Effect of Prophylactic Management of Hemophilia on Bleeding Episodes
Hemophilia A (factor VIII deficiency) and hemophilia B (factor IX deficiency) are the most common and serious congenital coagulation factor deficiencies with repeated hemarthroses leading to development of target joints. Continuous prophylaxis is regular infusion of factor concentrates at fixed dose at regular interval to prevent hemorrhages. The study was designed to assess the outcome of continuous prophylaxis in hemophilia on bleeding episodes. It was conducted from November, 2017 to April, 2018 in the Paediatrics Department of Midnapore Medical College, Paschim Medinipur, West Bengal on 33 boys from 4 to 18 years of age suffering from Hemophilia with frequent bleeding episodes. Prior to starting continuous prophylaxis all the patients’ target joint(s) were assessed based on Gilbert Score and bleeding episodes in the last 6 months were assessed based on Annualized Bleeding Rate and ISTH-BAT Score. All the children were provided prophylaxis therapy with plasma derived Factor concentrate twice per week at a dose of 20 + − 2 IU/Kg. All bleeding episodes of the children during prophylaxis were recorded. Bleeding episodes showed significant improvement only in children who could sufficiently adhere to continuous prophylactic therapy. Continuous prophylaxis reduces bleeding episodes in Hemophilia in twice weekly protocol provided the patients sufficiently adhere to continuous prophylaxis regimen.
KeywordsHemarthrosis Gilbert score ISTH-BAT score Prophylaxis
Compliance with Ethical Standards
All procedures performed in the study were in accordance with the ethical standards of the institutional research committee and with the 1964 Helsinki declaration and its later amendments. The study was explained to all participants and their parents. Informed consent was taken from all the individual children as well as their parents included in the study.
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