TY - JOUR
T1 - Respiratory Management of Patients With Neuromuscular Weakness
T2 - An American College of Chest Physicians Clinical Practice Guideline and Expert Panel Report
AU - Khan, Akram
AU - Frazer-Green, Lindsy
AU - Amin, Reshma
AU - Wolfe, Lisa
AU - Faulkner, Garner
AU - Casey, Kenneth
AU - Sharma, Girish
AU - Selim, Bernardo
AU - Zielinski, David
AU - Aboussouan, Loutfi S.
AU - McKim, Douglas
AU - Gay, Peter
N1 - Publisher Copyright:
© 2023 American College of Chest Physicians
PY - 2023/8
Y1 - 2023/8
N2 - Background: Respiratory 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 Methods: An 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. Results: Based on 128 studies, the panel generated 15 graded recommendations, one good practice statement, and one consensus-based statement. Interpretation: Evidence 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.
AB - Background: Respiratory 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 Methods: An 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. Results: Based on 128 studies, the panel generated 15 graded recommendations, one good practice statement, and one consensus-based statement. Interpretation: Evidence 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.
KW - airway clearance therapies
KW - clinical practice guidelines
KW - neuromuscular diseases
KW - noninvasive ventilation
KW - pulmonary function testing
KW - respiratory failure
KW - sleep-disordered breathing
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U2 - 10.1016/j.chest.2023.03.011
DO - 10.1016/j.chest.2023.03.011
M3 - Article
C2 - 36921894
AN - SCOPUS:85165899488
SN - 0012-3692
VL - 164
SP - 394
EP - 413
JO - CHEST
JF - CHEST
IS - 2
ER -