment for lung crackles, the causative fluid cannot be suctioned or immediately coughed out. Resolution simply must wait for treatment of the initial cause.

150  CASE STUDIES IN PEDIATRIC DYSPHAGIA

J:  Help us better understand wheezing. B:  “Wheezing,” which is classically associated with asthma. It is a whistling, musical sound caused by bronchospasm. “Bronchospasm” — tightening of the smooth muscles in the walls of medium and small airways. You might think you could hear that sort of sound unaided, but unless it is quite severe, you normally can’t. There are times when you put a stethoscope to the chest of a child who simply appears restless or uncomfortable, and you hear profound wheezing during their entire expiratory breath phase. Wheezing indicates that a patient may be doing a lot of work simply to breathe, so they’re focusing on that instead of what you may be trying to do with them. A third, and often obvious, breath sound is termed “rhonchi.” Rhonchi are gurgling or junky sounds generated by accumulated secretions in the upper, larger airways. Ronchi can usually be cleared by suctioning or, if the patient is able, getting them to cough. (See Figure 6–8 for further information on sharpening clinical skills in pulmonary issues.) J:  Can you help us understand respiratory decompensation? B:  That is a generic, descriptive term, but one that will be familiar to all clinical caregivers. It refers to any abnormal respiratory state rendering a patient incapable of sustaining oxygenation and ridding themselves of carbon dioxide. One of the things that we should keep in mind is that for children, the great majority of serious, life-threatening events originate with a respiratory event of some sort. For the most part, children

• Crackles (rales) — a “rice crispies in milk” sound; caused by small airways deep in the lung collapsing and audibly “popping” back open with each breath; likely due to pneumonia or fluid overload; normally, the fluids causing these sounds cannot be evacuated in the short term. • Ronchi (rattles) — due to fluid or secretions accumulating in larger airways; suctioning can often remove the offending fluids. • Wheezes — musical “whistling” sounds, normally heard on exhalation; can be audible without a stethoscope in severe cases; a classic sign of asthma; almost always due to airway spasm (bronchoconstriction); often relieved by administration of aerosolized bronchodilator medications (albuterol/Xopenex). Figure 6–8.  Understanding some basic pulmonary terminology.

6.  PULMONARY DIAGNOSES   151

have great hearts. Their respiratory systems are somewhat fragile in part because they’re small. All human ventilation has to do with gas flowing through tubes (airways) in the lungs. If a child’s already small airways are made even narrower by swelling, excessive secretions, or spasm, resistance to gas flow through those airways increases radically. When we get past the point of respiratory decompensation to actual respiratory failure marked by apnea or gasping, we move into the realm of intensive care where patients are normally too sick to receive SLP services. J:  That was a helpful explanation in understanding children’s airways. B:  I would summarize this whole topic by saying that the lungs are a very dynamic territory where ventilation and perfusion problems that occur can be life-threatening. This is where vasopressor medications, mechanical ventilation, and patient positioning, such as the act of “proning” with COVID patients, are used in attempts to match fresh oxygen-rich ventilatory gases with blood perfusion in as many segments of the lungs as possible. J:  You also talked about how RT roles vary in different institutions and the usefulness of knowing what RT roles are in the institution where you’re working. B:  Every hospital I have worked at in my career has been different. Some RT responsibilities are quite consistent. Aerosol medication delivery is what we do almost everywhere. Ventilator care and management is another responsibility that is almost always assigned to RTs. My current job at the children’s hospital provided me with my first opportunity ever to interact clinically with an SLP. I began with no idea what an SLP knew, or what they do clinically. I have been quite impressed with your profession after learning a bit about your base of skill and your expertise. My ignorance was mainly due to trach care at my previous hospitals being assigned almost exclusively to nursing staff. Over time at the hospital, that same division of care has changed so that our respiratory therapists are now involved in all aspects of trach care. That’s just one illustration of the differing roles you might find assigned to an RT. Therapists are not heavily involved with rehab in most institutions; therefore, RT and SLP professional paths may seldom cross. There are therapists who specialize in and do nothing but patient rehabilitation. However, they are a small subset of our profession. Not every therapist, therefore, is going to be everything to everybody. You may have to determine their responsibilities in your institution. Take that as another good reason to have a conversation with a friendly therapist.

152  CASE STUDIES IN PEDIATRIC DYSPHAGIA

J:  That’s a great idea. Another thing that you mentioned was that a lot of the children RTs treat are medically fragile with frequent admissions to the hospital. These are children and families that RTs come to know quite well. B:  We do, and that’s another common theme in most acute care institutions. We have a very lengthy history with a lot of children needing the rehabilitative services that SLPs offer. I can think of example after example where the familiarity we have with the respiratory needs of a patient and family would make us a valuable resource to call upon. We know the patient’s history. We know what they sound like when they are stable. We have probably seen them when they are not doing well. We worry about them, and we care. I’ve been fortunate almost my entire career to work at children’s hospitals where therapists are truly a different breed. It is not that they sort of like kids — they REALLY like kids, or they don’t stick around. So, as an SLP, you can be certain you will be working alongside someone willing to do all they can to help a patient and the family that loves and cares for them 24/7/365. J:  This is a little bit “off the cuff” (a little trach humor), but I think it would be helpful for the reader if you could talk through the trach weaning process. What is being looked at in considering a move toward decannulation? What factors are considered? Trach weaning or decannulation is the process of removing a tracheostomy tube when the patient no longer needs it. B:  Decannulation is considered when a patient no longer needs mechanical ventilation, is able to handle and clear their own airway secretions, and tolerates at least 24 hours with their stoma or the lumen of their trach occluded with a cap or plug. J:  Tell us about the team process in decannulation. B:  Prior to that 24-hour trial, which is usually done in an intensive care unit (ICU) setting, the trach tube itself has usually been downsized, and the surgeon or ENT managing the patient has determined there are no granulations or other anatomic issues that are likely to interfere. Decannulation tolerance is assessed in steps prior to the final 24-hour test without removing the trach. The stoma can be capped off for increasing periods while the patient is awake, alert, and monitored for distress. This can be accomplished with a speaking valve as well as with a simple plug that does not assist with vocalization. What’s surprising is how quickly the opening in the neck, the ostomy or stoma, begins to close after decannulation — usually within 24 to 48 hours.

6.  PULMONARY DIAGNOSES   153

J:  Give us a sense of the adjustments the child needs to make without a tracheostomy. B:  You’ve just given away the emergency airway a patient has depended on for a significant time, so you want to be ready when decannulation occurs. Most of the time everyone is confident what is going to happen due to the successful test steps that have been done, but no one assumes anything. You do, of course, have children with neuromuscular weakness, spinal muscular atrophy (SMA), for example. They may never have a truly effective cough. Can they handle that? Can their family handle that (Kolb & Kissel, 2015)? J:  What has been your experience in how families handle trach management? B:  Most children with a trach and their families get quite comfortable with it. It goes from absolute stark terror when the word is first mentioned with relation to their child to acceptance and, often, gratefulness. I can give you example after example of this because RT currently does the trach cardiopulmonary resuscitation (CPR) teaching at the children’s hospital for new trach families. After a trach is in place and the family has had a bit of time to get used to the new airway, one of the questions I like to ask to accentuate the positive is, “Is life better? Is your child breathing better now?” I know the answer is probably going to be yes. There is often an immense relief in no longer having to watch their child struggle to breathe and wondering if they might die in their sleep if a caregiver nods off from exhaustion. A trach can be very life enhancing, scary though it always is at first. J:  What are some responses that you see from children with trach use? B:  Kids will even play games with their own trach. It is a way a younger child can nonverbally say, “Hey, you’re not paying attention to me. Look here. I’ve got my trach tube in my hand. Now that I’ve got your attention, what are you going to do about it?” Going back to decannulation, episodes where a child purposely self-extubates might even add to a family’s level of comfort when the trach is to be discontinued. Their child has shown their own level of comfort with it all. J:  Can obstructive sleep apnea cause problems with decannulation? B:  Occasionally, I suppose, but only in rare and extreme cases where all the noninvasive continuous positive airway pressure (CPAP) and bilevel positive airway pressure (BIPAP) solutions have not worked. A long-term or permanent trach could then be considered. Noninvasive ventilation technology has gotten much more sophisticated in

154  CASE STUDIES IN PEDIATRIC DYSPHAGIA

the past decade, so I think there is almost always a nontrach solution that can be found. J:  Tell us more about lung anatomy. B:  There are 23 divisions of the airways from the trachea down to the terminal alveolus where oxygen and carbon dioxide gas exchange takes place. The larger airways are generally the first three or four divisions just below the larynx and vocal cords. You have the trachea, right and left mainstem bronchi, then segmental bronchi, one of which serves each of the six anatomically discrete lobes in the lungs. The airway division process continues until at the very end there are groups of little air sacs called alveoli. The alveoli are in very close proximity to blood vessels in the lungs and are where the all-important exchange of oxygen and carbon dioxide takes place. Airway sounds you might hear with the naked ear generally are coming from the three to four largest divisions of the airways. When you get down deep into the lung, you get the more high-pitched crackles or wheezing sounds. Rhonchi or rattles come from gas passing through secretions pooled in the larger airways. Rhonchi due to secretions can be a chronic condition for some long-term patients. At that point you turn to mom, you turn to dad, or you turn to grandma, and you say, “Is this unusually bad, or is this normal for your child? Should I be concerned? Are you concerned?” J:  I bet you get varying levels of response to do those sorts of things, right? B:  Well, the important point is the need to catch respiratory issues or signs of impending shock as early as possible. You don’t ever want to get to the point of full cardiorespiratory arrest because recovery from resuscitation is never guaranteed. It fails, or semi-fails, so many times, even in a hospital setting. We have a quality metric at the hospital that we follow. It is the number of days since we had a cardiopulmonary arrest outside critical care. Our current stretch (and record) is 650+ days and counting. This stretch follows previous long stretches of 592 and 535 days without an arrest. Doing math, that means in the past 5 years, we have dealt with only three full-arrest events outside ICU. That record is as phenomenal as it sounds. It demonstrates a very effective team effort by our physicians and by the nurses and therapists who are the most frequent caregivers at the patient’s bedside. Our (respiratory) therapists have made major contributions to keeping all our patients safe, which should reinforce the fact that we can help any SLP who might ask for clinical information or assistance. All clinical caregivers are required to have Pediatric Advanced

6.  PULMONARY DIAGNOSES   155

Life Support (PALS) certification. The first emphasis of that course is recognizing serious signs and symptoms early — before disaster. As an SLP, let’s say you are concerned that an infant undergoing a swallow study might aspirate or have laryngospasm. Where in the fluoroscopy suite is emergency oxygen and suction? Is everything connected and ready if needed? Will the tubing reach where the patient is located? How reliable is the patient’s heart rate and/or pulse ox monitoring? Is the monitor being watched by someone in the room? I submit that a good RT would have thought through each of those questions as part of their responsibility. One case that I will think about for the rest of my life involved my not blowing the whistle regarding a child being treated in our ED (emergency department). There are lots of reasons why I did not. In this case, the problem turned out to be profound septic shock; the outcome would likely have been no different if I had acted early, but I will always wonder. A message in that anecdote, for anyone working in a hospital setting, do not hesitate to ask for assistance or call a rapid response team for any patient condition that seriously concerns you. The ground rules for rapid response clearly state that no one will ever be penalized for being wrong. J:  You never get over those situations, do you? B:  No, you don’t. But such things are almost guaranteed to happen to everyone working in clinical care, and it can be amplified in pediatrics by the fact that the patients involved are children. We deal with that initial fear often in our department because we hire a lot of new therapists just out of school. They first must be brave enough to join us, but they must be brave yet again as they learn to work in the pediatric and neonatal intensive care units, the ED, or the medical/ surgical floors where problems can come at you from just about any direction. There is a lot to learn and a lot at stake in all those units, and only a portion of the learning from one unit will overlap with the others. We inform all new therapists when they are hired that being able to work independently in any unit that RT services will be a job requirement for them. J:  You have mentioned baseline function at a couple of different points. Walk us through just in a general sense how baseline of function is determined. B:  All right, well, this is an area where you sometimes accumulate a bunch of numeric readings, make a bunch of patient observations, and then try to make sense of it all. As an example, let’s take a child on a home ventilator. What do the numbers on the ventilator screen

156  CASE STUDIES IN PEDIATRIC DYSPHAGIA

say is happening? What does the child’s interaction with the ventilator say is happening? The numbers and your patient observations can be quite discordant at times. Let’s say the ventilator is cycling 40 to 45 times a minute but there is no apparent coordination between the child’s respiratory efforts and the ventilator. What’s going on? Or the opposite, with the ventilator cycling 10 times a minute, but a child is breathing 30 times a minute with no assisting response from the ventilator for 20 of the breaths they are initiating. What’s going on? J:  How do you pull all the information together? B:  Baseline respiratory assessment in these cases becomes an evaluation of the teamwork between the patient and their ventilator. Is the child comfortable with the ventilator settings they currently have? One of the concepts that we try to push is that as a therapist, you don’t just look at the ventilator, write down a bunch of numbers, then leave. We should do a true ventilator/patient assessment, then actively, if necessary, figure out whether anything needs to be assessed more fully or done differently. J:  That’s a good point. Seeing changes in behavior can be very informational. Let’s talk a bit more about patient safety. B:  We can look at trach patients where RT and SLP might meet at the bedside. At my hospital, we have worked long and hard on trach safety. If an SLP has a question about a trach, or is simply looking for confirmation of something, that’s a request that can be taken to almost any therapist at most institutions. J:  Tell us about how you have managed speaking valves with SLPs. B:  Speaking valves make me, as a respiratory therapist, cautious, and they are one of those areas where I think SLPs need a working knowledge of trach safety. What we are doing with a speaking valve is completely closing off a patient’s main expiratory pathway — the lumen of their trach tube. If the trach that’s involved has a cuff that is inflated in the trachea, at the time a speaking valve is attached to their trach tube, the result could be life-threatening. J:  Managing speaking valve placement must be challenging in an inpatient setting. B:  This is where caregiver teamwork becomes essential. SLPs can be very busy. Therapists often come in, do their thing, then hustle on to their next patient. Nurses are in and out doing their thing with the five or six patients they’ve been assigned. Family caregivers go to the cafeteria. There’s a lot of that going on, and suddenly, no one is

6.  PULMONARY DIAGNOSES   157

really monitoring a trach patient who may not be completely used to breathing with a speaking valve in place. J:  What are some other factors to consider with speaking valves? B:  Speaking valves serve an important developmental purpose, and at my hospital we try using them on patients in our NICU, even for some children who are still needing mechanical ventilation. A child should never be left inadequately monitored. The following questions should be asked: Is there a chance they might fall asleep and occlude their own airway without that being noted? Is there any chance a family member might unknowingly put a valve on a trach while the cuff is still inflated? Breathing with a speaking valve can also create increased resistance to exhalation that might fatigue a child and over time push them into trouble. We should all, as a team, be aware that there can be gray areas when it comes to children who are doing well, but are being introduced to new, home-oriented devices or routines. What type of monitoring is actually called for here? And most importantly, who will take responsibility for making sure there is monitoring? Those are key questions that someone on a team needs to think about and ask. J:  Well, that really touches on parental education and training and when do we, as therapists, need to become concerned enough about parental training and the use of equipment to seek advice or assistance. B:  I would say it is the same as calling a rapid response. You are never wrong to express a concern. At the hospital, one of the newest clinical committees that has been formed is our Airway Management Committee. At this point in its existence, it is primarily involved with trach care because there have been so many trach processes that need to be addressed. The committee is very much about families receiving the teaching they need to succeed when discharged home. J:  Have you seen progress in respiratory care? B:  We spend a lot of time teaching and having a family become comfortable with their child’s trach prior to being discharged home for the first time. We now have statistics that show that our teaching process has decreased subsequent emergency department (ED) visits and readmissions. We don’t see these kids as often now as we did 4 years ago, and that’s very encouraging. J:  What are some issues to solve moving forward? B:  One of the issues we are trying to address now involves the home care nursing staff many new trach families depend upon for support.

158  CASE STUDIES IN PEDIATRIC DYSPHAGIA

There is no guarantee that a private duty nurse will have had any experience with pediatric trach patients. I mean, care in the home is such a many-faceted problem for so many reasons. The good news is that we have had some success to date with our family education, and that we are seeking to do even better in the future. As for the pieces we have a lot of control over, we are aggressive. If a dad is taking the attitude that mom will take care of all that trach stuff, we come back with “No, Dad. You will also do trach changes here before your child goes home, because you will almost certainly be doing it all by your lonesome at some point down the road.” J:  Yeah, we can only do our best. Hopefully all the rest falls into place, right? B:  We spend a lot of time making sure that a family leaves the hospital with the right equipment in addition to the training they need for the responsibility they have as the ultimate caregivers for their child. We see most parents step up and become completely committed to the care of their child. And we should perhaps end by saying . . . and then there’s grandma. Thank heaven for the grandmas and grandpas of the world, because in so many cases, they will be watching and doing a lot of especially important help.

The following resources may be helpful when managing patients with pediatric dysphagia and respiratory problems: Donohue, C., Khalifa, Y., Mao, S., Perera, S., Sejdic´, E., & Coyle, J. L. (2021). Characterizing swallows from people with neurodegenerative diseases using high-resolution cervical auscultation signals and temporal and spatial swallow kinematic measurements. Journal of Speech, Language, and Hearing Research, 64(9), 3416–3431. https://doi.org/10.1044/2021_JSLHR-21-00134 Mir, M. J., & Hegland, K. W. (2021). A survey of speech-language pathologists’ experience with clinical cough assessment. Perspectives of the ASHA Special Interest Groups, 6(6), 1627–1640. https://doi.org/10.1044/2021_PERSP-21-00144

REFERENCES Kolb, S. J., & Kissel, J. T. (2015). Spinal muscular atrophy. Neurologic Clinics, 33(4), 831–846. https://doi.org/10.1016/j.ncl.2015.07.004

6.  PULMONARY DIAGNOSES   159

Lau, C. (2016). Development of infant oral feeding skills: What do we know?. The American Journal of Clinical Nutrition, 103(2), 616S–621S. https://doi​ .org/10.3945/ajcn.115.109603 Marpole, R., Blackmore, A. M., Gibson, N., Cooper, M. S., Langdon, K., & Wilson, A. C. (2020). Evaluation and management of respiratory illness in children with cerebral palsy. Frontiers in Pediatrics, 8, 333. https://doi.org/​ 10.3389/fped.2020.00333 Schwartz, T. H. (2021). Dysphagia, COVID-19, and the clinical swallow evaluation: Consistently inconsistent. Perspectives of the ASHA Special Interest Groups, 6(5), 1222–1226. https://doi.org/10.1044/2021_PERSP-21-00020

7 Traumatic Brain Injury

INTRODUCTION When I recall patients that I have served due to a traumatic brain injury, their stories are etched into my memory. The story behind Max’s case study is no different (Tucker, 2018).When working with this population, the intensity is often felt whether in an acute or outpatient setting due to the intense transitions these patients and their families are experiencing during their intervention. Morgan et al. (2010) cited a 46% occurrence of pediatric dysphagia cases in their sample of 157 children, with a much higher percentage identified in severe traumatic brain injury (TBI) cases (76%). Through a three-round Delphi survey, consensus-based recommendations were developed for speech, language, and swallowing disorders in pediatric traumatic brain injury, including optimal time of assessment and assessment tools and intervention strategies (Mei et al., 2018). Pediatric patients with traumatic brain injury are diverse. A basic understanding of neural motor control can be a good place to begin (Humbert & German, 2013). The first factor is the level of motor learning and feeding/swallowing experiences that a patient has prior to the neurological event. For example, you may be working with a 3- to 6-month-old who has just begun acquiring motor learning through a suck-swallowbreathe pattern of liquid intake. Alternatively, you may be working with a 7-year-old who has very intact motor processes for feeding and swallowing. Many other factors are important to consider, such as the area or 161

162  CASE STUDIES IN PEDIATRIC DYSPHAGIA

areas of the brain affected and rehabilitative progress during periods for spontaneous recovery of function. The case study provides an example of a preteen boy who had acquired all the motor planning skills necessary for oral feeding. He was also at a stage of rapid growth, continuing to grow in inches postinjury and during treatment. The brain injury that resulted due to a basilar artery aneurism affected cerebellar brain function and pharyngeal strength and coordination, profoundly affecting swallow function. The traumatic brain injury chapter is further supplemented by an interview with physiatrist Dr. Miriam Weinstein. Many years ago, I worked on a pediatric trauma brain injury team with Dr. Weinstein. She provides insight regarding aspects of brain injury, including latency in responses and the importance of external cuing in the rehabilitative process.

CASE STUDY See an image of Max beginning outpatient treatment in Figure 7–1.

Medical History and Current Status Initial evaluation at an outpatient facility 2 months after the basilar artery aneurism: Max, a previously healthy 11-year-old male, was taken to the emergency room by his parents. He was diagnosed with migraines and symptoms worsened after being sent home. He returned to the emergency department at a children’s hospital and received a head computed tomography (CT) scan because of a recent fall. The CT images revealed a concern of a bleed and Max was admitted for observation. While under observation, Max became unresponsive with difficulty breathing and experienced cardiac arrest with ventricular arrhythmia. He was given cardiopulmonary resuscitation (CPR). He suffered from a basilar artery aneurism. This resulted in locked-in syndrome; hemiparesis of the right dominant side; impaired functional mobility, balance, gait, and endurance; dysphagia; hydrocephalus; and trismus. A G-tube was placed for feeding and Max began receiving bolus feedings. Max’s mother provided the following medication list at the time of the evaluation: n Trazodone

100 mg

At night

n Lidocaine

5% patch

At night

7.  TRAUMATIC BRAIN INJURY   163

Figure 7–1.  Max beginning outpatient treatment.

n Bisacodyl

10 mg

At night

n Lidocaine-prilocaine

2.5%

As needed

n Polyethylene glycol

17 g

Every day

n Polyvinyl alcohol

Drops

As needed

n Lactobacillus rhamnosus

3 cap

Every day

n Baclofen

30 mg

3× a day

n Enoxaparin

50 mg

Every 12 hours

n Acetaminophen

576 mg

Every 6 hours p.m.

n Ranitidine

114 mg

2× a day

n Levetiracetam

1,200 mg

2× a day

n Diazepam

12.5 mg

As needed

See Max’s medical history timeline in Figure 7–2.

164  CASE STUDIES IN PEDIATRIC DYSPHAGIA

Max’s Case Study Timeline

• Outpatient referral 2-months post-injury for feeding and swallowing therapy • ER visit to local hospital due to headache at 11 years old • 2nd ER visit to a regional hospital, admitted after CT scan suggested the possibility of a brain bleed • Cardiac arrest, labored breathing and ventrical arrhythmia occurred • Diagnosed with a basilar artery aneurism • In-patient rehabilitative hospital stay for 2 months • Current diagnoses: locked-in syndrome, hemiparesis of the right dominant side, impaired functional mobility, balance, gait and endurance, dysphagia, hydrocephalus, and trismus Figure 7–2.  Max’s case study timeline.

Evaluation An oral mechanism examination revealed the following: Lips:  Asymmetrical; right-side paralysis; displayed left-side retraction, raised, and slight protrusion. Tongue:  Unable to view. Teeth:  Clenched, some grinding reported and noted. Dentition appeared within normal limits. Palate:  Unable to view. Jaw:  Muscle tightness observed on both sides but more severe on the right; immobile. Max was alert throughout the evaluation, as evidenced by answering yes/no questions gesturally. He responded to touch around his mouth and inside the left side of mouth through replying that he could feel the touch (a yes/no gestural response). He retracted his lips on the left side frequently throughout the evaluation, gestured through widening his eyes, and demonstrated increased lip retraction when told jokes. He utilized eye movement for communication. His family members explained, and the

7.  TRAUMATIC BRAIN INJURY   165

evaluator observed the following: Looking up meant “yes,” looking down meant “no,” fluttering up and down meant “kind of,” and looking up and down fast meant “I’m annoyed” or “stop.” His family also reported that Max used a letter-board to communicate. They were anticipating a loaner augmentative communication device that was ordered and scheduled to arrive following an evaluation at a local hospital. Max was observed to have labored breathing. His breathing seemed less labored as the evaluation continued. He was unable to swallow and cough when asked or when it was modeled. He was observed to cough reflexively on a couple of occasions during the evaluation. His cough was judged to be weak and nonproductive. His mother reported that after coughing up mucus, he could swallow, and if he was unable to swallow, he moved it to the side of his mouth to be suctioned out by a nurse or parent. During the 2-hour evaluation, Max was suctioned three times after asking him if he needed to be suctioned. Gathering Information to Prepare for Treatment Questions for Max’s parents: What types of changes have you seen over the past 2 months? We are very frustrated because during his inpatient stay, we feel like all they did with him is play games. We feel like nobody was working on improving his swallowing skills, like they just gave up on him. We really feel like it was a waste of time. How is Max doing with communicating pain and discomfort? Sometimes we just must guess that he is in pain and ask him lots of yes/no questions to know how to best position him. How does he sleep at night? He is sleeping well. How is he handling G-tube feedings? They seem to be going well. What are your goals for Max? We want him to be able to eat by mouth again. We know that he wants to eat, and he loved to eat before this happened. Additional questions to gain information about Max: What is the current G-tube feeding schedule? What are the ways to communicate to Max’s parents about how his muscles are currently functioning? Education should be a strong emphasis of treatment. Practice skills in providing verbal explanation for parents about sensory and motor responses.

166  CASE STUDIES IN PEDIATRIC DYSPHAGIA

Providing home programming for them will help them understand his progression. What are some concrete ways to explain sensory and motor muscle progression with Max’s parents? Providing information throughout the treatment session about the active oral movements that you are working toward can be helpful. For example, “I am exposing him to this smell and taste and mirroring motor movements. I would like to see increased motor control and active movement with his lips.” Max’s mother said, “The patient will tell you something different than the chart.” See Figures 7–3 and 7–4 for images of Max during the outpatient treatment process.

Max’s mother said, “The patient will tell you something different than the chart.”

Gaining a general sense of Max’s diagnosis will help in preparation. While the case cited is an adult case, it provides a broad sense of the potential cranial nerve involvement that should be further explored (e.g., visual, visual motor, jaw movement) through oral sensorimotor interven-

Figure 7–3.  Max during treatment.

Figure 7–4.  Max during treatment.

7.  TRAUMATIC BRAIN INJURY   167

tion (Giffin & Goadsby, 2001). See the cranial nerves involved in the oral phase of swallowing in Figure 7–5. In determining treatment direction for Max, sensory responses were the starting point. Sensory intervention should occur at the most basic level through olfactory and tactile stimulation (Madhavan & Etter, 2021). Incorporating principles of exercise physiology can also inform the treatment process (Huckabee & Burnip, 2018; Morgan, 2017). See the areas of concern identified during the evaluation process in Figure 7–6.

Structure

Afferent

Efferent

Lips

V2 (maxillary) V3 (lingual)

VII

Tongue

V3 (lingual)

XII

Mandible

V3 (mandibular)

V (muscles of mastication), VII

Palate

V, IX, X

IX, X

Buccal mucosa

V

V (muscles of mastication), VII

Figure 7–5.  Cranial nerves oral chart. Source: Comprehensive Management of Swallowing Disorders, Second Edition (p. 30) by Carrau, R. L., Murry, T., and Howell, R. Copyright © 2017 Plural Publishing, Inc. All rights reserved. Used with permission.

Oral Preparatory Phase Oral Transit Phase

Max’s Potential Areas of Concern Pharyngeal Phase Esophageal Phase

Figure 7–6.  Max’s potential areas of concern.

168  CASE STUDIES IN PEDIATRIC DYSPHAGIA

Treatment Treatment was initiated for Max’s oral and pharyngeal dysphagia. Initially, treatment focus was on sensorimotor skills. Sensory skills were targeted to encourage what were at first very subtle motor responses. After 3 to 4 weeks of treatment, the following progression of skills were reported: (a) decreased jaw tightness; (b) a weak, nonproductive cough but unable to cough on command; and (c) increased phonation throughout sessions. Increased exposure to tastes was trialed with signs or symptoms of physiological distress. See the cranial nerves involved in the pharyngeal phase of swallowing in Figure 7–7.

Reinitiation of Therapy Max came back in for services after a holiday break. Here is a note following intervention: Decreased jaw tightness today characterized by wider opening between teeth. Some movement on the right (twitching). Strong nonproductive

Structure

Afferent

Efferent

Tongue base

IX

XII

Epiglottis (lingual surface)

IX

X

Epiglottis (laryngeal surface)

X (internal branch of superior laryngeal nerve)

X

Larynx (to level of true vocal folds)

X (internal branch of superior laryngeal nerve)

X

Larynx (below true vocal folds)

X (recurrent laryngeal nerve)

X

Pharynx (naso- and oro-)

IX

X (except for stylopharyngeus, which is innervated by IX)

Pharynx (hypopharynx)

X (internal branch of superior laryngeal nerve)

X

Figure 7–7.  Cranial nerves pharyngeal chart. Source: Comprehensive Management of Swallowing Disorders, Second Edition (p. 31) by Carrau, R. L., Murry, T., and Howell, R. Copyright © 2017 Plural Publishing, Inc. All rights reserved. Used with permission.

7.  TRAUMATIC BRAIN INJURY   169

cough 2× reflexively. Phonation 3× today, no volume increase seen. Exposure to tastes today (e.g., lemonade) and swallow on command 2×. Appeared to attempt to initiate lip movement as evidenced by chin twitch. The family reported increased vocal use, volume, and some prosody. Also reported more tongue movement (up, down, side to side).

As more oral and pharyngeal volition became apparent, Max was a candidate for a VFSS. Max was expressing a desire for food, and his parents expressed interest in providing him with some preferred foods. What factors should influence getting a modified barium swallow study scheduled? In Max’s case, it was important to determine parent education and understanding of his acute risk for silent aspiration. A modified barium swallow appeared necessary to help them understand pharyngeal motor strength and coordination issues in a more concrete way. The clinician recommended a MBSS at the local hospital and called the primary care physician’s office to request an order given concerns for the pharyngeal phase of his swallow. See information about how to address issues identified through instrumental evaluation in Figure 7–8. Discharge to a rehabilitative facility was also under way, given Max’s progression of skills and potential. Pharyngeal skills were continuing to improve, but it was not clear whether Max could protect his airway without instrumental testing.

Problems identified

Maneuver

Decreased orolingual pressure (Fukuoka et al., 2013)

Effortful swallow

Decreased pressure in the upper pharynx (Huckabee & Steele, 2006)

Effortful swallow

Decreased tongue base retraction

Effortful swallowa

Decreased laryngeal elevation

Mendelsohn maneuver

Decreased airway protection (At the level of the vocal folds) (Guedes et al., 2017; Logemann et al., 1997)

Supraglottic swallow

Decreased airway protection (Facilitates closure of laryngeal vestibule) (Donzelli & Brady, 2004; Vose et al., 2014)

Super-supraglottic swallow

a

Effortful swallow may not be a good approach if pyriform sinus residue is identified in an instrumental study (Molfenter et al., 2018).

Figure 7–8.  How to address issues identified through instrumental evaluation.

170  CASE STUDIES IN PEDIATRIC DYSPHAGIA

A subsequent treatment note stated the following: Strong productive cough 1× reflexively. Phonation 2–3× today, minimal volume increase observed. Swallowed throughout session reflexively with 1- to 3-second response time. Observed second swallow 8× to clear throat. The following information was provided after a modified barium swallow study: Max was orally presented thin barium via an open cup and pudding barium via a spoon. Deep penetration was noted, with both consistencies resulting in aspiration. No cough reflex was elicited. Delayed swallowing reflex was present. The impression of the MBSS was listed as penetration and aspiration with thin barium and pudding barium. Following the MBSS, Max received insurance authorization for a rehabilitative hospital program. Discussion: At 3 years postinjury, Max’s parents reported, “He is eating now for pleasure.” Fortunately, his parents were strong advocates throughout the therapeutic process, and progression of skills occurred during his rehabilitative care. As sensorimotor intervention was initiated, Max gained increased jaw control. As he began to move his jaw, oral care improved. Eventually, lingual muscle function improved, leading to an ability to contain and propel bolus. Pharyngeal strength and coordination also eventually improved. See an image of Max straw jousting with dad in Figure 7–9.

Figure 7–9.  Max straw jousting with his dad.

7.  TRAUMATIC BRAIN INJURY   171

INTERVIEW WITH DR. MIRIAM WEINSTEIN Dr. Weinstein is a clinical associate professor at the University of Tennessee Health Science Center and is certified through the American Board of Physical Medicine and Rehabilitation. She has served countless families over the years, guiding them through the best rehabilitative plan as they develop. Several years ago, we served on a pediatric traumatic brain injury team together. Jennifer Wilson ( J):  Can you give me a brief bio? I’m sure it is going to be difficult because you’ve had quite a career. Dr. Miriam Weinstein (Dr. W):  I went to medical school at Louisiana State University in New Orleans. I started to go to a Physical Medicine and Rehabilitation and realized it was the perfect match. I went off to Rose F. Kennedy Children’s Evaluation and Rehabilitation Center at Albert Einstein College of Medicine and did a pediatric rehabilitation fellowship with Dr. Gabriella Molnar. At that time, we got crosstrained extensively in developmental pediatrics. I was an attending assistant professor at Einstein, then an attending at North Central Bronx and Montefiore Medical Center. I got the chance to work with some neat therapists. One of the speech therapists came back from an early talk by Dr. Jeri Logeman. She showed us videos and talked to us about modified barium swallow studies. I got extremely excited about that. I pioneered using them with kids. I arranged for my whole team, the occupational therapist, speech therapist, and me to go to a Jeri Logeman course. We said to Dr. Logemann, “You didn’t talk to us about kids.” She said back to us, “You’re evaluating kids?” She wanted to learn from us, when we were going to the guru! Years later, I ended up coauthoring with Mark Ylvisaker, a child psychologist, the first paper about pediatric swallowing studies (Ylvisaker & Weinstein, 1989). The journal article we submitted was guest edited by Jeri Logemann. It was the only article for a long time on the subject. It really took about 10 years or so for the field to catch up. I was out at Rancho Los Amigos for a while and working with severe traumatic brain injury trying to figure out how to unlock “locked-in” adults. I got interested in that. Eventually, I came back to doing pediatrics. I came to the University of Tennessee Graduate School of Medicine, back when they had a pediatric residency program. I was recruited by the pediatric department at Patricia Neal Rehabilitative Center. Now I’m in independent clinical practice as a clinical associate professor with the Graduate School of Medicine through the Department of Surgery.

172  CASE STUDIES IN PEDIATRIC DYSPHAGIA

J:  Tell us a little bit about developmental coordination disorder. Dr. W:  It is present in around 10% of the kids that you’re going to see. This is a problem that can be genetic, or it can be caused by prematurity. Any infant who was 32 weeks’ gestation or less should automatically be assumed to have this type of wiring issue. It’s characterized by underdeveloped brain circuits that carry the message in the brain from up through the cerebellum and into the thalamus and so on, into all the target areas of where this information needs to go. So that means that there is a lot of proprioceptive information from the body, and that includes the oral motor components that aren’t getting as completely to the brain areas that need to use them for motor planning, and this can cause delays in feeding, swallowing, and language. The speech therapist can be the first person to see these children. These motor planning issues can have incredibly significant implications for how well those kids are going to do in life in terms of predictive learning disabilities. About 50% of these kids have attention-deficit disorder. J:  With that developmental motor coordination disorder, we’re going to begin to see those kids with feeding and swallowing issues and that’s really going to be our first clue that there is that motor coordination issue, right? Dr. W:  They do have some feeding issues, and they sometimes have swallowing issues, but especially feeding issues because they don’t always develop a good chew method because of their general motor issues, so that’s something to be very aware of. I don’t think there’s enough areas of neuromotor muscular research going on these days. I took another look after I got your set of questions. It’s the problem of draining the swamp when you’re fighting alligators. Clinicians are terribly busy treating kids, and I don’t think that enough time has been spent on really doing prospective studies. I saw a lot of ones that got started, but no studies have reported their results. Almost no studies have reported results. There are problems with inadequate study design. That’s an ongoing frustration. See Figure 7–10 for resources to sharpen clinical skills in traumatic brain injury. J:  That’s a great point that there needs to be more research in neuromuscular rehabilitation, particularly in feeding and swallowing. Do you have advice for habilitative skills? Or developmental skills versus rehabilitative skills? You would need to alter your focus a little bit. If you’re working with someone with a traumatic brain injury who is doing motor relearning versus just the initial motor learning.

7.  TRAUMATIC BRAIN INJURY   173

Brain Injury Resource Center —TBI glossary http://www.headinjury.com/tbiglossary.htm Traumatic Brain Injury:  A Primer for Professionals https://doi.org/10.1044/leader.FTR1.07122002.4 Minimum Competency Recommendations for Programs That Provide Rehabilitation Services for Persons With Disorders of Consciousness: A Position Statement of the American Congress of Rehabilitation Medicine and the National Institute on Disability, Independent Living and Rehabilitation Research Traumatic Brain Injury Model Systems https://www.archives-pmr.org/article/S0003-9993(20)30093-9/pdf Figure 7–10.  Sharpening clinical skills in traumatic brain injury.

Dr. W:  You’ve got to understand that when a child has had traumatic brain injury, they automatically have had axon disruptions and they are dyspraxic. They need to be aggressively cued to be hands on. If you have the skills of Beckman’s oral motor exercises, I think that’s a big asset. That’s one thing. Another thing is trying to present them with targets. Dyspraxics often do better if you’re giving them a target, like something to blow on, through, or at. If you’re just asking them to imitate you, imitation can be a very weak area with them. Another thing to be very aware of is how slow their reaction times can be. You don’t necessarily want to go on to the next thing until you have really done a lot of work in chewing and done better assessment of what their response time is. Response times can be terribly slow. It’s not unusual for it to take even 30 to 40 seconds to get a response. We are just used to that. We think it’s been a long time since we waited for a child to respond in 5 seconds. But usually with an injured child, they have a window that’s consistent with how fast they respond. Over time, with good therapy, their response rate will speed up until hopefully they have something more approximating a normal reaction time. But in the beginning, when you’re starting with an involved child, you want to know what their reaction time is. It could even be as far out as a minute. When you have kids that are that far out, you’re not usually even getting much oral motor use of any kind. You’re looking more for gross motor skills at that point. Once they’re at the reaction time where you can work with them to start getting speech and language back, it’s usually faster than that, but it’s a lot slower than what you think it’s going to be. That’s especially important to be tuned into how much they need that hands-on approach

174  CASE STUDIES IN PEDIATRIC DYSPHAGIA

and input and objects to cue them. You should target more automatic things that elicit more of the movements that they used to make, like licking something. J:  Yeah, I think so. I think that is just that can be so tricky when you’ve got to think about the fact these are, these are skills that depend on the age of the child that you’re working with, who has brain injury. These are skills that should be internalized and automatic, particularly if you’re dealing with feeding and swallowing, and that’s so different than many of our kids who never really acquired skills in an oral motor function like rotary chew. Maybe they just haven’t acquired those skills in full use of intrinsic and extrinsic tongue muscles. I think that’s the thing that is just so notably different about working with brain injury in the pediatric population where we’re used to working on habilitative skills versus rehabilitative skills. Thank you for talking about it in neurological sense and that poor task initiation. J:  Are there areas of neuromuscular rehabilitation that we should be watching where there is some research and there’s some innovation and changes that will affect the way that we do rehabilitation? Dr. W:  I think that there’s beginning to be more attention for proprioceptive input and feedback as a valuable tool in trying to get neurorehabilitation done. They’ve been doing it in stroke, but I think that it has a real future in terms of starting to apply it to oral motor function. We want to explore how to cue kids so that you get more input peripherally to restart the circuits; especially in head injury, you’re going to assume at least some of the circuits are there to reestablish better circuits so that you can get better output. It’s more external than we’ve previously thought. There’s still a role for all the things that you’ve been doing. A lot of what good therapists have been doing uses these tools. I would just be saying to use them in a more conscious kind of way, tuning into a response to input. J:  That makes a lot of sense. What you’re saying is we intuitively do some of these things but to do them with a little more intentionality is good. Dr. W:  Exactly. Good therapists have great intuition. J:  What are some subtle neurological changes that are occurring in the children that we are working with that we should be aware of? What are some things that I need to look for that would suggest that there are some neurological changes?

7.  TRAUMATIC BRAIN INJURY   175

Dr. W:  Look at the posture. How well are they sitting? Has there been a change in how well they sit? Look at motor planning when they’re reaching out in space. Are they a little unsteady? Are you noticing that things that were a little easier for them are a little bit harder? Start looking for core coordination issues. Do they sound raspy or rattly? Try giving him a sip of water and listen. Are things going where they’re supposed to be going? You may start to have some concerns that maybe they are not. They are coming in with colds. Do they get fatigued more during a session? Those might be all things to look at. J:  How can we work together better? Dr. W:  I think effective communication. I love effective communication. We are each seeing a piece of the puzzle and I think if we share and if we can collaboratively solve problems. I was doing that with a therapist last week. We really felt good after we finished talking. We had a new plan, and we’ll see whether it works, but at least we felt like we were together and trying to solve some of the puzzles. J:  What kinds of qualities do you see in exceptional therapists? You’ve worked with many therapists, maybe some exceptional therapists throughout the years. What are some qualities that you have seen in therapists who have really made a difference? Dr. W:  Good clinicians are astute observers, and they have good instincts for things to try and how to interact with a child. They know what works and what doesn’t work. They stay flexible and creative in their approach. They stay up to date in their knowledge of techniques and are willing to seek more information. They go to courses or conferences so that they stay current. They are willing to communicate and they’re good listeners. They are good listeners to the parents, other clinicians, and the child. All these attributes are important components. J:  What advice would you give to therapists practicing in rural areas where there are not as many resources? Dr. W:  I would say network. Fortunately, currently you can network so much more easily than you could. Get a good mentor or two. When working with somebody senior to you, you’ll get a sense of whether they’re going to be willing and able to be a good mentor for you. Reach out to experts. It can’t hurt to ask somebody whose article you’ve read or conference you attend. You may have this case that’s puzzling you. Do they have any advice? And of course, go to conferences and courses and network. Bring a couple of your cases

176  CASE STUDIES IN PEDIATRIC DYSPHAGIA

that are challenging to you. You can have a little informal discussion with some people. You could explain your case and ask advice about what to try. Those are all ways to stay current when you’re not near the center of things. J:  Yes, it’s extremely hard to practice pediatric dysphagia with a child involved with a lot of comorbidities in a rural area, especially as a young clinician. Dr. W:  If you can have a good OT (occupational therapy), good PT available so that you have people who could cotreat, can be beneficial. Sometimes cotreating with someone who can do the positioning while you’re working on the oral motor can be invaluable. That extra pair of hands can be so helpful. Often, they are not there in a rural setting, you are working alone. That creates challenges. J:  I have learned so much through working on multidisciplinary teams. Dr. W:  It’s a good example of the fact that collegiality and sharing ideas and multidisciplinary approaches can really pay off.

REFERENCES Donzelli, J., & Brady, S. (2004). The effects of breath-holding on vocal fold adduction: Implications for safe swallowing. Archives of Otolaryngology — Head & Neck Surgery, 130(2), 208–210. https://doi.org/10.1001/archotol​ .130​.2.208 Fukuoka, T., Ono, T., Hori, K., Tamine, K., Nozaki, S., Shimada, K., . . . Domen, K. (2013). Effect of the effortful swallow and the Mendelsohn maneuver on tongue pressure production against the hard palate. Dysphagia, 28(4), 539–547. https://doi.org/10.1007/s00455-013-9464-y Giffin, N. J., & Goadsby, P. J. (2001). Basilar artery aneurysm with autonomic features: An interesting pathophysiological problem. Journal of Neurology, Neurosurgery and Psychiatry, 71(6), 805–808. https://doi.org/10.1136/ jnnp.71.6.805 Guedes, R., Azola, A., Macrae, P., Sunday, K., Mejia, V., Vose, A., & Humbert, I. A. (2017). Examination of swallowing maneuver training and transfer of practiced behaviors to laryngeal vestibule kinematics in functional swallowing of healthy adults. Physiology & Behavior, 174, 155–161. https://doi​ .org/10.1016/j.physbeh.2017.03.018. Huckabee, M.-L., & Burnip, E. (2018). Still rethinking rehab: Motor learning treatment approaches for dysphagia. Perspectives of the ASHA Special Inter-

7.  TRAUMATIC BRAIN INJURY   177

est Groups, 3(13), 146–156. https://doi.org/10.1044/2018_PERS-SIG132018-0006 Huckabee, M. L., & Steele, C. M. (2006). An analysis of lingual contribution to submental surface electromyographic measures and pharyngeal pressure during effortful swallow. Archives of Physical Medicine and Rehabilitation, 87(8), 1067–1072. https://doi.org/10.1016/j.apmr.2006.04.019 Humbert, I. A., & German, R. Z. (2013). New directions for understanding neural control in swallowing: The potential and promise of motor learning. Dysphagia, 28(1), 1–10. Logemann, J. A., Pauloski, B. R., Rademaker, A. W., & Colangelo, L. A. (1997). Super-supraglottic swallow in irradiated head and neck cancer patients. Head & Neck, 19(6), 535–540. https://doi.org/10.1002/(SICI)1097-0347​ (199709)19:63.0.CO;2-4 Madhavan, A., & Etter, N. M. (2021). How can speech-language pathologists think about sensation during swallowing evaluation and intervention? Perspectives of the ASHA Special Interest Groups, 6(3), 620–630. https://doi​ .org/10.1044/2021_PERSP-19-00170 Mei, C., Anderson, V., Waugh, M. C., Cahill, L., & Morgan, A. T. (2018). TBI Guideline Development Group. Evidence- and consensus-based guidelines for the management of communication and swallowing disorders following pediatric traumatic brain injury. Journal of Head Trauma Rehabilitation, 33(5), 326–341. https://doi.org/10.1097/HTR.0000000000000366. Morgan, A. T., Mageandran, S. D., & Mei, C. (2010). Incidence and clinical presentation of dysarthria and dysphagia in the acute setting following paediatric traumatic brain injury. Child: Care, Health, and Development, 36(1), 44–53. https://doi.org/10.1111/j.1365-2214.2009.00961.x Morgan, L. B. (2017). Exercise-based dysphagia rehabilitation: Past, present, and future. Perspectives of the ASHA Special Interest Groups, 2(13), 36–43. https://doi.org/10.1044/persp2.SIG13.36 Tucker, M. (2018). When others doubt; Maryville family holds to faith and hope as son recovers. The Daily Times. https://www.thedailytimes.com/ community/when-others-doubt-maryville-family-holds-to-faith-and-hopeas-son-recovers/article_1a86de93-2fa0-5b88-ad10-f0dcd9828c20.html Vose, A., Nonnenmacher, J., Singer, M., & González-Fernández, M. (2014). Dysphagia management in acute and sub-acute stroke. Current Physical Medicine and Rehabilitation Reports, 2(4), 197–206. https://doi.org/10.10​ 07/s40141-014-0061-2 Ylvisaker, M., & Weinstein, M. (1989). Recovery of oral feeding after pediatric head injury. Journal of Head Trauma Rehabilitation, 4(4), 51–63.

8 Ethical Considerations

INTRODUCTION Ethical treatment of pediatric dysphagia should be at the core of how we approach all intervention. It begins with constantly improving our education and providing evidence-based information to our clients, families, and caregivers and recognizing the role of self or parent caregiver advocacy throughout the intervention process (Ball & Riquelme, 2016; Brady et al., 2016; Wilson et al., 2020). We will explore this topic through grappling with a complex case and an interview with a leader in the field, Dr. Memorie Gosa. Continuing education is at the core of ethical dysphagia management. As leaders in dysphagia management point out, we should carefully consider whether the information that we are taking in is well vetted (Hazelwood & Pollack, 2021). Taking the time to self-reflect can also strengthen intervention. We must spend time and energy exploring biases and flawed perceptions that inhibit the rehabilitative process (Askren & Leslie, 2019). Tilley et al. (2020) looked at the influence of self-advocacy on the subjective well-being in people with intellectual disabilities. Through polling their perceptions, they found that self-advocacy improved external conditions, specifically through supportive social networks and a reported shift in self-concept. They cited a need for future research focusing on selfadvocacy for people with more profound intellectual disabilities (Tilley et al., 2020). 179

180  CASE STUDIES IN PEDIATRIC DYSPHAGIA

The following case study provides us with the opportunity to discuss oral management of a pediatric patient with multiple comorbidities contributing to swallowing difficulties, falling in the profound intellectual disability range. The patient should be looked at distinctly due to a few key factors. Her diagnosis of cortical vision impairment (CVI), severe to profound hearing loss, and cerebral palsy, in combination with childhood epilepsy, causes challenges in determining signs of physiological distress and makes expressive communication challenging. Still, we must consider communication and involve the caregiver, her best communication partner, as an integral team member. A whole-child perspective with the types of sensory differences (CVI, hearing loss) must be a key part in the care of this child. Working closely with a vision specialist in understanding the best presentation of objects, actions, and the best schemes for multisensory presentation should be a priority. Further understanding triggers that may overwhelm her sensory system and cause seizure activity is also important to consider. Integrating all these considerations during a virtual session can be quite challenging but was necessary during a pandemic. Following the case study, Dr. Memorie Gosa provides the reader with a discussion and resources to increase ethical practice in pediatric dysphagia. She began to gain insight through her work editing Assessing and Treating Dysphagia: A Lifespan Perspective (Suiter & Gosa, 2019). Ultimately, she cited patient education/reeducation and professional self-reflection as important means to ensure a patient-centered care model.

CASE STUDY Medical History Sadie was referred to an outpatient setting at 2 years of age for an evaluation by her otolaryngologist (ENT). See an image of Sadie at 2 years, 3 months of age in Figure 8–1. At the time of the initial evaluation, the following were some excerpts from her report: Sadie was diagnosed with cerebral palsy and infantile spasms at 4 months old. She later received a diagnosis of cortical vision impairment, was placed on a feeding tube, and had a tracheostomy. She was fitted with a Passy Muir valve (PMV). She had mild to severe hearing loss in the right ear and moderate to profound hearing loss in the left ear and was fitted with behind-the-ear hearing aids for both ears. A full-time nurse provided care throughout the day, on weekdays.

8.  ETHICAL CONSIDERATIONS   181

Figure 8–1.  Sadie — 2 years, 3 months old.

She was taking the following medications: Sabril, Banzel, Topamax, Keppra, clonazepam, Onfi, omeprazole, Zyrtec, scopolamine, and baclofen. See Sadie’s medical history timeline in Figure 8–2. The following was an observation of oral structures and function. She showed poor oral secretion control. Her tongue could not be adequately observed and assessed due to trismus. Jaw tightness was clear, and mobility was not observed. Sadie made vocalizations when her PMV was attached. She appeared to be turn-taking vocally with these vocalizations when her mother would speak to her. Sadie arrived in her stroller and took several minutes to open her eyes. She was placed on a ball and began to stretch and open her eyes. She appeared to look toward faces near her. Parental perceptions: Sadie’s mom expressed frustration during the initial evaluation. Discharge planning had been too quick with another outpatient provider and her case had been rejected by other providers who did not feel that they were qualified to provide services.

182  CASE STUDIES IN PEDIATRIC DYSPHAGIA

Sadie’s Case Study Timeline • Outpatient referral at 2 years old • Diagnosis of cerebral palsy and infantile spasms at 4 months • Later diagnosis of cortical vision impairment (CVI) and bilateral hearing impairment • A feeding tube was placed, a tracheostomy was also done • Previous VFSS suggested poor airway protection • Current diagnoses: cerebral palsy, childhood epilepsy, CVI, hearing impairment, chronic lung disease Figure 8–2.  Sadie’s case study timeline.

Preparing for Treatment What are some key findings from the evaluation that should be addressed in treatment? It was clear that Sadie was not swallowing often enough given the oral secretions observed. There were also concerns regarding the acute trismus. The level of trismus observed was a concern due to limited tongue use and oral hygiene. Upon further observation, it became clear that she had some abrasions intraorally on her gums due to tonic biting. Consider how to infer nonverbal communication in working with this client. What types of communication did mom infer through upper extremity or head movements? What were important treatment priorities for mom and Sadie? What are some other strategies to implement so that the client and caregiver have shared input during the treatment process? See images of Sadie during the treatment process in Figure 8–3. Establishing a Baseline for Secretion Management Begin to get a sense of suctioning intervals through open discussion with Sadie’s nurse and mom. How does this inform your treatment? It was also necessary to ascertain Sadie’s ability to tolerate use of the PMV as it was important in understanding pharyngeal potential. What kind of fluctuations of use were seen due to seizures and/or intermittent respiratory changes? Considering the information that Sadie’s mother provided regarding rejection from providers, the training and knowledge necessary to manage a medically fragile trached patient should be discussed. While

8.  ETHICAL CONSIDERATIONS   183

Figure 8–3.  Sadie during a treatment session.

equity of care was a problem, it is important that providers recognize their limitations in caring for complex patients. An active team with open interprofessional communication must be employed. Access to resources should also be guiding practice (Barnes & Toms, 2021; Jackson et al., 2018). (See Figure 8–4 for areas of concern identified through the evaluation process.)

Intervention Goals over the next few months were (a) increased volitional oral and pharyngeal movement, specifically increased swallowing and coughing, and (b) increased lingual response to multisensory stimulation (e.g., tastes, vibratory stimulation, oral motor tools). Sadie showed decreased jaw tightness and lingual movement increased. The primary obstacles were seizure activity, pulmonary health, and communication challenges due to poor motor control, as well as vision and hearing challenges. During this time, a vagal nerve stimulation device was implanted to help control seizures. Some important team members to provide best care included pulmonary, audiology (infrequent use of hearing aids as mom was concerned that they triggered seizures), and otolaryngology.

184  CASE STUDIES IN PEDIATRIC DYSPHAGIA

Oral Preparatory Phase Oral Transit Phase

Sadie’s Potential Areas of Concern Pharyngeal Phase Esophageal Phase

Figure 8–4.  Sadie’s potential areas of concern.

Progression of skills was inconsistent as tissue granulation around her tracheostomy site was becoming clear. Upper respiratory congestion was also a recurrent issue during the fall and early winter. The following goals were addressed as the treatment period ended: (a) Use switch activation in the emerging intent of request, (b) four to six visible swallows during a treatment session, and (c) decreased jaw tightness, as shown by an increased ability to provide input to tongue on two to three occasions during a treatment session. Instrumental Evaluation Through a VFSS and Airway Evaluation Sadie had a VFSS. Results suggested an unprotected airway and silent aspiration. Her ENT also performed an airway evaluation. Results are detailed in the following report. Operative Report Preoperative diagnosis:  Chronic tracheostomy, upper airway obstruction Postoperative diagnosis:  Chronic tracheostomy, upper airway obstruction Procedure:  Direct laryngoscopy with bronchoscopy with flexible fiberoptic laryngoscopy

8.  ETHICAL CONSIDERATIONS   185

Findings:  Normal vocal fold mobility, upper airway collapse with tongue base on posterior wall of oropharynx, retroflexed epiglottis. No subglottic stenosis. Complete superstomal collapse. Description of procedure: A flexible scope was passed through the right nasal cavity into the nasopharynx. The palate was completely off the back wall of the pharynx and there was no significant adenoid tissue. However, I gently pushed past the palate, and it was met by the tongue base on the posterior wall of the pharynx. We needed to perform a draw thrust to get the tongue off the back wall. This brought us to the supraglottic, where the epiglottis despite tongue thrust was retroflexed into the ball wall. I was able to get underneath the epiglottis. Sadie did not really respond to the scope in her. There was no vocalization or cries. The cords were in a lateral position. They did not move with respiration. I gently touched the supraglottis and they did come together in the midline when that occurred. The scope was removed. The patient was anesthetized and IV was started. The bed was turned 90 degrees, and shoulder roll and tooth guard were placed. A direct laryngoscope was used to examine the oral cavity, oropharynx, hypopharynx, and supraglottic and glottic larynx. There were no other changes. The larynx was a bit anterior, and her mouth did not open significantly wide to see the endolarynx well through the mouth. I sprayed the cords underneath the epiglottis with some xylocaine and placed a 3.5 ventilating bronchoscope into the supraglottis. This was gently passed through the cords into the subglottis; the subglottis was normal. There was a complete superstomal collapse. I was able to gently pass through this and remove the trach and advance the bronch. There were no other abnormalities in the airway. The bronch was removed, and the trach tube was replaced. The tooth guard was removed. A flexible scope was passed through the trach. It was a nice mid-tracheal position, not too long and not too short, and I kept it in place. The patient was returned to anesthesia, where she was to undergo the auditory brainstem response (ABR). Shift in Treatment Following this procedure, intervention was altered due to the global pandemic. It was recommended that her status be changed to NPO following the MBSS and airway evaluation. Another change that occurred was a transition to telepractice. This change was particularly challenging given that virtual learning is typically supported through increased visual information. Sadie’s cortical vision impairment helped clinicians to realize the need for maximal parent involvement and the importance of multisensory cuing through auditory and tactile input. It was also particularly important to have an enlarged, close view of Sadie during sessions to accurately assess

186  CASE STUDIES IN PEDIATRIC DYSPHAGIA

physiological stress with PMV use. Parental and nursing involvement were another vital component, as passive and active oral motor stimulation required a parent or caregiver. Fortunately, this patient’s parent was always sitting right next to her and able to support her sessions maximally. Sadie’s current medications included the following: Topamax, Keppra, clonazepam, Onfi, omeprazole, loratadine, baclofen, Vimpat, albuterol, and Pulmacourt daily and scopolamine every 3 days. Currently, her neurologists were working toward weaning her off seizure medications, and she had been using Epidiolex (cannabidiol) twice a day for managing seizures. A shared decision-making paradigm was incorporated in goal formation. Given so many medical comorbidities, including chronic lung disease, epilepsy, long-term use of a tracheostomy, and a global pandemic, Sadie’s mother and the therapy provider felt that the NPO status was important for reducing the need for hospitalization. Some questions that should be discussed in reviewing this case: 1. How often should children receive modified barium swallow studies? 2. Would water boluses be helpful for encouraging oral motor use? 3. What are the best protocols to put in place when serving medically fragile patients through telepractice? 4. How can we best support Sadie’s mother and nursing staff throughout the treatment process? As of this writing, Sadie has not been able to transition back into outpatient treatment. Some attempts have been made that resulted in overstimulation and increased seizure activity. Homebound status has been the best way to prevent hospitalization and the risk of hospitalization. See an image of Sadie at 3 years, 5 months old in Figure 8–5.

Discussion Throughout treatment, a cautious position was in place about progression from an NPO status due to various factors (e.g., erratic seizure activity, COVID-19 pandemic). Open discussions with the primary caregivers were necessary, incorporating education and shared decision-making. This case will allow the reader to consider how problems could occur if there was a lack of agreement or understanding of the modified barium swallow study findings. With pediatrics, medical statuses may change, and improved structure could lead to a more stable supportive airway for oral feeding.

8.  ETHICAL CONSIDERATIONS   187

Figure 8–5.  Sadie — 3 years, 5 months old.

In Sadie’s case, decannulation was not a possibility given her structural and neuromuscular involvement. Epilepsy was another factor that made progression difficult as seizure activity was not significantly decreased through medication and seizures were at times difficult to detect due to limited changes in affect and eye movements/appearance during seizures. Some questions to consider or discuss are as follows: 1. What are some ways to educate parents about modified barium swallow study results? 2. What is the best way to manage a case when there is a lack of agreement about care? 3. What are some factors that would need to improve for a progression from NPO to oral status? Additional topics such as risk feeding and palliative care were beyond the scope of this case. These topics have been primarily explored in the adult realm of treatment but are emerging as topics of discussion and future research in pediatric dysphagia (Radford, 2020; Smith, 2020). Find resources for sharpening skills in ethics in Figure 8–6.

188  CASE STUDIES IN PEDIATRIC DYSPHAGIA Sharpening Clinical Skills in Ethics Everyday ethics topics from ASHA Leader Blog Series https://www.asha.org/practice/ethics/everyday-ethics/ What Happens When Someone Files an Ethics Complaint? Answers to common member questions about the ASHA Board of Ethics and its adjudication and disciplinary procedures. When Should I Self-Report? The ASHA Code of Ethics and the Assistants Code of Conduct both have self-reporting requirements — here are details as to exactly when you need to self-report. Prohibitions Against Discrimination Under ASHA’s Code of Ethics Hypothetical scenarios explore discrimination claims under the Code of Ethics and how the ASHA Board of Ethics might evaluate them. Pitfalls to Watch for When Using Electronic Health Records Ethical issues can arise when navigating the use of EHRs in the delivery of hearing and speech-language services. Holding paramount the welfare of your clients/patients should be the guide for examining these ethical considerations. Top 10 Ethical Considerations in Using Telepractice Here are the top 10 ethical issues to consider before engaging in telepractice. Figure 8–6.  Sharpening clinical skills in ethics.

DR. MEMORIE GOSA INTERVIEW Memorie M. Gosa, PhD, CCC-SLP, BCS-S, is a pediatric speech-language pathologist, board-certified specialist in swallowing and swallowing disorders, and associate professor and chair of the Communicative Disorders Department at The University of Alabama. Her primary areas of research interest are the effects and use of thickened fluids in pediatric populations, intrinsic properties of thickened fluids, development of feeding and swallowing skills in infants and children, and diagnosis and management of dysphagia in pediatric populations.

Jennifer Wilson ( J):  What are some things that you learned through editing the text Assessing and Treating Dysphagia: A Life­span Perspective (Suiter & Gosa, 2019) regarding ethical management of dysphagia?

8.  ETHICAL CONSIDERATIONS   189

Memorie M. Gosa (M):  What came to mind with this question is the depth and breadth of our field. Dysphagia management is one small area that we work in. Then you broaden it out and you talk about managing and working with people that have dysphagia across the whole life span and consider all the different professionals and colleagues that were brought into this process. So of course, there’s the speech pathologists. But there’s also physicians, dieticians, social work, etc. The whole gamut of professional colleagues that are involved in this area of practice. When we begin to think about the ethical implications of that, we’re thinking across all these different disciplines that have different ethical codes that have different ethical standards, written out like ASHA’s (American Speech-Language-Hearing Association) Code of Ethics. Each profession has its own. With all those different standards, what it boils down to is putting our patient at the center of our care. We should take ourselves out of it, which can be challenging, taking our ego out of it. So, it’s not about my expertise. It’s not about what I think is best for this person. It really boils down to putting our patients, or our clients, at the center of the care. It’s not about what we want, it is about what they want. We can support autonomy, making sure that what we’re doing is not causing harm. We need to consider the evidence-based practice part of it, making sure that what we’re recommending and what we are providing for our patients is going to help them in the long run. In editing the book, bringing in all the different collaborators from all the different professions I think helped to center me in on the wide perspective that goes with helping people that have feeding and swallowing issues and the importance of putting them and what is important to them at the center of what we’re doing. J:  Great, that’s helpful to begin framing the complexity of the situation. What are some considerations for young professionals entering the workforce beginning to provide services in pediatric dysphagia? I was interviewing a young SLP recently, and she made pointed out that many of the students who are getting out there and starting their clinical fellowship year, that began working in private practices, are going to be working with the pediatric dysphagia population. That gets me excited about the fact that we really need to be doing the best we can with preparation for them at the university level. M:  A couple of thoughts. The young professionals in the environment that we work in now are more connected than we ever were. Twenty years ago, I went to my master’s program. There were eight other students in that program. It was small. We had the support of

190  CASE STUDIES IN PEDIATRIC DYSPHAGIA

our professors and our clinical educators at that time and then we graduated. We were not connected, unless we happened to run into each other, or I could call them on the phone. But I did not have daily interactions with the cohort that I had gone to school with in my graduate program. I didn’t have daily interactions with my professors or clinical educators. I went into a clinical fellowship year. I had my CFY supervisor, but she wasn’t there every day. It wasn’t the same kind of support system. Thinking about trying to find evidence or support, we were really limited to what was available in literature that we couldn’t always access. At that time. I could get the ASHA journals. With my ASHA dues I was able to access those, but in terms of having access to other disciplines’ data and those kinds of things, we just really didn’t. Flash forward 20 years and now we are so connected with social media and with web resources like ASHA’s practice portal and all these things that we can now access on a regular basis. I think one consideration there is that you have access to all kinds of information now. Not all the information is created the same way. Not all of it is vetted the same way, so a consideration I think for our young professionals where I didn’t have access to information and that was my struggle is that now you have information coming at you from all these different sources and it’s really up to our young professionals to consider and continue using those critical thinking skills that hopefully they honed in on in graduate school to help vet the information that they’re getting, because it’s not always peer reviewed. It has not always gone through a lot of vetting channels in some cases yet it’s freely available. It is great that we are connected and can have mentors across the country that are practicing in the specialized areas like pediatric dysphagia. I think it’s important that we continue to use those critical thinking skills that were learned in graduate school, that we continue to utilize those as we process the “fire hydrant,” full of information that’s coming at you all the time. That’s one thing. The other thing that I think is so important specific to pediatric dysphagia and dysphagia in general is that we can’t cut off our other skill sets in this area. Behavior is communication, especially when you’re working with infants and children. We are communication specialists. I think that’s what makes our field so unique is that we’re not just “swallow people.” We’re not just “feeding people.” The field of speech-language pathology is so broad and so encompassing. Our skills in communication really help us to be good feeding and swallowing specialists. We can’t cut that part off especially when you’re working with infants and children that don’t always have the most sophisticated communication skills. It’s up to us to use our knowledge of communication to help interpret what we’re

8.  ETHICAL CONSIDERATIONS   191

seeing in terms of those behavioral cues and respond appropriately. We must watch our assumptions about things. So often things are prescribed too, “Well that’s just a behavioral issue. That’s something that we need to break.” Maybe not. They’re communicating to us that something is not right, and it is up to us to figure that out. Then, to plan our intervention in a way that helps to alleviate the discomfort or the struggle that the child was experiencing. They communicate with us sometimes through their behavior. J:  What a great call for continuing education and lifelong learning. That’s a very helpful response. What do we need to be doing through training and research to improve and grow in ethical management of pediatric dysphagia? That’s a broad question. M:  Yeah, it’s a big question, because it’s a big job. I really appreciate the changes I’ve seen over the last 20 years. When I first started going to Dysphagia Research Society meetings, there were a handful of people there that studied pediatric-related issues, maybe like, five or six people that came on a regular basis. This small group was most interested in learning about advancing science in pediatrics. And over the last 20 years, that has really grown, and I see more people that are practicing in this area. With that, you have more questions that come up and there’s more opportunities for study. So, I think that is a good thing. In terms of training and research, we need more PhDs in this area, so we need more people that want to learn how to put good information into the world. That’s the thing that a PhD does. It teaches you how to create knowledge. In undergrad, you are learning all this information that other people have established. You’re soaking all that in. Graduate school is where we learn how to apply that information to very specific populations and disorders. The PhD is the next skill set where you then learn how to create knowledge and to put new knowledge out into the world. I really hope that there are clinicians that want to take that next step, to build that skill set. Our universities are desperate for more PhDs to fill the needs of our programs. We have highly qualified students all the time that are being turned away from graduate programs because there’s just not room for them. Our field is enjoying popularity. We are in the US News and World Report as a top 10 career (Bratcher, 2022). Those are all good things, but the training pipeline gets clogged because we just don’t have enough programs and resources in some cases to take in all the students that want to pursue speech pathology. I do hope that there are students that want to take that next step. In terms of improving our ethical management of families and children with pediatric dysphagia. I think the opportunity there is to

192  CASE STUDIES IN PEDIATRIC DYSPHAGIA

better understand what is typical, what is normal development across cultures. Food is a big part of someone’s cultural identity, and I think about when I had my own children and how that changed the way that I approached pediatric dysphagia. I didn’t have children when I first started in this area. Oftentimes we would do videofluoroscopy, or we would do the clinical swallow exam and we would make recommendations about changing something regarding the feeding situation. I never considered the impact on the mother and child and how stressful early infancy can be, especially for new moms. To say I never considered it is not true. I didn’t have the personal experience to understand really what that meant when I would say something like, “I think we need to do thickener at this time.” If mom was nursing, that meant then she had to pump instead of putting baby to breast. Consider the impact that was going to have on the motherchild relationship. I think learning more about what are culturally appropriate feeding practices across different populations will help us provide more culturally responsive care in this area. We have very limited information from observational studies about what is normal development. Primarily, those observational studies were done here in the United States. They were done with middle-class White families, and we work with more than that. To get a broader sense of what is typical development because development is going to be influenced by what foods the child is exposed to at home and all of that is wrapped up in cultural identity. I feel like that’s a big area for us to learn more. And that’s not just going to be speech pathologists in that area, like we’re going to depend on anthropologists and other disciplines to help us understand the impact of our recommendations across different cultural beliefs. J:  Yeah, I agree. That’s really been confounding to me through the years. And I’ve had lots of a-ha moments where I’ve realized that I need to think about my own cultural identity. Appreciating differences and integrating that into care can be really complicated. Those are some great points. What kind of core resources would you recommend to both more seasoned and young therapists who are specializing in pediatric feeding and swallowing to make sure that they are entering into their cases with an ethical mindset? M:  Begin by understanding the tenets of what ethics means. Those three core tenets that we talked about earlier, autonomy, nonmaleficence, and beneficence, so not doing harm, making sure we’re doing good, and providing for the autonomy of the person or the family that we’re serving. Understanding those principles is important.

8.  ETHICAL CONSIDERATIONS   193

The other piece that I think is so helpful is self-reflection. That’s a big part of ethical management and interventions. In culturally responsive care, we should make time and make space for self-reflection. When we’re recommending things, we should try to consider, why is it that a family is not following through with what we said? As a young clinician, not taking on perspectives of other people, I would jump to conclusions, thinking, “Oh well, they just didn’t do it.” There’s a reason they didn’t do it. Getting to the “why” is so important in terms of resources. Paula Leslie is a speech pathologist who writes a lot about ethical management of dysphagia. It’s usually couched in adult scenarios, but the information is relevant, across the life span. She is someone that I have learned a whole lot from in ethics. She’s written for SIG 13 and the ASHA journals, to name a few places that I have seen her work (Askren & Leslie, 2019; Leslie et al., 2021). I always find it meaningful the way that she presents the information and helps us get to that space for self-reflection. What did it mean? There was a framework that I ran across and I was getting ready for this, and it was proposed by Belinda Kenny. It was in a paper called “Food Culture, Preferences and Ethics in Dysphagia Management” (Kenny, 2015). She talks about this framework for shared decision-making. In the United States, we’ve talked about patient- and family-centered care. This shared decision-making framework is just a way to kind of focus us back on that concept of putting the family or the client themselves in the adult world at the center of those decisions. We’re not the food police. In early practice, I felt like that’s what we did. I did some work at HealthSouth as an early clinician, and they had this thing called rehab dining. That’s what I felt like my job was, to walk around and be like, “We can’t have that, and we have to put the thickener in your coffee.” If you have not ever tried to put thickener in an older person’s coffee and had them react back to you, I don’t think you can fully appreciate how insulting it is to people when we start messing with their food. But that’s not our job, right? We’re not the food police. Even though that’s what I felt like I was doing, that really wasn’t at the core of what I was supposed to be doing. In the shared decision-making framework, education is the first step, like showing, explaining, making sure that the patient understands what is at the root of the difficulty, the swallowing difficulty, the feeding difficulty, because dysphagia is not a standalone diagnosis. No one catches dysphagia. It is a symptom. It is an associated comorbidity of another problem. In infancy particularly, it’s often associated with other congenital issues or prematurity. Parents and caregivers need to understand what dysphagia is. How did it happen? How likely is

194  CASE STUDIES IN PEDIATRIC DYSPHAGIA

it to resolve depending on the state of disease or the process that the person is going through? That is the first part, the education piece. Then, understanding that that situation is dynamic. What they’re doing while they’re an inpatient may be very different than what they’re willing and able to do in an outpatient setting. It’s one scenario where they’re in the hospital and we’re bringing all the stuff to the bedside. They’re able to consume it without necessarily having to find the thickener or find the bottle system that we’ve recommended or have multiple systems ready to go so that they can feed throughout the day when they get home. The next part of that shared decision-making framework is this idea of repeat education. Understanding that things are dynamic, that they are going to change what the family or the older child agreed to do when they’re in an inpatient setting may be vastly different than what they’re willing to do in an outpatient setting. I think about our older kids and when they go back to school and in the lunchroom with all the things in front of them, then having to eat or do something that is wildly different than their friends and their peers and how that impacts their willingness to do those things. We should understand that there might have to be repeat education and that the person is allowed to change their mind, right? Like. The environment they’re in is going to influence what they are willing and able to do. That repeat education piece should be a part of treatment, particularly during transitions. The other piece that the last piece of that shared decision-making framework then is this idea of team meeting and family meeting. The team meeting to get the medical team on the same page to have this opportunity for reflection about the following: What is safe? What is appropriate? And then also, what is safe and appropriate for the client’s sociocultural well-being? It’s not just about what is safe for their body, their lungs, their physical well-being. We must consider that sociocultural piece too because feeding swallowing involves the food that we’re talking about, that we’re changing, that we’re asking them to do something different and that gets into their sociocultural identity. We must be willing to have that conversation with our team and then meet with the family to provide information about risks and benefits and let them decide what is going to work best for them with all the information. So not just the scare pieces. Like, they could develop pneumonia and come back in the hospital, but the full picture, the chances of them developing pneumonia are this in this population, in this setting. With a complete picture giving them that information and then letting the family be part of that care team to make the final decision about what’s best for them and their child.

8.  ETHICAL CONSIDERATIONS   195

J:  I was thinking about the fact that we live in such an instant gratification culture. Also, this kind of therapeutic intervention is dynamic because feeding and swallowing development in a normal sense can happen so quickly and can change so rapidly when you’re working with a child. I love the way you hit on the fact that this kind of intervention is so personal. It’s so attached to a parent or caregiver and who they are, and it’s also so attached to culture and the way we express ourselves is so through feeding others and the types of food that we eat, that there’s a lot of emotion embedded in this type of intervention. And the more you start unpacking that and unfolding it, yeah, it really makes you realize, well, there’s a lot of weight too, to doing this kind of intervention. No wonder it’s so challenging. Yeah. M:  You make a great point too, about just how quickly things change in that first year of life. Any time we’re intervening with an infant, we can’t wait 6 months to see them again. And that’s so often the recommendation, “We’re going to do this, and then we’ll see you again in 6 months?” They’re a completely different being and they’ve missed out on a large percentage of learning opportunities when we start restricting and don’t follow through with providing updated recommendations as the needs change, J:  A few of these interviews have brought to light, especially that aerodigestive chapter, how wonderful it is that there are these comprehensive centers that kids can go to who are medically complex and how cost-effective it is and how meaningful it can be to families, especially in rural areas. But like you just said, even with that happening, it all goes back to communication and the fact that, for that family really to get the kind of intervention they need, they really must have a local provider who’s communicating well with the comprehensive clinics. M:  Yeah, that was something that I experienced after I moved here because I’d always been part of a large pediatric hospital that had all the subspecialties on site and people would come there and you could just ask questions and do all the things in one day. And then I  moved. Tuscaloosa is not rural, but there’s 100,000 people here versus Memphis, where there’s a million plus people in the community. In Tuscaloosa, the nearest children’s hospital is an hour away and a lot of the families and the people that I have encountered here can’t get to Birmingham at the drop of the hat. It also takes all day, so then we’re talking about them missing work, their child missing school or preschool or their regular routine to go to those appointments. They are fantastic when we can get everyone together in the same room

196  CASE STUDIES IN PEDIATRIC DYSPHAGIA

and have that level of care. But then the drawback to that is it’s out of the child’s natural environment. It takes all day. Oftentimes, it requires resources that are beyond some families’ reach, to be able to, to drop a day’s work and go and do those things. Having local providers that can bridge the relationship between the two, between the family and the comprehensive care facility, is important.

REFERENCES Askren, A., & Leslie, P. (2019). Complexity of clinical decision making: Consent, capacity, and ethics. Seminars in Speech and Language, 40(3), 162–169. https://doi.org/10.1055/s-0039-1688838 Ball, K. A., & Riquelme, L. F. (2016). The graduate dysphagia course: Opening the door to new frontiers. Perspectives of the ASHA Special Interest Groups, 1(13), 104–112. https://doi.org/10.1044/persp1.SIG13.104 Barnes, G., & Toms, N. (2021). An overview of tracheostomy tubes and mechanical ventilation management for the speech-language pathologist. Perspectives of the ASHA Special Interest Groups, 6(4), 885–896. https://doi​ .org/10.1044/2021_PERSP-20-00105 Brady, S. L., Wiliams, L., Hakel, M., & Pape, T. (2016). Developing the talents of future dysphagia experts: A conceptual framework. Perspectives of the ASHA Special Interest Groups, 1(13), 97–103. https://doi.org/10.1044/ persp1.SIG13.97 Bratcher, E. H. (2022). Speech-language pathologist overview. U.S. News and World Report. https://money.usnews.com/careers/best-jobs/speechlanguage-pathologist. Hazelwood, R., & Pollack, L. M. (2021). Critical review of online resources frequently used by certified speech-language pathologists for dysphagia management. Perspectives of the ASHA Special Interest Groups, 6(6), 1618– 1626. https://doi.org/10.1044/2021_PERSP-21-00106 Jackson, A. J., Harendt, S. E., & Baker, C. D. (2018). A patient- and familycentered model of feeding and swallowing management for children with tracheostomies. Perspectives of the ASHA Special Interest Groups, 3(13), 101–112. https://doi.org/10.1044/persp3.SIG13.101 Kenny, B. (2015). Food culture, preferences and ethics in dysphagia management. Bioethics, 29(9), 646–652. https://doi.org/10.1111/bioe.12189 Leslie, P., Xia, B., & Yoo, J. (2021). It’s not such a small world after all: The intersection of food, identity, and the speech-language pathologist. ASHA Perspectives SIG 13 Swallowing and Swallowing Disorders, 6, 876–884. https://doi.org/10.1044/2021_PERSP-20-00276

8.  ETHICAL CONSIDERATIONS   197

Radford, C., Marshall, J., Herbert, A., Irving, H., & Weir, K. (2020). Risk feeding: An Australian pediatric palliative care perspective. Perspectives of the ASHA Special Interest Groups, 5(2), 515–521. https://doi.org/10.1044/​ 2020_PERSP-19-00032 Smith, P. A. (2020). Palliative care in dysphagia and dementia. Perspectives of the ASHA Special Interest Groups, 5(2), 506–510. https://doi.org/​10​.10​ 44/2020_PERSP-19-00038 Suiter, D. M., & Gosa, M. M. (2019). Assessing and treating dysphagia: A life­ span perspective. Thieme. Tilley, E., Strnadová, I., Danker, J., Walmsley, J., & Loblinzk, J. (2020). The impact of self-advocacy organizations on the subjective well-being of people with intellectual disabilities: A systematic review of the literature. Journal of Applied Research in Intellectual Disabilities: JARID, 33(6), 1151–1165. https://doi.org/10.1111/jar.12752 Wilson, J. J., Simmons, A. K., & McCarthy, J. H. (2020). Pediatric dysphagia: Survey results describing speech-language pathologists’ education and experience. Perspectives of the ASHA Special Interest Groups, 5(1), 236–245. https://doi.org/10.1044/2019_PERSP-19-00016

Conclusion Compiling these case studies and completing the expert interviews has provided me with professional growth that I would never have imagined. As I near my 30th year of professional practice, I am amazed by the way my work in the field of speech-language pathology has changed and expanded over the years. Pediatric dysphagia is a branch that I began to work in straight out of graduate school during my Clinical Fellowship Year. I sure did have a lot to learn! What I didn’t know then, and have become increasing aware of, is the importance of multidisciplinary teams. We have so much to learn from each other. Parents and caregivers should be key team members. Their voices should be heard in every part of the process, from when goals are created, home programming is put in place, or as medical concerns arise. Parents and caregivers know the child so much better than the professionals serving them know the child. Home programming must be incorporated into every session. Hopefully, through the messaging thread and retrospective interviews, the reader can gain a sense of how the parents and caregivers can be key players. I want to express a huge thanks to all of these families and clients for their transparency in sharing their stories. The case studies represent how cases were managed, which was sometimes complicated by external challenges (e.g., rural setting, global pandemic). When reading through cases, the reader is encouraged to contemplate different aspects of case management from goal direction to additional referrals to optimal instrumental measures. Management can always be improved as more resources are available. In conclusion, I firmly believe that we all learn best through storytelling. I hope these real-life stories can educate others to grow and learn in a branch of my profession that I have grown to love. I have found that every case in pediatric dysphagia is unique. We can all grow and learn through sharing our own journeys.

199

Index Note:  Page numbers in bold reference non-text material.

A Academy of Nutrition and Dietetics, 98 Acute care, 24, 58, 59, 74 126, 127, 152 ADD, 113 Adenoid, 71, 87, 185 hypertrophy, 60 ADHD, 49 Aerodigestive, 57, 58, 60, 61, 68, 70, 195 Ages and Stages Questionnaire, 52 Air pollution, 52 Alshawabkeh, Akram 51 Albuterol, 87 Alfamino, 59 Allergies, 16, 64, 81, 86, 87, 96, 107 Altricial, 46 American Journal of Speech Language Pathology — Perspectives, 50 American Speech Language Hearing Association, 9, 53, 58, 76, 188, 189, 190, 193 Amniotic fluid, 135 Amplification, 47 Anterior loss, 7, 60, 71 Anterior spillage, 59 Anterior sulcus, 59 Anterior-posterior lingual movement, 59 Antibiotics, 85 Anxiety, 91, 107, 112, 124, 129 APGAR score, 4 Appetite stimulant, 85, 87 Apraxia, 107

ASD, 128 ASHA Special Interest Group (SIG), 13 swallowing and swallowing disorders, 76 Aspiration, 21, 22, 24, 25, 30, 31, 32, 46, 58, 59, 60, 64, 68, 69, 70, 71, 72, 73, 106, 108, 118, 124, 126, 139, 143, 155, 169, 170, 184 ASQ, 52 Augmentin, 64 Autism, 28, 49, 52, 80, 85, 108, 112, 116 Aversion, 85, 140 Avoidant, 121 Azithromycin, 87

B Bacterial infections, 87 Base of tongue, 59, 60, 68 Battelle Developmental Inventory, 52 Bilateral grade 3 brain bleeds, 137 Binary expression profiles, 46 Blood sugar, 36 Bolus containment, 31 Bolus formation/movement, 60 Bolus organization, 5, 109 Bolus transit, 31 Brackett, Krisi, 34 Brain bleed, 106, 108 Brain integrity, 52 Breastfeeding, 4, 9, 10, 16, 21, 24, 75, 80, 92–101, 102, 192 Breastfeeding Peer Counselor Program, 99

201

202  CASE STUDIES IN PEDIATRIC DYSPHAGIA Broca’s area, 44 Brodmann’s area, 44 Bronchopulmonary dysplasia, 4, 107, 108

C CADET, 70 Cardiac, 45, 70, 73, 74, 75, 76 cardiac arrest 162 cardiac defect, 74 cardiac Feeding Protocol, 70 cardiac surgery, 74, 75 cardiac unit, 58 Cardiologist, 74 Cardiology, 39, 70, 72 Caregiver interruptions, 135 Casein, 87, 102 CASL-2, 115 Cat nap, 46 CBCL, 52 C-Diff, 107 Cell growth, 43 Central nervous system integrity, 52 Cerebral palsy, 58, 62, 65, 107, 108, 140, 180, 182 Cerebral spinal fluid, 135 CF, 80, 82, 83, 86, 87, 88, 91, 92 CFY, xi, 76, 190 Chapman, Denise 58, 69–76 Chemical toxins, 51 Chewy tubes, 65 Chiari malformation, 105, 107, 108 Child and maternal health, 92 Child Behavior Checklist, 52 Child environmental health centers, 53 child environmental health center program, 52 Child psychologist, 107 Chromosome 16 duplication, 29, 31 Chromosome X duplication, 29, 31 Chronic lung disease, 31, 137, 139, 186 Civil engineer, 51 Clinical fellowship year, xi, 76, 190 Clostridium difficile, 107 CNTNAP2, 46 Coaching, 90, 117, 124

Cognitive/behavioral approaches, 109 Colostomy bag, 86, 88 Colostrum, 93, 94 Community nutrition, 80, 92, 98 Compleat Pediatric Organic blends, 36 Complex Aerodigestive Evaluation Team, 70 Comprehensive Assessment of Spoken Language, 2, 115 Computed tomography, 137 Contamination threats, 51 Cortisol, 36 Cough response, 60 COVID-19, 142, 143, 145, 149, 151 Crackles, 142, 143, 149, 150 CRECER, 52 Cricopharyngeal dysfunction, 60 CT, 137 Cyproheptadine, 87 Cystic fibrosis, 80, 82, 83, 86, 87, 88, 91, 92

D Database of genomic variants, 50 Delayed swallow, 60, 170 Deoxyribonucleic acid, 43 Desensitize, 122, 123, 126 Diarrhea, 63 Dietician, 8, 36, 80, 86, 87, 92, 98, 99, 100, 189 Differentiation, 43 Digestive motility, 16 Dissolvable solids, 36 DNA, 43 Down syndrome, 27, 49 Dr. Brown’s, 25, 101 Drooling, 5, 59, 64, 109 Drug exposure, 12 Dysphagia Research Society, 1, 2, 191

E Ear infection, 37 Early intervention, xiii, 40, 41, 52, 53, 80, 124, 126, 140 Early interventionist, 8 ECHO, 53

INDEX   203

EEG, 137 EGD, 12 EI, xiii, 40, 41, 52, 53, 80, 124, 126, 140 Elecare formula, 30 Electroencephalogram, 137 Elopement, 12 Emesis, 11, 12, 81, 83, 84 Endocrinologist, 36 Endocrinology, 39 Endoscopy, 90, 111 ENT, xi, 180, 184 Environmental exposures, 52, 53 Environmental health, 51, 52, Environmental Influences on Child Health Program, 53 Environmental toxicologist, 53 Enzyme medications, 81 Enzymes, 86, 90 Esophageal motility disorders, 63 Esophagogastroduodenoscopy, 12 Esophagram, 73 Evidence-based, 2, 22, 58, 80, 179, 189 Expectorated, 59 Extrinsic muscles, 68 Extubated, 74

F Facial grimacing, 83, 88, 140 Failure to thrive, 71, 80, 82, 86, 88, 91, 93, 107, 108 Family mealtime coaching, 117 Fawning, 105, 120 Febrile seizure, 108, 111 Feeding Matters, ix,1, 2 Feeding tube, 34, 39, 60, 81, 117, 125, 130, 141, 142, 144, 180, 182 FEES, 33, 62, 74 Fetal demise, 106, 108 Fight or flight response, 120 Flexible Endoscopic Evaluation of Swallowing, 33, 62, 74 Flovent, 64, 139 Fluoroscopy time, 60 Food blends, 36, 86, 142 Food chaining, 11 Food refusal, 8, 11, 14, 32, 79

Food refusal behaviors, 8, 11, 12, 14, 79 Food repertoire, 80, 82, 83, 87, 91, 116 Food sensitivity, 16 Food textural differences, 110 Forkhead box protein P2, 28, 42, 43, 44, 45, 46, 47 Formula, 4, 11, 13, 14, 17, 30, 35, 71, 79, 81, 83, 87, 94, 98, 99, 100, 101, 102, 111, 140, 142, 143 FOXP2, 28, 42, 43, 44, 45, 46, 47 FOXP2 deletion, 45 Fragile x, 48, 49 Freeze, 120 Fundoplication, 32, 33, 34, 35, 36, 142 FXS, 48, 49

G Gag, 13, 30, 36, 37, 71, 72, 74, 82, 83, 84, 85, 87, 90, 144 Gastric motility, 12 Gastroenterologist, 8, 12, 15, 30, 64, 142 Gastroenterology, 8, 12, 30 Gastroesophageal medication, 15 Gastroesophageal reflux, 5, 6, 12, 79, 107, 139 Gastrointestinal, xi, 12, 14, 32 gastrointestinal function, 79, 137 gastrointestinal issues, 2, 79, 80, 96, 105 Gastrostomy tube, 40, 45 gastrostomy tube placement, 45 Gene expression, 28, 42, 44, 45, 46, 47, 48, 49, 54 Genetic deviation, 50 Genetic disorder, 28 Genetic specialist, 50 Genetic testing, 45, 50 GI, xi GJ-tube, 29 Glutamate receptor ionotropic N-methyl-D-aspirate 2A, 46 Gosa, Memorie, 179, 188–196 Grazing, 14, 91, 111 GRIN2A, 46

204  CASE STUDIES IN PEDIATRIC DYSPHAGIA Gross motor skills, 37, 63, 173 Growth trajectories, 41 G-tube, 29, 30, 31, 38, 40, 62, 64, 65, 66, 68, 69, 73, 74, 81, 82, 137, 142, 143, 162, 165 Gulping, 71 Gut microbiome, 79, 92 Gut microbiota, 79

Intraventricular hemorrhage, 135 Intubation, 73

H

K

Head circumference, 36 Health equity, 3, 92, 99 Heart disease, 118 Heterogeneity, 48 High-resolution pharyngeal manometry, 63 Home health, 30, 39, 41, 126, 137, 138, 144 Homozygous mutations, 43 Honey-thick liquid, 60 Human milk, 21, 102 Hydrocephalus, 137, 139, 162, 164 Hyperreactions, 122 Hypersensitivity, 82, 83, 84, 122, 140 Hypopharynx, 60, 61, 168, 185 Hypospadias, 29, 31 Hypothyroidism, 29, 137, 139

KE family, 42 Knockdown studies, 43 Knockout, 43

I

Magnetic resonance imaging, 68, 137, 147 Mandibular distraction, 62, 64, 68 Maron, Jan, 28, 41, 42, 44, 47, 50, 51, 54 Maroon spoon, 65 Maternal and Child Health, 92 Maternal drug use, 82, 137 Maternal psychosocial factors, 41 Maternal urine, 51 Mcgrattan, Katlyn, 3, 16–25 MCH, 92 Medically complex, 23, 73, 124, 195 Medically fragile, 17, 142, 143, 144, 145, 147, 152, 182 Medication usage, 79 Melatonin, 112 Mentor, 24, 75, 76, 175, 190 Metabolic function, 79

IBCLC, 92 Immune function, 36, 79 Immunology, 39 Inflammation, 32 Inguinal hernia, 29, 31, 107, 108 Inhaler, 63, 64 Intensivist, 74 Intrauterine space, 135 Interdisciplinary, 6, 15, 21, 28, 36, 38, 57, 72, 76, 83, 87, 106 Intermittent reflux, 11 International Board Certified Lactation Consultant, 92 Intestinal lining, 79 Intolerance, 73 Intraoral space, 68 Intrauterine growth restriction, 29, 31

J Jaw distraction, 59 Jaw micrognathia, 58 Jejunum, 74

L Lactation consultant, 80, 92, 94, 95, 97, 98, 99 Lactation training, 98 Laryngeal penetration, 30, 60 Latch, 10, 30, 140 Limited repertoire of foods, 109 Lingual movement, 60, 183 Liquid loss, 4, 5, 65 Lower esophageal sphincter, 142

M

INDEX   205

Metabolic processing, 101 Methamphetamines, 81 Mice, 43 Microarray analysis, 45 Microbial diversity, 79 Microbiome, 79, 80 Micrognathia, 58, 66 Milk protein, 87 Mode of delivery, 79 Moreland, Heidi Liefer 106, 117–128 Motor learning, 135, 161, 172 Motor planning, 38, 45, 109, 162, 172, 175 MRI, 68, 137, 147 Mucus, 32, 81, 87, 165 Multisensory, 34, 66, 67, 88, 109, 180, 183, Mutated, 44 Mutation, 43, 45, 48, 50

N NAS, 5, 15, 80 Nasogastric (NG) feeding tube, 40, 81, 82 National Child Traumatic Stress Network, 119, 126 National Hospitalist Organization, 72 National Institutes of Health, 53 Negative meal cycle, 129 Neglect, 80, 81, 86, 88 Neocate splash, 82, 87 Neonatal abstinence syndrome, 5, 15, 80 Neonatal saliva, 45, 46 Neonatologist, 28, 45 Neurexin, 1, 46 Neurobehaviorist, 85 Neurodevelopment, 41, 42 neurodevelopmental tests, 52 Neurologist, 64, 140, 186 Neurology, 30 Neuromuscular issues, 80, 105, 135, 137 NIH, 53 Nil per os, 36, 59, 65, 68, 74, 185, 186, 187 Nondysmorphic, 45

Non-nutritive suck, 52, 140 Normal infant feeding behavior, 16 Northeastern, 40, 41, 42 NPO, 36, 59, 65, 68, 74, 185, 186, 187 Nuk brush, 122 Nurse, 38, 39, 62, 65, 68, 74, 112, 116, 118, 154, 156,158, 165, 182 Nurse practitioner, 39, 74 Nutrient deficiencies, 93

O OBs, 98 Obstetricians, 98 Occupational therapist, 8, 127, 147, 171 Occupational therapy, 30, 36, 63, 107, 176 Olfaction, 147 Omeprazole, 64, 139, 186 Open heart surgery, 109 Oral care, 60, 170 Oral containment, 6, 11, 65, 88 Oral control, 60 Oral motor, 4, 36, 173, 176 Oral motor behaviors, 41 Oral motor exercises, 173 Oral motor function, 174 Oral motor movements, 10, 41, 42 Oral motor stimulation, 140, 186 Oral motor tools, 183 Oral organization/propulsion, 111 Oral phase dysphagia, 109 Oral preparation, 80 Oral propulsion, 14 Oral sequencing, 116 Oral sequencing/propulsion, 88 Oral stimulation, 60 Oral strength and coordination, 57 Oral suctioning, 65, 68 Oral transit time, 60, 68 Organization/propulsion, 37, 109, 111 Orkambi, 87 Orofacial motor impairment, 43 Orofacial movements, 43 Oropharyngeal dysphagia, 60, 109, 139

206  CASE STUDIES IN PEDIATRIC DYSPHAGIA Outpatient setting, 23, 58, 70, 72, 126, 139, 147, 161, 194 Outpatient therapy, 6, 106,126

P Paced bottle feeding, 97, 100 Palatal movement, 60 Pancreatic insufficiency, 81, 88 Pandemic, 142–144, 180, 185–186, 199 Passive leak, 60 Patent ductus arteriosus, 106, 137 Patent foreman ovale, 31 Pathogenic organisms, 79 PCR, 44 PDA, 106, 137 Pediasure, 35, 90, 108, 116 Pediatric Eating Assessment Tool, 10 Pediatric feeding disorder, 1, 2, 6, 8, 15 Pediatric intensive care unit, 128 Pediatrician, 6, 8, 51, 52, 53, 92, 93, 94, 96, 98, 99, 131 Penetration, 29, 30, 31, 32, 60, 69, 170 Perceived trauma, 118 Pesticide, 53 PFO, 31 pH probe, 32 Pharyngeal physiology, 16 Phenotype, 28, 43, 48, 50 Phenylketonuria, 96 Phthalates, 51, 52 Physiatrist, 64, 162 Physiatry, 39 Physical therapy, 30, 33, 107, 138 Physiological distress, 6, 65, 67, 88, 116, 168 Physiology, 41, 42, 62 PICU, 128 PKU, 96 Placental abruption, 62, 139 Plastics, 51, 53, 122 Pneumonia, 38, 70, 71, 141, 144, 149, 150, 194 PO (Per Os), 44, 50 PO tastes, 60 PO trials/tastes, 59, 60 Pocketed, 90

Polymerase chain reaction test, 44 Poor prenatal care, 137 Positioning, 34, 140, 151 Postnatal, 80 Post-traumatic stress, 121, 128 Potentially traumatic event, 105 Pragmatic language skills, 115 Pragmatic Language Test, 115 Premature, 15, 80, 135 Premature spillage, 60 Prenatal, 4, 52, 80, 137 Pressure equalization tubes, 62 Pressure transducer system, 52 Preterm infants, 41, 46, 51 Prevacid, 30, 83 PRISM III score, 128 Proliferation, 43 Propulsion, 5, 6, 11, 37, 65, 80, 88, 109, 110, 111, 116, 140 Proton pump inhibitors, 79 Protrusion, 64, 87, 164 Proventil, 64 Prozac, 107 PTE, 105 PTSD, 121, 128 Puerto Rico, 51 Pulmonary, 32 pulmonary comorbidities, 105 pulmonary diagnoses, 143 pulmonary sequelae, 38 Pulmonologist, 30, 32, 60, 64, 68, 86, 112, 141, 143 Pulmonology, 30, 39 Puree, 14, 15, 36, 37, 59, 60, 68, 82, 83, 123, 140 Pyloric stenosis, 108, 111 Pylorus, 111 Pyriform sinuses, 60, 169

R Raspy, 31,175 RD, 99–100, 189 Real food blends, 86, 142 Real foods, 99 Reference genes, 44 Reflux, 5, 6, 11–12, 18, 30, 32, 38, 63, 65, 66, 70–73, 79, 101, 107, 139

INDEX   207

Registered dietician, 98–100, 189 Regulation, 67 Resilient, 106, 118, 125, 137 Respiration, 9, 16, 60, 185 Respiratory distress, 63, 147 Respiratory therapist, 137, 138, 145–158 Responsive feeding, 117 Reverse transcriptome quantitative polymerase chain reaction, 44 Ribonucleic acid, 42, 44 Rice cereal, 13, 75 Rifton chair, 6 Right inguinal hernia, 29 RNA, 42, 44 Rooting reflex, 135 Rotary chew, 5, 7, 88, 174 Rotavirus, 36, 63 Rural, 23, 72, 73, 92, 175, 176, 195, 199

S Salivary FOXP2 expression levels, 44–45 Salivary samples, 47 Salivary transcriptome, 42 Sanfilippo syndrome, 58, 68–69 SAT, 44 Scalable and Accurate Targeted, 44 Secretion management, 32, 59, 60, 67, 91, 181,182 Secretions, 143, 146, 150, 151, 154 Seizures, 49, 108, 111, 137–139, 141–143, 180, 182–183, 186–187 Self-efficacy, 129 Self-esteem, 129 Sensory, 85, 90, 120, 166 sensory avoidant, 123 sensory differences, 120, 180 sensory exposure, 12 sensory factors, 47 sensory hypersensitivity, 140 sensory information, 41, 47 sensory intervention, 167 sensory issue, 71, 90, 96 sensory motor environment, 46 sensory problems, 120, 122

sensory processing disorder, 122 sensory responses, 165, 167 sensory skills, 168 sensory stimulation, 41 sensory system, 123, 180 sensory-based intervention strategies, 2 sensory-based issues, 123 Sequencing/propulsion, 88 Sequential oral sensory 11 Shunt, 137 Shunt revision, 137 SIDS, 100 Silent aspiration, 58, 60, 169, 184 Singular, 107 Sinus bacterial culture, 87 Smith, Rebecca, viii-xiii Social worker, 8 Song learning, 43 SOS, 11 Spectrum Pediatrics Feeding Tube Weaning Program, 117 Speech pathologist, 25, 51–52, 72–74, 76, 189, 192–193 Staph, 137 Staphylococcal, 137 Stimulant, 111 Stress cue, 22 Stridor, 71 Suck reflex, 135 Sucking physiology, 16–17 Suckling, 135 Suck-swallow sequence, 135 Suck-swallow-breath sequence, 38, 135 Suction catheter, 122 Suctioning, 44, 59, 64, 65, 68, 101, 119, 122, 145, 150, 155, 165, 182 Sudden infant death syndrome, 100 Swallow study, 14, 19, 23, 33 Syringe, 36, 59–60, 68, 82–84, 88, 90

T Texas Children’s Hospital, 76 Today show, 42 Tongue residue, 60 Tongue retraction, 59

208  CASE STUDIES IN PEDIATRIC DYSPHAGIA Tonic bite, 31 Tonsils, 60, 71, 87 Toxicant level, 52 Trace aspiration, 21 Tracheomalacia, 32, 58, 62, 65, 66 Traits, 48 Transcriptome, 42, 44, 47 Trauma informed care, 106, 113, 114, 116–132 Trisomy 12 with mosaicism, 27, 29, 31 Tube fed, 39, 40, 64, 83 Tube weaning, 117, 124 Tumbleform, 59 22q11.2 deletion syndrome, 29 Twin pregnancy, 106

U Undersensitivity, 82 Undernourished, 86 Upper airway collapse, 185 Upper airway inflammation, 73 Upper airway obstruction, 147, 184 Upper GI, 73 Upper respiratory congestion, 66, 184 Upper respiratory infections, 9

V Valleculae, 60 Vallecular residue, 60 Ventilator, 106

VFSS, 25, 29, 30, 31, 58, 59, 60, 62, 64, 65, 70, 169, 182 Video fluoroscopic swallow study, 25, 29, 30, 31, 33, 58, 73, 192 Vital capacity, 23 Vocal cords, 154 Vocal cord paralysis, 75, 76, 82 Vomiting, 30, 32, 72, 74, 85, 138

W Water and air quality, 51 Weight gain, 37, 39, 41, 71, 82, 93, 101 Weinstein, Miriam, 171–176 Whole foods, 98–99 WIC, 80, 92–94, 97–99 Winged helix, 43 Wojtowicz, Emily 92–101 Women, Infants, and Children, 80, 92–94, 97–99 World Health Organization, 36

Y Yost, Bob, 142, 145–158

Z Zantac, 107 Zebra finch, 43 Zero-pressure bottle, 101 Zimmerman, Emily 28, 40–54 Zyrtec, 87, 107, 181