Bleeding episodes in severely affected adolescent haemophiliacs and their management with replacement therapy

Aronstam, Anthony (1981) Bleeding episodes in severely affected adolescent haemophiliacs and their management with replacement therapy. University of Southampton, Doctoral Thesis.

Abstract

The consequence of undertreating haemophiliacs with Factor VIII is crippling deformity, but there are hazards associated with adequate therapy, which also strains human and financial resources. The work presented in this thesis attempts to rationalise the use of factor VIII. A review of the literature covers aspects of the disease itself, of transfusion therapy, and of the hazards of treatment. A linking section describes the history of the Lord Mayor Treloar College and Hospital where the work was undertaken. The work itself consists of retrospective observations and prospective studies made on severely affected adolescent haemophiliacs resident at the College between 1973 and 1979. The first section is an analysis of 4935 bleeding episodes, and information is presented on patterns of bleeding and transfusion requirements. The role of prophylactic treatment is described in the second section. Two double-blind controlled trials of prophylaxis are reported, as well as an analysis of prophylactic treatment during 1976 and 1977. The final section reports a double-blind controlled trial of the effect of three different dose regimes on the outcome of bleeds into the knee, ankle and elbow joints. The effect of past and continuing joint damage on outcome has also been studied. The major conclusions are that patterns of bleeding alter during adolescence and transfusion requirements vary with the site of bleeding. Prophylaxis is effective but usage of factor VIII escalates steeply with more intensive treatment. Finally, the underlying condition of the joint affects the response to different dosage regimes.

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