Haemophilia care in central Scotland 1980-94. I. Demographic characteristics, hospital admissions and causes of death

To estimate the resources required to manage patients with haemophilia in S cotland, we studied the demographic features, hospital admissions and cause s of deaths for individuals with haemophilia A and B and von Willebrand dis ease, treated with blood products, during the period 1980-94 living in cent ral Scotland. Data were obtained from 413 adults and children (93% ascertai nment). The age distribution in 1980 revealed a paucity of individuals over 60 years but the number in this age group increased over the study period. Of those with haemophilia A and B, 63 and two respectively, became HIV pos itive. Hospital admissions rose from 103 to 168 per annum; the number of an nual bed days utilized also increased, but there was marked annual fluctuat ion (790-1832). The rate of admission was greater for those with severe hae mophilia A and this increased during the 15-year period mainly due to the c linical consequences of human immunodeficiency virus (HIV) and hepatitis C virus (HCV). The admission rate for haemophilia B was significantly lower t han that for haemophilia A, and was similar for all degrees of severity of the disorder. Throughout the 15-year period the incidence of admissions for acute bleeds was constant, as was the average duration in hospital. For th ose with a factor VIII inhibitor, the rate of admission was about double th e rate of those without an inhibitor, although the duration of hospital sta y was similar for both groups. There were 61 deaths; the death rate increas ed during the study period principally due to HIV and HCV, and 12 patients died from haemorrhage. We conclude that: (i) the life expectancy for haemop hiliacs in Scotland was generally increasing, although HIV and HCV caused i ncreasing mortality and morbidity (as shown by the increase in hospital adm issions); (ii) hospital bed usage for the treatment of acute bleeds continu ed to be required, but fluctuated greatly; and (iii) the clinical impressio n that haemophilia B is less clinically severe than haemophilia A is confir med by objective data. The planning implications for haemophilia care in Sc otland and similar countries are discussed.