Decline in respiratory function and experience with long-term assisted ventilation in advanced Duchenne's muscular dystrophy

Chest. 1990 Apr;97(4):884-9. doi: 10.1378/chest.97.4.884.


We present 17 patients with advanced DMD who required long-term assisted ventilation. Eleven patients used part-time assisted ventilation. Five of the patients received BV and/or M-IPPV or N-IPPV between two and nine years before requiring full-time T-IPPV, while six others initially used part-time T-IPPV. One patient used all three modes before requiring full-time T-IPPV. Mean (+/- SD) FVC and rebreathe PCO2 at the outset of assisted ventilation were 0.62 +/- 0.20 L and 47.4 +/- 7.5 mm Hg, respectively. Clinical features were divided between symptoms suggesting respiratory muscle fatigue and sleep-related disordered breathing. We found that, while useful in early respiratory insufficiency, BV is associated with recurrent aspiration. In our experience, N-IPPV offers the safest and most convenient form of noninvasive ventilation. When the VC has decreased to about 300 ml, most patients will require full-time ventilation; T-IPPV is advised to provide airway access to suction secretions.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adolescent
  • Adult
  • Home Care Services
  • Humans
  • Intermittent Positive-Pressure Ventilation
  • Male
  • Muscular Dystrophies / complications*
  • Muscular Dystrophies / physiopathology
  • Respiration, Artificial*
  • Respiratory Insufficiency / etiology
  • Respiratory Insufficiency / physiopathology
  • Respiratory Insufficiency / therapy*
  • Vital Capacity