The respiratory management of patients with Duchenne muscular dystrophy: A DMD care considerations working group specialty article

David J. Birnkrant, Katharine M.D. Bushby, Raouf S. Amin, John R. Bach, Joshua O. Benditt, Michelle Eagle, Jonathan D. Finder, Maninder S. Kalra, John T. Kissel, Anastassios C. Koumbourlis, Richard M. Kravitz

Research output: Contribution to journalArticlepeer-review

109 Scopus citations

Abstract

In 2001, the Muscular Dystrophy Community Assistance, Research and Education Amendments (MD-CARE Act) was enacted, which directed federal agencies to coordinate the development of treatments and cures for muscular dystrophy. As part of the mandate, the Centers for Disease Control and Prevention (CDC) initiated surveillance and educational activities, which included supporting development of care considerations for Duchenne muscular dystrophy (DMD) utilizing the RAND/UCLA Appropriateness Method (RAM). This document represents the consensus recommendations of the project's 10-member Respiratory Panel and includes advice on necessary equipment, procedures and diagnostics; and a structured approach to the assessment and management of the respiratory complications of DMD via assessment of symptoms of hypoventilation and identification of specific thresholds of forced vital capacity, peak cough flow and maximum expiratory pressure. The document includes a set of Figures adaptable as "pocket guides" to aid clinicians. This article is an expansion of the respiratory component of the multi-specialty article originally appearing in Lancet Neurology, comprising respiratory recommendations from the CDC Care Considerations project.

Original languageEnglish (US)
Pages (from-to)739-748
Number of pages10
JournalPediatric Pulmonology
Volume45
Issue number8
DOIs
StatePublished - Aug 2010

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Pulmonary and Respiratory Medicine

Keywords

  • Consensus statement
  • Cough
  • Muscular dystrophy
  • Noninvasive ventilation
  • Prolonged survival
  • Pulmonary function

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