title: Respiratory Care Received by Individuals With Duchenne Muscular Dystrophy From 2000 to 2011.
abstract: BACKGROUND
  Duchenne muscular dystrophy (DMD) causes progressive respiratory muscle weakness and decline in function, which can go undetected without monitoring. DMD respiratory care guidelines recommend scheduled respiratory assessments and use of respiratory assist devices. To determine the extent of adherence to these guidelines, we evaluated respiratory assessments and interventions among males with DMD in the Muscular Dystrophy Surveillance, Tracking, and Research Network (MD STARnet) from 2000 to 2011.
  
  
  METHODS
  MD STARnet is a population-based surveillance system that identifies all individuals born during or after 1982 residing in Arizona, Colorado, Georgia, Hawaii, Iowa, and western New York with Duchenne or Becker muscular dystrophy. We analyzed MD STARnet respiratory care data for non-ambulatory adolescent males (12-17 y old) and men (≥18 y old) with DMD, assessing whether: (1) pulmonary function was measured twice yearly; (2) awake and asleep hypoventilation testing was performed at least yearly; (3) home mechanical insufflation-exsufflation, noninvasive ventilation, and tracheostomy/ventilators were prescribed; and (4) pulmonologists provided evaluations.
  
  
  RESULTS
  During 2000-2010, no more than 50% of both adolescents and men had their pulmonary function monitored twice yearly in any of the years; 67% or fewer were assessed for awake and sleep hypoventilation yearly. Although the use of mechanical insufflation-exsufflation and noninvasive ventilation is probably increasing, prior use of these devices did not prevent all tracheostomies, and at least 18 of 29 tracheostomies were performed due to acute respiratory illnesses. Fewer than 32% of adolescents and men had pulmonologist evaluations in 2010-2011.
  
  
  CONCLUSIONS
  Since the 2004 publication of American Thoracic Society guidelines, there have been few changes in pulmonary clinical practice. Frequencies of respiratory assessments and assist device use among males with DMD were lower than recommended in clinical guidelines. Collaboration of respiratory therapists and pulmonologists with clinicians caring for individuals with DMD should be encouraged to ensure access to the full spectrum of in-patient and out-patient pulmonary interventions.
authors: Department of Pediatrics, University of Arizona College of Medicine, Tucson, Arizona.
authors: Jennifer G
authors: JG
authors: Andrews
authors: New York State Department of Health, Albany, New York.
authors: Aida
authors: A
authors: Soim
authors: Department of Neurology, University of Rochester, Rochester, New York.
authors: Shree
authors: S
authors: Pandya
authors: New York State Department of Health, Albany, New York.
authors: Christina P
authors: CP
authors: Westfield
authors: Department of Neurology, University of Rochester, Rochester, New York.
authors: Emma
authors: E
authors: Ciafaloni
authors: New York State Department of Health, Albany, New York.
authors: Deborah J
authors: DJ
authors: Fox
authors: Department of Pediatrics, MetroHealth Medical Center, Cleveland, Ohio.
authors: David J
authors: DJ
authors: Birnkrant
authors: Department of Pediatrics, Weill Cornell Medical College, Cornell University, New York, New York.
authors: Christopher M
authors: CM
authors: Cunniff
authors: Department of Pediatrics, University at Buffalo, State University of New York, Buffalo, New York. dsheehan@upa.chob.edu.
authors: Daniel W
authors: DW
authors: Sheehan
mesh_terms: D000293:Adolescent; D002648:Child; D006801:Humans; D008297:Male; D020388:Muscular Dystrophy, Duchenne / Q000150:complications* / Q000503:physiopathology / Q000628:therapy; D011159:Population Surveillance / Q000379:methods*; D012121:Respiration, Artificial / Q000295:instrumentation / Q000379:methods / Q000706:statistics & numerical data*; D012131:Respiratory Insufficiency / Q000175:diagnosis* / Q000209:etiology / Q000628:therapy; D013997:Time Factors; D014139:Tracheostomy / Q000706:statistics & numerical data; D014481:United States; D012122:Ventilators, Mechanical / Q000706:statistics & numerical data*
keywords: Duchenne muscular dystrophy; clinical guidelines; hypoventilation; neuromuscular diseases; noninvasive ventilation; respiratory care; tracheostomy