Airway clearance techniques in neuromuscular disorders

...an overview of the evidence

In healthy individuals, mucociliary clearance and cough mechanisms are effective and efficient in defending against secretion encumbrance, but these mechanisms may become ineffective if the systems malfunction and/or in the presence of excessive bronchial secretions.

Individuals with weak or impaired inspiratory and/or expiratory muscles, with or without glottis closure issues (bulbar insufficiency, tracheostomy), will have decreased peak cough flow (PCF). Weakness of the inspiratory muscles leads to a progressive decrease in vital capacity (VC). Reduced ability to cough leads to secretion retention, predisposing to progessive respiratory morbity. Severe bulbar dysfunction and glottic dysfunction most commonly occurs in patients with amyotrophic lateral sclerosis (ALS), spinal muscle atrophy (SMA) type 1, and other rarer neuromuscular disorders (NMD). 

Inability to close the glottis and vocal cords results in complete loss of the ability to cough and swallow. Difficulty swallowing liquids may result in pooling of saliva and mucus in the pharynx. Alterations in alveolar ventilation, atelectasis, mucus plugging, and recurrent respiratory tract infections (RTI's), as a consequence of an ineffective cough; together with severe bulbar dysfunction, are the main causes of morbility and mortality in patients with NMD.

In this state of the art review it is recommended that PCF is routinely measured in patients with NMD. 

For proximal ACTs it is recommended to use MAC and assisted inspirations, as single breaths, AS and GPB, in patients with higher PCF. MI-E appears to be very effective in patients with lower PCF's (< 160 L/min).

Peripheral ACTs: all discussed techniques may be effective in patients with NMD and should be considered according to availability and local expertise in patients to mobilise the secretions prior to proximal ACTs.

> From: Chatwin et al., Respiratory Medicine 136 (2018-01-22 22:18:24) 98-110 . All rights reserved to The Author(s). Click here for the online summary.

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