Cystic fibrosis. End-stage care in Canada

Chest. 2000 Jul;118(1):80-4. doi: 10.1378/chest.118.1.80.


Objectives: To determine the circumstances in which individuals with cystic fibrosis (CF) die, the role of different caregivers, and the extent of palliative care for CF patients.

Design: Mailed survey of CF physicians.

Setting: CF centers in Canada.

Patients: All CF deaths in 1996 known to centers in Canada.

Results: The mean age (+/- SD) at death of the 45 individuals included in the study was 25.8 +/- 13.5 years. The major cause of death was respiratory (34 patients; 75.5%). Nutritional concerns were common. Lung transplantation was considered in 42 patients (93.2%), with 7 patients (17.1%) being entered on a list, but it was carried out in only 2 patients (4.4%). Autopsies were performed on only 10 patients (22.2%). Most patients died in hospital (37 patients; 82.2%), and 7 patients (15.6%) died in ICUs while receiving intermittent positive-pressure ventilation. Palliative care was never discussed in 10 patients (25%). In a further 16 patients (40%), it was not discussed until the last month before death.

Conclusions: Respiratory disease remains the most common cause of death in CF patients. Lung transplantation is frequently considered, but most patients die without having had a transplant. Discussions on end-of-life care could be considered sooner.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adult
  • Canada
  • Cross-Sectional Studies
  • Cystic Fibrosis* / microbiology
  • Cystic Fibrosis* / therapy
  • Female
  • Health Surveys*
  • Humans
  • Male
  • Palliative Care
  • Social Support
  • Sputum / microbiology
  • Terminal Care*