Christmas disease; Factor IX hemophilia
Standard treatment is infusion of factor IX concentrates to replace the defective clotting factor. The amount infused depends upon the severity of bleeding, the site of the bleeding, and the size of the patient.
To prevent a bleeding crisis, people with hemophilia and their families can be taught to administer factor IX concentrates at home at the first signs of bleeding. People with severe forms of the disease may need ongoing, preventive infusions.
Depending on the severity of the disease, factor IX concentrate may be given prior to dental extractions and surgery to prevent bleeding.
Hepatitis B vaccine is recommended for individuals with hemophilia B because they are at increased risk of developing hepatitis due to exposure to blood products.
For additional information, see hemophilia support group.
The outcome is usually good with treatment. Most people with hemophilia are able to lead relatively normal lives. A small number of people develop inhibitors of factor IX, and may die from loss of blood.
Patients with hemophilia B should establish regular care with a hematologist, especially one who is associated with a hemophilia treatment center. The ability to have quick and easy access to medical records documenting the patient's history of factor IX levels, factor transfusions (including the type and amount), complications, and amount of any inhibitors can be lifesaving in the event of an emergency situation.
Chronic joint deformities may occur from recurrent bleeding into the joint. These can be managed by an orthopedic specialist. However, joint replacement(s) may be needed.
Intracerebral hemorrhage (such as deep intracerebral hemorrhage and lobar intracerebral hemorrhage) may also occur.
Thrombosis may occur following use of factor IX concentrate.
Call your health care provider if:
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