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

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.

Hypotussic cough in persons with dysphagia: biobehavioral interventions and pathways to clinical implementation

“…In contrast, those for assisted inspiration augment inspiratory lung volumes in single or stacked breaths ( 57 ). The application of these techniques inflate the respiratory system to the maximal desired volume, thereby increasing the inspiratory capacity which is associated with an increased PEFR ( 114 ). Assisted inspiration and expiration techniques combine an inflated respiratory system and increased inspiratory capacity with thoracic compression and increased expiratory airflow to optimize PEFR ( 57 ).…”

Perioperative Management in Neuromuscular Diseases: A Narrative Review

“…There are also changes in lung distensibility and lung and chest wall elastic properties that decrease total respiratory system compliance (Crs) [74] and are postulated to be a result of rapid and shallow breathing pattern with increasing work of breathing that results in chronic micro atelectasis and decrease in both lung and chest wall compliance [5]. Reduced Crs may necessitate higher pressures to achieve adequate ventilation and tidal volumes with mechanical ventilation.…”

Pulmonaryfunctioninneuromusculardiseases

“…La disminución de la función tusígena en las ENM tiene relación a la debilidad muscular, la que repercute en la disminución de la capacidad inspiratoria y por consiguiente en disminución de la CV (30). Esta disminución de volúmenes derivara en muchos casos en microatelectasias y disminución de la distensibilidad toracopulmonar sobre todo en ENM de progresión lenta (como las distrofias) donde se ha visto que el mayor determinante de la reducción de volúmenes pulmonares está dado por la disminución de la distensibilidad, más que por la reducción de fuerza muscular respiratoria (31). Dado esto, se considera fundamental centrar el tratamiento respiratorio de las ENM en mantener una adecuada distensibi-lidad con técnicas de RVP.…”