The role of lung volume recruitment therapy in neuromuscular disease: a narrative review

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Abstract

Respiratory muscle weakness results in substantial discomfort, disability, and ultimately death in many neuromuscular diseases. Respiratory system impairment manifests as shallow breathing, poor cough and associated difficulty clearing mucus, respiratory tract infections, hypoventilation, sleep-disordered breathing, and chronic ventilatory failure. Ventilatory support (i.e., non-invasive ventilation) is an established and key treatment for the latter. As survival outcomes improve for people living with many neuromuscular diseases, there is a shift towards more proactive and preventative chronic disease multidisciplinary care models that aim to manage symptoms, improve morbidity, and reduce mortality. Clinical care guidelines typically recommend therapies to improve cough effectiveness and mobilise mucus, with the aim of averting acute respiratory compromise or respiratory tract infections. Moreover, preventing recurrent infective episodes may prevent secondary parenchymal pathology and further lung function decline. Regular use of techniques that augment lung volume has similarly been recommended (volume recruitment). It has been speculated that enhancing lung inflation in people with respiratory muscle weakness when well may improve respiratory system “flexibility”, mitigate restrictive chest wall disease, and slow lung volume decline. Unfortunately, clinical care guidelines are based largely on clinical rationale and consensus opinion rather than level A evidence. This narrative review outlines the physiological changes that occur in people with neuromuscular disease and how these changes impact on breathing, cough, and respiratory tract infections. The biological rationale for lung volume recruitment is provided, and the clinical trials that examine the immediate, short-term, and longer-term outcomes of lung volume recruitment in paediatric and adult neuromuscular diseases are presented and the results synthesised.

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Author and article information

Contributors

Nicole L. Sheers: URI : https://loop.frontiersin.org/people/1754305/overview

Rachel O’Sullivan: URI : https://loop.frontiersin.org/people/2338530/overview

David J. Berlowitz: URI : https://loop.frontiersin.org/people/902621/overview

Journal

Journal ID (nlm-ta): Front Rehabil Sci

Journal ID (iso-abbrev): Front Rehabil Sci

Journal ID (publisher-id): Front. Rehabil. Sci.

Title: Frontiers in Rehabilitation Sciences

Publisher: Frontiers Media S.A.

ISSN (Print): 2673-6861

ISSN (Electronic): 2673-6861

Publication date (Electronic): 26 July 2023

Publication date Collection: 2023

Volume: 4

Electronic Location Identifier: 1164628

Affiliations

[ ¹ ]Department of Respiratory and Sleep Medicine, Austin Health , Heidelberg, VIC, Australia

[ ² ]Institute for Breathing and Sleep , Heidelberg, VIC, Australia

[ ³ ]Department of Physiotherapy, Faculty of Medicine, Dentistry and Health Sciences, The University of Melbourne , Parkville, VIC, Australia

[ ⁴ ]Department of Physiotherapy, Christchurch Hospital , Canterbury, New Zealand

[ ⁵ ]Department of Medicine, Faculty of Medicine, Dentistry and Health Sciences, The University of Melbourne , Parkville, VIC, Australia

[ ⁶ ]Turner Institute of Brain and Mental Health, Monash University , Clayton, VIC, Australia

[ ⁷ ]Department of Physiotherapy, Austin Health , Heidelberg, VIC, Australia

Author notes

Edited by: Raffaele Scala, IRCCS San Donato Polyclinic, Italy

Reviewed by: Rodrigo Torres-Castro, University of Chile, Chile Oscar Henry Mayer, Children's Hospital of Philadelphia, United States

[* ] Correspondence: David J. Berlowitz david.berlowitz@ 123456austin.org.au

Abbreviations ACT, airway clearance technique; ALS/MND, amyotrophic lateral sclerosis/motor neurone disease; C _L, lung compliance; CPAP, continuous positive airway pressure; C _rs, total respiratory system compliance; DMD, Duchenne muscular dystrophy; EIT, electrical impedence tomography; ERV, expiratory reserve volume; FOT, forced oscillation technique; FRC, functional residual capacity; FVC, forced vital capacity; IC, inspiratory capacity; IPPB, inspiratory positive pressure breathing; LCI, lung clearance index; LIC, lung insufflation capacity; LVR, lung volume recruitment; MAC, manually assisted cough; MEP, maximal expiratory pressure; MI-E, mechanical insufflation–exsufflation; MIC, maximum insufflation capacity; MIP, maximal inspiratory pressure; NIV, non-invasive ventilation; NMD, neuromuscular disease; OEP, opto-electronic plethysmography; P _A, alveolar pressure; P _AW, airway pressure; PCF or CPF, peak cough flow; PEF, peak expiratory flow; P _ga, gastric pressure; P _oes, oesophageal pressure; P _pl, pleural pressure; PREM, patient reported experience measures; PROM, patient reported outcome measures; QoL, quality of life; REM, rapid eye movement; R _rs, respiratory system resistance; RTI, respiratory tract infection; SCI, spinal cord injury; SMA, spinal muscular atrophy; SNIP, sniff nasal inspiratory pressure; TLC, total lung capacity; VC, vital capacity; V _T, tidal volume; X _rs, respiratory system reactance; Z _rs, respiratory system impedence.

[ † ]

ORCID Nicole L. Sheers orcid.org/0000-0003-1847-4266 David J. Berlowitz orcid.org/0000-0003-2543-8722

Article

DOI: 10.3389/fresc.2023.1164628

PMC ID: 10410160

PubMed ID: 37565183

SO-VID: bfc8e380-17b0-4563-937a-66e7b1e4acd6

License:

This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) and the copyright owner(s) are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.

History

Date received : 13 February 2023

Date accepted : 16 June 2023

Page count

Figures: 4, Tables: 5, Equations: 0, References: 239, Pages: 0, Words: 0

Funding

Funded by: manuscript was adapted from the PhD thesis of NS “The effects of lung volume recruitment therapy on respiratory function and quality of life in people with neuromuscular disease”, undertaken at The University of Melbourne. NS received PhD funding in the form of a National Health and Medical Research Council/Motor Neurone Disease Research Institute of Australia co-funded Postgraduate Scholarship

Award ID: 2014/GNT1093831

Some content from this manuscript was adapted from the PhD thesis of NS “The effects of lung volume recruitment therapy on respiratory function and quality of life in people with neuromuscular disease”, undertaken at The University of Melbourne. NS received PhD funding in the form of a National Health and Medical Research Council/Motor Neurone Disease Research Institute of Australia co-funded Postgraduate Scholarship (2014/GNT1093831).

Custom metadata

section-at-acceptance Pulmonary Rehabilitation

Keywords: neuromuscular disease,amyotrophic lateral sclerosis,muscular dystrophy,lung volume recruitment,lung inflation,insufflation,breath stacking

Data availability:

Keywords: neuromuscular disease, amyotrophic lateral sclerosis, muscular dystrophy, lung volume recruitment, lung inflation, insufflation, breath stacking

The role of lung volume recruitment therapy in neuromuscular disease: a narrative review

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