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Sleep disordered breathing: Assessment and therapy in the age of emerging neuromuscular therapies
Author(s) -
Gurbani Neepa,
Pascoe John E.,
Katz Sherri,
Sawnani Hemant
Publication year - 2021
Publication title -
pediatric pulmonology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.866
H-Index - 106
eISSN - 1099-0496
pISSN - 8755-6863
DOI - 10.1002/ppul.24988
Subject(s) - medicine , spinal muscular atrophy , duchenne muscular dystrophy , neuromuscular disease , amyotrophic lateral sclerosis , polysomnography , myopathy , physical medicine and rehabilitation , intensive care medicine , muscular dystrophy , sma* , respiratory failure , sleep and breathing , breathing , pediatrics , physical therapy , disease , pathology , anesthesia , apnea , mathematics , combinatorics
Abstract The term neuromuscular disease (NMD) encompasses a large variety of disorders that result in abnormal muscle function. Although it may be conventional to relate the use of this term to the most common muscular diseases (Duchenne muscular dystrophy [DMD], spinal muscular atrophy [SMA], and amyotrophic lateral sclerosis, etc), it is important to extend the term to pathologies manifested by severe neurologic (brain and spinal cord) malformations and injuries. In many of these scenarios, there are common mechanisms that contribute to sleep disordered breathing (SDB) and respiratory insufficiency although comorbidities may be somewhat different. Advances in the understanding of these diseases and their natural history, and increasing availability of mechanical ventilation to these patients have improved survival. The development of novel genetic and molecular therapies (as in the cases of DMD, SMA, and X‐linked myotubular myopathy) provides an opportunity to use SDB as a reasonable outcome measure while also allowing the use of polysomnography as a validation tool in the assessments of effectiveness of therapies. We seek to provide an understanding of SDB in NMDs, and in the same light, would like to begin the conversation of thinking about weaning respiratory support when possible.