Cystic fibrosis deaths in the United States from 1979 through 1991. An analysis using multiple-cause mortality data

To analyze mortality trends among people who died with a diagnosis of cystic fibrosis from January 1, 1979, through December 31, 1991.

We reviewed death certificate reports in the Multiple-Cause Mortality Files compiled by the National Center for Health Statistics.

Of the 26,866,600 decedents in the study period, 6500 had a diagnosis of cystic fibrosis listed on their death certificates; of these, 6014 (92.5%) had cystic fibrosis listed as the underlying cause of death. The age-adjusted mortality rate decreased 21%, from 2.4 per 1 million in 1979 to 1.9 per 1 million in 1991, with similar decrements among males and females. The median age of death increased from 15 years in 1979 to 23 years in 1991. During the study period, whites were 6 times more likely to die with a diagnosis of cystic fibrosis than were blacks, and 8 times more likely than were people of other races. Comorbid conditions mentioned on death certificates included obstructive lung disease in 744 (11.5%), pneumonia in 1192 (18.3%), and right heart failure in 986 (15.2%).

From 1979 through 1991, the age-adjusted mortality rate for cystic fibrosis decreased and the median age of death among decedents with a diagnosis of cystic fibrosis increased. These results probably are due to improved treatment of the disease in children, although we cannot exclude other explanations for these findings, such as changes in death certification and coding or better diagnosis of the disease.