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Hemophilia is a rare inherited genetic disorder of blood clotting factors that primarily affects males. Individuals living with hemophilia don’t have enough of , or are missing one of the naturally occurring blood clotting factors found in blood. Two most common forms of hemophilia are A and B. Individuals with hemophilia A (also called as classical hemophilia), lacks clotting factor VIII completely or may not be in sufficient amounts where in the individuals with hemophilia B (also called as Christmas disease) lacks clotting factor IX. People with hemophilia do not bleed more profusely or bleed faster than normal as well they bleed for very longer period of time.
Hemophilia was identified as early as bibilicial times. Doctors in medieval times were familiar with it as well. In 1803, a Philadelphia doctor published the first description of hemophilia in the United states. But it was not until 30years later that hemophilia became widely recognized. Hemophilia later developed a reputation as the “royal disease” because it passes from Queen Victoria of England to her descendants throughout the royal houses of Europe. There are approximately 5,000 hemophiliacs worldwide.
The most significant advances in hemophilia treatment have been made in the last four decades. Baxter health care corporation introduced the first commercially available plasma derived factor concentrate in the mid 1960s. This was a major advancement over earlier formulations, which contained much lower concentrations of anti-hemophilic factor. In early 1970s, home treatment of hemophilia became widely available, offering people with hemophilia greater independence and reduced hospital stays. Today, recombinant DNA technology and the discovery of genes that control production of factor VIII and factor IX have led to the development of recombinant factors concentrates that do not rely on plasma at all.
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