• Akram Khan, Lindsy Frazer-Green, Reshma Amin, Lisa Wolfe, Garner Faulkner, Kenneth Casey, Girish Sharma, Bernardo Selim, David Zielinski, Loutfi S Aboussouan, Douglas McKim, and Peter Gay.
• Division of Pulmonary Allergy and Critical Care Medicine, Oregon Health and Science University, Portland, OR. Electronic address: khana@ohsu.edu.
• Chest. 2023 Aug 1; 164 (2): 394413394-413.

BackgroundRespiratory failure is a significant concern in neuromuscular diseases (NMDs). This CHEST guideline examines the literature on the respiratory management of patients with NMD to provide evidence-based recommendations.Study Design And MethodsAn expert panel conducted a systematic review addressing the respiratory management of NMD and applied the Grading of Recommendations, Assessment, Development, and Evaluations approach for assessing the certainty of the evidence and formulating and grading recommendations. A modified Delphi technique was used to reach a consensus on the recommendations.ResultsBased on 128 studies, the panel generated 15 graded recommendations, one good practice statement, and one consensus-based statement.InterpretationEvidence of best practices for respiratory management in NMD is limited and is based primarily on observational data in amyotrophic lateral sclerosis. The panel found that pulmonary function testing every 6 months may be beneficial and may be used to initiate noninvasive ventilation (NIV) when clinically indicated. An individualized approach to NIV settings may benefit patients with chronic respiratory failure and sleep-disordered breathing related to NMD. When resources allow, polysomnography or overnight oximetry can help to guide the initiation of NIV. The panel provided guidelines for mouthpiece ventilation, transition to home mechanical ventilation, salivary secretion management, and airway clearance therapies. The guideline panel emphasizes that NMD pathologic characteristics represent a diverse group of disorders with differing rates of decline in lung function. The clinician's role is to add evaluation at the bedside to shared decision-making with patients and families, including respect for patient preferences and treatment goals, considerations of quality of life, and appropriate use of available resources in decision-making.Copyright © 2023 American College of Chest Physicians. Published by Elsevier Inc. All rights reserved.

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