Patient and family participation in the management of respiratory failure in Duchenne's muscular dystrophy

Chest. 1989 Mar;95(3):519-24. doi: 10.1378/chest.95.3.519.

Abstract

Mechanical ventilation is now considered a therapeutic option for respiratory failure associated with Duchenne's muscular dystrophy (DMD). Through the monitoring of forced vital capacity and PCO2, prediction of impending respiratory failure is possible. This knowledge allows the patient to choose institution of mechanical ventilation before acute respiratory failure or no intervention and preparation for a natural death. To assist patients and families in this decision making, a special clinic was established. Thirty adolescent boys with DMD were followed up. Eighteen boys reached the end stage of their disease. Three died of cardiac failure. Prediction of respiratory failure was possible in 14 of the remaining 15; 11 of the 15 were able to make educated choices regarding respirator assistance or a natural death. The clinic has shown that in the majority of cases, when properly educated, patients and families can be active participants in life-and-death decisions.

MeSH terms

  • Adolescent
  • Adult
  • Counseling
  • Decision Making
  • Disclosure
  • Family
  • Humans
  • Male
  • Muscular Dystrophies / complications*
  • Muscular Dystrophies / psychology
  • Patient Education as Topic*
  • Respiration, Artificial
  • Respiratory Insufficiency / etiology
  • Respiratory Insufficiency / mortality
  • Respiratory Insufficiency / therapy*
  • Tracheostomy