Ventilator use in progressive neuromuscular disease: impact on patients and their families

Abstract

The question of extending the lives of patients with progressive neuromuscular disease by means of ventilator support is controversial. It has been documented that ventilators can prolong the lives of patients with Duchenne muscular dystrophy by between two and 25 years, but few studies have assessed the effects of their use on individual or family functioning, or the quality of survival. A sample of patients with progressive disease and families living in the community was surveyed to assess these effects. Most of the sample were satisfied with their decision to extend life with the ventilator, but they found the experience significantly stressful in the home. Both patients and families believed their health-care team could have met their needs more comprehensively. Recommendations are made for health-care professionals working with patients with late-stage neuromuscular disease who need to use ventilators.