Quality of Life in Advanced Cystic Fibrosis (CF) in a Transplantation Era

This study gathers empirical data on the experiences of cystic fibrosis (CF) patients, their families, and caregivers during the final years of life. The specific aim is to understand the quality of life and the social and logistical needs of CF patients and their families, and to understand how these factors may differ depending on the patient’s end-of-life trajectory. The approach for addressing these questions will be a longitudinal panel study of adult CF patients with advanced disease that employs three methods of data collection: (1) self-administered surveys of patients; (2) telephone interviews with patients, family members of patients who die, and clinicians; and (3) medical record abstraction. Expected outcomes from this research will be decreased suffering and enhanced quality of life for CF patients, guidelines for the appropriate use of technological interventions for patients both on and off the lung transplant list, new care models that can accommodate both the goals of transplantation and the goals of palliative care, better systems of care for the families of seriously ill CF patients, and increased understanding of the role of palliative care and technological intervention in other chronic illnesses.