Hemophilia

Hemophilia is a disorder where the blood does not clot normally. While the blood disorder usually occurs only in males, there are rare exceptions in which a female will have it. Symptoms of hemophilia include bleeding (which may occur internally in joints and muscles) and bruising. Treatment most often involves replacement therapy, in which the clotting factor missing in people with the disease is replaced.

What Is Hemophilia?

Hemophilia is a rare, inherited bleeding disorder in which the blood does not clot normally. People who have it may bleed for a longer period of time following an injury or accident. People with the disorder may also bleed internally, especially in the joints (such as the knees, ankles, and elbows).
 
Approximately 18,000 people in the United States have hemophilia, and about 400 babies are born each year with this disorder in the U.S. Although it usually occurs only in males, there are very rare exceptions in which a female will have the blood disorder.
 
People with hemophilia have problems with certain proteins in their blood, called clotting factors. Clotting factors help blood to clot. Hemophilia can occur if there is a low level of one of the clotting factors or if a clotting factor is completely missing.
 
When clotting factors are missing, or your body does not have enough clotting factors, it can take a long time for your blood to clot after an injury or accident.
 

Understanding Clotting and Clotting Factors

An injury (such as a cut) to a blood vessel causes a complex chain of events that will result in a blood clot. This is called the clotting process, which is also known as blood coagulation. Clotting is your body's reaction to bleeding to keep you from losing too much blood. Losing too much blood can be life threatening and can damage your internal organs.
 
Clotting factors are proteins in the blood that work with platelets -- small blood cell fragments -- to help the blood to clot. When blood vessels are damaged, clotting factors help the platelets stick together to plug cuts and breaks at the site of the injury.
 
In people with hemophilia, blood does not clot as it should due to missing or low levels of clotting factors. If this occurs, there may be heavy blood loss or injured body tissues and organs, which may result in permanent damage or even death.
 

Hemophilia Disease

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