QUALITY OF LIFE IN ADULTS AND ADOLESCENTS WITH SEVERE CYSTIC FIBROSIS

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This subproject is one of many research subprojects utilizing the resources provided by a Center grant funded by NIH/NCRR. The subproject and investigator (PI) may have received primary funding from another NIH source, and thus could be represented in other CRISP entries. The institution listed is for the Center, which is not necessarily the institution for the investigator. Cystic Fibrosis (CF) is one of the most common inherited fatal diseases; over the past thirty years, the median age of survival has improved from 14 years in 1969 to 29.1 years (95% CI's 27.5 to 31.6) in 1999. With this improved survival, this disease must now be considered a chronic and progressive lung disease. Unfortunately, there is very limited research on the utility and relative strengths of different methods to measure health-related quality of life in patients with CF. This application proposes a prospective cohort study of patients with severe CF. Four different health-related quality of life (HRQL) instruments will be assessed, including a recently developed CF specific quality of life instrument at 6 month intervals for a total of three years. The overall object of this proposal is to advance the state of the art in the measurement of HRQL in adolescent and adult patients with severe cystic fibrosis.