Hemophilia


Hemophilia is a rare genetic blood clotting disorder that primarily affects men. Those with the disorder have a deficiency in blood proteins called clotting factors, which are necessary to control bleeding in the body. There are approximately 17,000 people with the disorder in the United States, according to the National Hemophilia Foundation.

The disorder came to increased public awareness in the mid-1980’s at the beginning of the HIV/AIDS pandemic. At that time, one prominent personality, Ryan White, an Indiana teenager, became the human face of the disorder. White contr acted AIDS

as a result of a blood transfusion he received due to his having hemophilia.

Because of the absence of these clotting factors, those with the disorder, known as hemophiliacs, run a greater risk of unusual bleeding throughout the body, especially in muscles and joints. This bleeding may result from an injury or could begin for no apparent reason.
TYPES AND TREATMENT

Treatment for Hemophilia depends on the type and severity which one has. Before the disorder can be diagnosed a test on blood clotting factors is performed. The Thromboplastin Generation Test is performed in order to determine which clotting factors are present or absent in the blood.

Clotting factors may be replaced by injecting blood into the veins. Clotting factors in the blood are measured using percentages to determine the severity of the disorder. Those with the most severe form of hemophilia are forced to abide by a strict schedule to re-infuse their bodies with life-sustaining blood. In patients with this form of the disorder, bleeding typically happens at least one a week. Patients have less than 1 percent of the usual clotting factor in their blood.

Those with a mild or moderate type of the disorder may have between 1 and 5 percent of the usual clotting factor and may experience bleeding after a fall, sprain or strain. Those with the mildest form have a clotting factor of 5 percent or more and are often undiagnosed until major surgery or trauma.
TRANSMISSION AND PROGNOSIS

Although the majority of hemophiliacs inherit the disease from their mother, it has been estimated that about one-third of cases occur in people with no discernable genetic link to it. In these cases, a mutation in chromosomes that determine the blood’s ability to clot is the cause of the malady. A child with this form of mutation can either be a hemophiliac or a carrier. Women are usually carriers, but rarely get the disorder themselves. The life expectancy of those with hemophilia is normal or near normal. However, health complications from the disease can and sometimes do arise.
Material complied from WebMD.com and various sources contributed to this report.

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