ARTICLE IN PRESSCritical Care Guidelines and Consensus StatementsCHEST562358459506Respiratory Management of Patients With617628Neuromuscular Weakness6396410An American College of Chest Physicians Clinical Practice65112Guideline and Expert Panel Report6667136814as Akram Khan,MD;Lindsy Frazer-Green,PhD;Reshma Amin,MD;Lisa Wolfe,MD;Garner Faulkner,RRT;6915Kenneth Casey,MD;Girish Sharma,MD;Bernardo Selim,MD;David Zielinski,MD;Loutfi S.Aboussouan,MD;7016Q1Douglas McKim,MD;and Peter Gay,MD711718Endorsed by the American Academy of Sleep Medicine,the American Association for Respiratory Care,the American Thoracic7319Society,and the Canadian Thoracic Society7420Q4BACKGROUND:Respiratory failure is a significant concern in neuromuscular diseases(NMDs).7521This CHEST guideline examines the literature on the respiratory management of patients76723with NMD to provide evidence-based recommendations.7824METH:Anexpert panel conducted a systematicreview addressing the respiratory management7925of NMD and applied the Grading of Recommendations,Assessment,Devdopment,and Evalua-8026tions approach for assessing the certainty of the evidence and formulating and grading recom-81mendations.A modified Delphi technique was used to reach a consensuson the recommendations.8228RESULTS:Based on 128 studies,the panel generated 15 graded recommendations,one good 83practice statement,and one consensus-based statement.43085coNCLUSION:Evidence of best practices for respiratory management in NMD is limited and32is based primarily on observational data in amyotrophic lateral sclerosis.The panel found 87that pulmonary function testing every 6 months may be beneficial and may be used to initiate 8834noninvasive ventilation (NIV)when clinically indicated.An individualized approach to NIV 8935settings may benefit patients with chronic respiratory failure and sleep-disordered breathing 9orelated to NMD.When resources allow,polysomnography or overnight oximetry can help to 9guide the initiation of NIV.The panel provided guidelines for mouthpiece ventilation,9238transition to home mechanical ventilation,salivary secretion management,and airway 933940clearance therapies.The guideline panel emphasizes that NMD pathologic characteristics441represent a diverse group of disorders with differing rates of decline in lung function.The9642clinician's role is to add evaluation at the bedside to shared decision-making with patients9743and families,including respect for patient preferences and treatment goals,considerations of9844quality of life,and appropriate use of available resources in decision-making.9945CHEST2023;■()：■-10046101KEY WORDs:airway clearance therapies;clinical practice guidelines;neuromuscular diseases;10248noninvasive ventilation;pulmonary function testing;respiratory failure;sleep-disordered10349065breathing1045010551ABBREVIATIONS:ALS=amyotrophic lateral sclerosis;BT=botulinumventilation;NMD neuromuscular disease;ONO =overnight oximetry:10652toxin;COI conflict of interest;GRADE Gra
