Once called the Royal Disease, hemophilia is an inherited bleeding disorder. Those who have it lack...
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Once called the Royal Disease, hemophilia is an inherited bleeding disorder. Those who have it lack a protein, called a clotting factor, which is required for blood to clot. Your body has several clotting factors which are all necessary for proper blood clotting. When you cut your skin or a blood vessel a series of reactions occur to activate clotting factors and platelets. This creates a kind of glue that forms a clot that acts like a plug. When a clotting factor is missing, the chain reaction is disrupted, so it takes longer for bleeding to stop. This means a person bleeds longer after an injury and sometimes bleeds without an obvious cause. Over time, internal bleeding can damage tissues and organs. About 70-80% of bleeding incidents involve the joints and can cause damage similar to arthritis. The first treatments for hemophilia involved transfusions of the missing factor from donor blood. In the 1980s, many of those treated became infected with HIV and hepatitis. Fortunately, that risk has been virtually eliminated by new screening procedures and technologies. Scientists have also figured out how to create synthetic blood factors by cloning natural genes responsible for their production. And there are ongoing efforts to remove all human and animal proteins from treatments for hemophilia.