Pulmonary Rehabilitation [SECOND ed.] 9781351015578, 1351015575

Table of contents :
Cover
Half Title
Title Page
Copyright Page
Contents
Foreword
About the Book
Preface
Editors
Contributors
Part 1: The foundation of pulmonary rehabilitation
1. A framework for medical rehabilitation: Restoring function and improving quality of life
Introduction
A unifying model for rehabilitation: The International Classification of Functioning, Disability and Health
The approach to rehabilitation assessment
Unpacking the black box of rehabilitation: Important service delivery considerations and questions
Measuring the benefits of rehabilitation
Summary
Conflict of interest
References
2. Pulmonary rehabilitation: The development of a scientific discipline
Introduction
Definition and concepts
History
Summary
References
3. Key concepts in pulmonary rehabilitation
Definition and concept
Exercise training
Non-exercising interventions
Future perspectives for PR
Summary
References
4. Enhancing use and delivery of pulmonary rehabilitation
Introduction
Knowledge and awareness of pulmonary rehabilitation
Access, uptake and adherence to pulmonary rehabilitation
Use of pulmonary rehabilitation services compared to other effective therapies in COPD
Ensuring quality of PR programmes
Conclusion
Summary
References
5. Pathophysiological basis, evaluation and rationale of exercise training
Introduction
Pathophysiological basis of exercise training
Rationale of exercise testing
Exploring factors explaining exercise limitation
Rationale of exercise training
Benefits of exercise training
Summary
References
6. Education: Realizing the potential for learning in pulmonary rehabilitation
Overview
History of education in pulmonary rehabilitation
Examining outcomes from education
Educational design for pulmonary rehabilitation
Factors that influence learning
Digital pulmonary rehabilitation education
Summary
References
7. Self-management
Introduction
Definition of self-management interventions
Self-management interventions in COPD
Gap between the current best evidence, guidelines and practice
Model of care that includes pulmonary rehabilitation enhanced with self-management
Summary and clinical implications
References
8. Dual therapy: Pharmacologic management in pulmonary rehabilitation
Introduction
Bronchodilators
Supplemental oxygen and heliox
Anabolic agents
Nutritional supplements
Other pharmacologic agents
Summary
References
Part 2: Evaluation and management
9. Respiratory muscle function in rehabilitation
Introduction
Indications
Respiratory muscle assessment, theoretical considerations
Place of assessment of respiratory muscle function in some typical rehabilitation scenarios
Summary
References
10. Peripheral muscles
Introduction
Epidemiology and natural history
Causes
Structural and biological changes
Evaluation of limb muscle mass
Evaluation of limb muscle strength
Evaluation of muscle endurance
Effects of interventions on limb muscle function in COPD
Summary
References
11. Anxiety and depression in patients with chronic respiratory disease
Introduction
Depression
Anxiety
Barriers to depression and anxiety management in COPD
Treatment approaches for depression and anxiety in patients with COPD
Clinical tips
Summary
References
12. Dyspnoea
Introduction
What is dyspnoea?
Impact of dyspnoea in patients who have respiratory disease
Descriptors of dyspnoea
Measurements of dyspnoea for pulmonary rehabilitation
Effects of pulmonary rehabilitation on relieving dyspnoea
Responders versus non-responders
Mechanisms for relief of dyspnoea with pulmonary rehabilitation
Summary
References
13. Nutritional management in pulmonary rehabilitation
Introduction
Nutritional risk
Nutritional risk for development and progression of COPD
Nutritional risk for decreased physical performance
Nutritional risk for cardiovascular disease
Pharmaconutrients
Nutrition as part of integrated disease management
Summary
References
14. Balance impairment
Introduction
Balance and its assessment
Balance deficits in chronic lung disease
Balance assessment in pulmonary rehabilitation
Approaches to balance training as part of pulmonary rehabilitation
Conclusion
summary
References
15. Monitoring of physical activity
Physical activity: A clinically relevant (new) measure in pulmonary rehabilitation
Measurement of physical activity
Interpretation of PA in the context of pulmonary rehabilitation
Sedentary behaviour, a closely related concept
Summary
References
16. Monitoring health status
Why measure health status?
Measuring and monitoring health status
St. George’s Respiratory Questionnaire
Chronic Respiratory Questionnaire
COPD Assessment Test
Clinical COPD Questionnaire
Pulmonary rehabilitation and health status in COPD: Randomized, controlled trials
Comparison of pulmonary rehabilitation and pharmacologic therapy on health status in COPD
Pulmonary rehabilitation following exacerbations of COPD
Pulmonary rehabilitation and health status in respiratory patients with diseases other than COPD
Summary
References
Part 3: How, Who and Where?
17. Establishing a pulmonary rehabilitation programme
Introduction
The process
Outcome data
Conclusion
Summary
References
18. Quality assurance and control in pulmonary rehabilitation
Introduction
Quality control and assurance in PR
Quality standards
Pulmonary rehabilitation audit
Pulmonary rehabilitation certification and accreditation schemes
Pulmonary rehabilitation accreditation, certification and supporting processes in the US and UK
The dissemination of audit data and the development of quality improvement in PR
Broader impacts and future direction
Quality improvement resources
References
19. The ideal candidate
Introduction
Guidance criteria for inclusion in pulmonary rehabilitation
Responders and non-responders to PR
Contraindications to pulmonary rehabilitation
Adapting the pulmonary rehabilitation programme to different candidates
Final remarks
Summary
References
20. Rehabilitation team
Definition
Composition of the interdisciplinary PR team
Interdisciplinary pulmonary rehabilitation programme
Advantages
Barriers
Summary
References
21. Modalities of exercise training
Introduction
Exercise assessment tests
Modalities of exercise types
Exercise prescription
Supervision during training
Safety and recording during training
Oxygen supplementation
Summary
References
22. Physiotherapy and airway clearance
Introduction
Impairment of mucus elimination and clinical indications for airway clearance physiotherapy techniques
Control of mucus and airway clearance techniques
Mechanical respiratory muscle aids for secretion management
Summary
References
23. Smoking cessation
Introduction
Smoking cessation: An overview
Smoking cessation in pulmonary rehabilitation
Summary
References
24. Early rehabilitation following exacerbation of COPD
Introduction
Consequences of acute exacerbations
Early pulmonary rehabilitation in the exacerbation setting – the evidence base
Challenges in delivering early rehabilitation following exacerbation
Implementation of early rehabilitation after an exacerbation
Further research
Summary
References
25. Personalized rehabilitation
Introduction
Definitions
Personalized rehabilitation
Personalized comprehensive management
Awareness
Present evidence
Future
Summary
References
26. Pulmonary rehabilitation and primary care
Introduction
Epidemiology of the problem
Barriers to adaptation
Outcomes
Outlook
Summary
References
27. Home rehabilitation
What is the rationale for home-based rehabilitation?
Patient factors and perceptions
Alternative forms of rehabilitation
Supervision of participants
Access to staff
The evidence
Studies in the literature
Technology
Post exacerbation home-based studies
Maintenance programmes
Non-COPD populations
Conclusion
Summary
References
28. Telerehabilitation
Definition, rationale and opportunities for telerehabilitation
Telerehabilitation: Signals and models used
Telerehabilitation in COPD
Telerehabilitation in chronic respiratory insufficiency patients
Risk/disadvantages
Economic considerations (cost-effectiveness data)
Future directions and conclusions
Summary
References
29. Living with chronic lung disease: The experiences and needs of patients and caregivers
Introduction
Living with a chronic lung disease from the perspective of patients and caregivers
Pulmonary rehabilitation to support the family through the chronic lung disease journey
Real-world testimonials of patients and caregivers
Conclusion
Summary
Acknowledgements
References
Part 4: New Approaches to Exercise Training
30. Partitioned aerobic exercise training of ventilatory-limited patients with chronic respiratory disease
Overview
Background
Application
Summary
References
31. Whole-body vibration training
Introduction
WBVT platforms techniques
Summary
References
32. Neuromuscular electrical stimulation
Introduction
Principles of NMES
Clinical effects of NMES
Role in rehabilitation toolkit
Summary
References
33. A role for water-based rehabilitation
Introduction
Aquatic therapy
Water-based exercise for people with COPD
Conditions affecting the lower limbs and aquatic therapy
Special considerations for exercise in the aquatic environment
Special considerations for people with COPD for exercise in the aquatic environment
Summary
References
34. Sedentarism and light-intensity physical activity (in COPD)
Introduction
What do we know about sedentary time in people with COPD?
What are the health consequences of sedentary behaviour in the general population and those with COPD?
What is the physiological basis for sedentary behaviour being harmful?
Considerations when applying an intervention to reduce sedentary time in COPD
A behavioural science approach to sedentarism in COPD
Summary and conclusions
References
Part 5: Diseases Other than COPD
35. The multi-morbidity patient
Multi-morbidity in patients with COPD
The multi-morbid COPD patient: A model in rehabilitation
How multi-morbidity might impact outcomes of pulmonary rehabilitation
The effects of tailored pulmonary rehabilitation in multi-morbid patients
Conclusion
Summary
References
36. Is there any role for pulmonary rehabilitation in asthma?
Introduction
Patients
Types of intervention
Summary
References
37. Neuromuscular disorders
Introduction
Pathophysiology
What are respiratory muscle aids?
Patient evaluation
The intervention objectives
Long-term management
Glossopharyngeal breathing (GPB)
Oximetry monitoring and feedback protocol
Long-term outcomes
Extubation of unweanable patients
Decannulation of unweanable patients
Summary
References
38. Interstitial lung diseases
Introduction
Comprehensive symptom management
Pulmonary rehabilitation
Conclusion
Summary
References
39. Management of suppurative lung diseases
Background
Healthcare utilization in non-cystic fibrosis bronchiectasis
The implications of chronic sputum
Physiological consequences of bronchiectasis
Benefits of airway clearance techniques
Pulmonary rehabilitation in non-CF bronchiectasis
Physical activity in cystic fibrosis
Effect of cystic fibrosis on skeletal muscle dysfunction
Airway clearance techniques in cystic fibrosis
Summary
Financial support
Conflicts of interest
References
40. Rehabilitation in the intensive care unit
Introduction
Consequences of acute illness
Management of muscle weakness
Management of airway secretions
Prevention of cognitive impairment
Technical considerations
Conclusion
Summary
References
41. Chronic respiratory failure – pathophysiology
Introduction and definition
Overview
Causes of chronic hypercapnic respiratory failure
Chronic respiratory failure - diagnosis
Summary
References
Further Reading
42. Lung transplantation
Introduction
Rehabilitation before transplantation
Immediate post-transplant rehabilitation
Outpatient rehabilitation phase (hospital discharge up to 12 months post-transplant)
Long-term post-transplant phase (greater than 12 months post-transplant)
Conclusions and research needs
Summary
References
43. Lung volume reduction - old and new approaches
Introduction
Rationale for reducing lung volume reduction
Lung volume reduction surgery
Nonsurgical approaches to lung volume reduction
Summary
References
Part 6: Add-on interventions
44. Supplemental oxygen and heliox
Introduction
Dynamic hyperinflation of the lung during exercise
Supplemental oxygen breathing
Low-density gas mixture breathing
Relevance to rehabilitation programmes
Summary
References
45. Noninvasive ventilation during exercise training
Introduction
Exercise training for patients with chronic respiratory failure
Physiological effects of NIV on exercise
Noninvasive ventilation and exercise training
Technicalities
Future research
Alternative strategies
Summary
References
46. COPD patients requiring chronic nocturnal noninvasive ventilation
Introduction
Mechanisms of action
Chronic noninvasive ventilation in stable COPD
Chronic noninvasive ventilation after acute respiratory failure
Issues to be solved
Summary
References
47. More tools in the toolbox
Introduction
Music listening during PR
Singing
Tai Chi
Yoga
Active video games
Dance in COPD
Conclusion
Summary
References
48. Palliative care and end of life
Introduction
Palliative care
Dyspnoea
Fatigue
Cough
Pain
Integration of palliative care with pulmonary rehabilitation
Decision making
A place to die
Conclusion
Summary
References
49. Economical evaluation
Introduction
COPD
Interstitial lung disease and pulmonary fibrosis
Bronchiectasis
Summary
References
50. Pulmonary rehabilitation in the integrated care of the chronic respiratory patient
Introduction
Integrated care and the chronic care model
Similar, but not identical, concepts
Telemedicine
Pulmonary rehabilitation
Pulmonary rehabilitation and integrated care
Summary
References
51. Pulmonary rehabilitation in post-acute patients with COVID-19
Introduction
General recommendations
Principles of rehabilitation
Lessons from ARDS and other pandemics
Prevention of spread of infection
The intervention
Future directions
Conclusion
References
Index

Citation preview

1

Pulmonary Rehabilitation

Pulmonary Rehabilitation Second Edition

Edited by

Claudio F. Donner Nicolino Ambrosino Roger S. Goldstein

CRC Press Taylor & Francis Group 6000 Broken Sound Parkway NW, Suite 300 Boca Raton, FL 33487-2742 © 2021 by Taylor & Francis Group, LLC CRC Press is an imprint of Taylor & Francis Group, an Informa business No claim to original U.S. Government works International Standard Book Number-13: 978-1-138-49881-5 (Hardback) 978-1-351-01559-2 (eBook) This book contains information obtained from authentic and highly regarded sources. While all reasonable efforts have been made to publish reliable data and information, neither the author[s] nor the publisher can accept any legal responsibility or liability for any errors or omissions that may be made. The publishers wish to make clear that any views or opinions expressed in this book by individual editors, authors or contributors are personal to them and do not necessarily reflect the views/opinions of the publishers. The information or guidance contained in this book is intended for use by medical, scientific or health-care professionals and is provided strictly as a supplement to the medical or other professional’s own judgement, their knowledge of the patient’s medical history, relevant manufacturer’s instructions and the appropriate best practice guidelines. Because of the rapid advances in medical science, any information or advice on dosages, procedures or diagnoses should be independently verified. The reader is strongly urged to consult the relevant national drug formulary and the drug companies’ and device or material manufacturers’ printed instructions, and their websites, before administering or utilizing any of the drugs, devices or materials mentioned in this book. This book does not indicate whether a particular treatment is appropriate or suitable for a particular individual. Ultimately it is the sole responsibility of the medical professional to make his or her own professional judgements, so as to advise and treat patients appropriately. The authors and publishers have also attempted to trace the copyright holders of all material reproduced in this publication and apologize to copyright holders if permission to publish in this form has not been obtained. If any copyright material has not been acknowledged please write and let us know so we may rectify in any future reprint. Except as permitted under U.S. Copyright Law, no part of this book may be reprinted, reproduced, transmitted, or utilized in any form by any electronic, mechanical, or other means, now known or hereafter invented, including photocopying, microfilming, and recording, or in any information storage or retrieval system, without written permission from the publishers. For permission to photocopy or use material electronically from this work, please access www.copyright.com (http://www.copyright.com/) or contact the Copyright Clearance Center, Inc. (CCC), 222 Rosewood Drive, Danvers, MA 01923, 978-750-8400. CCC is a not-for-profit organization that provides licenses and registration for a variety of users. For organizations that have been granted a photocopy license by the CCC, a separate system of payment has been arranged. Trademark Notice: Product or corporate names may be trademarks or registered trademarks, and are used only for identification and explanation without intent to infringe. Library of Congress Cataloging-in-Publication Data Names: Donner, C. F. (Claudio F.), editor. | Ambrosino, N. (Nicolino), 1948- editor. | Goldstein, Roger, editor. | Donner, C. F. (Claudio F.). Pulmonary rehabilitation. Title: Pulmonary rehabilitation / edited by Claudio F. Donner, Nicolino Ambrosino, Roger S. Goldstein. Other titles: Pulmonary rehabilitation (Donner) Description: Second edition. | Boca Raton : CRC Press, [2020] | Preceded by Pulmonary rehabilitation / Claudio F. Donner, Nicolino Ambrosino, Roger Goldstein. 2005. | Includes bibliographical references and index. | Summary: “The new edition includes new sections on the development of PR as a discipline, global perspectives on quality control, early PR post exacerbation and personalized rehabilitation, innovative approaches to exercise, PR in interstitial lung disease and lung transplantation, and the latest research into the application of music, dance and yoga”-- Provided by publisher. Identifiers: LCCN 2020002769 (print) | LCCN 2020002770 (ebook) | ISBN 9781138498815 (hardback) | ISBN 9781351015592 (ebook) Subjects: MESH: Pulmonary Disease, Chronic Obstructive--rehabilitation | Respiratory Therapy--methods | Treatment Outcome Classification: LCC RC776.R38 (print) | LCC RC776.R38 (ebook) | NLM WF 600 | DDC 616.2/4--dc23 LC record available at https://lccn.loc.gov/2020002769 LC ebook record available at https://lccn.loc.gov/2020002770 Visit the Taylor & Francis Web site at http://www.taylorandfrancis.com and the CRC Press Web site at http://www.crcpress.com

Contents

Foreword ix About the Book

xi

Preface xiii Editors xv Contributors xvii Part 1 THE FOUNDATION OF PULMONARY REHABILITATION

1

1

3

2 3 4 5 6 7 8

A framework for medical rehabilitation: Restoring function and improving quality of life Julia Warden and Mark Bayley Pulmonary rehabilitation: The development of a scientific discipline Linda Nici and Roger S. Goldstein Key concepts in pulmonary rehabilitation Felipe V.C. Machado, Frits M.E. Franssen and Martijn A. Spruit Enhancing use and delivery of pulmonary rehabilitation Emily Hume, Carolyn L. Rochester and Ioannis Vogiatzis Pathophysiological basis, evaluation and rationale of exercise training Pierantonio Laveneziana and Paolo Palange Education: Realizing the potential for learning in pulmonary rehabilitation Felicity Blackstock and Suzanne C. Lareau Self-management Jean Bourbeau and Tanja W. Effing Dual therapy: Pharmacologic management in pulmonary rehabilitation J. Michael Nicholson and Richard Casaburi

11 21 29 41 53 63 75

Part 2  EVALUATION AND MANAGEMENT

87

9

89

10 11 12 13 14

Respiratory muscle function in rehabilitation Thierry Troosters, Michael I. Polkey and Rik Gosselink Peripheral muscles Luis Puente-Maestu, François Maltais, André Nyberg and Didier Saey Anxiety and depression in patients with chronic respiratory disease Abebaw Mengistu Yohannes Dyspnoea Pierantonio Laveneziana and Donald A. Mahler Nutritional management in pulmonary rehabilitation Rosanne J.H.C.G. Beijers, Emiel F.M. Wouters and Annemie M.W.J. Schols Balance impairment Marla K. Beauchamp

99 115 125 135 145

v

vi Contents

15 16

Monitoring of physical activity Heleen Demeyer, Thierry Troosters and Henrik Watz Monitoring health status Claire M. Nolan, William D.-C. Man and Richard L. ZuWallack

153 163

Part 3  HOW, WHO AND WHERE?

171

17

173

18 19 20 21 22 23 24 25 26 27 28 29

Establishing a pulmonary rehabilitation programme Michael D.L. Morgan and Sally J. Singh Quality assurance and control in pulmonary rehabilitation Michael Steiner, Chris Garvey, Sally J. Singh and Gerene Bauldoff The ideal candidate Francesca de Blasio, Rafael Mesquita and Enrico Clini Rehabilitation team Inês Machado Vaz, Sofia Viamonte and João Carlos Winck Modalities of exercise training Matthew Armstrong, Rebecca Crouch and Ioannis Vogiatzis Physiotherapy and airway clearance Miguel R. Gonçalves and Amanda J. Piper Smoking cessation Francesco Pistelli, Stefania Brogi and Laura Carrozzi Early rehabilitation following exacerbation of COPD William D.-C. Man, Claire M. Nolan and Milo A. Puhan Personalized rehabilitation Nicolino Ambrosino and Annia Schreiber Pulmonary rehabilitation and primary care Jonathan M. Raskin Home rehabilitation Sally J. Singh and Linzy Houchen-Wolloff Telerehabilitation Michele Vitacca and Michael K. Stickland Living with chronic lung disease: The experiences and needs of patients and caregivers Alda Marques and Roger S. Goldstein

183 195 203 209 219 231 241 247 253 257 271 281

Part 4  NEW APPROACHES TO EXERCISE TRAINING

295

30

297

31 32 33 34

Partitioned aerobic exercise training of ventilatory-limited patients with chronic respiratory disease Thomas E. Dolmage and Roger S. Goldstein Whole-body vibration training Rainer Gloeckl Neuromuscular electrical stimulation Matthew Maddocks and Isabelle Vivodtzev A role for water-based rehabilitation Renae J. McNamara and Jennifer A. Alison Sedentarism and light-intensity physical activity (in COPD) Kylie Hill, Zoe McKeough and Daniel F. Gucciardi

309 317 327 335

Part 5  DISEASES OTHER THAN COPD

347

35

349

36 37

The multi-morbidity patient Roberto Tonelli, Ernesto Crisafulli, Stefania Costi and Enrico Clini Is there any role for pulmonary rehabilitation in asthma? Elisabetta Zampogna, Martina Zappa, Antonio Spanevello and Dina Visca Neuromuscular disorders Miguel R. Gonçalves and John R. Bach

359 363

Contents vii

38 39 40 41 42 43

Interstitial lung diseases Nicolino Ambrosino Management of suppurative lung diseases J. Michael Nicholson, Roger S. Goldstein and Dmitry Rozenberg Rehabilitation in the intensive care unit Piero Ceriana and Nicolino Ambrosino Chronic respiratory failure – pathophysiology Mafalda Vanzeller, Marta Drummond and João Carlos Winck Lung transplantation Daniel Langer Lung volume reduction − old and new approaches Nathaniel Marchetti and Gerard Criner

373 379 391 399 409 419

Part 6  ADD-ON INTERVENTIONS

439

44

441

45 46 47 48 49 50 51

Supplemental oxygen and heliox Paolo Palange and Richard Casaburi Noninvasive ventilation during exercise training Nicolino Ambrosino and Lara Pisani COPD patients requiring chronic nocturnal noninvasive ventilation Marieke L. Duiverman and Peter J. Wijkstra More tools in the toolbox Annemarie L. Lee and Dina Brooks Palliative care and end of life Michele Vitacca and Nicolino Ambrosino Economical evaluation Roberto W. Dal Negro and Claudio F. Donner Pulmonary rehabilitation in the integrated care of the chronic respiratory patient Linda Nici and Richard L. ZuWallack Pulmonary rehabilitation in post-acute patients with COVID-19 Michele Vitacca, Mara Paneroni, and Nicolino Ambrosino

447 455 463 473 483 491 503

Index 511

Foreword

There is no field in the therapy of patients with respiratory diseases that has shown more practical evidence for its benefit than that of pulmonary rehabilitation. This has occurred over the last several decades because of the persistence of some unique health care professionals devoted to improving the quality of life of patients suffering from these illnesses. The scientific method has been applied to answer the many questions that in previous decades labeled the field of pulmonary rehabilitation as simple ‘art’. The new edition of this already classic book summarizes the result of hundreds of studies, many of which have been authored by the chapter writers since 2005 and complemented by their extensive personal experience in treating the ultimate beneficiaries—symptomatic patients with respiratory ailments. Unique to the readers of this book is the important contribution by a group of Italian colleagues who summarized their experience and recommendations related to pulmonary rehabilitation in this era of the COVID-19 pandemic. Even though we expect this pandemic to decrease and eventually disappear, the lessons learned should be extremely helpful and instructive, as it is likely that epidemics such as the one we have suffered will reappear in the future. One lesson gained from this experience is that we should always be prepared. A special recognition must be given to Drs Claudio Donner, Nicolino Ambrosino and Roger Goldstein, who gathered not only the most respected authorities in the field to contribute to this book, but had the foresight to organize its contents along an easy-to-grasp series of important headings. It starts with the foundation of the specialty, authored by several modern pioneers of the field and laying the ground work for subsequent major headings. The book then guides the reader in the appropriate tools to evaluate patients in those areas where the therapy will have its effect, or that will be needed to monitor during and after the program. In the third section, various experts review the basic questions of who, where, and when with excellent reviews on how to develop a program and expand areas such as the use of the home for rehabilitation and the potential use of telemedicine. The same section reviews the solid science that has proven to be the most important component of pulmonary rehabilitation, that of exercise training. This is further

expanded in the fourth section, where new approaches to this crucial component are explored. This section serves as an excellent base for those interested in exploring new areas of research. The subsequent section is also novel in providing actualized information about the value of pulmonary rehabilitation in diseases different from chronic obstructive pulmonary disease. This is extremely important because most of the solid evidence upon which we have built the programs is based on experience gained in patients with COPD. Once again, how effective the basic program is and how to modify it, if we have to, is ripe for more research and should stimulate younger generations of caregivers to plan the studies needed to expand the applicability of this highly effective therapy. The last section gathers several specific areas of importance that are often forgotten and should be in the minds of health care providers who participate in the care of these patients. This section includes the crucial issue of palliative care for compromised patients and the novel experience with rehabilitation in the COVID-19 era. The modern world in which we live allows us to access novel information from almost anywhere in the world. This has led to the narrow view that books are no longer needed. Nothing could be further from the truth, as this edition proves. There is a need to have an updated reference at hand, to summarize in one single volume the knowledge that arises from the wide-angle vision of experience. This book is a must for anyone interested in caring for patients with respiratory disease who are symptomatic with compromise in their lifestyle. Pulmonary rehabilitation has been proven to result in the largest potential impact on objective outcomes such as dyspnea, quality of life, and functional capacity in patients who qualify for the treatment. Its wide application still remains an elusive goal, and this book is one excellent effort to increase not only knowledge about the benefits of pulmonary rehabilitation, but also to spark an expansion in the number of champions ready to make rehabilitation a routine tool in treating their patients. Bartolome R. Celli, MD Professor of Medicine Harvard Medical School Boston, Massachusetts

ix

About the Book

On the wall in my office is a letter from a gentleman with COPD received in 2005, the same year the first edition of this important text on pulmonary rehabilitation was published. He opens the letter by telling me that his recent pulmonary rehabilitation program had ‘worked wonders for me – I would not have believed it possible’. He goes on to describe the new knowledge he had gained, and how he had been particularly struck by the critical role of the alveoli in lung health and disease. He was a mathematician, so he had gone home and calculated the surface area of a single alveolus. He included those results in his letter (by his calculation each alveolus 0.23 mm2, giving rise to over 70 m2 of surface area for gas exchange in the lung). This story will be familiar to many pulmonary rehabilitation practitioners using this book, who will have numerous testimonials (perhaps without the maths) from people who experienced similar benefits. Pulmonary rehabilitation is an intervention that can transform the lives of people with chronic lung disease and has transformed the understanding of healthcare professionals about the extraordinary things that people with chronic lung disease can do. In this second edition of Pulmonary Rehabilitation, the authors have presented the well-established scientific foundations of this treatment along with the many

advances that have occurred since 2005. These include new approaches to exercise training (e.g. partitioning, water-based training and neuromuscular stimulation) and a broader range of program components (e.g. physical activity monitoring and balance training). Pulmonary rehabilitation is extended into new settings and modes of delivery (primary care, home, telerehabilitation), and the importance of quality assurance is addressed. The voices of patients and caregivers are incorporated into this new edition to enhance our understanding of the experience of living with chronic lung disease and of undertaking pulmonary rehabilitation. Emerging knowledge on rehabilitation of COVID-19 survivors is included. The authors bring expertise from across the globe, and their work confirms that pulmonary rehabilitation is an essential component of comprehensive and integrated care. I am confident that the scientific rigor, clinical expertise, and insights contained in these pages will contribute to better lives for people with chronic lung disease. Anne E Holland PT PhD FThorSoc Professor Physiotherapy Monash University and Alfred Health Melbourne, Australia

xi

Preface

Since publication of the first edition of this book in 2005, there has been growing interest in the role of pulmonary rehabilitation (PR) for the management of chronic respiratory disease, which continues to increase as a major cause of global mortality and morbidity. Health providers and healthcare professionals, having become more aware of the improvements in function and quality of life associated with PR, are increasingly including it as an integral part of their approach to disease management. Since the joint European Respiratory Society and American Thoracic Society’s 2013 major statements on PR, published guidelines from professional associations around the world have endorsed PR as the prevailing standard of care for those with chronic respiratory conditions. PR substantially helps clinicians extend the pharmacological approach to chronic disease management by tackling some of the many associated non-respiratory impairments such as peripheral muscle dysfunction, cardiovascular disease, nutritional limitations and mental health disorders that are associated with chronic diseases. PR reduces exacerbation frequency and healthcare resource utilization. It also offers clinically important symptom relief and functional improvements for those with non-COPD respiratory conditions such as pulmonary fibrosis, pulmonary hypertension, suppurative lung disease and in the population preparing for and following lung transplantation, lobar resection or volume reduction surgery. Despite the rich fabric of information reported in excellent peer-reviewed publications, there are exciting ongoing challenges in relation to the location, duration, timing and content of PR. Clearly it must be both patient- and

society-centred and has to be modified within the context of local clinical and financial resources. Programmes must also be quality controlled to ensure fidelity to the model used. There is an increasing awareness of the importance of the impact of respiratory disease on caregiver burden. Newer modalities of distance monitoring, such as telemedicinesupported rehabilitation and web-based learning modules, are being encouraged to increase access and capacity as well as prolong the benefits of PR. With the increased interest in collaborative integrated care, clinicians are challenged to map the pathways that site PR in the patients’ journey between community services and acute care admissions. The increasing interest in PR has paralleled the advances in the development of patient-reported outcomes as well as patient-reported experiences. Although proven to improve quality of life and exercise capacity as well as reduce healthcare utilization, the possibility that PR may offer a survival advantage remains of interest. The authors have condensed information from reports spanning 2005 to 2019, published since the last edition. There are key points, take-home learning messages and chapter summaries. We have been fortunate in attracting some of the world’s most recognized experts in the field and we hope that this text serves as a learning tool and resource for the next generation of healthcare providers interested in participating and increasing learning in this evolving field. Claudio F. Donner Nicolino Ambrosino Roger S. Goldstein

xiii

Editors

Claudio F. Donner, FERS is currently medical director of Mondo Medico Multidisciplinary and Rehabilitation Clinic, Borgomanero (NO), Italy, prior to which he served as chief of the Division of Pulmonary Disease, Scientific Institute of Veruno, ‘S. Maugeri’ Foundation (1985–2006) and director of the Department of Pulmonary Rehabilitative Medicine of the ‘S. Maugeri’ Foundation throughout Italy. He has also been on the faculty of medical schools in Ferrara, Turin, and Novara. Dr Donner has served professional associations in a number of capacities including as president of the Italian Association of Hospital Pulmonologists (AIPO) (1995–1997) and of the Italian Interdisciplinary Association for Research in Lung Disease (AIMAR) (2003–2012). He has also been president of the non-profit Italian Foundation ‘World of Breath’ since 2013. Additional professional activities include service as head of the ERS Clinical Assembly (1996–1998), secretary general of the European Respiratory Society (ERS) (1998–2004), president (2002–2006) of the Pneumology Section & Board of the European Union of Medical Specialists (UEMS) (2002–2006), and member the UEMS Management Council (2004–2006). He was also international governor of the American College of Chest Physicians’ Italian Chapter (2009–2012). Commended for his involvement, Dr Donner has been honored with numerous awards, most recently receiving a fellowship of the European Respiratory Society and special recognition by AIPO Dr Donner is past editor-in-chief of the scientific journal Monaldi Archives for Chest Disease (1993–2002) and associate editor since 2017 as well as past co-editor (1990–2004) of the Italian Review of Respiratory Disease (1990–2004) and of Multidisciplinary Respiratory Medicine (2006–2016). He has been associate editor of Respiratory Medicine since 2005. He is also author of several textbooks and numerous original scientific papers (208 indexed in MEDLINE) on a wide range of topics including pathophysiologic mechanisms of exercise and CO2 retention, follow-up of chronic respiratory failure (CRF), lung mechanics in critical care, pulmonary rehabilitation, COPD genetics, sleep respiratory disorders and QoL in patients with CRF.

Nicolino Ambrosino, FERS is a specialist in internal ­medicine, pulmonary diseases and physiokinesitherapy and occupational medicine. He is a research consultant at Istituti Clinici Maugeri, Pavia, Italy. He is also chief editor of the Pulmonology journal. He was formerly research and clinical department director of Auxilium Vitae Rehabilitation Center Volterra, director of the Pulmonary and Respiratory Intensive Care Unit, Cardio-Thoracic Department, University Hospital, Pisa and director of the Pulmonary Division and Intermediate Intensive Care, S. Maugeri Foundation, Medical Center of Gussago. He has been also appointed professor at several Italian universities. Dr Ambrosino is one of the earliest contributors to the development of the use of noninvasive mechanical ventilation techniques in acute and chronic respiratory failure, pulmonary rehabilitation and respiratory intensive care units. His research activity has been devoted to respiratory critical care, pulmonary rehabilitation and home respiratory care. He has received several life achievement awards such as the ERS educational award and those from the ATS Pulmonary Rehabilitation Assembly, the ERS Assembly II, the Italian Association of Pulmonologist and Livorno Oscar. Roger S. Goldstein, FRCP (C), FRCP (UK) is a professor of medicine and physical therapy at the University of Toronto. He held the founding National Sanitarium Association’s Chair in Respiratory Rehabilitation Research from 2002– 2019. He is a senior scientist and heads the Respiratory Division at West Park Healthcare Centre, Toronto, Ontario, Canada, which specializes in the management of chronic respiratory conditions. Together with Dr Dina Brooks, he co-leads the postgraduate respiratory research programme at West Park, which has welcomed fellows from Canada and around the world. He was the founding scientific chair of the first Canadian Respiratory Conference. He has made numerous presentations and has published more than 200 articles in his field of research. He co-edited the first edition of Pulmonary Rehabilitation in 2005 and is the co-editor of the current second edition.

xv

Contributors

Jennifer A. Alison Sydney School of Health Sciences Faculty of Medicine and Health The University of Sydney and Sydney Local Health District NSW Ministry of Health Sydney, Australia Nicolino Ambrosino Istituti Clinici Scientifici Maugeri IRCCS U.O. di Pneumologia Riabilitativa Istituto Scientifico di Montescano (PV) Montescano, Italy Matthew Armstrong Department of Sport, Exercise and Rehabilitation School of Health & Life Sciences Northumbria University Newcastle, United Kingdom John R. Bach Department of Physical Medicine and Rehabilitation and  Department of Neurosciences Center for Ventilator Management Alternatives University Hospital Rutgers New Jersey Medical School Newark, New Jersey Gerene Bauldoff Clinical Nursing The Ohio State University Columbus, Ohio Mark Bayley Toronto Rehabilitation Institute University Health Network

and Division of Physical Medicine and Rehabilitation Department of Medicine University of Toronto Toronto, Ontario, Canada Marla K. Beauchamp School of Rehabilitation Science Department of Medicine McMaster University Hamilton, Ontario, Canada and West Park Healthcare Centre Toronto, Ontario, Canada Rosanne J.H.C.G. Beijers Department of Respiratory Medicine NUTRIM School of Nutrition and Translational Research in Metabolism Maastricht University Medical Centre Maastricht, The Netherlands Felicity Blackstock Department of Physiotherapy School of Science and Health Western Sydney University Sydney, Australia Jean Bourbeau Respiratory Epidemiology and Clinical Research Unit Research Institute of the McGill University Health Centre Department of Medicine McGill University Montreal, Quebec, Canada

xvii

xviii Contributors

Stefania Brogi Pulmonary Unit Cardiothoracic and Vascular Department University Hospital of Pisa Pisa, Italy Dina Brooks Department of Respiratory Medicine West Park Healthcare Centre and Department of Physical Therapy University of Toronto Toronto, Ontario, Canada and School of Rehabilitation Sciences Faculty of Health Sciences McMaster University Hamilton, Ontario, Canada Laura Carrozzi Department of Surgery, Medicine, Molecular Biology and Critical Care University of Pisa and Respiratory Pathophysiology and Rehabilitation Section Cardiothoracic and Vascular Department University Hospital of Pisa Pisa, Italy Richard Casaburi Rehabilitation Clinical Trials Center Los Angeles Biomedical Research Institute at Harbor UCLA Medical Center Torrance, California Piero Ceriana Istituti Clinici Scientifici Maugeri IRCCS U.O. di Pneumologia Riabilitativa Istituto Scientifico di Pavia Pavia, Italy Enrico Clini Department of Medical and Surgical Sciences University of Modena Reggio Emilia and University Hospital of Modena Modena, Italy Stefania Costi Department of Surgical Medical and Dental Department of Morphological Sciences related to Transplants, Oncology and Regenerative Medicine University of Modena and Reggio Emilia Modena, Italy

Gerard Criner Department of Thoracic Medicine and Surgery Lewis Katz School of Medicine at Temple University Philadelphia, Pennsylvania Ernesto Crisafulli Department of Medicine and Surgery Respiratory Disease and Lung Function Unit University of Parma Parma, Italy Rebecca Crouch College of Pharmacy and Health Sciences Campbell University Buies Creek, North Carolina Roberto W. Dal Negro National Centre for Respiratory Pharmacoeconomics and Pharmacoepidemiology CESFAR Verona, Italy Francesca de Blasio Department of Medical Science University of Turin Turin, Italy and Department of Medicine and Health Sciences ‘V.Tiberio’ University of Molise and Clinic Center S.p.A. Private Hospital Campobasso, Italy Heleen Demeyer Department of Rehabilitation Sciences Ghent University Ghent, Belgium and University Hospitals Leuven Respiratory Division Leuven, Belgium Thomas E. Dolmage Respiratory Diagnostic & Evaluation Services West Park Healthcare Centre Toronto, Ontario, Canada Claudio F. Donner Mondo Medico Multidisciplinary and Rehabilitation Outpatient Clinic Borgomanero (NO), Italy

Contributors xix

Marta Drummond Faculty of Medicine Porto University and Pulmonology Department São João Hospital Porto, Portugal Marieke L. Duiverman Department of Pulmonary Diseases/Home Mechanical Ventilation and Groningen Research Institute for Asthma and COPD University of Groningen University Medical Center Groningen Groningen, The Netherlands Tanja W. Effing Department of Respiratory Medicine Southern Adelaide Local Health Network and School of Medicine Flinders University Adelaide, Australia Frits M.E. Franssen Department of Research and Development CIRO+ Horn, The Netherlands and Department of Respiratory Medicine Maastricht University Medical Centre NUTRIM School of Nutrition and Translational Research in Metabolism Maastricht, The Netherlands Chris Garvey Pulmonary Rehabilitation and Sleep Disorders University of California San Francisco San Francisco, California Rainer Gloeckl Institute for Pulmonary Rehabilitation Research Schoen Klinik Berchtesgadener Land Schönau am Königssee, Germany Roger S. Goldstein Respiratory Rehabilitation Research Medicine and Physical Therapy University of Toronto and Respiratory Services West Park Healthcare Centre Toronto, Ontario, Canada

Miguel R. Gonçalves Noninvasive Ventilatory Support Unit Pulmonology Department Emergency and Intensive Care Medicine Department Center for Home Mechanical Ventilation São João University Hospital and Faculty of Medicine University of Porto Porto, Portugal Rik Gosselink Department of Rehabilitation Sciences KU Leuven and Pulmonary Rehabilitation, Respiratory Division University Hospital Leuven Leuven, Belgium Daniel F. Gucciardi School of Physiotherapy and Exercise Science and Physical Activity and Well-Being Research Group Faculty of Health Science Curtin University Perth, Western Australia, Australia Kylie Hill School of Physiotherapy and Exercise Science Faculty of Health Science Curtin University and Institute for Respiratory Research Sir Charles Gairdner Hospital Perth, Western Australia, Australia Linzy Houchen-Wolloff Department of Respiratory Science University of Leicester and Centre for Exercise and Rehabilitation Science NIHR Leicester Biomedical Research Centre – Respiratory Glenfield Hospital Leicester, United Kingdom Emily Hume Department of Sport, Exercise and Rehabilitation Northumbria University Newcastle, United Kingdom Daniel Langer KU Leuven Faculty of Movement and Rehabilitation Sciences and Department of Rehabilitation Sciences Research Group for Rehabilitation in Internal Disorders Respiratory Rehabilitation and Respiratory Division University Hospital Leuven, Belgium

xx Contributors

Suzanne C. Lareau College of Nursing University of Colorado Anschutz Medical Center Aurora, Colorado

François Maltais Institut universitaire de cardiologie et de pneumologie de Québec Université Laval Québec, Canada

Pierantonio Laveneziana Sorbonne Université INSERM UMRS1158 Neurophysiologie Respiratoire Expérimentale et clinique and Département Médico-Universitaire AP-HP Sorbonne Université Service des Explorations Fonctionnelles de la Respiration, de l’Exercice et de la Dyspnée des Hôpitaux Pitié-Salpêtrière Tenon et Saint-Antoine Paris, France

William D.-C. Man Harefield Respiratory Research Group Royal Brompton and Harefield NHS Foundation Trust and National Heart and Lung Institute Imperial College and Harefield Pulmonary Rehabilitation and Muscle Research Laboratory London, United Kingdom

Annemarie L. Lee Department of Physiotherapy Monash University and Institute for Breathing and Sleep Austin Health and Physiotherapy, Rehabilitation, Nutrition and Sport La Trobe University Victoria, Australia Felipe V.C. Machado Department of Research and Development CIRO+ Horn, The Netherlands and Department of Physiotherapy Laboratory of Research in Respiratory Physiotherapy (LFIP) State University of Londrina Londrina, Brazil Matthew Maddocks King’s College London London, United Kingdom Donald A. Mahler Geisel School of Medicine at Dartmouth Hanover, New Hampshire and Valley Regional Hospital Claremont, New Hampshire

Nathaniel Marchetti Department of Thoracic Medicine and Surgery Lewis Katz School of Medicine at Temple University Philadelphia, Pennsylvania Alda Marques Lab3R-Respiratory Research and Rehabilitation Laboratory of the School of Health Sciences (ESSUA) and Institute of Biomedicine (iBiMED) University of Aveiro Aveiro, Portugal Zoe McKeough Discipline of Physiotherapy Sydney School of Health Sciences Faculty of Medicine and Health The University of Sydney Sydney, New South Wales, Australia Renae J. McNamara Department of Physiotherapy Prince of Wales Hospital and Sydney School of Health Sciences Faculty of Health Sciences The University of Sydney and Woolcock Institute of Medical Research The University of Sydney Sydney, Australia Rafael Mesquita Department of Physiotherapy Federal University of Ceará Fortaleza, Brazil

Contributors xxi

Michael D.L. Morgan Department of Respiratory Science University of Leicester and Centre for Exercise and Rehabilitation Science NIHR Leicester Biomedical Research Centre – Respiratory Glenfield Hospital Leicester, United Kingdom J. Michael Nicholson Division of Respirology Western University London, Ontario, Canada Linda Nici The Warren Alpert Medical School of Brown University and Providence Veterans Affairs Medical Center Providence, Rhode Island Claire M. Nolan Harefield Respiratory Research Group and Harefield Pulmonary Rehabilitation Unit Royal Brompton and Harefield NHS Foundation Trust London, United Kingdom André Nyberg Department of Community Medicine and Rehabilitation Section of Physiotherapy Umeå University Umeå, Sweden Paolo Palange Department of Public Health and Infectious Diseases Sapienza University of Rome and Lorillard-Spencer Foundation Sapienza University of Rome Rome, Italy Amanda J. Piper Department of Respiratory and Sleep Medicine Royal Prince Alfred Hospital Camperdown, Australia and Faculty of Medicine and Health Science University of Sydney Sydney, New South Wales, Australia Lara Pisani Respiratory and Critical Care Unit Alma Mater Studiorum University of Bologna Sant’Orsola Malpighi Hospital Bologna, Italy

Francesco Pistelli Pulmonary Unit Cardiothoracic and Vascular Department University Hospital of Pisa Pisa, Italy Michael I. Polkey Department of Respiratory Medicine Royal Brompton & Harefield NHS Foundation Trust London, United Kingdom Luis Puente-Maestu Department of Respiratory Medicine University Hospital Gregorio Marañón Universidad Complutense de Madrid Medical School Madrid, Spain Milo A. Puhan Epidemiology, Biostatistics and Prevention Institute University of Zurich Zurich, Switzerland Jonathan M. Raskin Mount Sinai Beth Israel Medical Center Lenox Hill Hospital Mount Sinai Medical Center New York, New York Carolyn L. Rochester Section of Pulmonary, Critical Care and Sleep Medicine Department of Medicine Yale University School of Medicine New Haven, Connecticut Dmitry Rozenberg Department of Medicine Division of Respirology University of Toronto and Sandra Faire & Ivan Fecan Professorship in Rehabilitation Medicine Toronto General Hospital Research Institute University Health Network Toronto, Ontario, Canada Didier Saey Centre de Recherche Institut universitaire de cardiologie et de pneumologie de Québec Université Laval Québec, Canada

xxii Contributors

Annemie M.W.J. Schols NUTRIM School of Nutrition and Translational Research in Metabolism Department of Respiratory Medicine Maastricht University Medical Centre Maastricht, The Netherlands Annia Schreiber Division of Respirology Department of Medicine University Health Network Toronto, Ontario, Canada Sally J. Singh Department of Respiratory Science University of Leicester and Centre for Exercise and Rehabilitation Science NIHR Leicester Biomedical Research Centre – Respiratory Glenfield Hospital Leicester, United Kingdom Antonio Spanevello Dipartimento di Medicina e Chirurgia Malattie dell’Apparato Respiratorio Università degli Studi dell’Insubria Varese, Italy

Michael K. Stickland Pulmonary Division Department of Medicine Faculty of Medicine and Dentistry University of Alberta and G.F. MacDonald Centre for Lung Health Covenant Health Edmonton, Alberta, Canada Roberto Tonelli Department of Medical and Surgical Sciences University of Modena Reggio Emilia and University Hospital of Modena Modena, Italy Thierry Troosters Departement of Rehabilitation Sciences KU Leuven and Pulmonary Rehabilitation Respiratory Division University Hospital Leuven Leuven, Belgium

and Dipartimento di Medicina e Riabilitazione Cardio Respiratoria U.O. di Pneumologia Riabilitativa Istituti Clinici Scientifici Maugeri, IRCCS Tradate Tradate, Italy Martijn A. Spruit Department of Research and Development CIRO+ Horn, The Netherlands and Department of Respiratory Medicine Maastricht University Medical Centre NUTRIM School of Nutrition and Translational Research in Metabolism Maastricht, The Netherlands and Rehabilitation Research Center (REVAL) Biomedical Research Institute (BIOMED) Faculty of Rehabilitation Sciences UHasselt Diepenbeek, Belgium Michael Steiner Leicester Biomedical Research Centre-Respiratory Institute for Lung Health University of Leicester Leicester, United Kingdom

Mafalda Vanzeller Faculty of Medicine Porto University and Pulmonology Department São João Hospital Porto, Portugal Inês Machado Vaz Centro de Reabilitação do Norte/Centro Hospitalar Vila Nova de Gaia-Espinho Valadares, Portugal Sofia Viamonte Centro de Reabilitação do Norte/Centro Hospitalar Vila Nova de Gaia-Espinho Valadares, Portugal Dina Visca Istituti Clinici Scientifici Maugeri IRCCS Respiratory Rehabilitation of the Institute of Tradate (VA) and Department of Medicine and Surgery Respiratory Diseases University of Insubria Varese-Como, Italy

Contributors xxiii

Michele Vitacca Istituti Clinici Scientifici Maugeri IRCCS U.O. di Pneumologia Riabilitativa Institute of Lumezzane (Brescia) and Respiratory Rehabilitation Division Istituti Clinici Scientifici Maugeri IRCCS Lumezzane (BS) Lumezzane, Italy Isabelle Vivodtzev Department of Physical Medicine & Rehabilitation Harvard Medical School Boston, Massachusetts Ioannis Vogiatzis VA Connecticut Healthcare System West Haven, Connecticut and Department of Sport, Exercise and Rehabilitation School of Health & Life Sciences Northumbria University Newcastle, United Kingdom Julia Warden Toronto Rehabilitation Institute University Health Network and Division of Physical Medicine and Rehabilitation Department of Medicine University of Toronto Toronto, Ontario, Canada Henrik Watz Pulmonary Research Institute at Lungen Clinic Grosshandorf Airway Research Center North German Center for Lung Research Grosshansdorf, Germany Peter J. Wijkstra Department of Pulmonary Diseases/Home Mechanical Ventilation and Groningen Research Institute for Asthma and COPD University of Groningen University Medical Center Groningen Groningen, The Netherlands

João Carlos Winck Centro de Reabilitação do Norte/Centro Hospitalar Vila Nova de Gaia-Espinho Valadares, Portugal and Faculdade de Medicina do Porto Porto, Portugal Emiel F.M. Wouters NUTRIM School of Nutrition and Translational Research in Metabolism Department of Respiratory Medicine Maastricht University Medical Centre Maastricht, The Netherlands Abebaw Mengistu Yohannes Azusa Pacific University Department of Physical Therapy School of Behavioral and Applied Sciences Azusa, California Elisabetta Zampogna Istituti Clinici Scientifici Maugeri IRCCS Respiratory Rehabilitation of the Institute of Tradate (VA) Tradate (VA), Italy Martina Zappa University of Insubria Department of Medicine and Surgery Respiratory Diseases Varese-Como, Italy Richard L. ZuWallack Pulmonary and Critical Care St Francis Hospital (Trinity) Hartford, Connecticut and University of Connecticut School of Medicine Farmington, Connecticut and Frank H. Netter MD School of Medicine at Quinnipiac University North Haven, Connecticut

1

Part     The foundation of pulmonary rehabilitation

1 A framework for medical rehabilitation: Restoring function and improving quality of life 3 Julia Warden and Mark Bayley 2 Pulmonary rehabilitation: The development of a scientific discipline 11 Linda Nici and Roger S. Goldstein 3 Key concepts in pulmonary rehabilitation 21 Felipe V.C. Machado, Frits M.E. Franssen and Martijn A. Spruit 4 Enhancing use and delivery of pulmonary rehabilitation 29 Emily Hume, Carolyn L. Rochester and Ioannis Vogiatzis 5 Pathophysiological basis, evaluation and rationale of exercise training 41 Pierantonio Laveneziana and Paolo Palange 6 Education: Realizing the potential for learning in pulmonary rehabilitation 53 Felicity Blackstock and Suzanne C. Lareau 7 Self-management 63 Jean Bourbeau and Tanja W. Effing 8 Dual therapy: Pharmacologic management in pulmonary rehabilitation 75 J. Michael Nicholson and Richard Casaburi

1 A framework for medical rehabilitation: Restoring function and improving quality of life JULIA WARDEN AND MARK BAYLEY Introduction A unifying model for rehabilitation: The International Classification of Functioning, Disability and Health The approach to rehabilitation assessment Unpacking the black box of rehabilitation: Important service delivery considerations and questions

3 4 5

Measuring the benefits of rehabilitation Summary Conflict of interest References

7 8 8 8

6

KEY MESSAGES • The WHO International Classification of Functioning is a unifying framework for rehabilitation that addresses the impact of the health condition on body structure and function, activities of life and participation in usual roles. • Rehabilitation treatment is goal oriented, interdisciplinary and is focused on training

in remediation or compensation for an impaired function. • The ideal dose, timing, type, location and delivery model of rehabilitation should be based on the individual patient and address their unique physical, functional and psychological issues.

INTRODUCTION

and it complements the other three health strategies, that include prevention, cure and support (2). To put the four key health strategies in context, the primary goal of the preventive strategy is to prevent health conditions by targeting risk factors, for example controlling tobacco to reduce the incidence of lung cancer. The primary goal of the curative strategy is to cure health conditions, such as achieving remission in cancer, or controlling diseases such rheumatoid arthritis, typically with the use of biologically active medications. The primary goal of the rehabilitative strategy is to restore or optimize function, for example rehabilitating an individual with hemiparesis after stroke so that they are able to manage their basic activities of daily living independently, with or without the use of adaptive aids, and so that they are able to mobilize independently, whether it be with or without a gait aid. The primary goal of the supportive strategy is to optimize quality of life, for example achieving pain control and relieving mental distress in a palliative care patient with terminal cancer (1). Although conceptually distinct, the four health strategies have many related outcomes. For example, although the

Advances in curative medicine combined with demographic and epidemiological trends of population ageing and the shift to a higher incidence of chronic, non-­ communicable diseases, require that healthcare systems be able to respond to the increasing needs of people living with chronic conditions and impairments (1). Individuals are living longer, but with more disability, and what affects people more are the limitations in their independence and functioning arising from their health conditions. Rehabilitation is the field of medicine that focuses on improving quality of life and optimizing independence in daily activities. Based in the WHO International Classification of Functioning, Disability and Health (ICF), rehabilitation can be defined as the health strategy that aims to enable people with health conditions experiencing or likely to experience disability to achieve and maintain optimal functioning in interaction with the environment (2). It is a strategy that is relevant to all medical specialties and health professions (3),

3

4  A framework for medical rehabilitation: Restoring function and improving quality of life

rehabilitative strategy focuses on function, similar to the supportive strategy, it is also concerned with quality of life as a closely related outcome. Rehabilitation medicine also aims to optimize disease management, similar to the curative strategy, and it has a focus on minimizing the effects of the initial health problem, which is similar to a preventive strategy. The objective of this chapter, therefore, is to provide the reader with (a) a unifying model for the discipline of rehabilitation, (b) an overview of the rehabilitation assessment, (c) a peek into the black box of rehabilitation interventions and (d) insights into how rehabilitation outcomes are measured. It concludes with some of the key remaining research questions in the field.

or action by an individual, and activity limitation represents the difficulties an individual may have in executing activities. Participation is involvement in a life situations, and restriction refers to the problems an individual may experience with involvement in their usual roles in life, i.e. employment, parenting and recreation (4,5). Contextual factors in this model represent the complete background of an individual’s life and living and include two components: environmental factors (i.e. external to the individual) and personal factors (i.e. features of the individual such as gender, education or coping style). In the framework, an individual’s functioning in a specific domain is a complex interaction between their health condition and contextual factors (4).

A UNIFYING MODEL FOR REHABILITATION: THE INTERNATIONAL CLASSIFICATION OF FUNCTIONING, DISABILITY AND HEALTH

The discipline of rehabilitation

The ICF is the WHO framework for measuring health and disability of individuals and populations. It has been described as a unifying model for the conceptual description of the rehabilitation strategy (2). The overall purpose of the classification is to provide a framework for the description of health and health-related states, and the unit of classification is categories within health and health-related domains (4). The classification is based on a model of functioning and disability (Figure 1.1), in which Health condition is defined as a disease (acute or chronic), disorder, injury or trauma. Functioning is an umbrella term for body functions and structures and activities, and Participation signifies the positive aspects of the complex interaction between an individual with a health condition and their contextual factors (environmental and personal factors). Disability is an umbrella term for impairments, activity limitations and participation restrictions. It refers to the negative aspects of the classification. Body function and structure refers to physiological functions and anatomic parts of the body systems, and impairment is a loss or deviation from normal body functions and structures. Activity is the execution of a task

Rehabilitation is an inter-professional discipline, which deals with various disease entities with functional limitations (3). The first step involves diagnosing health conditions and assessing their impact on body structures, functioning and participation. Treatment can be focused on remediation, which refers to reducing impairments and stabilizing, improving or restoring function. However, in many cases it is focused on compensation for the absence or loss of body functions and structures, such as by providing assistive technology or teaching the individual to change their environment. Regardless of which of these two treatment approaches is most predominant, an individual will need training in remediation and/or compensation techniques to improve their function and wellbeing (Figure 1.2).

The rehabilitation team In rehabilitation, the various practitioners on the team work collaboratively to assist individuals in achieving as much independence as possible based on personal goals. The interdisciplinary rehabilitation team frequently includes: ●●

Medical doctors, for example those who specialize in physical medicine and rehabilitation, such as in stroke, brain injury, spinal cord, musculoskeletal or amputee

Health condition

Body functions and structures

Activity

Environmental factors

Participation

Personal factors

Figure 1.1 Interactions between the components of the International Classification of Functioning, Disability and Health (ICF) (4).

Remediation

Compensation

Training

Figure 1.2  Rehabilitation management.

The approach to rehabilitation assessment  5

●●

●●

●●

●●

●●

●●

rehabilitation; or respirologists, such as in pulmonary rehabilitation. In addition to physicians, the medical team often includes a registered nurse and a pharmacist. Physiotherapists, who, in pulmonary rehabilitation for example, are instrumental in assessing baseline exercise tolerance and lower limb strength as well as supervising exercise programmes and the use of breathing techniques. Occupational therapists, who play a role in assessing an individual’s ability to perform their activities of daily living and in assessing their upper body mobility, strength and endurance. They usually guide the individual in an upper extremity exercise programme, train them in energy conservation and relaxation, and often provide recommendations on home modifications and equipment or adaptive aids that can improve an individual’s safety and independence. A dietician, who assesses nutritional intake, guides nutritional supplementation as needed, provides counselling on the importance of a healthy diet and suggests modifications of eating habits as appropriate. A psychologist, who is important in addressing symptoms of depression or anxiety, which are common in individuals with chronic disease. Respiratory therapists, who will help optimize the use of prescribed interventions. Speech language pathologists, who have expertise in swallowing and communication.

Other members of the rehabilitation team to whom an individual may have access include social workers, behavioural therapists, recreational therapists and speechlanguage pathologists. The key is that all members of the interdisciplinary team work closely together to assess the patient, identify goals, implement treatment, assess progress and provide recommendations on transition from the programme to the next stage of their treatment/recovery, for example to a different rehabilitation setting, or on to a self-maintenance programme. The team members meet at regular intervals to evaluate the patient’s progress and to adjust the collaborative treatment plan as needed (5,6).

THE APPROACH TO REHABILITATION ASSESSMENT Functional history Consistent with the ICF, the traditional clinical history taken by a clinician with regard to the disease symptoms will be augmented by rehabilitation clinicians to examine the impact of the disease on functioning in three key domains of activities of daily living. The most basic level functioning is self-care abilities such as dressing, eating, mobility, transfers, hygiene and bowel and bladder continence. The next domain, instrumental activities of daily

living (IADL), concerns the skills involved in living in the community such as banking, meal planning, grocery shopping and driving. Perhaps the most challenging domain to recover is vocational and avocational pursuits that can be impaired by disease.

Assessing the individual’s environment The ICF highlights the importance of evaluating the person’s environment. Just as an astronaut cannot function in the hostile environment of space without a specialized suit, an individual’s environments can dictate their limitations. Clinicians must inquire into the individual’s living quarters, places of work and accessibility of their community. This may include examining the steps required for entry, size of kitchen or bathroom or walking distances for entry into buildings.

Assessment of the individuals psychological adjustment to disability Diseases and their concomitant limitations can undermine an individual’s psychological resources, causing anxiety and depression. Inquiring into an individual’s current understanding of the disease prognosis and their feelings about them is critical. Furthermore, premorbid coping style may be critical to rehabilitation. A coping style characterized by an orientation to problem solving appears to be more effective than one characterized by attributing outcomes to external factors. It is helpful to ask questions about how individuals have coped with previous life setbacks. Interestingly, in contrast to death, many individuals may have a form of ‘mobile grieving’ in that they are not imminently dying but grieve about the loss of previous abilities and preferred activities. Individuals may be found in any of the stages originally identified by Kubler-Ross (7) on any given day during their rehabilitation and don’t necessarily follow them in a linear fashion, i.e. denial (‘I don’t need to use that oxygen compensation yet’), bargaining (‘If I work hard in therapy, I will get better’), anger (‘I would have got better if I had better medication for my airways’), depression (‘I cannot live like this’) and acceptance. Rehabilitation clinicians are well advised to take note of the patient’s coping style and stage of adjustment as they engage in goal setting and treatment discussions while maintaining a realistic view of the prognosis.

Identification of patient’s goals Given the many possible foci of rehabilitation and the potential investment of time, it is essential that clinicians meet with patients and identify specific, measurable, achievable, realistic and time-limited (SMART) goals. This process of negotiation ensures individualized rehabilitation.

6  A framework for medical rehabilitation: Restoring function and improving quality of life

UNPACKING THE BLACK BOX OF REHABILITATION: IMPORTANT SERVICE DELIVERY CONSIDERATIONS AND QUESTIONS 1. Who benefits most and how to manage the referral/triage process to maximize access. Because many conditions will recover naturally, rehabilitation interventions must be measured against the normal pattern of recovery to determine if interventions expedite recovery over the natural rate of recovery. This raises important methodological questions for research and also has important ramifications for resource utilization and selection of patients for interventions. For example, mildly impaired patients may improve with simple instructions to perform their usual activities within the limits of tolerance and may not require specialized intervention. Conversely, extremely disabled individuals may not be able to fully participate, and rehabilitation may not change their ultimate outcome. In many conditions, rehabilitation is most beneficial to those with moderate disability who require the specialized intervention to make appropriate gains in therapy, but ongoing research is needed. This gap in evidence remains an important area for research in the rehabilitation field for many conditions. 2 . What is the ideal setting of delivery? Rehabilitation can be provided in a number of settings. Table 1.1 outlines considerations for feasibility of services, costs, availability of

professional expertise and effectiveness in certain subpopulations (8). 3. How should the rehabilitation team work together? a. Single service versus multidisciplinary: Some individuals may benefit from only single service; however, because of the multifaceted nature of most disabling conditions with physical, functional and emotional consequences, multidisciplinary teams are frequently important models of care. b. Group versus individual therapy: Many interventions can be offered in groups to improve feasibility and reduce costs. c. Goal setting processes: These are critical in developing a rehabilitation programme that is tailored to the individual’s personal situation and environment. Ideally, a member of the team should negotiate directly with the patient. d. Team communication and coordination processes: In  more complex patient cases, interprofessional team meetings are efficient ways of making sure that the treatment is consistent and tailored to the individual. Many teams structure their conferences around discussion of progress towards achievement of the key goals. e. Optimal complement of team members: The more complicated the disability is, the more specialized the training and inherent competencies required of the rehabilitation providers. The more routine the exercise and tasks required, the less specialized

Table 1.1  Advantages and disadvantages of rehabilitation setting Setting Inpatient rehabilitation

Outpatient hospital clinic

Community clinic Domiciliary (home-based)

Telerehabilitation

Advantages/optimal use • For most severely disabled who are unable to perform their self-care • Reduces mortality and institutionalization in complex populations such as hip fracture, stroke and spinal cord injury • Ensures continuity from inpatient to outpatient rehabilitation • Easy access to specialized equipment and expertise • Easy access to specialized equipment and expertise • Closer to patient’s home • Effective where individual needs to practice tasks in their own environment and allows for generalization of skills • Convenience for patients without transportation or family caregivers • Widespread adoption of mobile technology • Reduced travel costs for all • Potential to work with groups of patients • Remote monitoring of vital signs/effort • Monitors provide instant feedback and may reinforce achievement

Disadvantages • Most expensive • Risks of hospitalization including nosocomial infections etc. • Less opportunity for practice in patient’s environment • Transportation issues for patients • Less practice in own environment

• Reduced continuity from inpatient • Less practice in own environment • Reduced access to specialized equipment • Cost/Time for travel for professionals especially in rural settings

• Providers’ and patients’ discomfort with technology • No hands-on therapy and reduced ability to prevent falls • Technological failures • Access to special equipment • Confidentiality concerns

Measuring the benefits of rehabilitation  7

rehabilitation provider required. An example of this is the decision regarding whether a physiotherapy assistant or kinesiologist could provide therapy, or whether a more skilled and more expensive physical therapist is required. 4. What is the ideal timing of rehabilitation? In general, rehabilitation provided immediately after an individual achieves medical stability has been considered optimal. However, there are instances where intensive treatment extremely early after onset of illness or injury may not be ideal because of persistence of inflammatory factors and other considerations (34). Therefore, the question of the ideal time to intervene remains, in many disease populations, an unanswered question. Furthermore, for conditions such as respiratory disease likely to have exacerbations and remissions, the question remains as to whether intermittent rehabilitation may be important, as does the role of maintenance rehabilitation to maintain function in the face of ageing and deterioration. 5. What is the optimal dose and intensity of rehabilitation? In order to make changes at the cardiovascular and muscular levels, there needs to be a degree of effort or physiologic stress to induce change. Many exercise and skill acquisition studies have been completed in normal humans and not necessarily replicated in the context of individuals with disease. Therefore, the ideal number of task repetitions required to acquire new skills and capacity is not completely known. 6. What is the best mix of types of intervention in rehabilitation? Interventions can be classified as physical, functional, psychological, educational and medical. a. Physical approaches are perhaps the most common types of interventions that come to mind when thinking about rehabilitation. They include exercise, task-specific practice and training for skill acquisition. Also included are other types of physical interventions such as bracing, topical modalities for pain relief and range of motion such as ultrasound, laser or electrical stimulation to enhance muscle function. b. Biologic/Medical adjuncts to exercise: With the emergence of increasing awareness of genetic and other growth-stimulating agents, there is increasing opportunity to combine medications and biologically active compounds that might enhance muscle growth and joint function as well as neurological function when combined with practice and exercise. Furthermore, optimal medical management of issues such as pain and inflammation allows for better participation in rehabilitation activities (9,10). c. Psychological interventions are increasingly recognized as key components of the rehabilitation treatment. Cognitive behavioural interventions directed at optimizing psychological adaptation, reduction of anxiety and reframing the disability and its meaning for the individual are important strategies for enhancing the outcomes of other training. It may also be necessary to address the mild cognitive

impairment from small vessel disease of the brain that is frequently associated with cardiorespiratory disease. Mildly impaired cognition should not be a reason for exclusion from rehabilitation (11). Most cognitive assessment examines explicit learning which, for example, in the context of a mental status examination does not tap into the same learning systems involved in training to learn a task. Limiting access to rehabilitation based on performance on cognitive assessment may result in individuals not having access to rehabilitation who, with appropriate adaptations to the training, could benefit from multiple practice opportunities. d. Patient and family educational interventions are recognized as important elements of the self-­ management approach so that the individual can recognize patterns and manage their condition autonomously without healthcare professionals. Teaching individuals how to use their medications, assistive technology and how to adapt their activities in the community and plan ahead for their condition can be helpful in reducing fear as well as d ­isability. Caregiver education has also been frequently recognized as an important driver of rehabilitation outcomes. The presence of a healthy caregiver has been associated with better response to exercise, increased chance of discharge home and a range of other outcomes (12–16). 7. What is the role of assistive devices and technology? There are many types of devices that may help compensate for lost functions. Mobility assistive devices such as canes, walkers and wheelchairs are the most commonly prescribed devices, however there are many other technologies that may enhance function including orthotics to support joints, electrical stimulation and environmental modifications such as lifts, ramps and grab bars (5).

MEASURING THE BENEFITS OF REHABILITATION In keeping with the ICF, rehabilitation outcomes can be measured in a number of ways that reflect the various domains. The activity level has a number of domains of importance and is often more sensitive to rehabilitation interventions even when the bodily impairment has not changed. For example, after pulmonary rehabilitation, individuals may walk at a faster rate of speed or have enhanced endurance before stopping despite less obvious changes in body functions (Table 1.2). An emerging and critical element in the current fiscal environment where most healthcare systems are struggling with sustainability is that rehabilitation must be evaluated from an economic point of view (27–33). An important outcome of interventions is to measure the reduction in healthcare utilization, and economic loss due to time off work for patients and/or caregivers. These costs are significant, and rehabilitation activities directed at independence

8  A framework for medical rehabilitation: Restoring function and improving quality of life

Table 1.2  Examples of outcome measures ICF domain Body structure and function Activity

Participation Quality of life (QOL)

Examples Impaired ventilation Impaired muscle strength Disease stability Self-care Mobility Instrumental activities of daily living Vocational Extent of participation in usual roles in society Individual’s perception of healthrelated impact on QOL

Examples of potential measures Spirometry, oximetry Manual muscle grade Mortality Barthel Index or Functional Independence Measure (FIM) (17–20) 6-minute walk tests (21) Timed Up and Go (22) Nottingham Extended Activities of Daily Living (EADL) (23,24) Return to work status London handicap scale Perceived handicap questionnaire (25) EuroQol 5-Dimension Short Form-36 questionnaire (26)

could potentially significantly reduce such costs, resulting in a demonstrable benefit of rehabilitation and reducing the length of time that a person is disabled.

Persisting rehabilitation research questions Despite the emerging science of rehabilitation, there remain many unanswered questions: What is the ideal/ minimally effective dose and intensity of rehabilitation interventions? What is the optimal interprofessional model of care? What is the ideal balance between compensation and remediation? What is the sustainability of rehabilitation benefits? Does comprehensive self-management education work? How could tele-rehabilitation and artificial intelligence reduce the cost of rehabilitation? How can biologically active agents enhance the benefits of rehabilitation? How do we manage those with other comorbid conditions? To answer these important questions, we still need well-designed randomized controlled trials and active control groups, because any attention directed towards these patients may result in improvements, regardless of their nature.

SUMMARY In conclusion, the ICF provides a unifying framework for rehabilitation to frame assessment, treatment planning and outcome measurement. There are a number of key elements of rehabilitation including physical, educational, psychological, compensatory technology and medical approaches. The rest of this book will delve into how this framework informs the specifics of approaches to pulmonary rehabilitation. Source(s) of support. None.

CONFLICT OF INTEREST The authors have no conflicts of interest to declare related to this material.

REFERENCES 1. Stucki G, Bichenback J, Gutenbrunner C, Melvin J. Rehabilitation: The health strategy of the 21st century. J Rehabil Med. 2018;50:309–16. 2. Stucki G, Cieza A, Melvin J. The international classification of functioning, disability and health: A unifying model for a conceptual description of the rehabilitation strategy. J Rehabil Med. 2007;39:279–85. 3. Gutenbrunner C, Meyer T, Melvin J, Stucki G. J Rehabil Med. 2011;43:760–4. 4. World Health Organization. International Classification of Functioning, Disability and Health: ICF. World Health Organization; Geneva; 2001. 5. Stucki G, Kostanjsek N, Ustun B, Ewert T, and Cieza A. In: Frontera W, DeLisa J, Gans B, Walsh N, Robinson L, editors. DeLisa’s Physical Medicine and Rehabilitation: Principles and Practice, 5th ed. Lippincott Williams & Wilkins; 2010. pp. 301–24. 6. Cifu D, Kaelin DL, Kowalske JL et  al. Braddom’s Physical Medicine and Rehabilitation, 5th ed. Elsevier; 2016. 7. Kübler-Ross E. On Death and Dying. Routledge, ISBN 0-415-04015-9. 1969. 8. Furlan AD, Irvin E, Munhall C et al. Rehabilitation service models for people with physical and/or mental disability living in low- and middle-income countries: A systematic review. J Rehabil Med. 2018 Jun 15;50(6):487−98. 9. Dobkin BH. Behavioral, temporal, and spatial targets for cellular transplants as adjuncts to rehabilitation for stroke. Stroke. 2007 February 1;38(2):832–9. 10. Hansen MJ, Gualano RC, Bozinovski S, Vlahos R, Anderson GP. Therapeutic prospects to treat skeletal muscle wasting in COPD (chronic obstructive lung disease). Pharmacol Ther. 2006 January 1;109(1-2):​162–72. 11. Schou L, Østergaard B, Rasmussen Ls et al. Cognitive dysfunction in patients with chronic obstructive pulmonary disease--A systematic review. Respir Med. 2012 Aug;106(8):1071–81.

References 9

12. Cameron JI, Herridge MS, Tansey CM, McAndrews MP, Cheung AM. Well-being in informal caregivers of survivors of acute respiratory distress syndrome. Crit Care Med. 2006 January 1;34(1):81–6. 13. Cameron JI, Chu LM, Matte A et  al. One-year outcomes in caregivers of critically ill patients. N Engl J Med. 2016 May 12;374(19):1831–41. 14. Pereira S, Ross Graham J, Shahabaz A et al. Rehabilitation of individuals with severe stroke: Synthesis of best evidence and challenges in implementation. Top Stroke Rehabil. 2012 March 1;19(2):122–31. 15. Farquhar M. Carers and breathlessness. Curr Opin Support Palliat Care. 2017 September 1;11(3):165–73. 16. Figueiredo D, Cruz J, Jácome C, Marques A. Exploring the benefits to caregivers of a family-oriented pulmonary rehabilitation program. Respir Care. 2016 August 1;61(8):1081–9. 17. Wade DT, Collin C. The Barthel ADL Index: A standard measure of physical disability? Int Dis Stud. 1988;10:64–7. 18. Lorenzi CM, Cilione C, Rizzardi R et al. Occupational therapy and pulmonary rehabilitation of disabled COPD patients. Respiration. 2004;71(3):246–51. 19. Linacre JM, Heinemann AW, Wright BD, Granger CV, Hamilton BB. The structure and stability of the functional independence measure. Arch Phys Med Rehabil. 1994 February 1;75(2):127–32. 20. Pasqua F, Biscione GL, Crigna G et  al. Use of functional independence measure in rehabilitation of inpatients with respiratory failure. Respir Med. 2009 March 1;103(3):471–6. 21. Enright PL. The six-minute walk test. Respir care. 2003 August 1;48(8):783–5. 22. Podsiadlo D, Richardson S. The timed ‘Up & Go’: A test of basic functional mobility for frail elderly persons. J Am Geriatr Soc. 1991 February;39(2):142–8. 23. Nouri FM, Lincoln NB. An extended activities of daily living scale for stroke patients. Clin Rehabil. 1987;​ 1:301–30. 24. Okubadejo AA, O’Shea L, Jones PW, Wedzicha JA. Home assessment of activities of daily living in patients with severe chronic obstructive pulmonary disease on long-term oxygen therapy. Eur Respir J. 1997 July 1;10(7):1572–5.

25. Perenboom RJ, Chorus AM. Measuring participation according to the International Classification of Functioning, Disability and Health (ICF). Disabil Rehabil. 2003 January 1;25(11–12):577–87. 26. Ware JE, Jr, Sherbourne CD. The MOS 36-Item short-form health survey (SF-36). Med Care. 1992;​ 30:473–82. 27. Stenberg U, Vågan A, Flink M et al. Health economic evaluations of patient education interventions a scoping review of the literature. Patient Educ Couns. 2018 June 1;101(6):1006–35. 28. Rutten-van Mölken MP, Oostenbrink JB, Tashkin DP, Burkhart D, Monz BU. Does quality of life of COPD patients as measured by the generic EuroQol fivedimension questionnaire differentiate between COPD severity stages? Chest. 2006 October 1;130(4):​ 1117–28. 29. Golmohammadi K, Jacobs P, Sin DD. Economic evaluation of a community-based pulmonary rehabilitation program for chronic obstructive pulmonary disease. Lung. 2004 August 1;182(3):187–96. 30. Goldstein RS, Gort EH, Guyatt GH, Feeny D. Economic analysis of respiratory rehabilitation. Chest. 1997 August 1;112(2):370–9. 31. Dritsaki M, Johnson-Warrington V, Mitchell K, Singh S, Rees K. An economic evaluation of a self-management programme of activity, coping and education for patients with chronic obstructive pulmonary disease. Chron Respir Dis. 2016 February;13(1):48–56. 32. Celli BR, Decramer M, Wedzicha JA et al. An official American Thoracic Society/European Respiratory Society statement: Research questions in chronic obstructive pulmonary disease. Am J Respir Crit Care Med. 2015;191(7):e4–e27. 33. Stellefson M, Alber J, Paige S, Castro D, Singh B. Evaluating comparative effectiveness research priorities for care coordination in chronic obstructive pulmonary disease: A community-based eDelphi study. JMIR Res Protoc. 2015;4(3):e103. 34. Bernhardt J, Langhorne P, Lindley RI et  al. AVERT Trial Collaboration group, Efficacy and safety of very early  mobilisation within 24 h of stroke onset (AVERT): A randomised controlled trial. Lancet. 2015;​ 386:46–55.

2 Pulmonary rehabilitation: The development of a scientific discipline LINDA NICI AND ROGER S. GOLDSTEIN Introduction Definition and concepts History

11 11 12

Summary References

17 17

KEY MESSAGES • PR is the recommended standard of care for many chronic respiratory conditions. • PR increases exercise tolerance and self-efficacy. • PR reduces dyspnoea, fatigue, anxiety and depression.

• PR reduces unscheduled medical visits and hospitalizations. • Issues of access and capacity have resulted in a variety of delivery options. • PR is being augmented by music, dance, yoga and other creative options.

INTRODUCTION

education, designed to effect behavioural change. However, its implementation varies worldwide depending on local resources, healthcare system characteristics as well as the individual needs and goals of the patient. Despite this variability, the American Thoracic Society (ATS)/European Respiratory Society (ERS) has published a broad definition of pulmonary rehabilitation as follows: ‘Pulmonary rehabilitation is a comprehensive intervention based on a thorough patient assessment followed by patient-tailored therapies which include, but are not limited to, exercise training, education and behaviour change, designed to improve the physical and emotional condition of people with chronic respiratory disease and to promote the long-term adherence to health-enhancing behaviours’ (1). This definition highlights several important concepts. PR combines multiple different therapies that could and often should be given as part of good medical care. However, this intervention is much more than the sum of its parts. It must be individualized to the unique needs of the patient as determined by the diagnosis and severity of the respiratory disease itself, the presence of co-existing conditions, systemic manifestations and the psychological and social framework within which the patient operates. While exercise training remains the cornerstone of PR, in itself it is not sufficient to

Pulmonary rehabilitation (PR) is recommended by many medical professional societies as part of the standard medical management for patients with chronic respiratory disease. Using criteria-based patient selection, exercise training, self-management and educational and psychosocial interventions, significant benefits include improvements in exercise tolerance and health-related quality of life (HRQL) as well as reductions in symptom burden and healthcare utilization (1). PR as a distinct intervention was recognized in the 1960s. Since then, its effectiveness has been confirmed from anecdotal evidence of success through well-controlled clinical trials that have employed valid outcomes for exercise and HRQL. Emerging data now supports PR as effective for a wide spectrum of respiratory diseases across the range of disease severity and in non-traditional settings. This chapter reviews the history, evolution and maturation of PR as a scientific discipline.

DEFINITION AND CONCEPTS PR is a complex intervention, containing as its core the components of exercise training and self-management

11

12  Pulmonary rehabilitation: The development of a scientific discipline

provide optimal and long-term benefits. It must be coupled with educational efforts aimed at promoting self-management skills and positive health behaviour change. The initial more intensive programme should be followed by a maintenance phase, which ideally would be lifelong. Optimal treatment of the often-complex patient with chronic respiratory disease requires seamless care across settings and providers over the course of the disease. PR is designed to provide the most appropriate therapy for the right individual at the right time. Therapies may include smoking cessation, vaccination, regular exercise and physical activity in the home as well as in the community, fostering collaborative self-management strategies, use of supplemental oxygen, optimizing pharmacotherapy and medication adherence and, when needed, assisting patients with end-of-life decision making including palliative care and hospice services. Such an approach requires partnering for coordinated communication among health care providers, patients and their families (2,3).

HISTORY Some of the components of PR have been part of good medical care for centuries. During the mid-nineteenth century, organized programmes of sunshine, rest and nutrition were found to benefit patients with tuberculosis residing in European sanatoria (4). Soon after, it became clear that the judicious use of supervised exercise in between resting enabled patients to feel better. The first sanatorium in the United States opened in the Adirondacks in 1885, and the first Canadian sanatorium in Muskoka in 1897 (5). In addition to intensive hospital-based management, it was clear that ongoing management should include home support which became an early feature of chronic disease

management (Figure 2.1). In the 1950s and 1960s, clinicians became aware of physiological approaches such as diaphragmatic and pursed lip breathing (6) for those with chronic obstructive pulmonary disease (COPD) as well as the importance of organizing individual components of care into a comprehensive programme (7–9). Combined interventions, such as breathing and bronchial hygiene techniques, walking exercises and supplemental oxygen were first reported in the form of non-controlled or historically-controlled studies by enthusiastic pioneers such as Alvin Barach and Thomas Petty (10–12). PR was given its first official definition in 1974 by the American College of Chest Physicians, and in 1981 the ATS published its first official statement on PR (13,14). Although today the ATS plays a huge role in teaching, research and clinical care of patients with respiratory disease, critical illness and sleep disorders, it was actually established in 1905 as the American Sanatorium Association to address the management of those with tuberculosis. As part of the growth in PR and for some patients with severe disease, the application of supplemental oxygen was essential for reducing dyspnoea, increasing exercise tolerance and reducing mortality. Alvin Barach played a key role in the early use of supplemental oxygen through his invention of the ‘oxycane’ (15) (Figure 2.2). The subsequent landmark trials of oxygen therapy for patients with resting hypoxaemia established criteria for the prescription of supplemental oxygen in many jurisdictions across the world (16,17). Supplemental oxygen for those with resting hypoxaemia was noted to be life extending. However, the role of oxygen for isolated exercise desaturation or for isolated nocturnal desaturation still remains to be clarified. Notwithstanding early reports of success, the field of PR moved very slowly in the absence of well-defined,

Figure 2.1  An early visiting nurse offering home care for the supervision of patients with chronic tuberculosis. (From Baston A. Curing Tuberculosis in Muskoka. Canada’s First Sanatoria. Canada: Old Stone Books Limited; 2013, with permission.)

History 13

Mouthpiece Push button Transfilling orifice

Handle

Oxygen cane

Figure 2.2 Introduction of the oxygen cane by Alvin Barach, one of the forerunners of modern-day long-term oxygen therapy. (From Barach AL. Dis chest. 1959;35:229– 41, with permission.)

valid outcome measures that could assist with quantitative research essential for PR to become an evidence-based clinical intervention. In many respects this mirrors the development of many medical interventions in that widespread adoption follows appropriately designed clinical trials that provide indisputable evidence of effectiveness. The development of timed walking tests in 1976 (18) and their adaptation for COPD (19), as well as the development of field shuttle exercise testing (20–22) were key. The ­creation of disease-specific, patient-centred quality-of-life questionnaires for COPD (such as the Chronic Respiratory Questionnaire in 1987 and the St George’s Respiratory Questionnaire in 1992) (23–25) fuelled further interest in PR. Goldstein and colleagues published the first prospective randomized trial of 89 patients with severe COPD reporting improvements in exercise tolerance and health-related quality of life (HRQL) (26) soon to be followed by other highly successful trials (27–29). Such trials provided Grade 1A evidence of the improvements in dyspnoea, exercise and HRQL following PR.

It should be noted that in addition to its positive effects on symptoms and exercise capacity, PR was first shown by Emery and colleagues (30) to enhance cognitive function and improve psychological wellbeing, an observation subsequently confirmed in a recent systematic review (31) (Figure 2.3). In 1991, Casaburi and colleagues demonstrated that patients with COPD had a dose-dependent physiologic effect from exercise training (32), similar to conventional exercise training in healthy individuals. This study, although small, was groundbreaking since the conventional wisdom until then was that COPD patients were so ventilatory limited that their training could not be at a sufficiently high load to achieve physiologic benefits. In 1995, Ries and colleagues, in a study of 119 subjects, demonstrated that, compared to education alone, PR in addition to improving symptoms and exercise tolerance also improved self-efficacy for walking (33). Studies soon confirmed that COPD-associated peripheral muscle dysfunction, predominantly the consequences of disuse, can improve with exercise training. Maltais and colleagues reported on biopsy findings of the vastus lateralis muscle before and after endurance exercise training. The authors noted the increase in oxidative enzymes, such as citric synthetase, post training and also the inverse relationship between the increased oxidative enzymes and the decrease in lactic acid post training (34). In addition to the benefits seen in patient-centred outcomes, a study by Griffiths and colleagues in 2000, showed that, compared to usual care, clinically stable patients who underwent PR had reduced healthcare utilization, representing a cost saving attributable to this intervention. Although the number of hospital admissions did not change between study and control groups, the length of hospital stay was shorter in the PR group. Moreover, although the between-group difference in HRQL diminished over a year, the reduction in healthcare utilization persisted (35,36). Another important development in PR was the introduction of the minimal clinically important difference (MCID) which enabled test interpretation to extend beyond statistics to measure positive or negative changes identified by patients, which might result in a recommendation for or against a particular therapy (37). Subsequent trials tend to refer to the MCID when reporting results, although the MCID itself varies for a particular measure depending on how it is established. Traditionally, the educational component of PR was through didactic teaching. Recognition that the benefits of PR diminished over time, coupled with intense interest in how patients learn and are able to incorporate healthy behaviours, led to the active investigation and impressive results demonstrating that self-management education, including an action plan post-PR as well as active case management, led to a substantial reduction in healthcare utilization over the subsequent year. This multicentre study showed decreased unscheduled primary care and emergency department visits as well as decreased hospitalizations (38). These benefits of disease management have been confirmed in larger randomized trials in Europe and North America

14  Pulmonary rehabilitation: The development of a scientific discipline

Meta-analysis PR for anxiety and depression SMD (random) 95% CI

Study Anxiety Emery 1988 Griffiths 2000 Guell 2006 Subtotal (95% CI) Test for overall effect: Z = 2.67 (P = .008)

–4

–2

0

2

4

SMD (random) 95% CI

Study Depression Emery 1988 Griffiths 2000 Guell 2006 Subtotal (95% CI) Test for overall effect: Z = 3.24 (P = .001)

–4

–2

0

2

4

Figure 2.3  Meta-analysis of the impact of PR on anxiety and depression. (Adapted from Coventry PA, Hind D. J Psychosom Res. 2007;63(5):551–65.)

(39, 40) (Figure 2.4). A systematic review has pointed out that although it is likely that self-management is associated with a reduction in hospital admissions, the heterogeneity of study interventions and outcome measures reported make it very difficult to formulate clear recommendations regarding its best form and content (41). In 2001, the Global Initiative for Obstructive Lung Disease (GOLD) endorsed PR as standard therapy for

COPD, and in 2003 placed this intervention prominently in their treatment algorithm for stable COPD (42). PR is recommended in all major guidelines and statements for the treatment of COPD and other chronic respiratory diseases such as pulmonary fibrosis, pulmonary hypertension, asthma and cystic fibrosis (1). While it has traditionally been provided to stable patients with moderate-to-severe COPD in an outpatient setting,

Disease management reduces hospital admissions and ED visits 1

Mean admissions and ED visits

0.9 0.8 0.7 0.6

Usual care

0.5 0.4 0.3 0.2 Disease management

0.1 0 0

50

100

150

200

250

300

350

Figure 2.4  Disease management reduces hospital admissions and ED visits. (Adapted from Rice KL, Dewan N, Bloomfield HE et al. Am J Respir Crit Care Med. 2010;182:890–6.)

History 15

Meta-analysis early PR following acute exacerbation of COPD CRQ dyspnoea

Hospital readmission

0.002

0.1

Rehabilitation

1

10

500

–2

Control

–1

Control

0

1

2

Rehabilitation

6MW

–200 –100 Control

0

100 200 Rehabilitation

Figure 2.5  Meta-analysis of the impact of early post exacerbation PR. (From Puhan MA, Gimeno-Santos E, Scharplatz M et al. Cochrane Database Syst Rev. 2011:CD00530522, with permission.)

today there is also interest in the application of PR in other settings and at different acuities. A number of trials have reported that PR in the ICU reduces the time of ventilation, length of stay and effectiveness of recovery (43, 44). As a result, PR is now included as part of management of the critically ill patient with respiratory disease. Mann and colleagues have reported on the improved function and quality of life resulting from PR immediately post exacerbation, and a subsequent Cochrane review suggested a decrease in hospital readmissions and perhaps even reduced mortality associated with early PR (45,46) (Figure 2.5). COPD patients with less severe disease may also stand to benefit from PR. In a randomized controlled trial of 199 patients with moderate COPD (FEV1 60 (16)%) predicted, van Wetering and colleagues noted improvements in favour of the intervention group at the end of a 4-month intensive PR programme, followed by a 20-month maintenance programme. However, it should be noted that this was a long programme and the differences observed were small, most being below the MCID for the outcome measures used (47). Growth in the applicability and interest of PR has led to broadening interest in alternative settings, programme adjuncts, and complementary approaches. Pulmonary rehabilitation in the home or community setting results in similar reductions in dyspnoea and improvements in exercise capacity as do centre-based programmes. In 2008, Maltais and colleagues reported on a randomized controlled noninferiority trial of 252 patients receiving the same initial education but then randomized to 8 weeks of home versus outpatient PR. The authors reported non-inferiority in changes to HRQL and concluded that PR at home may be

a useful alternative to institutionally-based programmes (48). In 2011, these observations were extended in an evaluation by Stickland (49) of the use of telehealth technology to deliver PR at satellite sites. In a non-inferiority trial, 147 COPD patients enrolled in PR in rural areas were compared with 262 who had undergone PR at the central site. Each group received the same educational programme 2 days a week and exercised for 8 weeks under direct local supervision. The authors reported similar improvements in HRQL and in field exercise (12-minute walk) concluding that telerehabilitation may be a useful way of increasing access and capacity to PR in rural areas. Over the last 20 years there has been tremendous growth and inquiry in the field of PR. Programmes can be lengthened (50) or repeated (51) with good effect. Conventional exercise can be delivered through constant power endurance training or by interval training with similar results (52). Exercise can be augmented by reducing the work of breathing using positive pressure support (53), using hyperoxic gas mixtures (54), by replacing nitrogen with a mixture of helium and oxygen (55) (Figure 2.6) or by partitioning of muscle through one-legged exercise (56). For those with very severe disease, muscle function can be augmented through neuromuscular stimulation (57) and for others with severe exercise and balance impairment, whole body vibration may be beneficial (58). Conventional rehabilitation exercises may be augmented through music (59), singing (60), Tai Chi (61), yoga (62), dance (63) and active video games (64). It has become clear that although PR is a key contributor to the management of chronic respiratory conditions, it should not be an isolated intervention but part of comprehensive

16  Pulmonary rehabilitation: The development of a scientific discipline

What could happen to you: Adjuncts to exercise training

Heliox

H O X

L I

Y G

Hyperoxia

E

E

U M

N

Ventilatory assist Electrical muscle stimulation Figure 2.6  Adjuncts to exercise training. What could happen to you. Cartoon of some of the possibilities.

integrated collaborative care (65). Such an approach promotes communication among the healthcare providers in the community and hospital environments, with early and ongoing engagement of primary care and specialist physicians, the patient and the care manager. A number of studies have reported favourable outcomes using this approach first

Symptom monitoring Medical management

+ Decisionmaking

Action plan

Exacerbation management

+

Enhanced self-efficacy Problem solving Resource utilization Collaboration Emotional management Role management Goal setting

Knowledge

+

+

Skill acquisition, includes unsupervised exercise

External motivation Social interaction

+

Pulmonary Rehabilitation

Selfmanagement

Education

+

described by Wagner and colleagues (65, 66) but greater care integration is still required. Wagg and colleagues presented a schematic of where PR might fit within the spectrum of care that begins with an action plan post exacerbation through to include education, self-management, pulmonary rehabilitation and integrated care (67) (Figure 2.7).

+

Supervised exercise

Ongoing support

Integrated Care

+

Maintenance through care management

Figure 2.7  A spectrum of support for chronic obstructive pulmonary disease. (Modified from Rice KL, Dewan N, Bloomfield HE et al. Am J Respir Crit Care Med. 2010;182:890–6.)

References 17

Progress in pulmonary rehabilitation

2020

2010

Collaborative integrated care Augmentation of conventional PR Increasing access and capacity Adjuncts to exercise training PR in various settings The role of co-existing conditions

2000

Recognition by medical professional societies

1990

Formal evidence of effectiveness. Underlying principles of PR

1980

Recognition and use by enthusiastic pioneers

Figure 2.8  Past and future of pulmonary rehabilitation.

SUMMARY Pulmonary rehabilitation has evolved from anecdotal experience to being a recommended treatment for all patients with chronic respiratory conditions who remain symptomatic or have a decreased functional status despite otherwise optimal medical management. Formerly only offered to stable patients with severe disease, it is showing benefits during critical illness and early post exacerbation. Locations can be hospital based, community based, home based and at distance using tele-rehabilitation through satellite sites. Components of PR are being refined to individualize benefit and several adjunctive treatments offer the opportunity for ongoing maintenance of improved function. As a discipline, PR has advanced very substantially since its early days and offers increasingly greater benefits for patients with chronic respiratory disease (Figure 2.8).

REFERENCES 1. Spruit MA, Singh SJ, Garvey C et  al. An official American Thoracic Society/European Respiratory Society statement: Key concepts and advances in pulmonary rehabilitation. Am J Respir Crit Care Med. 2013;188:e13–64. 2. Nici L, Lareau S, ZuWallack R. Pulmonary rehabilitation in the treatment of chronic obstructive pulmonary disease. Am Fam Physician. 2010;82:655–60. 3. Nici L, ZuWallack R. An official American Thoracic Society workshop report: The integrated care of the COPD patient. Proc Am Thorac Soc. 2012;9:9–18. 4. Davos Platz: II. Sanatoriums and hotels. Br Med J. 1906;2:1407–10.

5. Baston A. Curing Tuberculosis in Muskoka. Canada’s First Sanatoria. Canada: Old Stone Books Limited; 2013. 6. Barach AL. Diaphragmatic breathing in pulmonary emphysema. J Chronic Dis. 1955;1:211–5. 7. Barach AL. Physiologic therapy of respiratory disease; with special reference to the management of pulmonary emphysema. N Y Med. 1946;2:21–6. 8. Haas A, Rusk HA, Zivan M. The results of a combined medical and rehabilitative program in tuberculosis; a preliminary report. Arch Phys Med Rehabil. 1954;35:77–86. 9. Casaburi R. A brief history of pulmonary rehabilitation. Respir Care. 2008;53:1185–9. 10. Barach AL. Breathing exercises in pulmonary emphysema and allied chronic respiratory diseases. Arch Phys Med Rehabil. 1955;36:379–90. 11. Petty TL, Nett LM, Finigan MM, Brink GA, Corsello PR. A comprehensive care program for chronic airway obstruction. Methods and preliminary evaluation of symptomatic and functional improvement. Ann Intern Med. 1969;70:1109–20. 12. Petty TL. Ambulatory care for emphysema and chronic bronchitis. Chest. 1970;58:(Suppl 2):441–8. 13. Hodgkin JE, Balchum OJ, Kass I et al. Chronic obstructive airway diseases. Current concepts in diagnosis and comprehensive care. JAMA. 1975;232:1243–60. 14. American Thoracic Society. Position statement of pulmonary rehabilitation. Am Rev Respir Dis. 1981;1136:663. 15. Barach AL. Ambulatory oxygen therapy: Oxygen inhalation at home and out-of-doors. Dis Chest. 1959;35:229–41.

18  Pulmonary rehabilitation: The development of a scientific discipline

16. Nocturnal Oxygen Therapy Trial Group. Continuous or nocturnal oxygen therapy in hypoxemic chronic obstructive  lung  disease: A clinical trial. Ann Intern Med. 1980;93:391–8. 17. Report of the Medical Research Council Working Party. Long term domiciliary oxygen therapy in chronic hypoxic cor pulmonale complicating chronic bronchitis and emphysema. Lancet. 1981;1:681–6. 18. McGavin CR, Gupta SP, McHardy GJ. Twelve-minute walking test for assessing disability in chronic bronchitis. Br Med J. 1976;1:822–3. 19. Butland RJ, Pang J, Gross ER, Woodcock AA, Geddes DM. Two-, six-, and 12-minute walking tests in respiratory disease. Br Med J (Clin Res Ed). 1982;284:1607–8. 20. Leger LA, Lambert J. A maximal multistage 20-m shuttle run test to predict VO2 max. Eur J Appl Physiol Occup Physiol. 1982;49:1–12. 21. Singh SJ, Morgan MD, Scott S, Walters D, Hardman AE. Development of a shuttle walking test of disability in patients with chronic airways obstruction. Thorax. 1992;47:1019–24. 22. Revill SM, Morgan MD, Singh SJ, Williams J, Hardman AE. The endurance shuttle walk: A new field test for the assessment of endurance capacity in chronic obstructive pulmonary disease. Thorax. 1999;54:213–22. 23. Guyatt GH, Berman LB, Townsend M, Pugsley SO, Chambers LW. A measure of quality of life for clinical trials in chronic lung disease. Thorax. 1987;42:773–8. 24. Jones PW, Quirk FH, Baveystock CM, Littlejohns P. A self-complete measure of health status for chronic airflow limitation. The St. George’s Respiratory Questionnaire. Am Rev Respir Dis. 1992;145:1321–7. 25. Guyatt GH, King DR, Feeny DH, Stubbing D, Goldstein RS. Generic and specific measurement of healthrelated quality of life in a clinical trial of respiratory rehabilitation. J Clin Epidemiol. 1999;52:187–92. 26. Goldstein RS, Gort EH, Stubbing D, Avendano MA, Guyatt GH. Randomised controlled trial of respiratory rehabilitation. Lancet. 1994;344:1394–7. 27. Wijkstra PJ, Altena R, Kraan J, Otten V, Postma DS, Koëter GH. Quality of life in patients with chronic obstructive pulmonary disease improves after rehabilitation at home. Eur Respir J. 1994;7:269–73. 28. Reardon J, Awad E, Normandin E, Vale F, Clark B, ZuWallack RL. The effect of comprehensive outpatient pulmonary rehabilitation on dyspnea. Chest. 1994;105:1046–52. 29. Zuwallack R. A history of pulmonary rehabilitation: Back to the future. Pneumonol Alergol Pol. 2009;77:298–301. 30. Emery CF, Leatherman NE, Burker EJ, MacIntyre NR. Psychological outcomes of a pulmonary rehabilitation program. Chest. 1991;100:613–7. 31. Coventry PA, Hind D. Comprehensive pulmonary rehabilitation for anxiety and depression in adults with chronic obstructive pulmonary disease: Systematic review and meta-analysis. J Psychosom Res. 2007;63(5):551–65.

32. Casaburi R, Patessio A, Ioli F, Zanaboni S, Donner CF, Wasserman K. Reductions in exercise lactic acidosis and ventilation as a result of exercise training in patients with obstructive lung disease. Am Rev Respir Dis. 1991;143:9–18. 33. Ries AL, Kaplan RM, Limberg TM, Prewitt LM. Effects of pulmonary rehabilitation on physiologic and psychosocial outcomes in patients with chronic obstructive pulmonary disease. Ann Intern Med. 1995;122:823–32. 34. Maltais F, LeBlanc P, Simard C et al. Skeletal muscle adaptation to endurance training in patients with chronic obstructive pulmonary disease. Am J Respir Crit Care Med. 1996;154:442–7. 35. Griffiths TL, Burr ML, Campbell IA et al. Results at 1 year of outpatient multidisciplinary pulmonary rehabilitation: A randomised controlled trial. Lancet. 2000;355:362–8. 36. Griffiths TL, Phillips CJ, Davies S, Burr ML, Campbell IA. Cost effectiveness of an outpatient multidisciplinary pulmonary rehabilitation programme. Thorax. 2001;56:779–84. 37. Redelmeier DA, Guyatt GH, Goldstein RS. Assessing the minimal important difference in symptoms: A comparison of two techniques. J Clin Epidemiol. 1996;49:1215–9. 38. Bourbeau J, Julien M, Maltais F et  al. Reduction of hospital utilization in patients with chronic obstructive pulmonary disease: A disease-specific self-management intervention. Arch Intern Med. 2003;163:585–91. 39. Casas A, Troosters T, Garcia-Aymerich J et  al. Integrated care prevents hospitalisations for exacerbations in COPD patients. Eur Respir J. 2006;28:123–30. 40. Rice KL, Dewan N, Bloomfield HE et al. Disease management program for chronic obstructive pulmonary disease: A randomized controlled trial. Am J Respir Crit Care Med. 2010;182:890–6. 41. Zwerink M, Brusse-Keizer M, van der Valk PDLPM et  al. Self management for patients with chronic obstructive pulmonary disease. Cochrane Database Syst Rev. 2014;(3):CD002990. 42. Global strategy for diagnosis, management, and prevention of COPD. Global Initiative for Chronic ­ Obstructive Lung Disease 2008 [cited 2008]. Available from: www.goldcopd.com. 43. Ambrosino N, Venturelli E, Vagheggini G, Clini E. Rehabilitation, weaning and physical therapy strategies in chronic critically ill patients. Eur Respir J. 2012;39(2):487–92. 44. Schweickert WD, Pohlman MC, Pohlman AS et  al. Early physical and occupational therapy in mechanically ventilated, critically ill patients: A randomised controlled trial. Lancet. 2009;373(9678): 1874–82. 45. Man WD, Polkey MI, Donaldson N, Gray BJ, Moxham J. Community pulmonary rehabilitation after hospitalisation for acute exacerbations of chronic obstructive pulmonary disease: Randomised controlled study. BMJ. 2004;329:1209.

References 19

46. Puhan MA, Gimeno-Santos E, Scharplatz M, Troosters T, Walters EH, Steurer J. Pulmonary rehabilitation following exacerbations of chronic obstructive pulmonary disease. Cochrane Database Syst Rev. 2011:CD00530522. 47. van Wetering CR, Hoogendoorn M, Mol SJ, Ruttenvan Molken MP, Schols AM. Short- and long-term efficacy of a community-based COPD management programme in less advanced COPD: A randomised controlled trial. Thorax. 2010;65:7–13. 48. Maltais F, Bourbeau J, Shapiro S et  al. Effects of home-based pulmonary rehabilitation in patients with chronic obstructive pulmonary disease: A randomized trial. Ann Intern Med. 2008;149:869–78. 49. Stickland M, Jourdain T, Wong EY, Rodgers WM, Jendzjowsky NG, Macdonald GF. Using Telehealth technology to deliver pulmonary rehabilitation in chronic obstructive pulmonary disease patients. Can Respir J. 2011;18:216–20. 50. Troosters T, Gosselink R, Decramer M. Short- and long-term effects of outpatient rehabilitation in patients with chronic obstructive pulmonary disease: A randomized trial. Am J Med. 2000;109:207–12. 51. Hill K, Bansal V, Brooks D, Goldstein RS. Repeat pulmonary rehabilitation programs confer similar increases in functional exercise capacity to initial programs. J Cardiopulm Rehabil Prev. 2008;28:410–4. 52. Beauchamp MK, Nonoyama M, Goldstein RS, Hill K, Dolmage TE, Mathur S, Brooks D. Interval versus continuous training in individuals with chronic obstructive pulmonary disease--a systematic review. Thorax. 2010;65:157–64. 53. Dolmage TE, Goldstein RS. Proportional assist ventilation and exercise tolerance in subjects with COPD. Chest. 1997;111:948–54. 54. O’Donnell DE, D’Arsigny C, Webb KA. Effects of hyperoxia on ventilatory limitation during exercise in advanced chronic obstructive pulmonary disease. Am J Respir Crit Care Med. 2001;163:892–8. 55. Eves ND, Sandmeyer LC, Wong EY et  al. Heliumhyperoxia: A novel intervention to improve the benefits of pulmonary rehabilitation for patients with COPD. Chest. 2009;135:609–18.

56. Dolmage TE, Goldstein RS. Effects of one-legged exercise training of patients with COPD. Chest. 2008;133:370–6. 57. Maddocks M, Nolan CM, Man WD et al. Neuromuscular electrical stimulation to improve exercise capacity in patients with severe COPD: A randomised doubleblind, placebo-controlled trial. Lancet Respir Med. 2016;4:27–36. 58. Gloeckl R, Heinzelmann I, Baeuerle S et  al. Effects of whole body vibration in patients with chronic obstructive pulmonary disease--a randomized controlled trial. Respir Med. 2012;106(1):75–83. 59. Lee AL, Desveaux L, Goldstein RS, Brooks D. The role of distractive auditory stimuli in the form of music in COPD – a systematic review. Chest. 2015;148:417–29. 60. Lord VM, Cave P, Hume VJ et  al. Singing teaching as a therapy for chronic respiratory disease – a randomised controlled trial and qualitative evaluation. BMC Pulm Med. 2010;10:41. 61. Ngai S, Jones AY, Tam WW. Tai Chi for chronic obstructive pulmonary disease (COPD). Cochrane Database Syst Rev 2016;6: CD009953. 62. Liu XC, Pan L, Hu Q, Dong WP, Yan JH, Dong L. Effects of yoga training in patients with chronic obstructive pulmonary disease: A systematic review and metaanalysis. J Thorac Dis. 2014;6:795–802. 63. Wshah A, Butler S, Patterson K, Goldstein RS, Brooks D. “Let’s Boogie”: Feasibility of a dance intervention in patients with chronic obstructive pulmonary disease. J Cardiopulm Rehabil Prev. 2019;39(5):E14−9. 64. Butler SJ, Lee AL, Goldstein RS, Brooks D. Active video games as a training tool for individuals with chronic respiratory disease: A systematic review. J Cardiopulm Rehabil Prev. 2019; 39(2):85−90. 65. Wagner E. Chronic disease management. What will it take to improve care for chronic illness? Effective Clin Pract. 1998;1:2–4. 66. Casas A, Troosters T, Garcia-Aymerich J et al., members of the CHRONIC Project. Integrated care prevents hospitalisations for exacerbations in COPD patients. Eur Respir J. 2006;28:123–30. 67. Wagg K. Unravelling self-management for COPD: What next? Chron Respir Dis. 2012;9(1):5–7.

3 Key concepts in pulmonary rehabilitation FELIPE V.C. MACHADO, FRITS M.E. FRANSSEN AND MARTIJN A. SPRUIT Definition and concept Exercise training Non-exercising interventions

21 22 23

Future perspectives for PR Summary References

24 25 25

KEY MESSAGES • Patient assessments may be performed at any point of the disease trajectory, including early disease, during or directly after an exacerbation. Assessments continue post PR to assess the effectiveness of a maintenance intervention. • A comprehensive PR programme should provide patient-tailored therapies guided according to patient’s initial assessment.

• Exercise training is the cornerstone of a PR programme. It is important to unravel the factors that contribute to exercise limitation in patients with chronic respiratory diseases. • Other non-exercising interventions and strategies should be investigated regarding their ability to enhance the benefits of exercise training and/or PR.

DEFINITION AND CONCEPT

extra-pulmonary, behavioural/lifestyle) that will guide the individualized treatment (1,2). The patient’s assessment may be performed at any point in the disease trajectory, including early disease as well as during or directly following an exacerbation. It should continue post PR to assess the effectiveness of maintenance interventions. While PR is beneficial in several chronic respiratory conditions, most evidence is derived from studies performed in patients with chronic obstructive pulmonary disease (COPD). Most COPD patients referred to PR present with more advanced disease. However, there is substantial evidence showing that patients with early disease also have lower limb muscle weakness (3), impairment in HRQoL (4), reduced exercise capacity and higher dyspnoea ratings at any given workload compared with healthy control subjects (5). Indeed, even patients with mild disease may present with physiological impairment at rest and during exercise, beyond the effects of natural ageing (6). This may be due to the lower physical activity levels which are observed in patients with a mild degree of airflow limitation (7–9). All these manifestations seem similar to the impairment in patients with more severe disease and thus can also be treated by PR. In general, patients with early disease also show improvements in exercise capacity and HRQoL after

The following definition of pulmonary rehabilitation was adopted by the ATS and ERS (1): ‘Pulmonary rehabilitation is a comprehensive intervention based on a thorough patient assessment followed by patient-tailored therapies, which include, but are not limited to, exercise training, education, and behavior change, designed to improve the physical and psychological condition of people with chronic respiratory disease and to promote the long-term adherence of health-enhancing behaviors.’ Accordingly, it is important to emphasize the need of a multi-professional workforce interdisciplinary team, including physicians, physiotherapists, respiratory therapists, nurses, psychologists, behavioural specialists, exercise physiologists, nutritionists, occupational therapists and social workers. The goals of PR include minimizing symptom burden, maximizing exercise performance, promoting autonomy, increasing participation in everyday activities, enhancing health-related quality of life (HRQoL) and effecting longterm health-enhancing behaviour change (1). To achieve these goals, an initial assessment of the patient should be performed in order to provide a wide view of the complexity, comorbidities, needs and affected traits (pulmonary,

21

22  Key concepts in pulmonary rehabilitation

PR (10). In addition, smoking cessation therapy has been shown to be effective in reducing the rate of decline in lung function (11), supporting the need for early diagnosis and lifestyle intervention. Although a recent exacerbation used to be a contraindication for initiating PR (12), recent systematic reviews showed high-quality evidence supporting moderate to large effects on HRQoL and exercise capacity and moderate-quality evidence supporting reductions in short-term mortality, number of hospitalization days and number of hospital readmissions after early PR (13,14). These effects appear to be maintained for at least 12 months after PR in patients with COPD hospitalized for an exacerbation (13). Therefore, early supervised PR is recommended for patients in the post-hospitalization phase. Additional studies will help clinicians understand the most effective strategy for exercise sessions, self-management education and other interventions. Despite marked improvements in exercise capacity, symptoms, and HRQoL following PR, its effects wanes after 12 months in the absence of maintenance intervention being offered (15,16). A systematic review identified seven randomized controlled trials that examined the effects of a maintenance intervention post PR and concluded that supervised exercise programmes after primary PR appear to be more effective than usual care for preserving exercise capacity in the medium term but not in the long term (17). However, one recent trial conducted a 3-year multicentre study comparing a supervised maintenance intervention to usual care after intensive PR in patients with moderate to severe COPD, and found that a simple maintenance intervention extends the benefits obtained with the PR programme, as measured by the multidimensional body mass index, airway obstruction, dyspnoea, exercise (BODE) index and exercise capacity, over at least 2 years (18). Notably, the dropout rate presented was high (more than 50%) during the 3 years (18). Thus, the optimal post-rehabilitation maintenance intervention and how to improve long-term adherence in patients with COPD remain to be defined. Considering the pronounced heterogeneity and complexity of patients with chronic respiratory disease who may be referred for PR and the diversity of stages of the diseases in which PR may be useful, the need for coordination and collaboration across the interdisciplinary team, added to a comprehensive and patient-centred approach, is essential. These needs mirror the integrated care model (19), whose concept ‘The continuum of patient-centred services organized as a care delivery value chain for patients with chronic conditions with the goal of achieving the optimal daily functioning and health status for the individual patient and to achieve and maintain the individual’s independence and functioning in the community’ (19) is more than suitable for PR, and can be applied to easily promote partnership among patients, their families and professionals to guide the right treatment to the right patient at the right time.

EXERCISE TRAINING Patients with chronic respiratory disease such as COPD frequently suffer from exercise intolerance, caused mainly by dyspnoea. Recently, a model of the dyspnoea-inactivity vicious circle theory in COPD was developed and externally validated (20). This model describes the sequence of events from airflow limitation and lung hyperinflation, key factors for the worsening of dyspnoea, which may be responsible for a decrease in physical activity (PA), and then deterioration of exercise capacity, which further enhances dyspnoea. It has been proposed that exercise capacity has a more central role in the vicious circle given that most of the effect of other variables (lung hyperinflation, physical activity, exacerbations) on dyspnoea are mediated by effects on exercise capacity (20). Due to this complex process, patients with COPD are inside a disease spiral and gradually begin to suffer from the effects of physical deconditioning (detraining). As detraining presents multiple different negative effects, mainly in cardiovascular and skeletal muscle systems, many factors may contribute directly or indirectly to exercise intolerance in these individuals. Consequently, before the prescription of exercise training, it is important to unravel the factors that limit exercise for each individual. A maximal cardiopulmonary exercise test may achieve this goal and further provide information regarding the safety of exercise and a suitable exercise prescription (21). Another important feature is the assessment of lower-limb muscle strength which can identify patients who are at increased risk for exercise intolerance and is used to prescribe adequate loads for resistance training (22). Considered to be the cornerstone of PR (23), multiple studies have demonstrated that exercise training is effective in improving lower-limb muscle function and exercise capacity in COPD (16,24–26) and other chronic respiratory diseases (27–29). Once exercise intolerance or reduced muscle strength is detected, independently of the cause, endurance and resistance training usually can be prescribed. There are other alternative exercise modalities (e.g. waterbased training, Nordic walking) that may also be beneficial to some subgroups of patients (30,31). In a comprehensive PR programme, the identification of the most suitable modality of exercise training for each patient is fundamental and still a challenge, since few studies tried to select subgroups of patients with COPD that could better respond from a particular exercise modality based on their initial clinical characteristics (tailored interventions). However, independently of the modality, the general principles of exercise training should be considered and are no different from those for healthy individuals or even athletes, in which application of progressive overload training is essential. Endurance training aims to improve exercise capacity and the muscles of ambulation in order to relieve dyspnoea and fatigue to allow an increase in PA. The most common forms of training are cycling (using a stationary cycle ergometer) or walking (either ground-based or on a treadmill).

Non-exercising interventions  23

Walking training has the advantage of being a functional exercise that can readily translate to improvement in walking capacity. Biking exercise, as a weight-supported exercise, places a greater specific load on the quadriceps muscles than walking (32), is less affected by obesity (33) and results in less exercise-induced oxygen desaturation (34). The modality of endurance exercise training (i.e. continuous or interval training) as well as the intensity and duration should be tailored to the individual patient. Interval training and continuous training appear to be equally effective in COPD (35). Interval training may be a useful alternative to continuous training, especially in symptom-limited individuals who are unable to tolerate high-intensity continuous training (36). This modality may result in significantly lower symptom scores (35) and less severe oxygen desaturation, despite high absolute training loads, thus maintaining the training effects of endurance training (35,37). The intensity can be controlled by objective measures (e.g. work rate, heart rate) as well as subjective measures (e.g. Borg dyspnoea or fatigue score, Rating of Perceived Exertion). A duration of 20 to 60 minutes per session for three to five times per week is required in order to achieve a volume of exercise that provides benefits for patients with chronic respiratory diseases. A programme duration of at least 8 weeks is recommended to attain a substantial effect (38). Another important goal of exercise training for patients with chronic respiratory diseases is optimizing muscle strength. For achieving this goal, a resistance training programme should be offered. Aerobic training alone does not provide benefit for increasing muscle strength and mass at the same extent as when resistance training is offered (25,40,41). In addition, resistance training can improve activities of daily living (ADL), such as arm-raises, sit-tostand and climbing stairs (42–44). For the progression of the resistance training, several variables can be manipulated (i.e. sequence of exercises, intensity, volume, training frequency, rest interval between sets and movement velocity) (45). As no specific guidelines are provided for patients with chronic respiratory diseases and the present literature does not inform clinicians as to which are the most effective resistance training settings for each subgroup of patients from this population, the recommendations of the American College of Sports Medicine to enhance muscle strength in adults are often followed (45). Until the latest statement (1) it was not clear whether and to what extent specific gains in upper limb function translate into improvements in broader outcomes. A recent systematic review on the effects of upper limb training reported that the addition of upper limb training to lower limb training made no difference to dyspnoea or HRQoL (46). Indeed, if an unsupported endurance upper limb training programme is provided, large improvements in unsupported endurance upper limb exercise capacity occur (46). This conclusion contrasts with the results of a recent randomized controlled trial in which the addition of upper limb resistance training showed to be more effective in improving exercise capacity, inspiratory muscle strength

and HRQoL in patients with COPD compared with a control group (endurance and respiratory training) (47). Due to these controversial findings, this topic is still of interest for studies; larger investigations are required to confirm the effects of upper limb training and compare the differences between upper limb endurance training and upper limb resistance training on patient-relevant outcomes (46). In summary, it is clear that exercise training for at least 4 weeks with or without education and/or psychological support is beneficial for improving HRQoL and exercise capacity compared to conventional care, to the point that no further trials are indicated to answer this question (48). However, offering a patient-tailored, individualized, comprehensive exercise training programme is decisive in enhancing the benefits of PR. For this, it is important to perform a holistic patient assessment with identification of the most suitable modality of exercise training for each patient.

NON-EXERCISING INTERVENTIONS A comprehensive intervention should not be limited to exercise training, but rather include other components to promote the overall health status of a patient to benefit from PR. Combining different strategies, such as self-management education, PA coaching, nutritional supplementation and counselling, psychological support, inspiratory muscle training (IMT) and occupational therapy, according to the needs of the patient identified during the initial assessment, is the optimal scenario.

Self-management education Self-management education refers to any formalized patient education programme aiming at teaching skills needed to carry out disease-specific medical regimens to guide health behaviour change for patients to control their disease and improve their well-being (49). The most included educational topics for educational programmes of the current management interventions for patients with COPD are smoking cessation, medications, exercise, breathing strategies, exacerbations, stress management, respiratory devices, action plans, nutrition, coping with chronic lung diseases, pathophysiology of chronic respiratory diseases and communicating with a healthcare provider (50). In patients with COPD, self-management education is associated with improvement in HRQoL, reduction in respiratory-related and all-cause hospital admissions and improvement in d ­ yspnoea (51).

Physical activity coaching PA coaching aims to merge the increase in capacity (exercise tolerance and muscle strength) provided after exercise training to promote behavioural changes to maintain the benefits of being active in the long term. In patients with COPD this is a common and promising strategy to stimulate higher levels of PA by using assessment and feedback of

24  Key concepts in pulmonary rehabilitation

PA combined with individualized PA goals and/or tailored motivational messages (52). Most studies have included PA counselling by using pedometers or telecoaching (by computer or mobile technology). However, recently, a new strategy consisting of combined behavioural strategies and unsupervised outdoor walking showed to be efficacious in increasing PA after 12 months in motivated COPD patients (53). For other chronic respiratory diseases, such as cystic fibrosis, there is limited evidence that PA counselling and exercise advice, undertaken over at least 6 months, to engage in a home exercise programme may result in improved PA participation (54).

Nutritional support Body composition abnormalities are prevalent in patients with COPD and probably in all advanced respiratory diseases (1). These abnormalities can be treated with nutritional supplementation and/or counselling. Depending on the body composition abnormality, intervention strategies will be oriented towards restoring energy and/or protein balance or decreasing energy balance while maintaining protein balance. There is moderate-quality evidence for nutritional supplementation in combination with exercise training for the management of malnourished patients with COPD (55). Recently, the potential of nutritional supplementation to enhance the efficacy of exercise training in muscle wasted patients with COPD was described with markedly beneficial effects on nutritional status, inspiratory muscle strength and PA (56). At present, the role of nutritional supplementation and/or counselling for obese individuals with COPD, as well as the effects of weight loss in obese patients on symptoms, lung function and exercise tolerance in obese individuals with COPD are still unclear. Recently, it has been shown that dietary energy restriction coupled with resistance exercise training results in improvements in body mass index, exercise tolerance and health status, whilst preserving skeletal muscle mass in obese patients with COPD (57).

Management of anxiety and depression The prevalence of symptoms of anxiety and depression reported in patients with COPD is up to 40% (58), and in patients with cystic fibrosis it is two times higher than reported in community populations (59). These symptoms are also common in other chronic respiratory diseases (60–62), and PR programmes should screen individuals to rule out untreated major depression or anxiety disorders since these symptoms may impact participation in, and reduce the benefit from, PR (63). Evidence supports that pulmonary rehabilitation leads to reduction in short-term anxiety and depression (58) and is effective to manage psychological morbidity in COPD (65). Improvements in anxiety and depression after PR are most likely to be observed in those presenting with significant baseline anxiety and depression (66). Some studies indicated that the addition

of sessions of psychotherapy to exercise training provides greater improvements in depression and anxiety than exercise alone (67,68).

Inspiratory muscle exercise Patients with chronic respiratory diseases, such as COPD, clearly present respiratory muscle weakness which is one factor associated to exercise limitation and perception of dyspnoea (69,70). When compared to no intervention or sham intervention, IMT as a standalone intervention demonstrates significant improvements in inspiratory muscle strength, inspiratory muscle endurance, functional exercise capacity, quality of life and significant reductions in dyspnoea (71,72). Recently, three randomized controlled trials aimed to investigate whether the addition of IMT could enhance the well-established benefits of pulmonary rehabilitation in patients with COPD (73–75). The results of two trials suggested that improvements in respiratory muscle function after IMT plus whole-body exercise training did not translate into additional improvements (73,74); conversely, in one study additional gains in endurance time and reductions in symptoms of dyspnoea were observed during an endurance cycling test (75). The search for a sub-population of COPD in which IMT can optimize the response to PR is still a topic for research. Interestingly, Augustin and colleagues recently showed that a reduced inspiratory muscle strength can also occur in patients with COPD who do not have static hyperinflation (76). Possibly, these may be good candidates for IMT.

Other interventions There are other interventions and strategies that should be investigated regarding their ability to enhance the benefits of exercise training and/or PR. For example, optimizing medical treatment before exercise training with bronchodilator therapy, long-term oxygen therapy, treatment of comorbidities, ADL training, partner involvement, noninvasive ventilation, neuromuscular electrical stimulation, smoking cessation therapies and balance training could be indicated, based on results of new studies, in order to maximize the effectiveness of PR according to specific patients’ needs and goals.

FUTURE PERSPECTIVES FOR PR Exercise-based rehabilitation is effective in improving exercise capacity and HRQoL in most chronic respiratory diseases, including non-cystic fibrosis bronchiectasis (77), interstitial lung disease (78), cystic fibrosis (79), asthma (80) and pulmonary hypertension (81). Indeed, most individuals enrolled in PR have COPD, and for these patients there is no need of further randomized controlled trials to confirm evidence that PR improves daily symptoms, exercise performance and health status, independently of disease stage and

References 25

complexity (48). A regular PR programme including exercise training and other interventions is safe and will benefit most patients with chronic respiratory diseases. Despite the definition of PR focusing on ‘patient-tailored therapies’, most studies have provided regular PR independently of patients’ initial assessments. Even though most patients respond to regular PR, some recent data show that the response may vary considerably between patients. A study including more than 2000 patients with COPD showed that patients can be clustered based on their multidimensional response to a comprehensive PR programme, and different groups of patients with a very good, good, moderate or poor response can be identified (82). These results raise a discussion regarding a possible need to redesign ongoing PR programmes in order to personalize the programme (82). Further research considering the concept of personalized medicine in PR is expected. ‘Personalized medicine’ refers to the tailoring of medical treatment to the individual characteristics of each patient. This requires the ability to classify individuals into subpopulations that differ in their susceptibility to a particular disease (or condition) or their response to a specific treatment (83). These can be applied in chronic respiratory diseases by identifying clinically meaningful and useful subgroups or phenotypes that exhibit similar underlying biologic or physiologic mechanism, clinical outcomes and therapeutic response profiles (84,85). For example, in asthma it has been proposed that patients may be stratified into several phenotypes based on the type of airway inflammation (supported by different molecular pathways), and the identification of severe eosinophilic asthma has been shown to select patients that present good clinical response to a specific pharmacological treatment (86). For patients with COPD, recent studies are also identifying different subgroups of patients according to key characteristics of the disease, such as lung function (76), physical activity (87), body composition (88,89) and comorbidities (90). However, the identification of phenotypes of COPD is still an urgent medical need (84) and longitudinal studies must validate the usefulness of the established phenotypes and explore sources of variability in phenotypes and  the temporal nature (39,64). Most studies are cross-­sectional and found that it is possible to discriminate groups with similar characteristics, but data are lacking regarding whether such phenotypes are associated to long-term outcomes or response to regular or personalized PR. As chronic respiratory diseases are usually complex and heterogeneous, stratifying patients into groups that share similar treatable traits is important for optimizing the efficacy of patient management.

SUMMARY This chapter provides a summary view of the latest American Thoracic Society (ATS)/European Respiratory Society (ERS) Statement on Pulmonary Rehabilitation. Key points are highlighted. Recent papers containing new

evidence on the effects and evolution of pulmonary rehabilitation (PR) are included. The content is displayed from the definition and concept to main considerations regarding exercise training (considered to be the cornerstone of PR). In addition, the authors discuss the main aspects of non-exercise interventions that are crucial to consider PR as an interdisciplinary intervention. Finally, future perspectives of studies and practice of PR are included.

REFERENCES 1. Spruit MA, Singh SJ, Garvey C et  al. An official American Thoracic Society/European Respiratory Society statement: Key concepts and advances in pulmonary rehabilitation. Am J Respir Crit Care Med. 2013 October;188(8):e13–64. 2. Agusti A, Bel E, Thomas M et  al. Treatable traits: Toward precision medicine of chronic airway diseases. Eur Respir J. 2016 February;47(2):410–9. 3. Seymour JM, Spruit MA, Hopkinson NS et  al. The prevalence of quadriceps weakness in COPD and the relationship with disease severity. Eur Respir J. 2010 July;36(1):81–8. 4. Miravitlles M, Soriano JB, García-Río F et al. Prevalence of COPD in Spain: Impact of undiagnosed COPD on quality of life and daily life activities. Thorax. 2009 October;64(10):863–8. 5. Ofir D, Laveneziana P, Webb KA, Lam Y-M, O’Donnell DE. Mechanisms of dyspnea during cycle exercise in symptomatic patients with GOLD stage I chronic obstructive pulmonary disease. Am J Respir Crit Care Med. 2008 March;177(6):622–9. 6. O’Donnell DE, Neder JA, Elbehairy AF. Physiological impairment in mild COPD. Respirology. 2016 February;21(2):211–23. 7. Shrikrishna D, Patel M, Tanner RJ et  al. Quadriceps wasting and physical inactivity in patients with COPD. Eur Respir J. 2012 November;40(5):1115–22. 8. Van Remoortel H, Hornikx M, Demeyer H et al. Daily physical activity in subjects with newly diagnosed COPD. Thorax. 2013 October;68(10):962–3. 9. Waschki B, Kirsten AM, Holz O et al. Disease progression and changes in physical activity in patients with chronic obstructive pulmonary disease. Am J Respir Crit Care Med. 2015 August 1;192(3):295–306. 10. Jácome C, Marques A. Pulmonary rehabilitation for mild COPD: A systematic review. Respir. Care. 2014 April;59(4):588–94. 11. Scanlon PD, Connett JE, Waller LA et al. Smoking cessation and lung function in mild-to-moderate chronic obstructive pulmonary disease. The Lung Health Study. Am J Respir Crit Care Med. 2000 February;161​ (2 Pt 1):381–90. 12. Wedzicha JA, Miravitlles M, Hurst JR et  al. Management of COPD exacerbations: A European Respiratory Society/American Thoracic Society guideline. Eur Respir J. 2017 March;49(3):pii: 1600791.

26  Key concepts in pulmonary rehabilitation

13. Ryrsø CK, Godtfredsen NS, Kofod LM et  al. Lower mortality after early supervised pulmonary rehabilitation following COPD-exacerbations: A systematic review and meta-analysis. BMC Pulm Med. 2018 September;18(1):154. 14. Puhan MA, Gimeno-Santos E, Cates CJ, Troosters T. Pulmonary rehabilitation following exacerbations of chronic obstructive pulmonary disease. Cochrane Database Syst Rev. 2016 December;12:CD005305. 15. Foglio K, Bianchi L, Bruletti G et al. Seven-year time course of lung function, symptoms, health-related quality of life, and exercise tolerance in COPD patients undergoing pulmonary rehabilitation programs. Respir Med. 2007 September;101(9):1961–70. 16. Griffiths TL, Burr ML, Campbell IA et al. Results at 1 year of outpatient multidisciplinary pulmonary rehabilitation: A randomised controlled trial. Lancet. 2000 January;355(9201):362–8. 17. Beauchamp MK, Evans R, Janaudis-Ferreira T, Goldstein RS, Brooks D. Systematic review of supervised exercise programs after pulmonary rehabilitation in individuals with COPD. Chest. 2013 October;​ 144(4):1124–33. 18. Güell MR, Cejudo P, Ortega F et al. Benefits of longterm pulmonary rehabilitation maintenance program in patients with severe chronic obstructive pulmonary disease. Am J Respir Crit Care Med. 2017 March;​ 195(5):622–9. 19. Nici L, ZuWallack R; American Thoracic Society Subcommittee on Integrated Care of the COPD Patient. An Official American Thoracic Society Workshop Report: The Integrated Care of the COPD Patient. Proc Am Thorac Soc. 2012 March;9(1):9–18. 20. Ramon MA, Ter Riet G, Carsin A-E et  al. The dyspnoea-inactivity vicious circle in COPD: Development and external validation of a conceptual model. Eur Respir J. 2018 September;52(3):pii: 1800079. 21. American Thoracic Society; American College of Chest Physicians. ATS/ACCP Statement on cardiopulmonary exercise testing. Am J Respir Crit Care Med. 2003 January;167(2):211–77. 22. Maltais F, Decramer M, Casaburi R et  al. An official American Thoracic Society/European Respiratory Society statement: Update on limb muscle dysfunction in chronic obstructive pulmonary disease. Am J Respir Crit Care Med. 2014 May;89(9):e15–62. 23. Nici L, Donner C, Wouters E et al. ATS/ERS Pulmonary Rehabilitation Writing Committee. American Thoracic Society/European Respiratory Society Statement on pulmonary rehabilitation. Am J Respir Crit Care Med. 2006 June;173(12):1390–1413. 24. Sala E, Roca J, Marrades RM et al. Effects of endurance training on skeletal muscle bioenergetics in chronic obstructive pulmonary disease. Am J Respir Crit Care Med. 1999 June;159(6):1726–34. 25. Bernard S, Whittom F, Leblanc P et  al. Aerobic and strength training in patients with chronic obstructive

pulmonary disease. Am J Respir Crit Care Med. 1999 March;159(3):896–901. 26. Maltais F, LeBlanc P, Simard C et al. Skeletal muscle adaptation to endurance training in patients with chronic obstructive pulmonary disease. Am J Respir Crit Care Med. 1996 August;154(2 Pt 1):442–7. 27. Holland AE, Hill CJ, Conron M, Munro P, McDonald CF. Short term improvement in exercise capacity and symptoms following exercise training in interstitial lung disease. Thorax. 2008 June;63(6):549–54. 28. Nishiyama O, Kondoh Y, Kimura T et al. Effects of pulmonary rehabilitation in patients with idiopathic pulmonary fibrosis. Respirology. 2008 May;13(3):394–9. 29. Newall C, Stockley RA, Hill SL. Exercise training and inspiratory muscle training in patients with bronchiectasis. Thorax. 2005 June;60(11):943–8. 30. McNamara RJ, McKeough ZJ, McKenzie DK, Alison JA. Water-based exercise in COPD with physical comorbidities: A randomised controlled trial. Eur Respir J. 2013 June;41(6):1284–91. 31. Breyer M-K, Breyer-Kohansal R, Funk G-C et  al. Nordic walking improves daily physical activities in COPD: A randomised controlled trial. Respir Res. 2010 August;22;11:112. 32. Man WD, Soliman MG, Gearing J et al. Symptoms and quadriceps fatigability after walking and cycling in chronic obstructive pulmonary disease. Am J Respir Crit Care Med. 2003 September;168(5):562–7. 33. Maatman RC, Spruit MA, van Melick PP et al. Effects of obesity on weight-bearing versus weight-supported exercise testing in patients with COPD. Respirology. 2016 April;21(3):483–8. 34. Poulain M, Durand F, Palomba B et al. 6-Minute walk testing is more sensitive than maximal incremental cycle testing for detecting oxygen desaturation in patients with COPD. Chest. 2003 May;123(5):1401–7. 35. Vogiatzis I, Nanas S, Roussos C. Interval training as an alternative modality to continuous exercise in patients with COPD. Eur Respir J. 2002 July:20(1):12–9. 36. Maltais F, LeBlanc P, Jobin J et al. Intensity of training and physiologic adaptation in patients with chronic obstructive pulmonary disease. Am J Respir Crit Care Med. 1997 February;155(2):55–61. 37. Puhan MA, Büsching G, Schünemann HJ, VanOort E, Zaugg C, Frey M. Interval versus continuous highintensity exercise in chronic obstructive pulmonary disease: A randomized trial. Ann Intern Med. 2006 December;145(11):816–25. 38. Beauchamp MK, Janaudis-Ferreira T, Goldstein RS, Brooks D. Optimal duration of pulmonary rehabilitation for individuals with chronic obstructive pulmonary disease - a systematic review. Chron Respir Dis. 2011 8(2):129–40. 39. Pinto LM, Alghamdi M, Benedetti A, Zaihra T, Landry T, Bourbeau J. Derivation and validation of clinical phenotypes for COPD: A systematic review. Respir Res. 2015 April;18(16):50.

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40. Ortega F, Toral J, Cejudo P et  al. Comparison of effects of strength and endurance training in patients with chronic obstructive pulmonary disease. Am J Respir Crit Care Med. 2002 September;166(5):669–74. 41. Mador MJ, Bozkanat E, Aggarwal A, Shaffer M, Kufel TJ. Endurance and strength training in patients with COPD. Chest. 2004 June;125(6):2036–45. 42. Panton LB, Golden J, Broeder CE, Browder KD, Cestaro-Seifer DJ, Seifer FD. The effects of resistance training on functional outcomes in patients with chronic obstructive pulmonary disease. Eur J Appl Physiol. 2004 April;91(4):443–9. 43. Costi S, Crisafulli E, Antoni FD, Beneventi C, Fabbri LM, Clini EM. Effects of unsupported upper extremity exercise training in patients with COPD: A randomized clinical trial. Chest. 2009 August;136(2):​ 387–95. 44. Janaudis-Ferreira T, Hill K, Goldstein RS et al. Resistance arm training patients with COPD: A randomized controlled trial. Chest. 2011 January;139(1):151–8. 45. American College of Sports Medicine. Progression models in resistance training for healthy adults. Med Sci Sport Exerc. 2009 March;41(3):687–708. 46. McKeough ZJ, Velloso M, Lima VP, Alison JA. Upper limb exercise training for COPD. Cochrane Database Syst Rev. 2016 November;11:CD011434. 47. Silva CMDSE, Gomes Neto M, Saquetto MB, Conceição CSD, Souza-Machado A. Effects of upper limb resistance exercise on aerobic capacity, muscle strength, and quality of life in COPD patients: A randomized controlled trial. Clin Rehabil. 2018 December;​ 32(12):1636–44. 48. McCarthy B, Casey D, Devane D, Murphy K, Murphy E, Lacasse Y. Pulmonary rehabilitation for chronic obstructive pulmonary disease. Cochrane Database Syst Rev. 2015 February;23;(2):CD003793. 49. Bourbeau J, Nault D, Dang-Tan T. Self-management and behaviour modification in COPD. Patient Educ Couns. 2004 March;52(3):271–7. 50. Stoilkova A, Janssen DJA, Wouters EFM. Educational programmes in COPD management interventions: A systematic review. Respir Med. 2013 November;107​ (11):1637–50. 51. Zwerink M, Brusse-Keizer M, van der Valk P et  al. Self management for patients with chronic obstructive pulmonary disease. Cochrane Database Syst Rev. 2014 March;3:CD002990. 52. Mantoani LC, Rubio N, McKinstry B, MacNee W, Rabinovich RA. Interventions to modify physical activity in patients with COPD: A systematic review. Eur Respir J. 2016 July;48(1):69–81. 53. Arbillaga-Etxarri A, Gimeno-Santos E, BarberanGarcia A et al. Long-term efficacy and effectiveness of a behavioural and community-based exercise intervention (Urban Training) to increase physical activity in patients with COPD. A randomised controlled trial. Eur Respir J. 2018 October;52(4):pii: 1800063.

54. CoxNS,AlisonJA,HollandAE.Interventionsforpromoting physical activity in people with cystic fibrosis. Cochrane Database Syst Rev. 2013 December;(12):CD009448. 55. Ferreira IM, Brooks D, White J, Goldstein R. Nutritional supplementation for stable chronic obstructive pulmonary disease. Cochrane Database Syst Rev. 2012 December;12:CD000998. 56. van de Bool C, Rutten EPA, van Helvoort A, Franssen FME, Wouters EFM, Schols AMWJ. A randomized clinical trial investigating the efficacy of targeted nutrition as adjunct to exercise training in COPD. J Cachexia Sarcopenia Muscle. 2017 October;8(5):748–58. 57. McDonald VM, Gibson PG, Scott HA et al. Should we treat obesity in COPD? The effects of diet and resistance exercise training. Respirology. 2016 July;21(5):​ 875–82. 58. Coventry PA. Does pulmonary rehabilitation reduce anxiety and depression in chronic obstructive pulmonary disease? Curr Opin Pulm Med. 2009 March;15(2):​143–9. 59. Quittner AL, Goldbeck L, Abbott J et al. Prevalence of depression and anxiety in patients with cystic fibrosis and parent caregivers: Results of The International Depression Epidemiological Study across nine countries. Thorax. 2014 December; 69(12):1090–7. 60. Batal O, Khatib OF, Bair N, Aboussouan LS, Minai OA. Sleep quality, depression, and quality of life in patients with pulmonary hypertension. Lung. 2011 April;189(2):141–9. 61. Ryerson CJ, Arean PA, Berkeley J et  al. Depression is a common and chronic comorbidity in patients with interstitial lung disease. Respirology. 2012 April;​ 17(3):525–32. 62. Akhtar AA, Ali MA, Smith RP. Depression in patients with idiopathic pulmonary fibrosis. Chron Respir Dis. 2013 August;10(3):127–33. 63. Garrod R, Marshall J, Barley E, Jones PW. Predictors of success and failure in pulmonary rehabilitation. Eur Respir J. 2006 April;27(4):788–94. 64. Bourbeau J, Pinto LM, Benedetti A. Phenotyping of COPD: Challenges and next steps. Lancet Respir Med. 2014 March;2(3):172–4. 65. Fan VS, Meek PM. Anxiety, depression, and cognitive impairment in patients with chronic respiratory disease. Clin Chest Med. 2014 June;35(2):399–409. 66. Harrison SL, Greening NJ, Williams JE, Morgan MD, Steiner MC, Singh SJ. Have we underestimated the efficacy of pulmonary rehabilitation in improving mood? Respir Med. 2012 June;106(6):838–44. 67. Emery CF, Schein RL, Hauck ER, MacIntyre NR. Psychological and cognitive outcomes of a randomized trial of exercise among patients with chronic obstructive pulmonary disease. Health Psychol. 1998 May;17(3):232–40. 68. de Godoy DV, de Godoy RF. A randomized controlled trial of the effect of psychotherapy on anxiety and depression in chronic obstructive pulmonary disease. Arch Phys Med Rehabil. 2003 August;84(8):1154–7.

28  Key concepts in pulmonary rehabilitation

69. Gosselink R, Troosters T, Decramer M. Peripheral muscle weakness contributes to exercise limitation in COPD. Am J Respir Crit Care Med. 1996 March;​153(3):976–80. 70. Hamilton AL, Killian KJ, Summers E, Jones NL. Muscle strength, symptom intensity, and exercise capacity in patients with cardiorespiratory disorders. Am J Respir Crit Care Med. 1995 December;152(6 Pt I):2021–31. 71. Gosselink R, De Vos J, Van Den Heuvel SP, Segers J, Decramer M, Kwakkel G. Impact of inspiratory muscle training in patients with COPD: What is the evidence? Eur Respir J. 2011 February;37(2):416–25. 72. Beaumont M, Forget P, Couturaud F, Reychler G. Effects of inspiratory muscle training in COPD patients: A systematic review and meta-analysis. Clin Respir J. 2018 July;12(7):2178–88. 73. Schultz K, Jelusic D, Wittmann M et  al. Inspiratory muscle training does not improve clinical outcomes in 3-week COPD rehabilitation: Results from a randomised controlled trial. Eur Respir J. 2018 January;​ 51(1):pii: 1702000. 74. Beaumont M, Mialon P, Le Ber C et al. Effects of inspiratory muscle training on dyspnoea in severe COPD patients during pulmonary rehabilitation: Controlled randomised trial. Eur Respir J. 2018 January;51(1):pii: 1701107. 75. Charususin N, Gosselink R, Decramer M, Demeyer H, McConnell A, Saey D. A randomised controlled trial of adjunctive inspiratory muscle training for patients with COPD. Thorax. 2018 October;73(10):942–50. 76. Augustin IML, Spruit MA, Houben-Wilke S et al. The respiratory physiome: Clustering based on a comprehensive lung function assessment in patients with COPD. PLOS ONE. 2018 September;13(9):e0201593. 77. Lee AL, Hill CJ, McDonald CF, Holland AE. Pulmonary rehabilitation in individuals with non-cystic fibrosis bronchiectasis: A systematic review. Arch Phys Med Rehabil. 2017 April;98(4):774–82e1. 78. Dowman L, Hill CJ, Holland AE. Pulmonary rehabilitation for interstitial lung disease. Cochrane Database Syst Rev. 2014 October;(10):CD006322. 79. Radtke T, Nevitt SJ, Hebestreit H, Kriemler S. Physical exercise training for cystic fibrosis. Cochrane Database Syst Rev. 2017 November;11:CD002768.

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4 Enhancing use and delivery of pulmonary rehabilitation EMILY HUME, CAROLYN L. ROCHESTER AND IOANNIS VOGIATZIS Introduction 29 Knowledge and awareness of pulmonary rehabilitation 30 Access, uptake and adherence to pulmonary rehabilitation 32 Use of pulmonary rehabilitation services compared to other effective therapies in COPD 34

Ensuring quality of PR programmes Conclusion Summary References

34 35 35 36

KEY MESSAGES • PR is a recommended effective treatment for patients with chronic respiratory diseases providing physiological, symptom-reducing, psychological and health economic benefits. • PR is severely underutilized worldwide due to poor patient referral, uptake and completion of PR programmes, as well as suboptimal patient access to PR. • Strategies to increase referral rates to PR focus on improving knowledge and awareness of PR among healthcare professionals, patients and payers.

• Improving patient access to PR is important to enhance uptake and completion of PR programmes. Further research is needed regarding novel models of PR delivery; combining PR and cardiac rehabilitation programmes is another strategy to improve patient accessibility. • To ensure PR quality, it is imperative that the core components and quality of PR programmes are maintained by following evidence-based clinical guidelines and measuring key performance indicators.

INTRODUCTION

for the management of chronic respiratory disease (3), as it significantly reduces healthcare utilization by stabilizing or reversing systemic manifestations of chronic lung disease (4,5). The strong evidence base regarding the efficacy of PR has led to it being endorsed as a key component of comprehensive care for not only COPD (6), but also for interstitial lung disease and pulmonary hypertension (7,8). People with asthma, cystic fibrosis, non-cystic fibrosis bronchiectasis, lung cancer and those preparing for or recovering from lung transplantation can also benefit from PR (9). Despite its well-established benefits and guideline recommendations for its use, PR is severely underutilized in practice worldwide. Data from numerous countries show that only 3%–16% of eligible patients with COPD are referred to PR (10–16). For example, out of more than 33,000 Medicare beneficiaries with COPD in the United States,

Pulmonary rehabilitation (PR) is ‘a comprehensive intervention based on a thorough patient assessment followed by patient-tailored therapies that include, but are not limited to, exercise training, education, and behaviour change, designed to improve the physical and psychological condition of people with chronic respiratory disease and to promote the long-term adherence to health-enhancing behaviours’ (1). The 2019 Global Initiative for Chronic Obstructive Lung Disease (GOLD) document concludes that PR is the most effective therapeutic strategy to improve shortness of breath, health status and exercise capacity for patients with chronic obstructive pulmonary disease (COPD) (2). As well as clear benefits for patients, PR has also been shown to be one of the most cost-effective strategies

29

30  Enhancing use and delivery of pulmonary rehabilitation

2.6% received PR in 2003, and only 3.7% received it as of 2012 (16). In 2013/14 in England and Wales, 15% of eligible patients were referred, but of these individuals, only 69% attended an initial assessment (10). In the United States, referral rates to PR following acute COPD exacerbation are especially low, approximately 2%–3% (17). While data are thus far lacking, it is likely that PR referral rates for people with non-COPD respiratory disorders are even lower than those for individuals with COPD. The recent American Thoracic Society (ATS)/ European Respiratory Society (ERS) policy statement on PR has attributed poor utilization of PR to multiple factors including lack of healthcare professional, payer, patient and caregiver awareness and knowledge of PR; insufficient funding and limited resources for PR programmes; and multifactorial issues surrounding patient access to PR programmes. All of these issues widen the gap between scientific evidence and actual delivery of PR services (18). There is also a need to ensure the quality of PR programmes. The Theoretical Domains Framework (TDF) incorporates a number of behaviour change theories that can be used to help explain issues relating to implementation of best practice in healthcare settings (19). When applied to PR in a review of 48 studies, environment (e.g. travel, waiting time), knowledge (e.g. referral processes) and beliefs about consequences (e.g. expectations of outcomes) were the most frequently reported domains to hinder or facilitate participation (20), reinforcing the conclusions of the ATS/ERS policy statement (18). Furthermore, a global survey including 430 centres in 40 countries showed that within and between continents, PR programmes are heterogeneous in terms of content and organizational characteristics (21). The setting of PR programmes, composition of the team, case mix of individuals, programme completion rates, methods of referral and types of reimbursement all vary widely (21). This makes international benchmarking challenging and limits the degree to which research findings can be extrapolated to other models of PR. Accordingly, this chapter will address the key factors that impede patients’ referral, uptake and completion of PR programmes, as well as review evidence that has targeted these factors. Actions that will be proposed encompass enhancing healthcare professional, patient and payer knowledge and awareness of PR; increasing access and uptake to PR and ensuring quality of PR programmes.

 NOWLEDGE AND AWARENESS OF K PULMONARY REHABILITATION Increasing healthcare professionals’ knowledge and awareness to enhance patient referral to PR To initiate patient uptake of PR, healthcare professionals from primary, community, secondary and tertiary healthcare settings are required to identify eligible patients and

refer them to a programme. Thus, it is important for healthcare professionals to have a clear understanding of the science, process and benefits of PR in order for this referral process to occur. The National COPD Audit Programme in England and Wales estimated that in 2013/14 446,000 patients with COPD were eligible for referral to PR, however only 68,000 referrals were made (10). A recent review focusing on healthcare professional barriers and enablers to PR referrals worldwide identified a total of 29 barriers to PR referral (22). The two reported most commonly were a dearth of knowledge of and/or providers’ disbelief in the benefits of PR, and low knowledge of the referral process. Other frequently reported barriers include low knowledge of patient eligibility, low awareness (no reminders as part of clinical workflow), belief that it would be too difficult to change patient behaviour and ambiguity around whose role it is to refer patients (22–24). A focus on pharmacotherapy rather than non-pharmacologic treatments, reliance on patients to take the initiative, perception of patients as lacking motivation for PR and lack of resources to discuss PR are other reported barriers to PR referral (25). Also, despite healthcare professionals’ awareness of diagnosis and treatment guidelines for conditions such as COPD, evidence-based treatment is often not applied (26). On the other hand, the main enabler to referral identified was PR training, mentoring or experience in outpatient clinics (23,25,27,28). At present, exposure to the process, indications and benefits of PR are not universally compulsory features of education for healthcare professionals providing care for patients with respiratory disease (18), and most clinicians lack direct exposure to PR in the context of their daily practice. This may contribute significantly to the lack of knowledge and awareness of PR, as any extensive training has to be undertaken at the discretion of the healthcare professional. Recommendations and actionable items to foster healthcare professionals’ knowledge and awareness of PR were proposed in the ATS/ERS policy statement on PR (18). Further facilitators of PR referral include PR awareness campaigns (23,27), reminders or prompts (e.g. computer pop-ups, posters and/or mugs) for clinicians during patient appointments (23,29) and having a streamlined referral process (24,27). A recent systematic review of interventions to increase referral rates to PR reported education and learning support as key features of helpful interventions targeted at healthcare professionals (30). For instance, implementation of an educational programme for 154 general practitioners (GPs) and their staff (nurses, secretaries and laboratory technicians) led to a small, yet statistically significant increase in PR referral of 3.5% (14). Educational sessions included meetings with a consultant focusing on GOLD guidelines, regional meetings with 30 GPs and pulmonary specialists to discuss guidelines and a symposium with workshops for all GPs and staff. An alternative intervention that has elicited a significant increase (5%) in patient referral across 18 general practices was based on the Institute for Healthcare Improvement’s ‘Breakthrough Series’ collaborative model

Knowledge and awareness of pulmonary rehabilitation  31

(31). This intervention encompassed collaborative learning sessions for clinical staff, tools to facilitate practice changes such as a COPD flow sheet, COPD registry and patient selfmanagement worksheet and feedback from patient focus groups (32). Although intervention studies to increase referral to PR are heterogeneous, they highlight the need for core formal training and educational sessions on PR for clinicians who treat patients with chronic respiratory disease. When looking specifically at PR following hospitalization for an acute COPD exacerbation, implementation of an evidence-based discharge care bundle increased referrals to PR by 54.4% in London hospitals (33). Items in the bundle included PR, smoking cessation, patient information materials, inhaler use and an outpatient follow-up appointment with specialist, which all had to be signed off by the nurse and patient prior to hospital discharge (33). This suggests that setting key discharge processes in the discharge documents can improve compliance with optimal evidencebased healthcare practice, including referrals to PR. Overall, possible interventions to target the barriers to PR referral by healthcare professionals include training on the process; indications and benefits of PR; provision of hands-on experience with PR within pulmonary outpatient clinics; PR awareness events; streamlined referral processes and/or discharge procedures; reminders such as computer pop-ups/computerized decision support or other visual aids during reviews and monitoring of quality indicators and referrals (22,23,28,30,34). Key barriers and facilitators of referral to PR are shown in Table 4.1. Ideally also, referrals of suitable patients to PR should be included in national Table 4.1 Barriers and facilitators to PR referral by healthcare professionals Barriers • Low knowledge of the process, indications and benefits of PR • Low knowledge of how to refer to PR • Low knowledge of patient eligibility criteria • Ambiguity around whose role it is to refer patients • Focus on pharmacotherapy rather than nonpharmacologic treatments • Lack of resources to discuss PR • Perception of patients lacking motivation for PR

Facilitators • Include PR as a compulsory feature of the training curriculum for healthcare professionals treating patients with respiratory diseases • Increase provision of mentoring and hands on experience in PR for health care professionals • Streamlined referral process and hospital discharge procedures • PR awareness events • Reminders or prompts during patient appointments (e.g. computer pop-ups or other visual aids) • Monitoring of referrals

quality metrics for healthcare professionals who care for people with chronic respiratory diseases. However, there is a need for further high-quality research into interventions that target barriers to and facilitators of PR referral.

Increasing patient knowledge and awareness While it is the duty of healthcare professionals to refer patients to PR, lack of patient awareness also contributes to patients not seeking referral to PR (18). Although figures may vary between countries, evidence suggests that patient awareness of PR is very low (35–39). For instance, in a European study, 85% of patients thought that drugs were the only treatment option for COPD and only 7.5% of patients stated that they had heard about PR (36). Enhancing patient understanding of the health benefits of PR is imperative for patients to be able to make an informed decision on their participation and to request access to PR programmes. Many patients with a high symptom burden and limited physical function may find the idea of exercise frightening or counterintuitive and/or perceive they will not gain health benefits, and therefore will try to avoid it, without comprehending the significant benefits it could yield (20,37,40,41). Others may view themselves as ‘not sick enough’ to need PR (20,37,41). A patient survey conducted in 29 countries in Europe and America, involving 1685 individuals with chronic respiratory diseases, reported that 22.4% of respondents had not been told about PR by their healthcare provider (42). Other challenges that were described included a lack of information to decide whether to participate (9.6%), uncertainty regarding the benefits PR would provide (8.9%) and concerns that PR may be painful (2.2%) (See Figure 4.1) (42). This coincides with issues raised in the recent ATS/ ERS policy statement on PR (18), which highlights the need for national and international patient advocacy groups to develop and disseminate language, education level and culturally appropriate education materials in various formats (e.g. written, internet-based) for patients. In addition, communication campaigns using social media platforms could be used to heighten public awareness of PR (43). Recently, to this end, in the United States, a website to foster public awareness of PR has been developed (44); this project was funded by a private family foundation and developed in collaboration with the ATS. This form of promotion will also help family members, caregivers and friends to become more aware of PR and encourage individuals with chronic respiratory diseases to participate. An intervention exploring the use of a quality improvement tool to empower patients to actively engage in decisions regarding their care increased patient referral rates by 7.4% (45). This involved a patient-held scorecard containing six care quality indicators derived from the British National Institute for Clinical Excellence (NICE) standards, which was co-designed by both patients and primary care staff. This allowed patients to compare the quality of their current

32  Enhancing use and delivery of pulmonary rehabilitation

Family not supportive Worried it might be painful Did not qualify for the service in my area Doctor did not think it would help Lack of encouragement from staff Service is too far away Low self-confidence or anxiety Not covered by insurance No PR service in the area Not sure if it would help Not enough information Other Never heard of PR 0%

5%

10%

15%

20%

25%

Figure 4.1  Patient perspectives on challenges to taking part in PR. Data reproduced from table published in ERJ Open Res., involving 1685 patients across 29 countries.

care against national recommended guidelines. Findings from this preliminary study indicate that improving the transparency and patient understanding of care options and national recommendations can help to build a mutual partnership between patients and their clinicians, leading to increased referral rates.

Increasing payer knowledge and awareness Despite PR being established as one of the most cost-effective therapies for patients with chronic lung disease, funding for PR programmes is insufficient in significant part due to suboptimal payer knowledge and awareness. This lack of funding leads to limited programme availability and capacity for eligible patients and has potential to restrict the quality and subsequent effectiveness of PR programmes. The problem of insufficient payer support is an enormous challenge, because it is the area wherein healthcare professionals have least control over the outcome. There may, however, be opportunities to make ‘inroads’ to gain increased funding and reimbursement for PR over time. The ATS/ERS PR policy statement emphasized the need for improved communication between healthcare professionals, professional societies (e.g. ATS, ERS and others), payers and health policy authorities regarding the process, benefits and cost-effectiveness of PR (18). Therefore, respiratory disease societies should collaborate with primary care providers and patient advocacy groups to develop ways to communicate the benefits, costs and value of PR to payers. For example, PR programmes should routinely maintain a registry and database of their patient enrolment and clinical outcomes to demonstrate programme efficacy for payers. Programmes must also effectively detail programme costs to payers to advocate for reasonable reimbursement. Real-world studies demonstrating cost-effectiveness of programmes and potential cost-savings related to avoidance of costly acute events such as disease exacerbations and

hospitalizations are needed. Combined efforts of healthcare professionals, patients and the general public such as the National Institutes of Health Town Hall Meetings that resulted in the development of the National Action Plan for COPD in the United States (46), may also promote payer recognition of the benefits and support for PR funding over time.

ACCESS, UPTAKE AND ADHERENCE TO PULMONARY REHABILITATION Poor accessibility to PR programmes represents a significant barrier to the delivery and uptake of PR. Data from the England and Wales audit showed that of the 68,000 people referred for PR, only 69% attended their pre-assessment, following which 10%–15% of patients ultimately enrolled in a PR programme (10). Factors that limit access to PR include insufficient programme capacity as well as geographical location leading to unacceptable and unaffordable travel distances for some patients. There is wide geographic variability in access to PR. A recent rigorous analysis of geographic location of PR programmes in the United States demonstrated significant disparities in access to PR services (47). A total of 2068 hospital-based outpatient PR programmes were identified; 1366 US counties (or equivalents) were found to have at least one programme, whereas programmes (and in some cases even hospitals) were lacking altogether in 1776 counties (47). Rural areas were particularly under-served. The geographic access to PR does vary across countries. For example, a recent analysis in Belgium demonstrated that PR programmes are available in the majority of Belgian hospitals (48). However, variability in payer funding impacted the scope of the PR provider teams, interventions and services offered, and outcomes measured in the programmes (48). Moreover, socioeconomic and racial disparities impact patients’ access (49) and adherence to PR (50). Even in areas where PR programmes are available, most programmes’

Access, uptake and adherence to pulmonary rehabilitation  33

resources are limited such that they enrol fewer than 135 patients per year (21,51). One study demonstrated that only 0.8% of Canadians with moderate-to-severe COPD had access to PR (52). Suboptimal local logistics and infrastructure also impact access to PR. For instance, elderly, frail patients who lack social support may find it particularly difficult to attend a programme, especially if disabled parking or adequate transport is expensive and/or not available (40). Importantly also, access to PR is limited, at least in some countries, by an insufficient number of healthcare professionals trained in the delivery of PR. Requirements for training in PR vary across countries and for the different healthcare disciplines (physicians, nurses and other allied healthcare professionals). Since training in PR is often not required, healthcare professionals may not have exposure to PR during training such that they develop an interest in pursuing it as a career option. The impact of local and regional availability of PR providers on access to PR worldwide is an area in urgent need of research.

Increasing access to pulmonary rehabilitation The barriers surrounding accessibility highlight the need for novel solutions to increase patients’ access to and uptake of PR (18). One potential approach to increase access to PR is to add and combine PR to existing cardiac rehabilitation programmes (53). Many patients with lung disease have concurrent cardiovascular disease, and cardiac rehabilitation programmes tend to be better-resourced than PR programmes, with significantly shorter waiting times between referral and enrolment onto a programme (10,54), at least in the UK. Addition of PR content into cardiac rehabilitation programmes could increase the total number of centre-based PR programmes and the number of patients who could be accommodated locally within them. Alternative modes of providing multidisciplinary rehabilitation such as home-based programmes or tele-rehabilitation are also potential solutions to increase PR access that may be suitable for some patients. Current research within this area demonstrates positive results for individuals with COPD (55). For example, in a study among COPD patients, 8 weeks of home-based PR consisting of one home visit and weekly telephone calls from a physiotherapist led to shortterm improvements in functional exercise capacity and health-related quality of life (56). In another study, 7 weeks of home-based PR consisting of daily walking and three-timesweekly resistance exercise, supported by a health manual and two telephone calls from PR staff, led to significant gains in Chronic Respiratory Disease Questionnaire (CRQ) dyspnoea scores; however, non-inferiority compared with centre-based PR could not be confirmed in this trial (57). In addition, several observational studies and randomized controlled trials (RCTs) have demonstrated feasibility and efficacy of tele-rehabilitation (58–60). In one RCT, tele-rehabilitation was equally effective at reducing the

risk for acute COPD exacerbation and hospitalizations as hospital-based outpatient PR (61). In another RCT, participants in tele-rehabilitation following hospitalization for COPD exacerbation had lower 30-day all-cause and COPDrelated readmissions, and longer time to first readmission compared to a control group (62). A recently published Cochrane review protocol (63) planning to evaluate the effects of tele-rehabilitation on exercise capacity, breathlessness and health-related quality of life compared to traditional, centre-based PR will provide more robust evidence on the efficacy of tele-rehabilitation and facilitate its translation into clinical practice if found to be effective. Another novel model that has potential to increase access to PR is provision of physiotherapist-supervised group exercise training and education in local public community gyms. One pilot study evaluating this approach demonstrated feasibility, improvements in 6-minute walk test distance and Medical Research Council (MRC) dyspnoea scores, high completion rates, and decreased wait times for centre-based PR programmes (64). Further work is needed to validate and confirm the efficacy of this approach. Novel approaches to expand PR services are especially needed in rural areas. Innovative programmes have been piloted with some success, including the ‘Breathe Easy, Walk Easy’ programme in rural Australia (65), and a community-based programme in rural Appalachia in the United States, wherein a family foundation partnered with a federally qualified nonprofit community health centre to establish new PR programmes in rural areas (66). Despite these positive findings, there is a need for more evidence from pragmatic clinical trials to support the efficacy of these novel approaches, and to identify which patients are most suitable for them. Moreover, not all studies of novel approaches to PR have shown positive results, and importantly, in most countries they are not yet formally recognized, resourced, funded or reimbursed by healthcare systems. Novel models are also not yet defined by quality standards for process or performance metrics, and characteristics that determine or support which patients are optimally suited to individual models of PR are not yet outlined. As such, at present, these novel approaches remain largely investigative, with their use confined to the research setting. In considering novel models of PR, it is also important to consider patients’ views and preferences, since patient engagement is essential to achieve PR uptake and adherence. Evidence in this area is, to date, sparse, but qualitative interviews have suggested patients value programme convenience, flexibility and local availability (67), but feel that safety, collaborative and supportive interactions with PR programme staff and peers, and the social aspects of exercising and learning are important (67,68).

Interventions to increase uptake to pulmonary rehabilitation A recent systematic review revealed that two studies incorporating interventions to improve patient education and

34  Enhancing use and delivery of pulmonary rehabilitation

promote partnership between patients and healthcare professionals significantly increased patient uptake to PR (30). Harris et al. (69) found an 18% increase in PR uptake among patients of lower socioeconomic status following implementation of a patient manual summarizing evidence on COPD designed to stimulate discussion between the patient and doctor, compared to no increase seen in the control group. Furthermore, Zwar et al. (70) reported a 21.5% increase in uptake to PR compared to a control group following implementation of a partnership model of care in which GPs, nurses and patients worked collectively to develop and implement a 6-month personalized care plan, which was delivered by the nurses at the patient’s home. These studies suggest that facilitating a constructive healthcare professional-patient relationship may enhance patients’ uptake of evidence-based care. Key factors limiting patient access to PR and potential solutions to increase access are shown in Table 4.2. Although studies to date are encouraging, existing evidence is as yet insufficient to guide clinical practice on interventions for improving patient uptake of PR. This was highlighted in a systematic review by Jones et al. (71) which identified only one study that assessed the efficacy of an intervention to increase patient uptake and completion of PR (72). The intervention included provision of a tablet computer to patients attending outpatient PR, which included a training diary, exercise videos and training results. Use of the tablet increased PR completion by 11%, however this was not statistically significant. The dearth of evidence in this area poses a major barrier to addressing continuing challenges relating to patient uptake and completion of PR programmes. Therefore, there is a need for high-quality studies implementing interventions that target the key barriers to PR uptake amongst people with COPD, as well as those with non-COPD respiratory disorders. Table 4.2  Factors that contribute to poor patient access to PR and potential solutions Factors limiting patient access to PR • Lack or cost of transport • Geographical location • Lack of parking • Inconvenient timing of PR sessions • Limited capacity of programmes • Lack of social support

Potential solutions • Novel PR program models should be developed and studied (e.g. community based, home based or tele-rehabilitation) • New PR programmes should be created in geographic areas where demand exceeds capacity • Combine PR and cardiac rehabilitation programmes • Increase healthcare professional training and exposure to PR to enhance the number of staff trained in delivering PR

USE OF PULMONARY REHABILITATION SERVICES COMPARED TO OTHER EFFECTIVE THERAPIES IN COPD Widely-utilized healthcare recommendations such as immunizations are fostered by high levels of organizational, community, payer, clinician and patient support (28,34). In seeking to understand the reasons behind the poor availability and uptake of PR, a recent study compared PR to alternative COPD therapies such as bronchodilators and oxygen therapy (73). Unlike PR, these therapies are used widely in developed countries. When comparing the efficacy of PR to bronchodilators, PR yielded significantly greater benefits in terms of exercise tolerance, dyspnoea and quality of life, well exceeding the minimal clinically important difference of gain in each of those outcome domains. In addition, PR has been shown to be more cost-effective than bronchodilator therapy, therefore this should not be a major barrier to its availability and uptake from the provider’s perspective (3). One possible reason that PR is so underutilized in comparison to other therapies is that in contrast to PR, bronchodilators are supported by product marketing and have extensive clinical research to demonstrate the product’s benefits. Although the benefits of PR are also supported by extensive clinical research, PR programmes generally have much less support for implementation, and lack backing of and promotion by large corporate entities. While there is no obvious solution to this disparity, one health outcome that is high priority for patients, healthcare professionals and payers is survival (73). Emerging retrospective analysis of data from clinical trials of PR implemented following hospitalizations for COPD exacerbations suggests a survival benefit of PR (74,75). A well-designed prospective RCT confirming a survival benefit of PR would likely drive increased demand for PR referrals and PR programme availability.

ENSURING QUALITY OF PR PROGRAMMES Best practice recommendations for PR are outlined in the 2013 ATS/ERS statement on PR (1), the 2013 British Thoracic Society (BTS) quality standards (76) and additional documents (77–79). As stated in the ATS/ERS policy statement (18), to meet the requirements for PR, the programme should, at a minimum, include comprehensive assessment at baseline and post PR, a structured and supervised exercise training programme, educational/behavioural content intended to foster long-term health enhancing behaviour and recommendations to adopt home-based exercise and physical activity. Furthermore, staff should be proficient at delivering PR and be able to demonstrate this. Where feasible, a broader-based patient assessment and additional outcome measures including (but not limited to) functional status, balance, anxiety and depression, knowledge and self-efficacy, smoking status and nutritional status should be undertaken (80,81). Importantly, patients have varying disease management and rehabilitative needs as well

Summary 35

as differential responses to PR (82). These varying needs depend on the underlying disease, disease severity, symptoms, physical fitness, exercise capacity and functional status, physical activity levels, comorbidities, hospitalizations, nutritional and psychosocial issues and patients’ self-efficacy to manage their condition. Individual patients’ needs and goals must be considered and addressed. Thus, ‘one size does not fit all’; while some people may be wellsuited to undergo PR in a setting with streamlined staff and limited equipment and/or space, others have complex needs for which a multidisciplinary team of providers and a broad spectrum of treatment interventions are needed. Indeed, while PR has always been patient-centred, and the exercise training individualized to patients’ abilities and needs, the practice of PR is evolving towards a more multifaceted, personalized approach (83–85), wherein a broadbased patient assessment guides decision making regarding the approaches to rehabilitation undertaken. Individual patient factors may impact success for specific approaches. For example, in one study, patients’ self-efficacy predicted whether they would achieve gains in 6-minute walking distance after participation in an exercise training-only intervention (without accompanying educational sessions) (86). Further research is needed to identify patient characteristics and other factors that predict success of individual models of PR delivery for the varying aspects and components of patients’ needs. As noted previously, a global survey of 40 countries showed that differences exist in the content and organizational aspects of PR programmes within and among countries (21). For instance, the number and type of healthcare professionals varied between PR programmes. In European programmes, chest physicians, physiotherapists, occupational therapists, social workers, psychologists and cardiologists were more frequently employed. In contrast, dieticians, exercise physiologists and respiratory therapists were common in North American programmes. Further differences were seen in the setting, the case mix of individuals, methods of referral, completion rates and methods of reimbursement. These observed differences highlight the need to consider a set of uniform processes and performance metrics to enable monitoring and international benchmarking of PR. In reality, however, this is challenging due to differences in PR structure and content, in significant part dictated by health systems in different countries. A strategy to ensure quality standards have been met by PR programmes is to complete regular audits or, alternatively, implement a certification process. Currently, the American Association of Cardiovascular and Pulmonary Rehabilitation (AACVPR), the Royal College of Physicians in the UK (in collaboration with the National Asthma and COPD Audit Programme, BTS and British Lung Foundation), and some countries in Europe offer a certification process for PR services (18). Introducing an international accreditation would help to ensure PR quality on a worldwide level and improve the disparity of PR delivery between different countries. Thus, international respiratory

societies such as the ERS Pulmonary Rehabilitation and Chronic Care and Physiotherapists groups, the AACVPR and ATS Pulmonary Rehabilitation Assembly would benefit from discussing and developing key performance and process indicators for various models of PR (e.g. home- vs. centre-based, etc.) (18,40). Furthermore, collaboration among these societies to expand existing national PR registries so that they can be used worldwide will provide data for benchmarking and demonstrate the positive impact of PR. This should help to further encourage patients to seek and healthcare professionals to provide PR referral, and should promote funding support for PR services.

CONCLUSION There is an urgent need to expand implementation and delivery of PR services to people with chronic respiratory diseases. Broader participation of suitable candidates would not only improve the health of individuals, but has potential broader implications for improving public health, disease management and reducing healthcare costs. Increasing healthcare professionals, patients, general public and payers’ knowledge and awareness of PR, and expanding access to PR will take time and will require a multifaceted approach. The optimal strategies to increase access, to secure increased funding and to implement novel models of PR will vary across countries and healthcare systems and must be done in a manner sensitive to cultural norms. It is essential that the core components and quality of programmes be maintained, and efficacy of programmes regarding proven expected PR outcomes be demonstrated.

SUMMARY Pulmonary rehabilitation (PR) is a comprehensive intervention designed to improve the physical and psychological condition of patients with chronic respiratory disease and promote long-term adherence to health-enhancing behaviours. It is now recognized as one of the most cost-effective strategies for managing chronic respiratory disease and is recommended in clinical guidelines worldwide. Despite its well-established benefits and recommended use, PR is significantly underutilized worldwide, with data showing that only 3%–16% of eligible patients with chronic obstructive pulmonary disease receive PR. The poor utilization of PR stems from low referral rates of patients to PR services. Strategies to increase referral to PR focus primarily on increasing the knowledge and awareness of PR among not only healthcare professionals, but also patients and payers. Limited accessibility of PR in terms of programme capacity, geographical location, poor local logistics and infrastructure can negatively impact patient uptake and adherence to PR programmes. Potential solutions to improve patient accessibility include novel models of PR such as home-based training, tele-rehabilitation or walking programmes; however, more high-quality clinical trials are required to support the efficacy of these approaches and determine patient

36  Enhancing use and delivery of pulmonary rehabilitation

suitability. Furthermore, combining PR with cardiac rehabilitation programmes, which tend to be better resourced, may be another solution that should be explored. Finally, it is important that PR quality is maintained by ensuring clinical guidelines are adhered to and key performance indicators are measured.

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65. Johnston CL, Maxwell LJ, Boyle E, Maguire GP, Alison JA. Improving chronic lung disease management in rural and remote Australia: The Breathe Easy Walk Easy programme. Respirology (Carlton, Vic). 2013;18(1):161–9. 66. Doyle D, Tommarello C, Broce M, Emmett M, Pollard C. Implementation and outcomes of a community-based pulmonary rehabilitation program in rural Appalachia. J Cardiopulm Rehabil Prev. 2017;37(4):295–8. 67. Lahham A, McDonald CF, Mahal A et al. Home-based pulmonary rehabilitation for people with COPD: A qualitative study reporting the patient perspective. Chron Respir Dis. 2018;15(2):123–30. 68. Fischer MJ, Scharloo M, Abbink JJ et al. Participation and drop-out in pulmonary rehabilitation: A qualitative analysis of the patient’s perspective. Clin Rehabil. 2007;21(3):212–21. 69. Harris M, Smith BJ, Veale AJ, Esterman A, Frith PA, Selim P. Providing reviews of evidence to COPD patients: Controlled prospective 12-month trial. Chron Respir Dis. 2009;6(3):165–73. 70. Zwar NA, Hermiz O, Comino E et al. Care of patients with a diagnosis of chronic obstructive pulmonary disease: a cluster randomised controlled trial. Med J Aust. 2012;197(7):394–8. 71. Jones AW, Taylor A, Gowler H, O’Kelly N, Ghosh S, Bridle C. Systematic review of interventions to improve patient uptake and completion of pulmonary rehabilitation in COPD. ERJ Open Res. 2017;3(1):​ 00089–2016. 72. Ringbaek TJ, Lavesen M, Lange P. Tablet computers to support outpatient pulmonary rehabilitation in patients with COPD. Eur Clin Respir J. 2016;3:31016. 73. Casaburi R. Pulmonary rehabilitation: Where we’ve succeeded and where we’ve failed. COPD. 2018;15(3):​ 219–22. 74. Ryrsø CK, Godtfredsen NS, Kofod LM et al. Lower mortality after early supervised pulmonary rehabilitation following COPD-exacerbations: A systematic review and meta-analysis. BMC Pulm Med. 2018;​18(1):154. 75. Lindenauer PK, Stefan MS, Priya A et al. Association between pulmonary rehabilitation following hospitalization for COPD and 1-year mortality. C17 Am J Respir Crit Care Med. 2019;2019:A4269. 76. Bolton CE, Bevan-Smith EF, Blakey JD et  al. British Thoracic Society guideline on pulmonary rehabilitation in adults: Accredited by NICE. Thorax. 2013;​ 68(Suppl 2):ii1–ii30. 77. Marciniuk DD, Goodridge D, Hernandez P et  al. Managing dyspnea in patients with advanced chronic obstructive pulmonary disease: A Canadian Thoracic Society clinical practice guideline. Can Respir J. 2011;​ 18(2):69–78. 78. Ries AL, Bauldoff GS, Carlin BW et  al. Pulmonary rehabilitation: Joint ACCP/AACVPR evidencebased clinical practice guidelines. Chest. 2007;131(5 Suppl):4s–42s.

References 39

79. Singh SJ, ZuWallack RL, Garvey C, Spruit MA. Learn from the past and create the future: The 2013 ATS/ERS statement on pulmonary rehabilitation. Eur Respir J. 2013;42(5):1169–74. 80. Singh S. Approaches to outcome assessment in pulmonary rehabilitation. Clin Chest Med. 2014;35(2):​353–61. 81. Spruit MA, Wouters EFM. Organizational aspects of pulmonary rehabilitation in chronic respiratory diseases. Respirology (Carlton, Vic). 2019;24(9):838−43. doi:10.1111/resp.13512. 82. Spruit MA, Augustin IML, Vanfleteren LE et  al. Differential response to pulmonary rehabilitation in COPD: Multidimensional profiling. Eur Respir J. 2015;​ 46(6):1625–35.

83. Houben-Wilke S, Augustin IM et  al. COPD stands for complex obstructive pulmonary disease. Eur Respir Rev. 2018;27(148):180027. 84. Wouters EFM, Wouters BBREF, Augustin IML, HoubenWilke S, Vanfleteren LEGW, Franssen FME. Personalised pulmonary rehabilitation in COPD. Eur Respir Rev. 2018;27(147):170125. 85. Troosters T, Blondeel A, Janssens W, Demeyer H. The past, present and future of pulmonary rehabilitation. Respirology (Carlton, Vic). 2019;24(9):830−7. 86. Blackstock FC, Webster KE, McDonald CF, Hill CJ. Self-efficacy predicts success in an exercise trainingonly model of pulmonary rehabilitation for people with COPD. J Cardiopulm Rehabil Prev. 2018;38(5):333–41.

5 Pathophysiological basis, evaluation and rationale of exercise training PIERANTONIO LAVENEZIANA AND PAOLO PALANGE Introduction Pathophysiological basis of exercise training Rationale of exercise testing Exploring factors explaining exercise limitation

41 42 43 44

Rationale of exercise training Benefits of exercise training Summary References

46 46 48 48

KEY MESSAGES • Exercise training is a key intervention in pulmonary rehabilitation and improves exercise capacity and tolerance. • The patient undergoing rehabilitation needs to be evaluated with an exercise testing modality that matches the sophistication of the rehabilitative intervention. • Pulmonary rehabilitation leads to substantial benefits in dyspnoea, exercise capacity, health-related quality of life and healthcare utilization. • These benefits are of greater magnitude than that seen with other medical therapies such as bronchodilators.

They are achieved without discernible improvements in traditional measures of physiological impairment, such as the FEV1. • This apparent paradox is explained by the fact that PR addresses the systemic effects of chronic respiratory disease, including peripheral muscle dysfunction, physical inactivity leading to deconditioning, anxiety and depression and maladaptive behaviours such as a sedentary lifestyle and poor adherence to prescribed therapies.

INTRODUCTION We decided to start this chapter by challenging the reader with three questions; the answers may be sometimes provocative; however, they stimulate the scientific and clinical discussion around this topic:

3. Should the intensity of exercise training be reduced when the patient is not able to cope with the proposed training intensity?   Answer: No, other modalities of training, such as interval training, can be used when the patient cannot cope with a high training intensity.

1. Which tests are essential in the design of an exercise training programme?   Answer: Incremental exercise test and skeletal and respiratory muscle force testing are fundamental. 2. Is exercise training a guarantee to meet the patient’s needs?   Answer: No, improvements in exercise capacity and skeletal muscle force should be implemented in the daily life of the patient. This is the task of the multidisciplinary rehabilitation team. Exercise training is, however, the hallmark of a rehabilitation programme.

Pulmonary rehabilitation (PR) has evolved into a highly scientific discipline with a firm physiologic basis. Exercise training is a key intervention, and improving exercise tolerance is a major goal (1,2). Exercise training does improve exercise capacity and tolerance. However, this is clearly only the first step in improving patient participation in everyday life. Maximizing exercise tolerance will enhance the patient’s potential to increase physical activity in daily life, and it may boost the patient’s confidence and self-efficacy to be physically active (1,2). The patient undergoing 41

42  Pathophysiological basis, evaluation and rationale of exercise training

rehabilitation needs to be evaluated with an exercise testing modality that matches the sophistication of the rehabilitative intervention (3). Existing tools available to measure exercise capacity or tolerance range from simple, low-cost field tests requiring little equipment to cardiopulmonary exercise testing (CPET), which requires specialized equipment and trained technical staff, and carries higher associated costs (3). As many PR programmes lack access to CPET resources, the use of CPET is often limited to specialized centres. Nonetheless, the commonly used low-cost field tests requiring little equipment have serious deficiencies as compared to CPET in evaluating scientifically and accurately the goals to be achieved by exercise testing in the context of PR, which are the following (3): 1. Accurately measure exercise tolerance – Peak oxygen uptake in an incremental test has well-defined normal values. The anaerobic threshold has the advantage of being an effort-independent measure of exercise tolerance. 2. Assess mechanisms of exercise limitation – Patterns of ventilatory and gas exchange responses to incremental exercise testing can define specific pathophysiologic entities. It can be determined whether exercise is limited by cardiovascular, gas exchange or ventilatory function (or a combination of these). Additional manoeuvres can be added to the test to clarify pathophysiology, e.g., inspiratory capacity manoeuvres to assess dynamic hyperinflation. Some assessments require arterial blood sampling, though noninvasive assessments can sometimes suffice, e.g. some exercise VE/VCO2 ranges allow normality or abnormality of VD/V T to be predicted with confidence. 3. Define contraindications to an exercise programme – In the elderly rehabilitation population, comorbidities are common. Cardiac disease is particularly prevalent. An exercise electrocardiographic evaluation complemented by simultaneous gas exchange measurements provides important information as to the safety of an exercise training programme. Cardiac ischaemia, arrhythmias and abnormal blood pressure responses are often present and cannot be predicted from resting measurements. 4. Determine the need for oxygen supplementation − Oxygen supplementation during exercise improves exercise tolerance and prevents hypoxaemia. The oxygen dose (nasal cannula litre flow) necessary to prevent hypoxaemia cannot be predicted from resting measurements. Exercise desaturation is less likely to occur if the exercise involves cycling than if it involves walking. Treadmill exercise testing is especially useful to assess the need for supplemental oxygen if the exercise training programme involves walking tasks. 5. Provide an exercise prescription – High-intensity exercise training provides superior physiologic training benefits as  compared to low-intensity training. An incremental exercise test can provide an initial exercise intensity

target, measured either as a work rate target or a heart rate (HR) target. 6. Measure improvement in exercise tolerance – Constant work rate exercise testing can provide a sensitive measure of exercise tolerance improvement. A necessary prerequisite, however, is that a work rate (WR) is individually designed that yields a pre-intervention exercise duration in the range of approximately 3–8 minutes. This can generally be obtained in ∼60% of patients (mostly COPD) by assigning a WR equal to about 75% of the peak WR in a preceding incremental exercise test, though WR adjustment may be necessary to obtain an exercise duration in the desired range, for example by adjusting the WR (5 watts lower or 5 watts greater) and repeating the test. In COPD patients it has been suggested that 5 watts adjustments (up or down) are sufficient to reset an initial endurance time outside of 3–8 minutes in an additional 30% of the patients.

PATHOPHYSIOLOGICAL BASIS OF EXERCISE TRAINING Training programmes that include exercising of the leg muscles performed with walking, stationary cycling or treadmill exercise are commonly used in sedentary populations (4) and patients suffering from COPD, chronic heart failure (CHF) and pulmonary arterial hypertension (PAH) to improve exercise performance, reduce symptom perception (shortness of breath and leg discomfort) and improve quality of life (3–5). Since exercise training has specific effects, the training programme can be modified based on the patients daily life requirements but this is − in the opinion of the authors − only after the format of the training is set out based on the patient’s exercise limitations (Figure 5.1). Hence ­k nowing the exercise tolerance and the factors limiting exercise performance seems  crucial in the development of successful ­exercise training (6). In general, there is consensus that a number of requirements need to be fulfilled in a rehabilitation session. The duration of the cumulated exercise bouts needs to be at least 30 minutes, and in general whole-body exercise is advised (4). To obtain physiological benefits of exercise, the training intensity is advised to be high. Shortterm effects on functional exercise tolerance and healthrelated quality of life are also seen after training at lower training intensity (1,2). Several modifications can be made to ‘classic’ endurance training. The training prescription is driven by the result of an incremental maximal exercise test as well as the assessment of peripheral and respiratory muscle strength (Figure 5.1). These consist of modification of the training format (e.g. interval training) or the training modality (e.g. resistance training). In patients who may require it, inspiratory muscle training can be added to a comprehensive rehabilitation programme. Ventilatory demands of whole-body exercise can be alleviated by oxygen supplementation or by ventilatory

Rationale of exercise testing  43

Optimal bronchodilatation and safety to perform exercise

Predominantly cardiovascular limitation

Predominantly ventilatory limitation

No Exercise tolerance enhanced by NIMV

Respir. muscle weakness

Yes

Constant work rate test @ 70% Wmax

Whole body endurance TR

Yes

Exercise tolerance enhanced by O2? Yes

Consider TR with NIMV

>10 min

Skeletal muscle weakness?

Desaturation?

Hypogonadism?

Yes

O2 supplements

Consider testosterone supplements

Yes

8/10? Patient achieves predicted VO2 or evidence of a plateau in VO2? Evidence of a ventilatory limitation: breathing reserve 150 mL) VT plateau RR > 50–55 breaths/min (if restrictive pattern) VT = IC or >60% VC (if restrictive pattern) HR peak < HR predicted with or without Gas exchange abnormalities VD/VT ↑ P(A–a)O2 ↑ Decrease of PaO2 ≥ 10 mmHg Decrease of SpO2 ≥ 4% and/or SpO2 peak ≤ 88% PaCO2 peak > 45–50 mmHg

Step 4

Cardiovascular and/or pulmonary vascular BR > 15%–20% VO2/HR < 70% Flat (and declining) VO2/HR trajectory Abnormal HR/VO2 slope (>50) Chronotropic incompetence with or without

Other(s) Anaerobic threshold 14%) of the 720 citations. A search for the word education, on the other hand, yielded 7 of 137 and 4 of the 720, both representing 0.75 and the UCOPD has been shown to be responsive to pulmonary rehabilitation (15,19). The MCID has also not been reported. While these knowledge questionnaires have been available, unfortunately, rarely have they been used as outcome measures in research. The work that has been done to determine if education alone as an intervention has affected outcomes has been undertaken from several approaches. In a study of 53 COPD (n = 26 in the intervention at 1 year) and 71 asthma patients (n = 32 at 1 year), education was provided both in a group setting as well as individually (20). The outcomes targeted were health-related quality of life (HRQoL) with the Saint George’s Respiratory Questionnaire (SGRQ) (21) and spirometry. After 1 year, the asthma group had improved both HRQoL scores and spirometry, but no improvement in these outcomes was observed in the COPD group. In similar work, the effect of education alone was tested in a community setting for its effects on HRQoL using the Sickness Impact Profile (SIP) (22). A total of 213 COPD patients from one community who completed an educational programme were compared to 325 COPD patients who were not exposed to a formal education programme (23). The results after 1 year showed no statistically significant difference between groups in HRQoL. Was the limitation to these studies selecting quality of life as an outcome? For example, it is unclear how education alone would have a profound

56  Education: Realizing the potential for learning in pulmonary rehabilitation

effect on HRQoL unless we understood if the instruments were powered sufficiently to detect change following education. However, it is unlikely that lectures alone would impact a person’s quality of life unless they resulted in a change in behaviour leading to better health. Other studies targeted both education and some form of training. In one study, education and training were tested related to dyspnoea management strategies (24). One might expect a change because training (behaviour change) was part of the intervention. The outcomes measured included HRQoL, 6-mile walk distance (6-MWD) and dyspnoea. Dyspnoea was measured with six different measures. The treatment group (n = 46) received 6 weeks of education and training on dyspnoea while the controls (n = 43) received 6  weeks of general health lectures (not specific to lung disease). At 6 weeks, there was no difference in dyspnoea, HRQoL or walk distance. Another study looked at individual training pertaining to physical activity (25). In a 6-month PR programme, 80 patients were randomized to either individual physical activity education or sham attention. The physical activity counselling included eight individual sessions, each lasting 20–30 minutes, over the 6-month PR programme. Physical activity was evaluated with two activity monitors. Neither intervention nor control group increased their physical activity from baseline at 3 and 6 months. Limited work has been done to determine the unique contribution of the educational component as a result of PR programmes in randomized controlled trials. This is significant given the previously mentioned plethora of studies on exercise. Two studies have evaluated education in PR, one in 2005 and one 2014. In the study by Norweg et al. (26), 43 subjects with COPD were randomized to one of three groups: exercise training alone (n = 12 completers), exercise training plus activity training (n = 11) and exercise training with education lectures (n = 10). Scores on a HRQoL instrument (Chronic Respiratory Disease Questionnaire [CRQ]) (27) and distance walked (6-MWD) were two of several outcomes; however, there was no educational outcome measured. Measures were taken at baseline, 6 weeks following the intervention and 12, 18 and 24 weeks. The HRQoL scores improved in the group with exercise and activity training more than the education group with training (p < 0.05). There was no difference seen in the improvement in 6-MWD between groups. In a study by Blackstock et  al. (28), 267 subjects were randomized to an 8-week education with exercise training (n = 113 completers) or exercise training alone (n = 85). The outcomes included the same measures as Norwig and colleagues: the CRQ and 6-MWD at baseline, 8 weeks, 24 and 48 weeks. Both groups showed similar increases in distance walked and HRQoL at 8 and 24 weeks. However, no difference was observed in an education measure, the Health Education Impact Questionnaire (heiQ) (29), between the group that received education and the one that did not, once again suggesting that HRQoL does not uniquely change with education. Nor, in the latter case, did the non-pulmonary-specific education measure improve.

EDUCATIONAL DESIGN FOR PULMONARY REHABILITATION Education has the ultimate goal of supporting a person to transform how they interact with their world such that their interactions lead to positive outcomes. In the context of health education, it is anticipated that these positive outcomes relate to improved health status and quality of life. Learning is the foundation of transforming health behaviour, and through experiential educational activities, knowledge, skills and attributes can be developed (30). Therefore, in designing educational activities we need to think about what cognitive (knowledge), psychomotor (skill) and affective (attributes and attitudes) outcomes we are striving to support our patients to achieve such that they can change their health behaviours and positively impact their health and quality of life (31). To design and implement educational activities, three key components are necessary in sequential order: (i) articulated learning objectives or outcomes, (ii) delivery of educational activities and (iii) assessment of attainment of learning with reflection on what further e­ ducation is needed (31,32). In pulmonary rehabilitation, step ii − delivery of educational activities − has been the focus of the education component (32). This may be a fundamentally flawed approach to education in pulmonary rehabilitation though. Learners need to understand what the outcomes of learning will be (step i), and they need feedback and time to reflect on whether they are learning (step iii), to redirect themselves if they are not achieving the learning outcomes desired. Education in pulmonary rehabilitation is no exception to this. When formulating learning outcomes, the designer of the education first needs to determine whether the ­outcome of learning is cognitive, psychomotor or affective. The articulated learning outcome(s) forms the foundation for deciding on what activities will support achievement of the outcome. Educational activities to support the development of knowledge will be very different from the ­activities to support development of skill or attribute/behaviour. Cognitively focused learning outcomes centre around memory and recall of theoretical information; ‘knowing facts’. Psychomotor skill development contains both a memory and recall component, but also a motor control aspect. Simply knowing what to do for a psychomotor skill is insufficient for successful learning of the skill. This fact is brought home by the high rate of incorrect inhaler use by patients who were ‘taught’ how to use their inhaler. Psychomotor skill development requires knowing and performance, and as such, learning outcomes focus on capability of completing specified tasks. Finally, affective learning outcomes centre around changing attitudes, beliefs, behaviours, communications, emotional responses and metacognition (i.e.  capacity to think about one’s thinking). Outcomes for the affective domain of learning often refer to formulation and implementation of plans, critical appraisal of one’s health status, problem solving, decision making and self-reflection on actions taken. Table  6.2 provides exemplars of learning outcomes for appropriate oxygen

Educational design for pulmonary rehabilitation  57

Table 6.2  Example of designing learning outcomes, educational activities and assessment of learning Cognitive (knowledge)

Psychomotor (skill)

Educational activities

Recall the prescribed dosage of oxygen needed to improve health status and life expectancy 1. Verbal discussion on impact of oxygen therapy with healthcare professional 2. Written information on dosage

Accurately use oxygen at the dosage prescribed using a cylinder, concentrator and/or liquid as a stationary and portable oxygen systems 1. Observe demonstration of technique to apply nasal prongs and turn on oxygen therapy equipment 2. Practice applying nasal prongs to mannequin, and then self 3. Practice turning on oxygen equipment with peer and/or healthcare provider observing

Resources required

1. Paper, pens, printed handout

Assessment for learning

1. A short quiz on oxygen needs of the body (e.g. O2 saturation ≥90%)

Assessment of learning

1. Presentation on oxygen needs to the next learners commencing PR

1. Mannequin 2. Mirror 3. Nasal prongs 4. Cylinder (stationary and portable) with regulator 5. Concentrator (stationary and portable) 6. Liquid (stationary and portable) 1. Using smartphone, video of self-application of oxygen therapy (including nasal prongs) which is sent to healthcare provider for feedback and confirmation 1. Consistently observed correct application of nasal prongs with prescribed dosage while attending PR

Learning outcomes

Affective (attitudinal) Routinely monitor health status and reflect on effectiveness of oxygen therapy

1. Small group, peer discussion on observed changes in health status and oxygen levels 2. Creation of a recording template (or download an application to smart device with reminders) to record symptoms and health state Note: where the patient creates their own diary – designing a tool that suits the person 1. Structured peer mentorship questions to facilitate group discussion 2. Smartphone or examples for creation of personalized record

1. Documenting flow rates and comparing to peers 2. Discuss recordings with healthcare professional and receive feedback if any changes are made 1. Presentation of diary and selfreflections to healthcare professional

Source: Adapted from Blackstock FC et al. Ann Am Thorac Soc. 2018;15:769–84. Abbreviation:  PR, pulmonary rehabilitation.

therapy use at home, showcasing the variation in wording between cognitive, psychomotor and affective outcomes. Presently, learning outcomes or thresholds for completion of pulmonary rehabilitation have not been established, and research in this area is needed. In the absence of an understanding of learning needed to be achieved by people completing PR, patient goals can be used. To design educational activities, one’s imagination is all that limits what can be created. Lectures, peer discussions, written materials, online content, one-to-one supported reflections and skills practice are some commonly used activities to develop specific cognitive and psychomotor attributes in pulmonary rehabilitation (6). Unfortunately, no direct scholarly comparisons of educational design features have been conducted to provide direction on which activities provide the most impactful learning for patients completing PR. In the absence of the evidence to guide best practice, educational design should be framed in learning theory. There are many theories on how people learn that

provide a framework for designing educational activities, with behaviourism, cognitivism and constructivism being the three most cited theories. Behaviourism theorizes that learning occurs with direct instruction supported by positive or negative affirmation of actions – reinforcement and repetition for rote learning (33). Simplistically, behaviourism is based on observable changes in behavioural patterns where a person learns by repeated reward for a desired action and punishment for undesirable actions. The learner depends on instruction for acquisition of knowledge, and confirmation from the instructor that the knowledge and new behaviour are correct. Cognitivism builds on the theory of behaviourism, theorizing that change in behaviour occurs through sequential development of an individual’s cognitive abilities (34). That is, learning is occurring as a process of step-wise thoughts in a person’s mind that each add to the last thought. Finally, constructivism is based on the theory that a person learns by making meaning of experiences they have (35). Knowledge, skills and attributes are

58  Education: Realizing the potential for learning in pulmonary rehabilitation

constructed by people through experiences and reflections on those experiences. In the constructivism philosophy of education, an educator is therefore a facilitator of learning, not a teacher or transmitter of knowledge (35). In designing education, it is important to consider which learning theory will underpin the learning experience, as different activities will be chosen depending on which theory is applied. For the purpose of education in PR, constructivism theory seems most intuitive to frame the design and implementation of learning activities. For a person to selfmanage their chronic respiratory disease, simply knowing is not enough. Deep understanding and shifts in beliefs, health behaviours and lifestyle are often needed for effective self-management. Experiential learning, where patients are immersed in activities that allow them to apply prior knowledge to a learning activity/situation and construct new knowledge and meaning from this are more likely to be impactful. For example, peer discussions, practical application of skills and simulations of health scenarios where patients can practise decision-making could all be educational activities aligned with experiential learning philosophy. These activities could all be considered active learning, with ‘diagnosis’ of learner needs and interests, mutual planning between learner and teacher, and sequential activities that link learning outcomes to each other and support the person to develop themselves over time. Didactic lectures and written material generally do not provide an environment for active participation and experiential learning, and reconsideration of their relevance in pulmonary rehabilitation is needed (36). However, as previously stated, there is no literature that has directly compared different learning activities or frameworks to determine which is more impactful. Such future research would greatly benefit educators in PR. It is also important to note that in experiential learning, structure to learning activities is still required with clearly articulated learning outcomes and opportunities for assessment of self and feedback from others. Educational design theory is a complex area that experts have studied for decades. This chapter, however, can only provide a very brief overview of design theory. Many texts, websites and literary pieces are available to support the healthcare professional in learning about learning (37–41). To design rich, meaningful learning experiences for patients in the education component of PR, healthcare professionals are not exempt from learning needs. They are encouraged to learn themselves, by identifying their own learning needs and sourcing resources to transform their knowledge on education in PR. As previously outlined, scholarly exploration of best educational design features is also now needed to create truly impactful education in PR. Personal reflection on understanding of learning by healthcare professionals will foster future innovation in educational design.

learning) are often present. Older patients also are at high risk for cognitive impairment from dementia. Added to that is this older age group are at risk for visual difficulties, fine motor impairment and health literacy issues. The challenges are further complicated by cultural and ethnic issues that may create barriers to learning.

FACTORS THAT INFLUENCE LEARNING

Depression and anxiety

Patients with COPD face several challenges, given that hypoxaemia, depression and anxiety (all of which affect

The prevalence of depression and anxiety in COPD patients is substantial. Estimates are that depressive symptoms exist

Hypoxaemia and cognition In order to maximize the educational experience, patients must be able to retain, interpret and apply the information. One of the more frequent issues facing patients with respiratory disease is the effects of their respiratory condition on oxygen levels. Reports of the effects of hypoxaemia in COPD patients were presented over 50 years ago, with subsequent work confirming these effects during the Nocturnal Oxygen Therapy Trial (NOTT). Findings from the NOTT in 121 subjects with hypoxaemia (PaO2 51 mm Hg), showed that approximately 80% displayed disturbances in abstracting ability (reasoning) and complex perceptual motor integrative abilities (42). Another study of 100 COPD patients with mild hypoxaemia (PaO2 66 mm Hg), compared to 25 normal controls showed significant memory deficits (immediate p < 0.05), and delayed deficits (p < 0.01) (43). Memory and reasoning are fundamental to identifying symptoms and the need to seek healthcare by affecting the cognitive capabilities of an individual − the capabilities that underpin the psychomotor skills and broader attributes needed for successful health behaviour change. A number of screening tests are available to evaluate for cognitive deficits (32). More recently, an association has been reported between lung disease and dementia (44). These findings are further support for the effects of lung disease on cognitive impairment from cerebral small vessel disease, stroke and hypoxaemia previously reported (45,46). Unfortunately, there is little evidence that changes in cognition can be reversed. There is some suggestion that exercise may improve cognition; however, the results to date are mixed (47–49). Given the unlikely ability to change the cognitive state of patients, teaching strategies must be adapted to the patient’s learning. Screening for cognitive function is therefore important if we are to approach successful learning. In the instance of memory deficits, providing repetitive verbal along with written instructions and sharing this information with the patient’s caregiver can be effective. Also, the concept of encoding (storing and recalling information from cognitive learning) is important by linking information to what the patient already knows and presenting information in a logical order for ease of comprehension and repeating the information. This is particularly useful in teaching inhaler use, given the multiple steps inhaled devices require patients to learn to maximize deposition of medication.

Digital pulmonary rehabilitation education  59

in 25% of patients with COPD compared to 12% in a general population (50), and symptoms of anxiety are 10%–55% among inpatients and 13%–46% among outpatients (51). Depression and anxiety have also been associated with coping style, whereby those with higher levels of symptoms of depression and anxiety coped by withdrawal and exhibited lower levels of self-efficacy (52). Coping styles are relevant to learning, more so in the setting of depressive symptoms and anxiety. In a study of 698 patients with COPD (53), coping was measured with the Utrecht Coping List (UCL) (54) and symptoms of depression and anxiety with the Hospital Anxiety and Depression Scale (HADS) (55). Patients with symptoms of depression scored low for active confronting and increased their use of avoidance and passive reaction pattern (all p < 0.001). Those with symptoms of anxiety also demonstrated issues with coping style. Similar to those with symptoms of depression, those with high anxiety scores also demonstrated lower active confronting style, avoidance and passive reaction pattern compared to controls (all p < 0.001). Seeking social support was not significantly different and coping style was not associated with HRQoL (using the SGRQ). Coping was also evaluated following PR using the aforementioned measures (56). COPD patients (n = 303) improved their coping skills for active confronting (p < 0.05), while levels of avoidance (p < 0.05), passive reaction pattern and reassuring thoughts decreased (both 5 GAD-2 = 2 DASS-A ≥ 7 BAI ≥ 19 AIR ≥ 8

1 5–10 5 4

1 7 21 14

MCID 1.5 N/E 3.64 5 4.9

Response to PR or CBT Yes Yes N/E Yes Yes Yes

Abbreviations: AIR, Anxiety Inventory for Respiratory Disease; BAI, Beck Anxiety Inventory; DASS-A, Depression Anxiety Stress ScalesAnxiety; HADS-A, Hospital Anxiety Depression Scale-Anxiety; MCID, minimally clinically important difference; N/E, not examined.

Treatment approaches for depression and anxiety in patients with COPD  119

anxiety, symptoms of dyspnoea and the neural mechanism underlying the increased perception of respiratory sensation (55). Anxiety and COPD may create a vicious cycle of breathlessness. Feelings of breathlessness can provoke panic and intense thoughts of fear, which can make individual feel more anxious and can make it even harder to breathe (56). The chronic, progressive nature of the disability in COPD may increase anxiety. Acute exacerbations and hospitalizations are a source of stress and anxiety for both patients and their families. Furthermore, not having confidante and lifetime depressive disorders may also cause elevated anxiety, especially when intertwined with social and behavioural factors such as cigarette smoking (55,57).

BARRIERS TO DEPRESSION AND ANXIETY MANAGEMENT IN COPD Overlapping physical- and respiratory-related symptoms between depression, anxiety and COPD create diagnostic challenges (2–5). They include anhedonia, sleep problems, stress, hopelessness and fatigue. Diagnosing an anxiety disorder in the presence of COPD is further complicated by patients experiencing difficulty in articulating their symptoms, knowledge gaps about their symptoms, and the presence of coexisting cognitive disorders (2–5,57). Furthermore, the fear of rejection and stigma by family members or wider society possibly inhibits patients from seeking treatment for anxiety and depression (2–5,56,57). Overstrained healthcare professionals do not routinely assess anxiety and depression using validated screening tools (58). In addition, limited access to mental health treatment may extend the chronicity of dysfunction (58). In one study, three-quarters of COPD patients declined antidepressant drug therapy, fearing addiction, difficulty discontinuing such medications, and side effects (26,59). These numerous barriers highlight the need for personalized healthcare, such as a care manager who engages with the patient, explains the nature of both their COPD and concomitant anxiety disorder, and facilitates medication adherence as part of a CoCM. It increases patient engagement, which in turn may result in a better healthcare experience, satisfaction and improved patient outcomes.

TREATMENT APPROACHES FOR DEPRESSION AND ANXIETY IN PATIENTS WITH COPD As a significant proportion of COPD patients suffer from coexisting depression and anxiety, multimodal treatment strategies that incorporate psychological, physiological and social factors are required.

Pulmonary rehabilitation Pulmonary rehabilitation (PR) is a cornerstone in the rehabilitation of patients with chronic respiratory diseases. PR is defined as ‘comprehensive intervention based on a thorough

patient assessment followed by patient-tailored therapies that include, but are not limited to, exercise training, education, and behaviour change, designed to improve the physical and psychological condition of people with chronic respiratory disease and to promote the long-term adherence to healthenhancing behaviours’ (60). PR improves limb muscle functioning, exercise capacity, QOL, and self-efficacy. It reduces anxiety and depressive symptoms as well as healthcare utilization in patients with chronic respiratory diseases (60). In a prospective uncontrolled study of 557 patients with COPD and clinically significant symptoms who participated in an 8-week PR programme, PR reduced depression, anxiety, stress and dyspnoea as well as increasing exercise capacity and QOL (18). The mental health improvements correlated with the reductions in breathlessness. This encouraging study did not evaluate long-term effects of PR in reducing anxiety and depressive symptoms. In a systematic review (61), three RCTs (n = 269 patients with COPD) showed that PR reduced short-term depression and anxiety and also improved health-related QOL compared with usual care (with or without education). Emery and colleagues demonstrated that 10 weeks of exercise, education and stress management intervention was effective compared to education plus stress management to reduce depression and anxiety in patients with COPD (62). However, the three trials exhibited widely varying effect sizes. In addition, the efficacy of a PR maintenance programme and the identification of any long-term benefits remain unknown. Larger, more homogeneous studies with healthcare utilization metrics will help optimize the PR intervention. DROPOUT RATES FROM PULMONARY REHABILITATION

Despite the documented benefits, 25%–43% of those who commence PR drop out (43,63–65). Factors contributing to dropout of patients include physical disability, psychological morbidity and social factors (Table 11.3). Non-completers had a lower exercise capacity, measured by the incremental shuttle walk test, and elevated symptoms of dyspnoea and anxiety compared to completers (43). Logistic regression showed that a high load of anxiety symptoms related to increased dropout from PR. Intriguingly, only a third of COPD patients with baseline elevated anxiety symptoms responded to PR, implying the chronicity of anxiety symptoms may relate to dropout. Combinations of PR and psychological therapy for patients with elevated symptoms of anxiety will be helpful. Furthermore, healthcare professionals should encourage participants the positive aspects of PR such as participation is an opportunity for improvement, a safe and multidisciplinary nature of the PR programme. In addition, depressed or anxious COPD patients may lack the motivation and energy to adhere to exercise and related activities. Thus, the role of psychiatrist or psychologist to be involved in supporting patients in PR is worthy endeavor is advisable, where appropriate, to consider the location of the PR programme, in order to be close to a community gymnasium and/or public transport, and to also consider availability of volunteer(s) support (63,65).

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Table 11.3 Factors that contribute to dropout from pulmonary rehabilitation in patients with chronic respiratory diseases (43,63–65) Physiological and psychological markers Severity of lung function impairment Acute exacerbations Severity of dyspnoea Anxiety Depression Low self-efficacy Stressful life events Increased physical disability Psychosocial and demographic characteristics Lack of transportation Distance travelling to PR services Women Younger age Low education status Smoking Living alone Lack of improvement and social factors

Behavioural therapy Behavioural therapy is a composite term describing various therapies to treat mental health disorders (e.g. depression, anxiety, panic and phobias). Behavioural therapy comprises cognitive behavioural therapy (CBT), counselling, meditation and yoga, and relaxation and self-management exercises. CBT specifically addresses a patient’s thought processes and beliefs that induce excessive worry, anxiety and depressed mood. Several studies demonstrate that CBT effectively treats anxiety and depressive symptoms in patients with COPD (66–68). In an 8-week RCT, Kunik and co-workers compared the impact of CBT versus education on anxiety, depression and QOL in patients with COPD (66). The weekly 1-hour group CBT session followed a format focusing on anxiety and depression, designed for enhancing coping skills. The specific skills included relaxation, decreasing anxiety-related avoidance, thought modification, problem solving and sleep management, as well as a skills review. The weekly group education session comprised 45 minutes of lecture and 15 minutes of discussion. The education covered breathing strategies and airway management, pathophysiology of lung disease, medications including use of oxygen, avoidance of environmental irritants, nutrition, exercise, smoking cessation and end-of-life planning. Both interventions reduced anxiety and depression at 8 weeks and 12 months of follow-up. Interestingly, one in three patients with COPD and baseline elevated depression and anxiety failed to complete the 8-week course of either CBT or education. Hynnien et al. (67) compared seven weekly 2-hour group CBT sessions (n = 25) to usual care (n = 26) in patients with COPD and high anxiety and depressive symptoms.

The CBT intervention included psychoeducation awareness, relaxation, cognitive therapy (e.g. to identify and challenge depressive patterns of thought or anxiety-related ruminations in favour of more functional thought patterns), behavioural activation (e.g. identify and replace passive behaviours with pleasant activities that increase the sense of mastery), fear-based exposure (e.g. replacing avoidance with graded exposure to anxiety-provoking situations and activities, thereby increasing tolerance and reducing anxiety) and sleep management skills. Compared to the control group, CBT resulted in significant improvements for both depression and anxiety which were maintained after 8 months of follow-up. In patients with COPD and elevated symptoms of anxiety, Heslop-Marshall and colleagues compared brief CBT (four intervention sessions) delivered by respiratory nurses to self-help leaflets. Four brief CBT sessions effectively ameliorated clinically meaningful anxiety symptoms, with a sustained benefit at 1 year (68). In addition, they demonstrated the financial benefits gained by CBT through reduced emergency care and hospitalizations in a cost-effective manner (68). However, in long-term follow-up of CBT depressive symptoms were not reduced. A Cochrane systematic review identified three RCTs in an outpatient setting, comparing CBT versus usual care in predominantly male COPD patients (69). The review indicated a low quality of evidence for the use of CBT in ameliorating anxiety in patients with COPD. However, a more recent Cochrane systematic review included 13 RCTs with 1500 participants examining the efficacy of CBT in patients with chronic respiratory diseases (70). The authors concluded that psychological therapies using the CBT approach may potentially be effective in reducing depressive symptoms (70). The authors also highlighted the relatively small effect sizes and low quality of evidence due to heterogeneity of the studies.

Pharmacological therapy Antidepressant drug therapy is effective in the treatment of clinical depression in patients with chronic diseases (71), and international guidelines advocate antidepressant drug therapy in the treatment of major depression, specifically among older adults with chronic diseases. The National Institute for Health and Care Excellence (NICE) guidelines advocate the use of selective serotonin reuptake inhibitors as the first line of treatment over tricyclic antidepressants, because of their higher safety records in the treatment of depression (72). However, a systematic review by Yohannes and Alexopoulos demonstrated that antidepressants were inconclusive in the treatment of major depression in patients with COPD (73). The inability to demonstrate efficacy was partly explained by small sample sizes and absence of a controlled study design. Furthermore, significant numbers of patients with COPD did not complete the pharmacotherapy intervention because of temporary side effects including dry mouth, blurred vision, nausea and vomiting,

Clinical tips  121

worsening anxiety, dizziness, excessive fatigue and sedation (73). In addition, some patients failed to see the value and lacked knowledge of antidepressant drug therapy, and feared stigmatization. Similar observations from studies in other mood disorders implicate disease misconceptions and inadequate patient education as potential barriers to initial participation and successful conclusion in clinical trials. Thus, simply offering an antidepressant drug is not the answer, but a collaborative care approach would potentially overcome these barriers. Such an approach by a case manager would explain to patients why comorbid depression requires treatment, address patient concerns and provide patient support during and beyond the intervention period. A pioneer study published in 1995 showed that incorporating ‘a multifaceted intervention consisting of collaborative management for depression by the primary care physician and a consulting psychiatrist, intensive patient education, and surveillance of continued refills of antidepressant medication improved adherence to antidepressant regimens in patients with major and with minor depression’ (74).

Case Study 11.1 Mr Patrick is a 79-year old man with a 30-year history of COPD and forced expiratory volume in 1 second of 40% predicted. In the past 12 months, he has been hospitalized six times and required emergency care consultations on three occasions. His past history also includes major depression, anxiety and panic, chronic heart failure and osteoarthritis. He recurrently experiences acute depressive episodes enduring up to 2 weeks, which include low energy, feeling hopeless, and suicidal ideation without suicide attempt. He smoked two packs of cigarettes a day for 25 years, lives alone and drinks alcohol occasionally during social gatherings. In outpatient consultation, Mr. Patrick reported sad mood, loss of interest in pleasurable activities, tiredness, hopelessness and being tired of living. Mr Patrick is anxious and experiences fear of his progressive breathlessness and excessive tiredness. Contributors to Mr Patrick’s potential demise include discoordinated care, recurrent depressive symptoms and persistent smoking. Therefore, Mr. Patrick could benefit from an in-depth physical examination, psychological assessment and psychiatric consultation culminating in accurate diagnosis and an appropriate, comprehensive treatment plan. Given the severity of his respiratory impairment and depression, ideal CoCM would include CBT (with behavioural therapy and smoking cessation), pharmacological therapy (antidepressant drug) to stabilize his mood, self-management (e.g. physical activities and walking exercise) and personalized interventions and problem-solving therapy (e.g. how to manage the negative effects of stressful events) (75,76,80,81). Close collaboration between the psychiatrist, care manager, chest physician and primary care provider will optimize Mr Patrick’s ability to gain benefits from COPD and depression care using the CoCM.

Collaborative care A collaborative care model (CoCM) approach for depression treatment includes planning, involvement of the interdisciplinary care team, and a healthcare system that provides infrastructure and resources needed to deliver a patient-centred intervention. In recognition of these challenges and barriers to psychiatric care, the Improving Mood Promoting Access to Collaborative Treatment (IMPACT) trial is a randomized control trial for late-life depression (75). Intervention patients had access to a primary care physician, depression care manager and psychiatrist for up to 12 months. The care manager provided education, care management and support for either antidepressants managed by the patient’s primary care physician or a brief psychotherapy for depression (76). The intervention group showed greater reduction in depressive symptoms, more satisfaction with depression care, and improvement in functional activities and QOL compared to the usual care group. The diverse content of a CoCM typically comprises a multi-professional approach to patient care, structured management, scheduled patient follow-up and enhanced interprofessional communication (74–76). The CoCM overcomes important challenges and provides an opportunity to question patients about their behaviours and actions, typically by a care manager or healthcare professional who can support and encourage patients to adhere to their prescribed medication and rehabilitation programme. The CoCM model is cost effective in the treatment of anxiety and depression in older patients with other chronic diseases (77–79). Thus, future research should replicate the efficacy of CoCM specifically in patients with COPD and comorbid depression and/or anxiety. In the meantime, the addition of CoCM, seems the best way forward as a strategy to treat moderate to severe depression and anxiety for patients with chronic respiratory diseases, as exemplified in Case Study 11.1.

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Patients disabled with chronic respiratory diseases, such as including advanced COPD, have frequent episodes of hospitalization and emergency care utilization. They also experience distressing symptoms of depression and anxiety as well as dependency on caregivers. These experiences accelerate the deterioration in their health status, worsen their respiratory symptoms and increase the burden of disease on the healthcare system. Periodic screening of patients with chronic respiratory diseases using validated screening tools for depression and anxiety is useful, especially following stressful events such as loss of loved ones or a hospital admission. Screening is potentially the most effective strategy to detect and treat anxiety and depression in patients with chronic respiratory diseases.

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PR and CBT tend to improve dyspnoea, anxiety and depression in patients with COPD, at least in the short term. Therefore, PR and CBT should be first line of treatment for patients with chronic respiratory diseases who experience mild to moderate anxiety and/or depression. In the case of a COPD patient presenting with moderate to severe depression, the management strategy should include proactive consultation with a psychiatric team to formulate the diagnosis and supervise treatment, as well as involvement of a care manager with mental health training to address specific aspects of treatment, identify barriers and facilitators, coordinate care with multifaceted strategies and recognize the patient’s and family’s needs for long-term follow-up.

SUMMARY Coexisting anxiety and depression frequently occur in patients with chronic respiratory diseases. They are closely interrelated to chronic respiratory disease through similar factors, such as low socioeconomic status, and psychological and behavioural factors (e.g. smoking). Unmanaged depression and anxiety are particularly distressing for patients with COPD. A multimodal treatment approach that interrupts the pathways linking depression and anxiety with excess healthcare burdens, such as CoCM, seems most appropriate but also warrants further prospective RCTs with large sample sizes.

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12 Dyspnoea PIERANTONIO LAVENEZIANA AND DONALD A. MAHLER Introduction What is dyspnoea? Impact of dyspnoea in patients who have respiratory disease Descriptors of dyspnoea Measurements of dyspnoea for pulmonary rehabilitation

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Effects of pulmonary rehabilitation on relieving dyspnoea Responders versus non-responders Mechanisms for relief of dyspnoea with pulmonary rehabilitation Summary References

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KEY MESSAGES • Dyspnoea is a subjective experience of breathing discomfort that consists of qualitatively distinct sensations that vary in intensity. • The mechanisms of dyspnoea are complex and multifactorial: there is no unique central or peripheral source of this symptom. • The effective management of exertional dyspnoea remains a major challenge for caregivers, and modern

treatment strategies that are based on attempts to reverse the underlying chronic condition are only partially successful. • Pulmonary rehabilitation is one of the key interventions in leading to substantial benefits in dyspnoea, exercise capacity, health-related quality of life and healthcare utilization.

INTRODUCTION

with COPD. The effective management of exertional dyspnoea remains a major challenge for caregivers, and modern treatment strategies that are based on attempts to reverse the underlying chronic physiological mechanisms of dyspnoea during exercise, its impact for patients with respiratory disease, its measurements for pulmonary rehabilitation (PR), the effects of PR on relieving dyspnoea and the mechanisms for relief of dyspnoea with PR.

Dyspnoea is a complex, multifaceted and highly personalized sensory experience, the source and mechanisms of which are incompletely understood. Activity-related dyspnoea is usually the earliest and most troublesome complaint for which patients with cardiopulmonary diseases seek medical attention. This symptom progresses relentlessly as the disease advances, leading invariably to avoidance of activity with consequent skeletal muscle deconditioning and an impoverished quality of life. It is estimated that up to a quarter of the general population and half of severely ill patients are affected by it. In patients with chronic obstructive pulmonary disease (COPD), dyspnoea has been shown to be a better predictor of mortality than forced expiratory volume in 1 second (FEV1). In patients with heart disease referred for clinical exercise testing, it is a better predictor of mortality than angina. Dyspnoea is also associated with decreased functional status and worse psychological health in older individuals living at home. It is also a factor in the low adherence to exercise training programmes in sedentary adults and in patients

WHAT IS DYSPNOEA? Breathing is an unconscious activity in which the medulla in the brain stem sends electrical signals to the respiratory muscles to control frequency and tidal volume of breathing. This automatic process occurs normally 12–14 times every minute without a thought. However, breathing is also under voluntary control, as the cerebral cortex can instruct the person ‘to hold your breath’ or to take fast and deep breaths. The word dyspnoea is derived from dys (difficult) and pnoea (breathing). It is a medical word that is used to refer to an individual’s complaint that ‘I feel short of breath’. 125

126 Dyspnoea

Dyspnoea is defined by the American Thoracic Society and European Respiratory Society as a ‘subjective experience of breathing discomfort that consists of qualitatively distinct sensations that vary in intensity’ (1,2). The three major qualities of dyspnoea are work/effort of breathing, chest tightness and unsatisfied inspiration (1,2). In simple terms, dyspnoea is a warning signal that the interaction between the respiratory system and the brain is not working properly. A neurobiological model may be used to explain the perception of dyspnoea (3). In brief, stimulation of chemoreceptors, mechanoreceptors and receptors located in respiratory muscles causes afferent impulses to be sent to brainstem respiratory centres that automatically adjust breathing based on appraisal of blood gases, acid-base, and mechanical status of the respiratory system (3). Two common stimuli are hypoxaemia, which activates carotid bodies (peripheral chemoreceptors) and hyperinflation, both static as well as dynamic, which can activate mechanoreceptors in the lungs and respiratory muscle receptors. Integration and processing of respiratory inputs from sensory receptors occurs in the central nervous system (Figure 12.1). Neuroimaging has shown that the anterior insular cortex, anterior cingulate cortex, amygdala, dorsolateral prefrontal cortex and cerebellum are activated in responses to stimuli in the laboratory (4). This model is useful to understand how PR is effective in relieving dyspnoea by different mechanisms (5). Different pathways appear to process distinct respiratory sensations. For awareness of the intensity or sensory domain of dyspnoea, afferent information from respiratory muscle receptors is relayed into the medulla and then projected to the ventroposterior thalamus, from where projections ascend to the primary and secondary somatosensory cortices (6,7). This is called ‘discriminative processing’. For awareness of unpleasantness, or affective domain of dyspnoea, afferent information from activation of airway and lung receptors is relayed via the vagal nerve to the medulla and then processed to the amygdala and medial dorsal areas of the thalamus. These projections ascend to the insular and cingulate cortices which are part of the limbic system. This is called ‘affective processing’. Then, direct efferent impulses are sent via the phrenic and thoracic nerves to the diaphragm and intercostal muscles, respectively, that control frequency of breathing and tidal volume. The perception of dyspnoea is considered to result from an imbalance between the demand to breathe and the ability to breathe. This has been called ‘neuromechanical dissociation’ (1–3). Certainly, patients with respiratory disease exhibit a range of dyspnoea responses. There are ‘high perceivers’ who report higher than expected ratings based on objective data, while there are ‘low perceivers’ who indicate little if any breathing discomfort despite severe physiological impairment (8). Psychological factors such as anxiety, panic and depression can also affect the experience of dyspnoea (9,10). The respiratory system is modulated continuously by excitatory and inhibitory neuropeptides that act from sensory neurons to central networks (3). Endogenous opioids

Corollary discharges

Somatosensory cortex

Motor cortex

Limbic cortex

Medulla motoneurone relay Respiratory afferents

Motor commands Lungs Bronchi Pleura Vessels

Thorax

Respiratory muscles PaO2 PaCO2 pH

Figure 12.1 Integrative mechanisms at the origin of dyspnoea. Respiratory command derives from inputs from both the motor cortex and the medulla. These commands are integrated at the spinal level and transmitted to the muscular effectors of the respiratory system. The subsequent activation of the respiratory muscles will generate afferent inputs that are fed back to the respiratory command centres and the somatosensory cortex. The comparison of the corollary discharge and the ensuing afferent feedback may present a mismatch, and dyspnoea will occur when a negative affective sensation is attributed to this mismatch by the limbic cortex, which will also be influenced and modulated by memory and the prevailing environment. (Adapted from Laviolette L, Laveneziana P. Eur Respir J. 2014;43(6):1750–62, with permission.)

are inhibitory neuropeptides that affect respiratory rhythm and modulate the perception of dyspnoea. In clinical studies, patients with asthma and those with COPD reported higher ratings of breathlessness with different noxious respiratory stimuli (bronchoconstriction, exercise and resistive load breathing) when naloxone was administered to block opioid receptor signalling compared with normal saline (11–13). It is possible, although unproven, that other inhibitory and/or excitatory neuropeptides may also modulate the perception of dyspnoea (14).

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IMPACT OF DYSPNOEA IN PATIENTS WHO HAVE RESPIRATORY DISEASE About 30% of presumably healthy individuals 65 years of age or older report breathlessness while walking on a level or up an incline (14). It is likely that the 30% prevalence of dyspnoea in the elderly is multifactorial, including possible undiagnosed cardiac or respiratory disease, decreased physical activity with low fitness levels and increased body mass index. Breathing difficulty is a strong predictor of mortality in the elderly who have no known cardiorespiratory disease (15), in those with COPD independently of their FEV1 (16) and in those admitted to chest pain units for suspected acute coronary syndrome (17). Of 2258 patients with COPD and FEV1, 5% over the past 6 months is considered clinically significant, taking natural variations into account. Weight changes and BMI classification do not consider body compositional shifts, including amount and distribution of fat mass, lean mass and bone mineral content. Based on well-established adverse effects of low fat-free mass (FFM = lean mass + bone mineral content) on physical performance and survival, age- and sex-adjusted FFM index (FFMI = FFM/height2) lower than the 10th percentile is defined as abnormally low. For most normal-tounderweight Caucasian COPD patients this corresponds to a FFMI 5% in 6 months and FFMI