Palliative and end-of-life care in cystic fibrosis: what we know and what we need to know

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Abstract
This review will examine what is now known about palliative and end-of-life care for cystic fibrosis (CF) patients, including the changing demographics and context of such care, and then outline a brief research agenda to guide further work in this area. We have now entered an era of more invasive end-of-life care in CF, due in part to changes in the demographics of death in CF and in part to the wider pursuit of lung transplantation. Recent single-center studies suggest that lung transplant listing changes the location and intensity of end-of-life care and complicates end-of-life care decision-making for the patient, family, and clinician. Further studies have demonstrated a high burden of daily symptoms in CF, especially pain and depression. In addition, there is evidence of an increasing burden related to the expanding daily treatment regimen for CF. The time has come for national, multicenter studies of palliative and end-of-life care practices in CF, followed by systematic evaluation of the efficacy of existing interventions. Studies of the symptoms and treatment burdens in CF should continue with an emphasis on interventions to improve health-related quality of life throughout the lifespan of children and adults with CF.

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