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Cystic fibrosis. End-stage care in Canada
I Mitchell1, E Nakielna, E Tullis
1Department of Paediatrics, University of Calgary, Canada. ian.mitchell@crha-health.ab.ca
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.