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On this World Hemophilia Day, we share the stories of five people who live with hemophilia, the struggles, and their triumphs over this ancient, debilitating disease.

Hemophilia is a hereditary disease that interferes with the body's ability to make the clotting factors that stop bleeding. Primarily a disease that is passed from mother to son, hemophilia is usually a condition in which a male's X-chromosome doesn't have a gene for making a clotting factor essential for forming fibrin to stanch the flow of blood after an injury to a blood vessel.

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Women, who have two X chromosomes, typically have at least one X chromosome that has a copy of the gene needed to encode the protein, and are almost always (unless they have both a mother who carries the disease and a father who has the disease) immune to the disease.

Hemophilia in History

Hemophilia has shaped history. Britain's Queen Victoria, who is an ancestor to almost all the royalty of Europe, carried the disease and passed it on to her sons. Her granddaughter Victoria Eugenie, who later became a Queen of Spain, bore two sons born with the disease who later died of bleeding after accidents.

Queen Victoria's granddaughter Alexandra, the last Empress of Russia, bore a son, Nicholas II, who according legend received treatment from the mysterious figure Rasputin, famous for his role in the Bolshevik Revolution. But as you can see in the five personal stories we'll share in a moment, hemophilia is no longer a "royal" disease.

No Longer a Death Sentence

Hemophilia was once a death sentence, the average life expectancy being only 11 years. With the use of blood-derived clotting factors, however, since 1980 most people who have hemophilia can now expect to lead an almost-normal lifespan. Living with hemophilia, however, is not yet normal. Here are five stories of five hemophiliacs who live courageously and well with the disease.

The Val Bias Story

Val Bias, now in his 50's, is president of the National Hemophila Foundation. Coming out of the womb with a purple knot on his head, Bias had two older brothers died of the disease and two more brothers who are unaffected. Bias has the more severe form of the disease, hemophilia B, in which there is essentially no ability for the body to make clotting factor IX and even slight injuries result in severe bleeding.

Bias, who is an African-American, points out that hemophilia strikes people of all races, and that even women and girls can come down with the disease. Hemophilia runs in families, but 1/3 of all cases appear in families with no history of the disease.

Since hemophiliacs have to be given blood products on a weekly basis and the blood supply has not always been tested for viral conditions, it's both possible and common for hemophiliacs to contract hepatitis B, hepatitis C, and HIV, and Bias has contracted all three. After treatment, Bias no longer has hepatitis B.

Even though hemophilia is a difficult disease, Bias told Karen Barrow of the New York Times, it is important to point out that having hemophilia doesn't mean you can't ever have fun. Unable to ride the subway and suffering severe joint pain, Bias still is an avid river rafter.

Continue reading after recommendations

  • Barrow, K. Changed, But Not Defined, by Hemophilia. New York Times, 30 January 2012.
  • Holstein K, Marx G, Lentz B, Bokemeyer C, Langer F. Successful eradication of a FVIII inhibitor in a 60-year-old patient with mild haemophilia A using single-agent prednisolone. Hamostaseologie. 2012. 32 Suppl 1:S48-51.

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