International comparison of median age at death from cystic fibrosis

Study objectives: To compare international trends in mortality from cystic fibrosis. Design: Comparison of trends in median age at death using national mortality data. Setting: Data from 10 countries in North America, Europe, and Australasia. Participants: All persons registered as having died of cystic fibrosis in specified years from 1980 to 1994. Interventions: Comparison of relative odds of death at the international median age at death for the year of death between countries for two periods of time; from 1980 to 1987 (10 countries) and from 1980 to 1994 (7 countries). Measurements and results: The international median age at death increased from 8 years in 1974 to 21 years in 1994. Median age at death also increased within all countries, was consistently highest in the United States, and varied significantly by a factor of > twofold between countries. Women were significantly more likely to die at a younger age than the median age at death than men. Conclusions: Median age at death from cystic fibrosis is increasing, but our findings imply that clinically significant differences in survival with cystic fibrosis persist between countries.