Palliative care in cystic fibrosis

Although advances in therapy for cystic fibrosis (CF) have dramatically increased the average life span of patients, the disease is still uniformly fatal. Little attention has been paid to methods of palliative care for patients with cystic fibrosis in the medical literature. The primary palliative care issue in end-stage CF is the management of dyspnea. An observational study done at Children's Hospital in Boston suggested that doses of morphine in the range of 5 mg per hour can control the end-stage dyspnea in CF of over 50% of patients; the rest required increasing doses with the highest dose required being 30 mg/hour. The use of mechanical ventilation at the end of life is increasing in CF, particularly for those patients awaiting lung transplantation, and it appears that the consensus against the use of aggressive care at the end of life in CF may be eroding. The use of various modes of assisted ventilation in end-stage CF will add new challenges to the compassionate provision of end-of-life care. In addition to these medical issues, palliative care in CF presents some unique psychosocial issues: there may be more than one family member with the disease, and there is an involved patient community also affected by end-of-life plans. A family-centered approach to end-of-life care for patients with CF is essential.