Hemophilia

Hemophilia

Signs and Symptoms
Diagnosis
Treatment

Treatment

Our health care team can help you decide which is the best treatment option for you. We rely on your input to help develop a plan of care that will keep you healthy, active and able to live successfully with the challenges of hemophilia.

The present goal of hemophilia therapy is to raise factor levels, decrease the frequency and severity of bleeding episodes and prevent complications of bleeding with the use of clotting factor concentrates. Clotting factor concentrate, also called "factor," is a dried powder form of the clotting factor. It is mixed with water to form a liquid before it is given. Some clotting factor products, called plasma-derived factor products, are made from donated human blood plasma. Others, called recombinant factor products, are made in a laboratory and do not use human blood proteins. Recombinant factor products are safer since they decrease the potential transmission of viruses from donated blood.

Clotting factor concentrate is injected into your vein soon after you have had an injury or show signs of bleeding. When clotting factor is given, it immediately circulates in the blood so the body can use it immediately to form a blood clot. Once the blood clot is established and the bleeding has stopped, the body begins to reabsorb the blood that has leaked into the tissues and joints.

If you do not receive prompt treatment, extra blood can pool in the joint or soft tissue and cause pain and swelling that takes longer to go away. Over time, repeated bleeding into a joint can lead to severe joint damage and arthritis. Early treatment will minimize the risk of joint damage.

If you have mild hemophilia, you may only need to be treated episodically, that is, each time you experience a bleeding episode. If your hemophilia is moderate or severe, your hemophilia may require more frequent treatment.

Home Therapy

All factor treatments are infused or injected intravenously into a vein. At first, you will be treated at a hemophilia treatment center, your doctor's office or an emergency room. Later, you may be taught how to administer the factor at home. Devices, called ports, can be surgically inserted under the skin in the chest area to make it easier to administer clotting factor products.

Whether or not you are on home treatment, you should always have factor concentrate at home, since you may need to take it to the emergency room when you need a treatment. If the decision is made to infuse factor to treat your bleeding episode, the most important thing you can do is to administer it as soon as possible. If there is a delay, however, you can apply ice to help shrink the size of the leaking blood vessels, limit the amount of bleeding into joints or tissues and prevent a small bleed from becoming a larger one. To avoid ice burn, place a cloth, such as a washcloth, between the ice and your skin.

After you receive factor treatment for a joint bleed, rest, ice, compression and elevation (or "RICE") are required. You may also benefit from support devices, such as crutches, following a bleed into the knee or ankle, or a sling, following a bleed into a muscle or joint in the arm. Depending on the site of the bleed, you may have to limit activities for a few days after a bleed.

Over-the-Counter Medications

Acetaminophen, sold under the brand names Tempra and Tylenol, is recommended as a safe pain reliever for people with hemophilia. Follow the directions carefully and be sure to take only the recommended amount of the medicine.

Never take any product with aspirin, or acetylsalicylic acid, in it. Aspirin can interfere with clotting. Many common household remedies, such as Alka-Seltzer, contain aspirin, so read labels very carefully before you take any medication. Ibuprofen, such as Advil, Aleve and Motrin, also may interfere with clotting; these should be used only if you have discussed their side effects with your doctor If you have any questions about what is or is not safe for you to take, talk to your doctor or hemophilia medical staff.

Experimental Treatments

Although safe recombinant clotting factors now allow people with hemophilia to live a long, full life, researchers are still attempting to find a cure for the disease. Technically, hemophilia can be cured through a liver transplant, but the risks involved in the surgery and the requirement for lifelong medications to prevent rejection of the new liver may outweigh the benefits.

Researchers also are currently working on a way to insert a normal copy of the factor VIII or factor IX gene into the cells of patients with hemophilia to raise factor levels enough to decrease the frequency and severity of bleeding episodes and to prevent the complications of bleeding. So far, these approaches have not led to long-term increases in factor level, but it is hoped that they will be effective in the future.

Reviewed by health care specialists at UCSF Medical Center.
Last updated May 8, 2007