LIFE-EXPECTANCY OF DUTCH HEMOPHILIACS, 1972-1985

The 1ife-expectancy of hemophiliacs has risen during the last decades from (for severe hemophilia) a mere 16 yrs in 1940, 23 yrs in 1964 to almost normal after coagulation factor preparations became available. Still, many hemophiliacs encounter ratings or refusals when applying for life-insurance. We carried out three mail surveys (1972, 1978, 1985) among Dutch hemophiliacs to establish excess mortality caused by hemophilia. Follow-up for non-respondents was carried out with help from the municipal authorities, while information on the deceased was obtained from the hemophilia treatment centers. To compare mortality with the general population, we constructed Kaplan-Meier reference curves for population groups with the same age and sex distribution, using national death tables. Pending completion of the follow-up for the 1972-1985 interval, this abstract supplies the results for the 1978-1985 period. The 1978 cohort included 578 individuals, with a mean age of 26 yrs (general male population: 33 yrs). None were lost to follow-up, 22 (3.8%) had died during the 7i yrs of observation. In the reference group 1.9% mortality would have been expected. So, overall relative mortality is increased twofold compared to the general male population (95% confidence interval 1.3-3.0). Patients with an inhibitor excluded, excess mortality did not differ much for severe, moderate and mild hemophilia (relative mortality: 2.3, 1.8, 1.5). In 11 cases hemorrhage was the cause of death, but in 6 cases this was associated with an underlying lethal disorder. In 9 cases the cause of death was not related to hemophilia and in 2 it remained unknown. An inhibitor was present in 4 (18%) of these 22 patients, but in only 4% of the total group of 578. In the 1972 survey 43% of the respondents who had applied for a life-insurance (n=199) reported a rating or a refusal, in 1978 47% of 227 and in 1985 53% of 676. The excess mortality caused by hemophilia is small and similar to that due to smoking cigarettes. Moreover, the excess may be caused in part by a carry-over effect from the time before modern treatment was introduced.