Hemophilia

The Basics

Written by the doctors and editors at UpToDate пїЅ пїЅ

What is hemophilia? " пїЅ Hemophilia is a condition that keeps blood from clotting normally. If blood doesnt clot normally, people can bleed very easily or much more than normal. The bleeding can sometimes be life-threatening. пїЅ пїЅ

People with hemophilia are missing a protein in the blood called a "clotting factor. " пїЅ Without this, the blood can't clot normally. There are 2 types of hemophilia (called "A " пїЅ and "B " пїЅ), depending on which clotting factor is missing. пїЅ пїЅ

Hemophilia is a life-long condition that people are born with. It is caused by an abnormal gene. Sometimes, parents pass this abnormal gene to their child. In some of these cases, parents might not know they have the abnormal gene, because they don't have any symptoms. Boys and men are most likely to have hemophilia; it is very rare in girls or women. пїЅ пїЅ

Hemophilia can be mild or severe. There is no cure for the disease, but it can be managed with different treatments. пїЅ пїЅ

What are the symptoms of hemophilia? " пїЅ Symptoms depend on how mild or severe a person's hemophilia is. Some children start having symptoms from birth. For example, a baby boy might bleed much more than normal if he has a procedure to remove the skin that covers the tip of the penis (called "circumcision " пїЅ). Other children, especially those with mild hemophilia, start having symptoms later on. пїЅ пїЅ

People with mild hemophilia usually bleed more than normal after an injury, procedure, or surgery. пїЅ пїЅ

People with severe hemophilia also bleed more than normal after an injury, procedure, or surgery. But they can also have bleeding that happens for no reason at all. This type of bleeding usually happens in a joint. The joints most often involved are the ankles, knees, and elbows. Blood in a joint can cause pain, swelling, stiffness, and trouble moving the joint. Over time, repeated bleeding in a joint can lead to long-term joint pain and damage. пїЅ пїЅ

Symptoms can also happen from bleeding in other parts of the body. These can include: пїЅ пїЅ

Blood in a bowel movement
Blood in the urine
Belly pain (from bleeding into the belly wall)
Bruises or bleeding in a muscle

Is there a test for hemophilia? " пїЅ Yes. Your doctor or nurse can check for hemophilia by doing different blood tests. пїЅ пїЅ

Sometimes, babies are tested for hemophilia before they are born. Pregnant women who have male relatives with hemophilia can choose to test their unborn baby for the disease. пїЅ пїЅ

How is hemophilia treated? " пїЅ Treatment depends on how severe a person's hemophilia is. It also depends on whether the person is going to have a procedure or surgery. пїЅ пїЅ

The main treatment for hemophilia is called "factor replacement therapy. " пїЅ This involves getting the clotting factor that the body is missing. There are different types of replacement clotting factors. Some are made from human blood, and others aren 't. Replacement therapy goes into a vein. People can get this treatment in the hospital, or they can give it to themselves (or have someone else give it to them) at home. пїЅ пїЅ

Replacement therapy is used in 2 ways: пїЅ пїЅ

To treat a bleed when it happens
On a regular basis, to prevent bleeding

Some people with hemophilia take other types of medicines, too, that can help with clotting. пїЅ пїЅ

What else should I know about replacement therapy? " пїЅ In the past, factor replacement therapy made from human blood sometimes had viruses in it. This could include HIV, the virus that causes AIDS, which could then infect the person being treated for hemophilia. Today, replacement therapy is made in a safer way and does not contain HIV. пїЅ пїЅ

What else can people with hemophilia do to avoid bleeding problems? " пїЅ To avoid bleeding problems, people with hemophilia should: пїЅ пїЅ

See their doctor for regular visits
Let every doctor who takes care of them know they have hemophilia, and which type ( "A " пїЅ or "B " пїЅ)
Follow their doctor's instructions about treatment and which activities or sports to avoid (if any)
Learn the signs and symptoms of bleeding, and how to treat it
NOT take aspirin or medicines called NSAIDs without the input of a doctor " пїЅ Common NSAIDs include ibuprofen (sample brand names: Advil, Motrin) and naproxen (sample brand names: Aleve, Naprosyn).
Plan ahead for any procedures or surgery that could cause bleeding
Take good care of their teeth and see their dentist for regular visits
Take their replacement therapy with them when travelling

If your child has hemophilia, you should let caregivers, daycare, or the school know. Teach them which symptoms to watch for, and how to treat a bleed. пїЅ пїЅ

How can I learn more about hemophilia? " пїЅ To learn more, talk with your doctor or nurse. The Hemophilia Foundation also has a lot of information. Their website is www.hemophilia.org. Their toll-free phone number in the US is 1-800-424-2634. пїЅ пїЅ

All topics are updated as new evidence becomes available and our peer review process is complete. пїЅ пїЅ

This topic retrieved from UpToDate on: Nov 04, 2014. пїЅ пїЅ

Topic 83610 Version 3.0 пїЅ пїЅ

Release: 22.8 - C22.206 пїЅ пїЅ

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Hemophilia

The Basics

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