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.