Cystic fibrosis (CF), the most common lethal inherited illness among Caucasians in North America, is undergoing a revolution. The advent of lung transplantation, while offering a chance at extended life for many patients, has undone the previous consensus on the appropriate treatment of end-stage symptoms, the use of assisted ventilation, and the management of psychosocial suffering. In order to answer the call of the Institute of Medicine for the integration of palliative care into chronic and ultimately fatal illnesses, a comprehensive study of the care of patients with end-stage cystic fibrosis is proposed. The purpose of the proposed study is to gather empirical data on the experiences of CF patients, their families, and caregivers during the final years of life.
The specific aim i s to understand the quality of life and the social and logistical needs of CF patients and their families, and to understand how the these factors may differ depending on the patient's end-of-life trajectory. The approach for addressing these questions will be a three-year longitudinal panel study of adult CF patients with advanced disease. Ten CF centers from across the United States will enroll adult patients with a predicted life expectancy of two to three years; these patients will be followed in a prospective manner for 36 months. The study will employ 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 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, ultimately fatal illnesses. ? ?
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