Hemophilia is a rare inherited bleeding disorder in which the blood does not clot normally. Persons with hemophilia may bleed for a longer time than others after an injury or accident. They also may bleed internally, especially in the knee, ankle and elbow joints.
Babies born with hemophilia are missing or have a low level of a protein needed for normal blood clotting or blood coagulation. The protein is called a clotting factor. People with hemophilia can have one of the following problems with clotting factors:
When clotting factors are missing, or the body does not have enough of these factors, it can take a long time for blood to clot after an injury or accident.
About 18,000 people in the United States have hemophilia. Each year, about 400 babies are born with the disorder. Hemophilia usually occurs in males, with only very rare exceptions.
The main treatment for hemophilia is “replacement therapy” - giving or replacing the clotting factor that is too low or missing. Concentrates of the clotting factor are infused, or injected directly into the bloodstream. The specific factors used to treat hemophilia are:
Replacement therapy can be used for the following:
The type of treatment received depends on several things. Here are the treatments usually given for mild, moderate, or severe hemophilia:
Mild hemophilia - Replacement therapy is usually not needed for mild hemophilia. A medication called desmopressin (DDAVP), however, is sometimes given to raise the body’s levels of factor VIII. Since the effect of desmopressin wears off with chronic use, it is applied prior to dental work, participation in sports and other similar activities, to prevent or reduce bleeding. Desmopressin does not help in hemophilia B.
Moderate hemophilia - Those that may need treatment only when bleeding occurs need to learn to recognize signs and symptoms of bleeding so that they can get treatment as quickly as possible. They may also have treatment to prevent bleeding that could occur when participating in some activity.
Severe hemophilia - Long-term or shorter term preventive therapy is needed for those with severe hemophilia to prevent bleeding that could cause permanent damage to joints, muscles, or other parts of the body. Some people with severe hemophilia receive treatment only when bleeding occurs, however.
When bleeding occurs, it is important to get treatment as soon as possible. Delayed treatment can lead to complications. Learn to recognize signs of bleeding, and make sure that it is treated quickly.
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