End-of-life care in adults with cystic fibrosis

Background: Cystic fibrosis (CF) is a life-limiting congenital disease, with most patients dying at a young age of progressive lung disease. Lung transplantation offers hope for many but may not occur. There is little to guide the provision of palliative care to this young population who maintain hope in the possibility of lung transplantation. Methods: To inform the development of an appropriate model of palliative care, a medical record review of CF patients dying within a 5-year period without lung transplantation was undertaken. The aspects of care that were preventative, therapeutic, or palliative were quantified. Results: Of the 20 records studied, all died in hospital. Only 15% of patients had a do-not-resuscitate order agreed to more than 1 week prior to death, increasing to 90% at the time of death (median of 2 days before death). Opioids were prescribed for 1 patient (5%) at 1 week prior to death, increasing to 85% of patients in the last 24 hours of life (median of 36 hours before death). During the last 24 hours of life, intravenous antibiotics continued in 85%, and assisted ventilation in 90% of subjects. Conclusion: We conclude that the circumstances surrounding the death of patients with CF holds challenges for their effective palliative care. CF patients continue life prolonging and preventative treatments until the last hours of life. There is an urgent need to examine palliative care approaches that may usefully coexist with maintaining transplantation options in the end-of-life care of this population.