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.
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