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    Understanding Hemophilia

    Understanding Hemophilia -- Diagnosis and Treatment

    How Do I Know if I Have Hemophilia?

    Normally, when bleeding begins, a complex series of chemical events produces a "plug" to stop the bleeding; this plug is called a fibrin clot. The fibrin clot is the end-product of many different "clotting factors" reacting in the blood. Hemophilia is an inherited condition in which one of these clotting factors (mainly factor VIII or IX) is absent from the blood, so that it does not clot normally. If your health care provider suspects that you have hemophilia, you may be given blood tests to examine how well your blood creates this clot. A lab mixes your blood with specific chemicals in a test tube, to produce a fibrin clot. If such tests are abnormal, other blood tests are done to determine the amounts of factors VIII and IX in the blood. These tests help doctors diagnose the type of hemophilia and its severity.

    What Are the Treatments for Hemophilia?

    Treatments for hemophilia include:

    • Receiving clotting factors replacement therapy
    • Medication
    • Treatment for joint bleeding and other problems associated with hemophilia

    The treatments you need will depend on the type of hemophilia and the severity of hemophilia. For instance, if you have mild hemophilia, you may need treatment only when you've been injured or in preparation for surgery. However, if you have severe hemophilia and bleed frequently, you may need regular treatment to help prevent bleeding and help protect your joints from deformity and disability.

    Clotting Factors for Hemophilia

    People with hemophilia receive the appropriate clotting factor (factor VIII or factor IX). The clotting factor is given intravenously (through a needle in your vein) to stop or prevent bleeding. Factor preparations come from two sources:

    • Donated blood plasma
    • Synthetic (DNA-derived)

    Newer types of these clotting factor products have been developed so they can last longer in the body. This means that they do not have to be used as often.

    One of these, Adynovate, is used to replace clotting factor VIII to help prevent and control bleeding in adults and children ages 12 and older with hemophilia A.

    In 2016, the FDA approved Idelvion, Coagulation Factor IX (Recombinant), Albumin Fusion Protein, to help control and prevent bleeding in children and adults with hemophilia B. Delivered by IV, Idelvion can be effective for up to two weeks and is delivered either as needed to prevent bleeding episodes or to manage the frequency of bleeding episodes.

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