Hemophilia and HIV

What is Hemophilia and How Does it Intersect with HIV?

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Prior to routine screening of the donated blood supply, people receiving blood and blood products were put at considerable risk for acquiring HIV. In fact, since the earliest part of the AIDS crisis in the 1980s and 90s, the risk of blood-to-blood transmission was considered so high as to place hemophiliacs as among the high levels of risk (a situation brought to world's attention with the highly publicized cases of Ricky Ray, Ryan White and Elizabeth Glaser).

 

What is Hemophilia?

Hemophilia is a genetic bleeding disorder characterized by lower than normal clotting factors circulating in the blood. With these abnormally low levels of clotting factors, blood clotting is prolonged which places the patient at risk for abnormal bleeding.

People living with hemophilia often need hospitalization for bleeding into joints such as the elbows and knees or abnormal bleeding after trauma or breaks in the skin. Because hemophilia is genetically linked to sex determining genes, hemophilia almost exclusively strikes males.

Why are Hemophilia and HIV Associated?

Prior to 1992, there was not a screening tool available to guarantee that donated blood products were HIV-free. Unfortunately, people living with hemophilia require regular transfusions of clotting factors in order to maintain a normal blood clotting system.

Therefore, those hemophilia patients receiving untested and unscreened clotting factor prior to 1992 were considered at an extreme risk for contracting HIV via the very blood products that were saving their lives.

To add to the already high risk was the way that blood supplies had been pooled, arbitrarily mixing blood donations from different donors rather based simply on blood type, meaning that even those donations that were negative were contaminated with HIV-infected blood.

The Story of Ricky Ray

Ricky Ray and his two brother were all hemophiliacs and received regular transfusions of blood products to maintain their clotting system.

Unfortunately, all three contracted HIV from what was believed to be HIV tainted blood products. They were not alone.

Over 10,000 people living with hemophilia contracted HIV in this manner during the early parts of the epidemic. What made matters worse was that it was later revealed that agencies ignored warnings that HIV was spreading rapidly though the hemophilia population and did nothing to pre-screen donors. .

The Ricky Ray story is a tragic one. After being diagnosed with HIV, Ricky and his brothers were kicked out of school for fear they would spread their HIV to other students. Eventually they were forced to go into hiding after their house was burned down by unknown assailants.

So outrageous was this injustice that, in 1998, Congress passed the Ricky Ray Hemophilia Relief Fund Act, paying restitution to those hemophilia patients who contracted HIV from July 1, 1982 to December 31, 1987.

What's the Status of Hemophilia and HIV Today?

Today, there are extensive screening tools in place that prevent HIV infected blood from entering into the blood supply.

By the late 1990s, following the advent of universal blood and tissue screenings, as well as the introduction of newer-generation HIV tests, the estimated risk of acquiring HIV from blood transfusions was roughly one out of 600,000 cases. By 2003, that risk was seen to be around 1 in 1.8 million.

Furthermore, from 1999 to 2003, only three Americans out of estimated 2.5 million blood recipients were confirmed to have acquired HIV the from transfusion of bloods following a false negative HIV screening.

Sources:

U.S. Department of Health and Human Services (DHHS). "Ricky Ray Hemophilia Relief Fund; Federal Register." Washiington, D.C.; September 29, 2005.

Schreiber, G.; Busch, M.; Kleinman, S.; et al. "The risk of transfusion-transmitted viral infections. The Retrovirus Epidemiology Donor Study." New England Journal of Medicine. June 27, 1996; 334(26):1685-1690.

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