title: The respiratory management of patients with duchenne muscular dystrophy: a DMD care considerations working group specialty article.
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.
authors.affiliation: Department of Pediatrics, MetroHealth Medical Center, Cleveland, Ohio 44109, USA. dbirnkrant@metrohealth.org
authors.forename: David J
authors.initials: DJ
authors.lastname: Birnkrant
authors.forename: Katharine M D
authors.initials: KM
authors.lastname: Bushby
authors.forename: Raouf S
authors.initials: RS
authors.lastname: Amin
authors.forename: John R
authors.initials: JR
authors.lastname: Bach
authors.forename: Joshua O
authors.initials: JO
authors.lastname: Benditt
authors.forename: Michelle
authors.initials: M
authors.lastname: Eagle
authors.forename: Jonathan D
authors.initials: JD
authors.lastname: Finder
authors.forename: Maninder S
authors.initials: MS
authors.lastname: Kalra
authors.forename: John T
authors.initials: JT
authors.lastname: Kissel
authors.forename: Anastassios C
authors.initials: AC
authors.lastname: Koumbourlis
authors.forename: Richard M
authors.initials: RM
authors.lastname: Kravitz
mesh_terms: D058109:Airway Management; D006801:Humans; D020388:Muscular Dystrophy, Duchenne / Q000150:complications* / Q000503:physiopathology*; D017410:Practice Guidelines as Topic; D012120:Respiration Disorders / Q000175:diagnosis* / Q000209:etiology / Q000628:therapy*; D014797:Vital Capacity