When Carrie Koenig was growing up in Pennsylvania, her family’s future was uncertain. Her father was one of 20,000 people in the United States diagnosed with an inherited bleeding disorder called hemophilia. Because of the long-term, debilitating nature of the disease, each day Carrie’s father and his family wondered whether he would be well enough to work, walk or care for himself.

About the disorder

From the Greek haima (“blood” or “shed blood”) and philia (in this case, “tendency to”), hemophilia is a rare and chronic genetic disorder. A person with hemophilia has minimal or no ability to produce a protein that aids significantly in the blood clotting process. Where Carrie’s dad could certainly survive a papercut, he and those like him could suffer internal bleeds in their joints, muscles, and other locations. These “bleeds” could result in severe pain, devastating joint damage and, in some cases, death.

The history of hemophilia is fraught with hope and devastation. Prior to the discovery of plasma and cryoprecipitate treatments in the 1960s–70s, people with hemophilia would spend weeks or months in the hospital with a bleed and no medication to stop it. In the late 1970s, researchers extracted the protein hemophiliacs were missing from blood donations. Carrie’s dad now had a treatment he could administered at home that provided some certainty to himself and his family.

HIV’s impact

However, tragedy struck the hemophilia community in the early 1980s as the same treatment that provided certainty to Carrie’s dad began to harbor killers. Blood donations that included HIV (the virus that causes AIDS) and hepatitis C virus were used inadvertently to produce the hemophilia treatment. Despite concerns over tainted blood being used to produce hemophilia treatments, patients were encouraged to continue to use these products.

“Today the U.S. blood supply is safer, as a result of this small community that would not be forgotten.”

It is estimated that 90 percent of people with the most severe form of hemophilia were infected with one or both of the viruses. Adults and children with hemophilia infected with HIV were shunned and isolated due to the stigma then associated with HIV infection. Thousands of hemophiliacs died, most well before their time, including well-known figures like teenager Ryan White.

When investigations into the tragedy proved it could have been greatly lessened or averted, the affected families and individuals instigated a grassroots campaign to demand justice. Their efforts would force changes to regulations regarding monitoring of the national blood supply. Today the U.S. blood supply is safer, as a result of this small community that would not be forgotten.

Today’s outlook

A child born with hemophilia today, such as Carrie’s son Nicholas, is expected to live as long as his non-hemophilia counterpart. Researchers have advanced safe and effective treatment options, and gene therapy could result in a cure for the disease.

Nonetheless, the repeal of the Affordable Care Act and failure to re-implement certain of its provisions could change all of this. Thus, this community of people and the advocacy organizations that support them are committed to averting another tragedy by making their voices heard, and providing a more certain future for Carrie’s father and her son.