New Guidance for Respiratory Management of Neuromuscular Disease

In an American College of Chest Physicians clinical practice guideline and expert panel report, published in CHEST, recommendations are presented for the respiratory management of patients with neuromuscular disease (NMD), including the use of pulmonary function testing every 6 months and initiating noninvasive ventilation (NIV) when clinically indicated. Akram Khan, MD, and colleagues developed recommendations to address the respiratory management of NMD. Fifteen graded recommendations, one good practice statement, and one consensus-based statement were generated based on 128 studies. Evidence was limited on best practices for respiratory management in NMD and is mainly based on observational data in amyotrophic lateral sclerosis. Pulmonary function tests every 6 months may be beneficial and have use for initiation of NIV when clinically indicated. Patients with chronic respiratory failure and sleep-disordered breathing related to NMD may benefit from an individualized approach to NIV. Polysomnography or overnight oximetry can help guide initiation of NIV, when resources allow. The authors also provide guidance for mouthpiece ventilation, transition to home mechanical ventilation, salivary secretion management, and airway clearance therapies.