What is hemophilia?

The most common bleeding problem in people who have severe hemophilia is bleeding into a joint (hemarthrosis). This often happens without an injury. Symptoms include warmth, tingling, or swelling of the joint. Symptoms of bleeding into a muscle include bruising, swelling, tenderness, muscle hardening, and pain.

There are two main types of hemophilia:

• Hemophilia A, also called classic hemophilia, is caused by a lack of active clotting factor VIII (8). It is the most common type of hemophilia.
• Hemophilia B, also called Christmas disease, is caused by a lack of active clotting factor IX (9). It is less common than Hemophilia A.

Hemophilia usually runs in families and almost always affects males.

Hemophilia can be treated by replacing missing blood clotting factors. This can be done with clotting factor replacement therapy. Replacement therapy can prevent or treat bleeding episodes.

You may need to take medicines that help prevent bleeding. You might take medicines at certain times, such as before you have surgery or dental work. Talk to your doctor about what options may be right for you.

Hemophilia treatment centers are available at most large medical centers. They are an excellent resource to help you and your family get the best care for this condition.

Your doctor may ask about your medical history and your family's medical history. You may need to have some tests, such as a blood test or a genetic test.

If your doctor thinks that you may have a problem with blood clotting, your doctor will take a blood sample. The sample will be used in tests that check for the amount of clotting factor. If the level is low, then more tests will find out the type of hemophilia and how severe it is.

How severe the disease is depends on how much clotting factor is produced and when bleeding most often occurs.

Mild hemophilia.

Bleeding problems might not be noticed unless there is a lot of bleeding after a major injury or surgery.

Moderate hemophilia.

Bleeding problems are common and often follow a fall, sprain or strain.

Severe hemophilia.

Bleeding problems often happen one or more times a week for no reason.

If hemophilia runs in your family and you are planning to have children, ask your doctor about tests that can show if you are a carrier. (Only females can be carriers.) This will allow you to make informed decisions about pregnancy and prenatal care.

You can take steps at home to prevent bleeding episodes and improve your health.

• Learn how to recognize bleeding episodes so you can start treatment right away.
• Stay at a healthy weight. Additional stress on joints can trigger bleeding episodes.
• Get regular exercise. Choose activities that will keep your muscles and joints in good shape, such as swimming, tai chi, or walking.
• Don't take nonprescription medicines unless your doctor tells you to. And don't take aspirin or other nonsteroidal anti-inflammatory drugs (NSAIDs), such as ibuprofen and naproxen. These can affect the clotting action of your blood.
• Prevent injuries and accidents around your home.

Hemophilia A and B are caused by changes (mutations) in genes. These changes affect how much clotting factor a person has and how well it works.

In hemophilia, blood does not clot properly. This usually happens because your body does not have enough of a certain kind of clotting factor. This makes it harder for bleeding to stop. People with hemophilia may bleed a lot after cuts, during surgery, or even after a fall. Some people have abnormal bleeding inside their bodies for no clear reason.

Doctors often recommend acetaminophen, such as Tylenol, for pain relief in people who have hemophilia. Acetaminophen doesn't reduce swelling. But it's safer than other medicines.

Nonsteroidal anti-inflammatory drugs (NSAIDs) can cause problems. Examples are aspirin and ibuprofen. NSAIDs can cause bleeding in the stomach or intestines. They can interfere with blood clotting and affect the function of the cells that first plug a wound (platelets). Acetaminophen doesn't have these side effects. Be safe with medicines. Read and follow all instructions on the label.

Medicines that people with hemophilia should not take include:

• Aspirin.
• Ibuprofen, such as Advil or Motrin.
• Medicines that contain salicylate. This ingredient is closely related to aspirin. Alka-Seltzer, Pepto-Bismol, and many cough medicines contain salicylates.
• Other NSAIDs. An example is Aleve. These medicines are designed to reduce swelling and reduce pain.