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Hemophilia

Treatment



Various types of factors VIII and IX are available to replace a patient's missing factors. These are administered intravenously (directly into the patient's veins by needle). These factor preparations may be obtained from a single donor, by pooling the donations of as many as thousands of donors, or by laboratory creation through highly advanced genetic techniques.



The frequency of treatment with factors depends on the severity of the individual patient's disease. Patients with relatively mild disease will only require treatment in the event of injury, or to prepare for scheduled surgical or dental procedures. Patients with more severe disease will require regular treatment to avoid spontaneous bleeding.

While appropriate treatment of hemophilia can both decrease suffering and be life-saving, complications of treatment can also be quite serious. About 20% of all patients with hemophilia A begin to produce chemicals within their bodies which rapidly destroy infused factor VIII. The presence of such a chemical may greatly hamper efforts to prevent or stop a major hemorrhage.

Individuals who receive factor prepared from pooled donor blood are at risk for serious infections which may be passed through blood. Hepatitis, a severe and potentially fatal viral liver infection, is frequently contracted from pooled factor preparations. Most frighteningly, pooled factor preparations in the early 1980s were almost all contaminated with Human Immunodeficiency Virus (HIV), the virus which causes AIDS. Currently, careful methods of donor testing, as well as methods of inactivating viruses present in donated blood, have greatly lowered this risk, but not before huge numbers of hemophiliacs were infected with HIV. In fact, some statistics show that, even today, the leading cause of death among hemophiliacs is AIDS.

Resources

Books

Andreoli, Thomas E., et al. Cecil Essentials of Medicine. Philadelphia: W. B. Saunders Company, 1993.

Berkow, Robert, and Andrew J. Fletcher. The Merck Manual of Diagnosis and Therapy. Rahway, NJ: Merck Research Laboratories, 1992.

Hay, William W., et al. Current Pediatric Diagnosis and Treatment. Norwalk, CT: Appleton & Lange, 1995.


Rosalyn Carson-DeWitt

KEY TERMS

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Amplification

—A process by which something is made larger. In clotting, very few chemical released by the initial injury, result in a cascade which produces increasingly larger quantities of different chemicals, resulting in an appropriatelysized, strong fibrin clot.

Fibrin

—The final substance created through the clotting cascade, which provides a strong, reliable plug to prevent further bleeding from the initial injury.

Hemorrhage

—Very severe, massive bleeding which is difficult to control.

Additional topics

Science EncyclopediaScience & Philosophy: Habit memory: to HeterodontHemophilia - Normal Blood Clotting, The Defect In Hemophilia, How Hemophilia Is Inherited, Symptoms Of Hemophilia - Diagnosis