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Motor neurone disease/amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disorder with no known cure, where death is usually secondary to progressive respiratory failure. Assisting people with ALS through their disease journey is complex and supported by clinics that provide comprehensive multidisciplinary care (MDC). This review aims to apply both a respiratory and a complexity lens to the key roles and areas of practice within the MDC model in ALS. Models of noninvasive ventilation care, and considerations in the provision of palliative therapy, respiratory support, and speech and language therapy are discussed. The impact on people living with ALS of both inequitable funding models and the complexity of clinical care decisions are illustrated using case vignettes. Considerations of the impact of emerging antisense and gene modifying therapies on MDC challenges are also highlighted. The review seeks to illustrate how MDC members contribute to collective decision-making in ALS, how the sum of the parts is greater than any individual care component or health professional, and that the MDC per se adds value to the person living with ALS. Through this approach we hope to support clinicians to navigate the space between what are minimum, guideline-driven, standards of care and what excellent, person-centred ALS care that fully embraces complexity could be., Educational Aims: To highlight the complexities surrounding respiratory care in ALS.To alert clinicians to the risk that complexity of ALS care may modify the effectiveness of any specific, evidence-based therapy for ALS.To describe the importance of person-centred care and shared decision-making in optimising care in ALS., Competing Interests: Conflict of interest: D.J. Berlowitz received no financial support for this work and reports no financial or personal conflicts of interest connected or unconnected with this work. D.J. Berlowitz has previously received competitive grant funding from Motor Neurone Disease Research Australia and the Medical Research Future Fund for unconnected work. S. Mathers received no financial support for this work and reports no financial or personal conflicts of interest connected or unconnected with this work. S. Mathers has previously received competitive grant funding from Motor Neurone Disease Research Australia, Fight MND and NHMRC for unconnected work. K. Hutchinson received no financial support for this work and reports no financial or personal conflicts of interest connected or unconnected with this work. A. Hogden received no financial support for this work and reports no financial or personal conflicts of interest connected or unconnected with this work. K.A. Carey received no financial support for this work and reports no financial or personal conflicts of interest connected or unconnected with this work. M. Graco received no financial support for this work and reports no financial or personal conflicts of interest connected or unconnected with this work. M. Graco has previously received competitive grant funding from Motor Neurone Disease Research Australia (MND Research Post-Doctoral Fellowship). B-M. Whelan received no financial support for this work and reports no financial or personal conflicts of interest connected or unconnected with this work. B-M. Whelan has previously received competitive grant funding from Motor Neurone Disease Research Australia for unconnected work (project grant funding). S. Charania received no financial support for this work and reports no financial or personal conflicts of interest connected or unconnected with this work. S. Charania has previously received a competitive scholarship from The Prince Charles Hospital for unconnected work (PhD funding in the area of Motor Neurone Disease). F. Steyn received no financial support for this work and reports no financial or personal conflicts of interest connected or unconnected with this work. F. Steyn has previously received competitive grant funding for unconnected work (project grant funding). This includes industry-funding for preclinical testing of compounds for the treatment of ALS. P. Allcroft received no financial support for this work and reports no financial or personal conflicts of interest connected or unconnected with this work. A. Crook received no financial support for this work and reports no financial or personal conflicts of interest connected or unconnected with this work. A. Crook has previously received competitive grant funding from Motor Neurone Disease Research Australia and the University of Technology Sydney for unconnected work (project funding and funding towards conference attendance). N.L. Sheers received no financial support for this work and reports no financial or personal conflicts of interest connected or unconnected with this work. N.L. Sheers has previously received competitive grant funding from Motor Neurone Disease Research Australia for unconnected work (project grant funding and funding towards conference attendance)., (Copyright ©ERS 2023.)

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., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (© 2023 Sheers, O'Sullivan, Howard and Berlowitz.)

Introduction: Reduced lung volumes are a hallmark of respiratory muscle weakness in neuromuscular disease (NMD). Low respiratory system compliance (C rs ) may contribute to restriction and be amenable to lung volume recruitment (LVR) therapy. This study evaluated respiratory function and the immediate impact of LVR in rapidly progressive compared to slowly progressive NMD., Methods: We compared vital capacity (VC), static lung volumes, maximal inspiratory and expiratory pressures (MIP, MEP), C rs and peak cough flow (PCF) in 80 adult participants with motor neuron disease ('MND'=27) and more slowly progressive NMDs ('other NMD'=53), pre and post a single session of LVR. Relationships between respiratory markers and a history of respiratory tract infections (RTI) were examined., Results: Participants with other NMD had lower lung volumes and C rs but similar reduction in respiratory muscle strength compared with participants with MND (VC=1.30±0.77 vs 2.12±0.75 L, p<0.001; C rs =0.0331±0.0245 vs 0.0473±0.0241 L/cmH 2 O, p=0.024; MIP=39.8±21.3 vs 37.8±19.5 cmH 2 O). More participants with other NMD reported an RTI in the previous year (53% vs 22%, p=0.01). The likelihood of having a prior RTI was associated with baseline VC (%predicted) (OR=1.03 (95% CI 1.00 to 1.06), p=0.029). Published thresholds (VC<1.1 L or PCF<270 L/min) were, however, not associated with prior RTI.A single session of LVR improved C rs (mean (95% CI) increase = 0.0038 (0.0001 to 0.0075) L/cmH 2 O, p =0.047) but not VC., Conclusion: These findings corroborate the hypothesis that ventilatory restriction in NMD is related to weakness initially with respiratory system stiffness potentiating lung volume loss in slowly progressive disease. A single session of LVR can improve C rs . A randomised controlled trial of regular LVR is needed to assess longer-term effects., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2022. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ.)