Results

Mortality of patients with hemophilia

We studied mortality, causes of death and life expectancy of hemophilia patients between 1992 and 2001(1). We compared these findings with those of previous cohorts, together spanning the periods before, during and after the use of potentially contaminated clotting products. We performed a prospective cohort study among 967 patients with hemophilia A and B Death rates, overall and cause-specific, were compared to national mortality figures for males adjusted for age and calendar period as Standardized Mortality Ratio (SMR’s).
Between 1992 and 2001, 94 (9.7%) patients had died. Mortality was 2.3-times higher in hemophilia patients than in the general male population (SMR 2.3 95 % confidence interval 1.9-2.8). In patients with severe hemophilia life expectancy decreased from 63 years (1972-1985) to 59 years (1992-2001). Exclusion of virus-related deaths resulted in a life expectancy at birth of 72 years.
AIDS was the main cause of death (26%) and 22% of deaths were due to hepatitis C. In patients not affected by viral infections mortality was still slightly higher than in the Dutch male population. Thus mortality of patients with hemophilia is still increased; this is largely due to the consequences of viral infections.

Hepatitis C and health-related quality of life in patients with hemophilia

Hepatitis C has a negative effect on health-related quality of life (HRQoL). It is not clear whether hepatitis C affects HRQoL of patients with hemophilia. The objective of this study was to assess the effect of hepatitis C virus (HCV) infection on HRQoL in patients with hemophilia. We used data of the Hemophilia in the Netherlands-5 study. HRQoL was determined by using the self-administered SF-36 questionnaire. Patients were eligible for the study if they completed the SF-36, had been treated with clotting factor products before 1992, and had reported their hepatitis C status. Fifty-five percent (333/602) of the study population had a current HCV infection. Hemophilia patients infected with HCV scored lower on the HRQoL domains of general health and vitality than hemophilia patients who had never been infected with HCV.

Bleeding problems of carriers of hemophilia

Bleeding in carriers of hemophilia was investigated. A wide range of factor VIII and IX levels was observed both in carriers and non-carriers. In carriers extreme lyonisation may lead to low clotting factor levels. A postal survey was performed in all women tested for carriership of hemophilia between 1985 and 2001.We compared bleeding after trauma and medical interventions in carriers and non-carriers. Clotting factor levels lower than 0.60 IU/ ml were increasingly associated with prolonged bleeding from small wounds, prolonged bleeding after tooth extraction, tonsillectomy and operations. We showed that carriers of hemophilia have a higher risk of bleeding compared to non-carriers especially after medical interventions.
Currently we are investigating the risk of excessive bleeding related to delivery in carriers of hemophilia.

Other projects are still ongoing.