CHEST Guidelines-Respiratory Management of Patients With Neuromuscular Weakness

Respiratory Management of Patients With Neuromuscular Weakness

Pdf Summary

The American College of Chest Physicians, alongside several endorsing organizations, has released guidelines addressing respiratory management in patients with neuromuscular diseases (NMDs). Respiratory failure poses a significant risk in NMDs, often necessitating a spectrum of respiratory support strategies. The guidelines, derived from a systematic review of 128 studies, offer 15 recommendations and emphasize an individualized approach, particularly in managing chronic respiratory failure.<br /><br />The guidelines recommend pulmonary function tests (PFTs) every six months for patients at risk of respiratory complications, with particular attention to vital capacity and inspiratory pressure measurements. Noninvasive ventilation (NIV) is advised for those with chronic respiratory failure or sleep-related breathing disorders. Initiation of NIV should consider factors like respiratory parameters and polysomnography results where appropriate.<br /><br />Key management aspects include tailored NIV settings, mouthpiece ventilation as an adjunct to nocturnal mask NIV, and early considerations for invasive mechanical ventilation via tracheostomy if NIV fails or complications arise. Management of sialorrhea, often distressing in NMDs, should begin with anticholinergic agents, escalating to botulinum toxin therapy or radiation therapy if needed.<br /><br />Additionally, airway clearance therapies are deemed vital due to increased respiratory morbidity in NMDs. Techniques such as lung volume recruitment, manually assisted cough, and mechanical insufflation-exsufflation (MI-E) offer secretion management solutions.<br /><br />Future research should focus on optimizing the frequency of respiratory assessments, evaluating telemedicine's impact on NIV management, and exploring innovative technologies like artificial intelligence in tailoring respiratory support. The guidelines highlight a need for shared decision-making with patients and caregivers to account for quality of life and treatment preferences, recognizing the diverse progression patterns across NMDs.

Keywords

respiratory management

neuromuscular diseases

respiratory failure

pulmonary function tests

noninvasive ventilation

airway clearance therapies

sialorrhea management

mechanical insufflation-exsufflation

shared decision-making

telemedicine