Treatment:

Standard treatment involves replacing the missing clotting factor. The amount of factor VIII concentrates needed depends on the severity of the bleeding, the site of the bleeding, and the size of the patient.

Mild hemophilia may be treated with desmopressin (DDAVP), which helps the body release factor VIII that is stored within the lining of blood vessels.

To prevent a bleeding crisis, people with hemophilia and their families can be taught to give factor VIII concentrates at home at the first signs of bleeding. People with severe forms of the disease may need regular preventive treatment.

Depending on the severity of the disease, DDAVP or factor VIII concentrate may be given before having dental extractions or surgery in order to prevent bleeding.

Immunization with hepatitis B vaccine is necessary because of the increased risk of exposure to hepatitis due to frequent blood infusions.

Patients who develop an inhibitor to factor VIII may require treatment with other clotting factors such as factor VIIa, which can help with clotting even without any factor VIII.

Support Groups:

For additional resources, see hemophilia support group.

Expectations (prognosis):

The outcome is usually good with treatment. Most people with hemophilia are able to lead relatively normal lives.

Patients with hemophilia 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.

Complications:

Chronic joint deformities may occur from bleeding into joints. This complication can be managed by an orthopedic specialist. However, joint replacement may be needed.

Intracerebral hemorrhage is another possible complication.

Repeated transfusions may slightly raise the risk for HIV and hepatitis , however, continued improvements in blood screening procedures makes blood products safer than ever.

Calling your health care provider:

Call your health care provider if:

• Symptoms of a bleeding disorder develops
• A family member has been diagnosed with hemophilia A
• You have hemophilia A and you plan to have children