The Basics
Written by the doctors and editors at UpToDate � �
What is hemophilia? " � 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. � �
People with hemophilia are missing a protein in the blood called a "clotting factor. " � Without this, the blood can't clot normally. There are 2 types of hemophilia (called "A " � and "B " �), depending on which clotting factor is missing. � �
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. � �
Hemophilia can be mild or severe. There is no cure for the disease, but it can be managed with different treatments. � �
What are the symptoms of hemophilia? " � 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 " �). Other children, especially those with mild hemophilia, start having symptoms later on. � �
People with mild hemophilia usually bleed more than normal after an injury, procedure, or surgery. � �
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. � �
Symptoms can also happen from bleeding in other parts of the body. These can include: � �
- 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? " � Yes. Your doctor or nurse can check for hemophilia by doing different blood tests. � �
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. � �
How is hemophilia treated? " � 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. � �
The main treatment for hemophilia is called "factor replacement therapy. " � 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. � �
Replacement therapy is used in 2 ways: � �
- 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. � �
What else should I know about replacement therapy? " � 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. � �
What else can people with hemophilia do to avoid bleeding problems? " � To avoid bleeding problems, people with hemophilia should: � �
- See their doctor for regular visits
- Let every doctor who takes care of them know they have hemophilia, and which type ( "A " � or "B " �)
- 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 " � 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. � �
How can I learn more about hemophilia? " � 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. � �
All topics are updated as new evidence becomes available and our peer review process is complete. � �
This topic retrieved from UpToDate on: Nov 04, 2014. � �
Topic 83610 Version 3.0 � �
Release: 22.8 - C22.206 � �
� � 2014 UpToDate, Inc. All rights reserved. � �
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