Treatment:

Standard treatment involves replacing the missing clotting factor. The amount of factor VIII concentrates needed depends on how severe the bleeding is, 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 preventative treatment.

Depending on the severity of the disease, DDAVP or factor VIII concentrate may be given before having 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 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):

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.

People with hemophilia A should establish regular care with a hematologist (blood doctor), especially a doctor who is associated with a hemophilia treatment center. The ability to have quick and easy access to medical records documenting the level of Factor VIII that the person has had, the history of factor transfusions (including the types and amounts), any complications, and the type and amount of any inhibitors can be lifesaving in the event the person with hemophilia is in an emergency situation.

Complications:

• Chronic joint deformities, caused by recurrent bleeding into the joint, may be managed by an orthopedic specialist. These problems sometimes require joint replacement.
• Recurrent transfusions may increase the risk of contracting HIV and hepatitis. Since 1985, however, blood screening procedures were improved for detecting the HIV virus. And new heat processing treatment makes factor VIII material free of the HIV virus and thus safe for use.
• Intracerebral hemorrhage is another possible complication (see deep intracerebral hemorrhage, lobar intracerebral hemorrhage).

Calling your health care provider:

• If symptoms of a bleeding disorder develops
• (Call for screening) if a family member has been diagnosed with hemophilia A
• If you have hemophilia A and you plan to have children