Guidelines Developed for Respiratory Management of Neuromuscular Disease

THURSDAY, March 16, 2023 (HealthDay News) -- In an American College of Chest Physicians clinical practice guideline and expert panel report, published online March 13 in CHEST, recommendations are presented for the respiratory management of patients with neuromuscular disease (NMD), including the use of pulmonary function testing every six months and initiating noninvasive ventilation (NIV) when clinically indicated.

Akram Khan, M.D., from the Oregon Health & Science University in Portland, 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.

The authors note that there is limited evidence of best practices for respiratory management in NMD, which is mainly based on observational data in amyotrophic lateral sclerosis. Pulmonary function tests every six 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.

"Respiratory muscle weakness is a serious concern in patients with neuromuscular diseases. It can lead to inadequate ventilation, nighttime hypoventilation and the inability to mobilize secretions, which is frequently the cause of death in this population," Khan said in a statement. "We anticipate this guideline will standardize and improve the care provided to patients with neuromuscular diseases and subsequent weakness."

Financial ties to Cytokinetics and Biogen were disclosed.

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