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. Patients who develop an inhibitor to factor VIII may require treatment with other clotting factors such as factor VIIa, which can aide clotting even in the absence of factor VIII.
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.
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, especially prior to 1985 when blood screening procedures were improved for detecting the HIV virus. However, new heat processing treatment makes factor VIII material free of the HIV virus and thus safe for use.
