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Posted by Doctor Pediatric Center - Vinmec Times City International Hospital
Hemophilia is an X-linked hereditary bleeding disease, caused by decreased or abnormal function of factor VIII (Hemophilia A) or factor IX (Hemophilia). In Vietnam, it is estimated that there are 6000 patients and 30,000 carriers of the disease gene. Treatment of hemophilia is infusing factor VIII or IX deficiency to the patient.
1. Principles of treatment
When there is suspicion of bleeding, RICE should be applied to support hemostasis (RICE stands for English words: Rest = rest, Ice = ice, Compression = compression, Elevation = elevation lesions):
Adding factor VIII/IX is enough to stop the patient's bleeding as soon as possible when bleeding occurs. Factor VIII/IX is a biological product that can be obtained from the following sources: Factor VIII is found in fresh plasma, fresh frozen plasma, frozen factor VIII concentrates of human plasma origin, factor VIII concentrates. Recombinant Factor IX Source: Fresh plasma, fresh frozen plasma, precipitated plasma, factor IX concentrate of human plasma origin, factor IX concentrate recombinant Factor VIII/IX to be achieved depends on the site of bleeding, the extent of bleeding and monitoring of the effectiveness of treatment.
2. How to calculate the dose of clotting factor
Hemophilia A: Factor VIII needed (UI)= (VIIIcđ –VIIIbn)% x P(kg)/2 Hemophilia B: Factor IX needed(ui) = (IXcđ – IX bn)% x P(kg) VIIIIcđ, IX cd: Is the concentration of factor VIII, IX to achieve (%). The required concentrations of these factors depend on the site of bleeding. VIII patient, IX patient: Is the concentration of factor VIII, IX just before treatment of the patient (%) P is the weight of the patient (kg) The half-life of factor VIII is from 8-12 hours so When bleeding is heavy, dangerous sites need to add factor VIII every 8-12 hours. The half-life of factor IX is 18 to 24 hours, so add factor IX every 24 hours.
3. Hemophilia still needs supportive treatment
Fibrinolytic inhibitors such as Tranexamic Acid (Transamin) Desmopressin increase the concentration of factor VIIII. Indicated for mild and moderate hemophilia. Corticosteroids in cases of arthritis have stopped bleeding. Pay attention to applying ice, compressing bandages, elevating the injury site, sedating pain relief. Treatment of complications: Iron supplementation, HC block transfusion in severe anemia. Use antibiotics when there is an infection. Treatment of hepatitis B, C if available Treatment when the patient needs surgery: Surgery and procedures such as tooth extraction, tonsillectomy ... need to add factor VIII or IX enough before proceeding.
4. Management of Hemophilia patients
Each patient needs to be registered and managed at the Hemophilia centers. Assessed and guided patients to rehabilitation exercises after each bleeding episode Assessed musculoskeletal function at the rheumatology or rehabilitation specialist 1 year / time Oral examination 1 year / time Assessment status bleeding and use of coagulation factors, quality of life of patients every year. Patients need to be pedigree to identify people who are likely to have the disease or carry the disease gene in the family for genetic counseling. Patients with hemophilia should be fully vaccinated, especially hepatitis A and B vaccines if not already infected. Hemophilia is a disease that requires lifelong monitoring and treatment, so it requires close cooperation between the patient and the patient's family (especially children). The responsibilities of the hematologist are to treat diseases and complications, and to manage patients from an early age so that hemophilia patients have the best quality of life.
Vinmec International General Hospital with a system of modern facilities, medical equipment and a team of experts and doctors with many years of experience in medical examination and treatment, patients can rest assured to visit. examination and treatment of hemophilia at the Hospital.
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