hemophilia

A detailed Summary of hemophilia

Hemophilia is a genetic bleeding disorder. People who have hemophilia have a deficiency or an absence of a coagulation protein. A blood clotting factor is deficient or absent. Bleeding is most often into joints, such as the knee, elbow, or ankle, but bleeding can occur anywhere in the body. People with hemophilia bleed longer, not faster. The severity of hemophilia varies greatly. Hemophilia A and Hemophilia B are the most common genetic bleeding disorders. Hemophilia A is observed in 80 percent of hemophiliacs and is a deficiency or absence of Factor VIII. It can also be referred to as "classic" hemophilia. In the second most common, hemophilia B, factor IX is missing. This is also known as the "Christmas Disease" because of the surname of the first patient studied.

Hemophilia was identified as early as biblical 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 30 years later that hemophilia became widely recognized. Hemophilia later developed a reputation as the "royal disease" because it passed from Queen Victoria of England to her descendants throughout the royal houses of Europe.

Babies with hemophilia usually have no difficulty during the birth process, however circumcision may produce prolonged bleeding. During the first few months of life, a baby with hemophilia has few problems because ones ability to move around is limited. As the baby learns to walk one will fall and sustain many small, superficial bumps and bruises. Bleeding into soft tissue area of the arms and legs is frequent and usually not serious. Superficial bruises are seen easily and are often raised, but usually do not require treatment.

Clotting factor is one of 12 or more proteins found in blood that work together to make blood clot. They are designated by Roman numerals I through XIII. When the body detects bleeding, clotting factors are switched on in a specific order, each sending an activating message to the next. Factor VIII is one of the clotting factor proteins that helps produce the fibrin clot.

There are two important points to keep in mind: A fresh bleeding episode can start if the clot becomes dislodged and natural reactions in the body cause a clot that is no longer needed to "break down" or to be dissolved. The processes begin approximately five days after the initial clot was formed and occasionally a bleed may restart at the site. Although proficient, sometimes one infusion is not enough and one must be prescribe a follow up treatment.

All blood donors in the U.S. are tested for blood borne viruses and all blood products are tested for hepatitis and the virus that causes AIDS. In addition, concentrates are treated and purified in several ways to reduce the risk of hepatitis and virtually eliminate the risk of AIDS transmission. Monoclonal products are more pure than heated concentrates, and recombinant factor is considered the most pure.

After about 24 hours the body has essentially used up all the clotting factor, at which time the clot is well established and the body begins to get rid of the blood which has leaked into the surrounding tissues, so that eventually the swelling subsides.

As a child with hemophilia matures, one is more active and will have more bleeding episodes. The first sign of bleeding deep in a muscle may be a reluctance to use the limb. The child may become irritable as bleeding continues. When the symptoms are present, examination of the limb should be done carefully but do not in a manner where the limb should be moved forcibly is one resists. After an examination the opposite limb at the same time has been completed, one can detect possible differences in swelling and/or skin temperature.

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Approximate Word count = 2034
Approximate Pages = 8 (250 words per page double spaced)

Category: Science