Hemophilia A

Alternative names:

classical hemophilia; factor VIII deficiency hemophilia

Treatment:

Standard treatment is infusion of factor VIII 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.

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

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

Depending on the severity of the disease, DDAVP or factor VIII concentrate may be given prior to dental extractions and surgery to prevent bleeding.

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

Support groups:

The stress of illness can often be helped by joining a support group where members share common experiences and problems. See hemophilia - support group.

Expectations (prognosis):

With treatment, the outcome is good; most people with hemophilia are able to lead relatively normal lives. A small percentage of people with hemophilia will develop inhibitors of factor VIII and may die from loss of blood.

Complications:

• Chronic joint deformities, caused by recurrent bleeding into the joint, may be managed by an orthopedic specialist.
• Recurrent transfusions may increase the risk of contracting AIDS and hepatitis, especially prior to 1985 when blood screening procedures were improved for detecting the AIDS virus. However, new heat processing treatment makes factor VIII material free of the AIDS virus and thus safe for use.
• Intracerebral hemorrhage is another possible complication (see deep intracerebral hemorrhage, lobar intracerebral hemorrhage).

Calling your health care provider:

Call your health care provider if symptoms of a bleeding disorder develop.

Call for an appointment with your health care provider (for screening) if a family member has been diagnosed with hemophilia A.

Call for an appointment with your health care provider if you have hemophilia A and you plan to have children.