Cystic fibrosis: what do patients know, and what else would they like to know?

As the survival of patients with cystic fibrosis (CF) improves, it becomes increasingly important to address such issues as employment, reproduction, and quality of life in adulthood. In this study, 22 patients and 20 parents completed a questionnaire about the natural history, treatment and genetics of cystic fibrosis. Patients and parents knew as much as each other about the natural history of cystic fibrosis. Patients under 13 knew significantly less about the genetics and treatment. All found probability questions about heredity difficult. All the patients indicated that they would like further information on the future and how they would cope, through CF literature, organized patient groups and, in adolescence, would prefer to obtain information from medical staff rather than parents. Patients had received considerable information about the disease and its treatment from parents and clinical staff, but few had discussed the day to day problems of living with cystic fibrosis. This study has identified specific areas of ignorance in patients with cystic fibrosis, areas that need addressing in view of the improved survival associated with this disease.