About Hemophilia

What is Hemophilia?

Hemophilia is an inherited disease that prevents the blood from clotting properly. People with hemophilia have a deficiency of a blood protein, also called a "clotting Factor," that is necessary to clot the blood and stop bleeding. A person does not bleed more profusely or more quickly than other people; however, he bleeds for a longer time. External wounds are usually not serious. Far more important is internal bleeding (hemorrhaging). These hemorrhages are in joints, especially knees, ankles and elbows; and into tissues and muscles. When bleeding occurs in a vital organ, especially the brain, a hemophiliac's life is in danger.

The severity of hemophilia can vary depending on the amount of coagulation Factor in the blood of a person with hemophilia. People with hemophilia can have mild, moderate or severe forms of the bleeding disorder.

• People with mild hemophilia have 5 to 30% of the normal amount of clotting Factor. They usually experience bleeding only after surgery or severe injury.
• People with moderate hemophilia have 1 to 5% of the normal amount of clotting Factor. These persons might bleed for a long time after surgery, a bad injury or dental work.
• People with severe hemophilia have less than 1% of the normal amount of clotting Factor. These people tend to bleed more often several times a month and sometimes for no clear reason. Bleeding may occur into muscles and joints.

Hemophilia affects people of all races, colours, and ethnic origins. The most severe forms of hemophilia affect almost only males. Females can be seriously affected only if the father is a hemophiliac and the mother is a carrier. This is extremely rare.

Hemophilia A and B

Originally, hemophilia was thought to be a single disease. In the 1950s, two separate forms of hemophilia were discovered: hemophilia A and hemophilia B.

Hemophilia A

Hemophilia A is the most common form of hemophilia, and accounts for about 80% of all cases of hemophilia. People with hemophilia A, also called "classic hemophilia," have a deficiency in clotting Factor VIII. The clotting Factor is either missing or there is not enough of it.

Hemophilia A happens in every race and population in the world. Hemophilia A is found almost only in males, and happens in about 1 out of every 10,000 people.

Hemophilia B

People with hemophilia B have a deficiency in coagulation Factor IX. Hemophilia B is also called Christmas disease, named for the first person diagnosed with a Factor IX deficiency. It is the second most common form of hemophilia, and happens in about 1 out of every 50,000 people. Like hemophilia A, hemophilia B is found almost only in males.