Hemophilia

What is hemophilia?

Hemophilia is a bleeding disorder. It is a rare condition that does not allow the blood to clot normally.

How does it occur?

Hemophilia usually is inherited. This means that it is a problem with the genes and it can be passed from parents to children. When you have hemophilia, the body has little or no blood-clotting factor. Clotting factor is needed for blood to clot normally.

There are several types of clotting factors and there are different types of hemophilia. The type of hemophilia depends on which clotting factor is low or missing. The 2 main types of hemophilia are hemophilia A and hemophilia B.

• Hemophilia A is the most common form of hemophilia. It occurs when clotting factor 8 is low or missing.
• Hemophilia B was originally called Christmas disease. It is the second most common form of hemophilia. It happens when factor 9 is low or missing.

Because of the way hemophilia is inherited, it is much more common in males than females. The disease is usually passed to children by mothers who carry the trait but do not have symptoms. In most cases a man with the disease cannot pass it on to his sons. All of his daughters, however, may pass it on to their children.

What are the symptoms?

The main symptom is bleeding that lasts longer than normal after injuries or surgery. You may also have internal bleeding, especially into the joints (knees, ankles, and elbows). Examples of abnormal bleeding are:

• a lot of bleeding or bruising after minor injuries
• easy bleeding, such as nosebleeds
• bleeding in the mouth from a cut or bite or from losing a tooth

The first signs in a child may be:

• bruising beneath the skin as a child starts to crawl or walk
• bleeding gums when the child gets new teeth

How is it diagnosed?

Most cases of hemophilia are diagnosed in the first 2 years of a child's life. The diagnosis is based on family history, unusual bleeding, and blood tests. In some cases, especially if the symptoms are mild, the diagnosis may not be made until a child is several years old.

Hemophilia is classified as mild, moderate, or severe, depending on how much clotting factor is in the blood.

How is it treated?

There is no cure for hemophilia but there are treatments that can allow a fairly normal lifestyle. Bleeding is controlled by adding blood-clotting factors to the blood. Treatment choices include:

• Mild hemophilia A: A medicine called desmopressin (DDAVP) may be given to help the body release more clotting factor 8. Regular shots of DDAVP may be given at home to help prevent bleeding. You may learn how to give the shots yourself.
• Moderate to severe hemophilia A or hemophilia B: Blood products from donated blood, or genetically engineered products called recombinant clotting factors, may be slowly dripped or injected into a vein to control the bleeding. The slow drip or injection of medicine into a vein is called an infusion.

In some cases your healthcare provider may recommend regular infusions of replacement products to help prevent bleeding.

Usually you will be able to treat minor cuts at home with a bandage and pressure on the wound. If the bleeding does not stop, you will need to talk to your provider or go to the emergency room.

How long will the effects last?

Hemophilia lasts a life time. Healthcare providers hope that some day they will be able to cure hemophilia with genetic treatments.

How can I take care of myself?

• Exercise regularly in sports like biking, swimming, and walking. This will keep your muscles strong and protect your joints from injury. Avoid contact sports, such as football or soccer, and other activities that are more likely to cause internal bleeding.
• Don’t take aspirin, ibuprofen, or naproxen. These medicines can cause your stomach to bleed. Use acetaminophen when you need to take medicine for pain.
• Take care of your teeth. If you practice good dental hygiene, you may be able to avoid bleeding when you need dental care.
• Make sure your friends, family, and coworkers know that you have hemophilia.
• Wear a medical alert bracelet or necklace. If you need emergency care, the healthcare providers will then know about your condition.
• Hemophilia treatment centers are located in many areas of the US. These centers provide treatment, education, and support to people who have hemophilia and to their families.
• For more information, visit the following Web sites:
  • National Institute of Health Web site:
    http://www.nhlbi.nih.gov
  • The National Hemophilia Foundation Web site:
    http://www.hemophilia.org.