The history of the diagnosis and therapy of cystic fibrosis (CT ) parallels the history of the major forces shaping clinical medicine in the 20th century. The story of cystic fibrosis can be used as an incisive case study of disease definition and negotiation, medical specialization and sub-specialization, the rise of genetic diagnoses and attempts at gene therapy, the uses of invasive technology in children, the role of private and public funding in directing scientific work, the ethical issues surrounding end of life care, and the competing metaphors of medical practice employed in the treatment of serious childhood illness. The principal aim of this proposal is the research and writing of a book on the history of cystic fibrosis in the 2Oth century, with particular emphasis on the social factors which have defined both the disease and the experience of illness. The principal sources will be published records of clinical and basic science research on CF as well as clinical records of patient care at a large CF Center; in addition, materials from the archives of the National Library of Medicine will be used for documentation of the activities of the major foundations supporting CF research and clinical care. The principal investigator is an experienced CF clinician as well as a medical ethicist with a strong interest in social medicine. His previous work has focused on the interaction of technology and terminal care for CF patients, as well as the ethical issue arising out of advances in therapy.
