Cystic fibrosis. End-stage care in Canada.
OBJECTIVE
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.


METHODS
Mailed survey of CF physicians.


METHODS
CF centers in Canada.


METHODS
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.
Mitchell
I
I
Department of Paediatrics, University of Calgary, Canada. ian.mitchell@crha-health.ab.ca
Nakielna
E
E
Tullis
E
E
Adair
C
C
D000328:Adult; D002170:Canada; D003430:Cross-Sectional Studies; D003550:Cystic Fibrosis / Q000382:microbiology / Q000628:therapy; D005260:Female; D006306:Health Surveys; D006801:Humans; D008297:Male; D010166:Palliative Care; D012944:Social Support; D013183:Sputum / Q000382:microbiology; D013727:Terminal Care