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Guides & Guidelines

Respiratory management of the patient with Duchenne muscular dystrophy

key information

source: Pediatrics

year: 2018

authors: Sheehan DW, Birnkrant DJ, Benditt JO, Eagle M, Finder JD, Kissel J, Kravitz RM, Sawnani H, Shell R, Sussman MD, Wolfe LF

summary/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.

organization: Oishei Children's Hospital and The University at Buffalo, USA; MetroHealth Medical Center and Case Western Reserve University, USA; University of Washington, USA; University of Newcastle, UK; University of Pittsburgh Medical Center Children's Hospital of Pittsburgh and University of Pittsburgh, USA; The Ohio State University Wexner Medical Center, USA; Duke University, USA; Cincinnati Children's Hospital Medical Center, USA; Nationwide Children's Hospital and The Ohio State University, USA; Shriners Hospital for Children, USA; Northwestern University, USA

DOI: 10.1542/peds.2018-0333H

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