Hemophilia in Sweden. Studies on demography of hemophilia and surgery in hemophilia and von Willebrand's disease.

Abstract

Demography: All known hemophiliacs in Sweden, 564 cases, were subjected to a demographic survey in 1980. The ratio of hemophilia A/B was 81/19. The severe cases constituted 30% and the mild 54%. Mean and median ages for mild and moderate hemophiliacs did not differ from those of Swedish males but were approximately 10 years lower in the severe cases. The prevalence was 7 per 100,000 population. The incidence remained constant at 1.7 (+/- 0.1) per 10,000 live-born males in the period 1957-1978. Mortality: In the period 1957-1980, there was a substantial increase in the median age at death among 118 deceased hemophiliacs. This increase was most pronounced in severe hemophilia, from 19 to 50.5 years. Intracranial hemorrhage accounted for one third of the deaths. Six deaths were attributed to hepatic dysfunction caused by hepatitis. Age related deaths (ischemic heart disease and malignancy) increased from 8 % to 32 %. Longevity: Median life expectancy of hemophiliacs in Sweden increased faster than the median life expectancy of the male population. In the beginning of the century, severe hemophiliacs faced a median life expectancy of about 11 years which had increased to 58 years by 1969-1980. Median life expectancy in mild and moderate hemophilia was 72 years, only three and a half years less than the corresponding age for Swedish males. Surgery in hemophilia and von Willebrand's disease: Three hundred and twelve operations (225 in hemophilia and 87 in von Willebrand's disease) were performed on 180 patients (121 hemophiliacs) in the period 1956-1983 at the General Hospital in Malmö. During the first years reconstructive orthopedic surgery dominated among young, severely affected, hemophiliacs but in recent years surgical diseases unrelated to the bleeding disorders increased. Ninety-seven operations were indicated by ailments caused by the bleeding disorders, mainly hemophilic arthropathy and pseudotumours. In another 72 operations the bleeding disorders were considered a contributing factor, mainly gastric surgery for ulcers, hysterectomy indicated by menometrorrhagia and subdural hematomas. Reconstructive orthopedic surgery was almost exclusively performed in hemophilia but gastric surgery was more common in von Willebrand's disease. The operations claimed three fatalities. It was considered essential to extend the substitution therapy to at least two weeks, especially in orthopedic surgery and in severe hemophilia. Furthermore, it was of the utmost importance to have access to laboratory facilities for repeated determinations of VIII:C/IX:C in hemophilia, Duke bleeding time in von Willebrand's disease, and to exclude the presence of inhibitors.