Understanding Hemophilia

How Do I Find Out If I Have It?

If your doctor suspects that you have hemophilia, the doctor will perform blood tests to examine how well your blood coagulates. Normally, when bleeding begins, a complex series of chemical events produces a "plug" to stop the bleeding; this plug is called a fibrin clot. Diagnosis of hemophilia usually involves carefully controlled evaluation of the length of time it takes blood, mixed with specific chemicals in a test tube, to produce a fibrin clot. If such screening tests are abnormal, other blood tests are run to determine the amounts of factors VIII and IX in the blood. These tests help doctors diagnose the type of hemophilia and its severity.
What Are the Treatments?

If you have hemophilia, you need to receive the appropriate clotting factor (factor VIII or factor IX) intravenously (through a needle in your vein) to stop or prevent bleeding. Factor preparations are manufactured from donated blood plasma or are synthetic (DNA-derived) recombinant factor VIII or factor IX. For persons with mild hemophilia A, there is a medication called desmopressin acetate (DDAVP) that can temporarily increase the concentration of factor VIII in your blood. DDAVP can be given intravenously, through an injection, or in the form of nasal spray.

The frequency of your treatments will depend on the severity of your condition. For instance, if you have mild hemophilia, you may need treatment only when you've been injured or in preparation for surgery. However, if you have severe hemophilia and bleed frequently, you may need regular, prophylactic infusions of clotting factors to prevent bleeding and protect your joints from deformity and disability.
Contamination of Blood Products

If in the past you received clotting factor that came from donated blood, you ran the risk of contracting a blood-borne disease. In fact, in the late 1970s and 1980s a large number of people with hemophilia acquired the virus that causes acquired immune deficiency syndrome (AIDS) as well as viral hepatitis. Now, potential blood donors are carefully screened with state-of-the-art tests that dramatically decrease the chance that anyone with AIDS or hepatitis donates blood.

All donated blood is carefully tested for viruses and is processed to inactivate any unrecognized viruses. So your chance of contracting any disease through treatment is extremely low. Still, if you have hemophilia it is important for you to receive immunizations for hepatitis A and B to prevent you from becoming infected with these viruses.
Other Complications

Another possible complication is that your immune system may begin to recognize the administered clotting factor as foreign and then destroy it, making your treatment ineffective. Your doctor will want to monitor your (or your child's) blood for such a reaction.
Ancillary Treatment for Joint Bleeding

For bleeding joints, doctors recommend resting and icing the affected joint to decrease pain and swelling. As pain and swelling subsides, physical therapy may help you recover joint mobility and strength.
Physical Activities

If you or your child has hemophilia, your doctor will discuss the types of physical activities that are appropriate and what kinds of activities may be too dangerous. Your doctor's advice depends on the severity of the factor deficiency.