Abstract:
:In 2010, Care Considerations for Duchenne Muscular Dystrophy, sponsored by the Centers for Disease Control and Prevention, was published in Lancet Neurology, and in 2018, these guidelines were updated. Since the publication of the first set of guidelines, survival of individuals with Duchenne muscular dystrophy has increased. With contemporary medical management, survival often extends into the fourth decade of life and beyond. Effective transition of respiratory care from pediatric to adult medicine is vital to optimize patient safety, prognosis, and quality of life. With genetic and other emerging drug therapies in development, standardization of care is necessary to accurately assess treatment effects in clinical trials. This revision of respiratory recommendations preserves a fundamental strength of the original guidelines: namely, reliance on a limited number of respiratory tests to guide patient assessment and management. A progressive therapeutic strategy is presented that includes lung volume recruitment, assisted coughing, and assisted ventilation (initially nocturnally, with the subsequent addition of daytime ventilation for progressive respiratory failure). This revision also stresses the need for serial monitoring of respiratory muscle strength to characterize an individual's respiratory phenotype of severity as well as provide baseline assessments for clinical trials. Clinical controversies and emerging areas are included.
journal_name
Pediatricsjournal_title
Pediatricsauthors
Sheehan DW,Birnkrant DJ,Benditt JO,Eagle M,Finder JD,Kissel J,Kravitz RM,Sawnani H,Shell R,Sussman MD,Wolfe LFdoi
10.1542/peds.2018-0333Hsubject
Has Abstractpub_date
2018-10-01 00:00:00pages
S62-S71issue
Suppl 2eissn
0031-4005issn
1098-4275pii
peds.2018-0333Hjournal_volume
142pub_type
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