title: Twenty-four hour noninvasive ventilation in Duchenne muscular dystrophy: a safe alternative to tracheostomy.
abstract: BACKGROUND
  Almost all patients with Duchenne muscular dystrophy (DMD) eventually develop respiratory failure. Once 24 h ventilation is required, either due to incomplete effectiveness of nocturnal noninvasive ventilation (NIV) or bulbar weakness, it is common practice to recommend invasive tracheostomy ventilation; however, noninvasive daytime mouthpiece ventilation (MPV) as an addition to nocturnal mask ventilation is also an alternative.
  
  
  METHODS
  The authors' experience with 12 DMD patients who used 24 h NIV with mask NIV at night and MPV during daytime hours is reported.
  
  
  RESULTS
  The mean (± SD) age and vital capacity (VC) at initiation of nocturnal (only) NIV subjects were 17.8±3.5 years and 0.90±0.40 L (21% predicted), respectively; and, at the time of MPV, 19.8±3.4 years and 0.57 L (13.2% predicted), respectively. In clinical practice, carbon dioxide (CO2) levels were measured using different methods: arterial blood gas analysis, transcutaneous partial pressure of CO2 and, predominantly, by end-tidal CO2. While the results suggested improved CO2 levels, these were not frequently confirmed by arterial blood gas measurement. The mean survival on 24 h NIV has been 5.7 years (range 0.17 to 12 years). Of the 12 patients, two deaths occurred after 3.75 and four years, respectively, on MPV; the remaining patients continue on 24 h NIV (range two months to 12 years; mean 5.3 years; median 3.5 years).
  
  
  CONCLUSIONS
  Twenty-four hour NIV should be considered a safe alternative for patients with DMD because its use may obviate the need for tracheostomy in patients with chronic respiratory failure requiring more than nocturnal ventilation alone.
authors: Canadian Alternatives in Non-invasice Ventilation Program, Respiratory Services and The Ottawa Hospital Sleep Centre, Ottawa, Ontario, Canada. dmckim@ottawahospital.on.ca
authors: Doug A
authors: DA
authors: McKim
authors: Nadia
authors: N
authors: Griller
authors: Carole
authors: C
authors: LeBlanc
authors: Andrew
authors: A
authors: Woolnough
authors: Judy
authors: J
authors: King
mesh_terms: D000328:Adult; D006801:Humans; D020388:Muscular Dystrophy, Duchenne / Q000628:therapy*; D063087:Noninvasive Ventilation / Q000379:methods; D012189:Retrospective Studies; D013997:Time Factors; D014139:Tracheostomy; D055815:Young Adult