Respiratory management of the patient with Duchenne muscular dystrophy

Daniel W. Sheehan, David J. Birnkrant, Joshua O. Benditt, Michelle Eagle, Jonathan Finder, John Kissel, Richard M. Kravitz, Hemant Sawnani, Richard Shell, Michael D. Sussman, Lisa F. Wolfe

Research output: Contribution to journalArticle

2 Citations (Scopus)

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.

Original languageEnglish (US)
Pages (from-to)S62-S71
JournalPediatrics
Volume142
DOIs
StatePublished - Oct 1 2018
Externally publishedYes

Fingerprint

Duchenne Muscular Dystrophy
Guidelines
Ventilation
Clinical Trials
Patient Transfer
Respiratory Muscles
Survival
Muscle Strength
Patient Safety
Centers for Disease Control and Prevention (U.S.)
Neurology
Respiratory Insufficiency
Publications
Quality of Life
Medicine
Pediatrics
Phenotype
Drug Therapy
Lung
Therapeutics

All Science Journal Classification (ASJC) codes

  • Pediatrics, Perinatology, and Child Health

Cite this

Sheehan, D. W., Birnkrant, D. J., Benditt, J. O., Eagle, M., Finder, J., Kissel, J., ... Wolfe, L. F. (2018). Respiratory management of the patient with Duchenne muscular dystrophy. Pediatrics, 142, S62-S71. https://doi.org/10.1542/peds.2018-0333H

Respiratory management of the patient with Duchenne muscular dystrophy. / Sheehan, Daniel W.; Birnkrant, David J.; Benditt, Joshua O.; Eagle, Michelle; Finder, Jonathan; Kissel, John; Kravitz, Richard M.; Sawnani, Hemant; Shell, Richard; Sussman, Michael D.; Wolfe, Lisa F.

In: Pediatrics, Vol. 142, 01.10.2018, p. S62-S71.

Research output: Contribution to journalArticle

Sheehan, DW, Birnkrant, DJ, Benditt, JO, Eagle, M, Finder, J, Kissel, J, Kravitz, RM, Sawnani, H, Shell, R, Sussman, MD & Wolfe, LF 2018, 'Respiratory management of the patient with Duchenne muscular dystrophy', Pediatrics, vol. 142, pp. S62-S71. https://doi.org/10.1542/peds.2018-0333H
Sheehan DW, Birnkrant DJ, Benditt JO, Eagle M, Finder J, Kissel J et al. Respiratory management of the patient with Duchenne muscular dystrophy. Pediatrics. 2018 Oct 1;142:S62-S71. https://doi.org/10.1542/peds.2018-0333H
Sheehan, Daniel W. ; Birnkrant, David J. ; Benditt, Joshua O. ; Eagle, Michelle ; Finder, Jonathan ; Kissel, John ; Kravitz, Richard M. ; Sawnani, Hemant ; Shell, Richard ; Sussman, Michael D. ; Wolfe, Lisa F. / Respiratory management of the patient with Duchenne muscular dystrophy. In: Pediatrics. 2018 ; Vol. 142. pp. S62-S71.
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