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Sabtu, 07 November 2009

If you ride a skateboard or play a sport, you probably know about bruises and banged-up knees and elbows. But for guys with a rare bleeding disorder called hemophilia , minor cuts and bruises can be a big deal.

What Is Hemophilia?

Hemophilia is a disease that prevents blood from clotting properly, so a person who has it bleeds more than someone without hemophilia does. It's a genetic disorder, which means it's the result of a change in genes that was either inherited (passed on from parent to child) or occurred during development in the womb. "Hemo" means blood and "philia" means a tendency toward. A person who has hemophilia has a tendency to bleed excessively. Hemophilia affects mostly boys, although it's very rare: Only about 1 in every 5,000 boys is born with it. The disease can affect people of any race or nationality.

When most people get a cut, the body naturally protects itself. Sticky cells in the blood called platelets go to where the bleeding is and plug up the hole. This is the first step in the clotting process. When the platelets plug the hole, they release chemicals that attract more sticky platelets and also activate various proteins in the blood known as clotting factors. These proteins mix with the platelets to form fibers, and these fibers make the clot stronger and stop the bleeding.

Our bodies have 12 clotting factors that work together in this process (numbered using Roman numerals from I through XII). Having too little of factors VIII (8) and IX (9) is what causes hemophilia. A person with hemophilia will only lack one factor, either factor VIII or factor IX, but not both.

There are two major kinds of hemophilia. About 80% of cases are hemophilia A, which is a factor VIII deficiency. Hemophilia B is when factor IX is lacking.

Hemophilia is classified as mild, moderate, or severe, based on the amount of the clotting factor in the person's blood. If someone produces only 1% or less of the affected factor, the case is called severe. Someone that produces 2% to 5% has a moderate case, and someone that produces 6% to 50% of the affected factor level is considered to have a mild case of hemophilia. In general, a person with milder hemophilia may only bleed excessively once in a while, whereas severe hemophilia puts someone at risk for having bleeding problems much more often.

Most people with hemophilia discover they have the condition when they are babies or young kids. Sometimes the disease is so mild that a guy doesn't even know he has it until he has minor surgery — like getting his tonsils or appendix out — and it's found in blood tests that doctors perform before surgery.