Hemophilia: The Royal Disease
National Hemophilia Foundation
Hemophilia is a rare bleeding disorder in which the blood doesn’t clot normally. It is often called the “Royal Disease” because Queen Victoria of England (1837-1901) was a carrier of the hemophilia gene and passed the disease on to several royal families.
Victoria’s eighth child, Leopold, had hemophilia and suffered from frequent hemorrhages, which were reported in the British Medical Journal in 1868. Leopold died at the age of 31 of a brain hemorrhage. Leopold’s daughter, Alice, was a carrier, and her son, Viscount Trematon, was born with hemophilia. Viscount died in 1928, of a brain hemorrhage similar to the one that killed his grandfather.
References to excessive and unexplained bleeding have been made since antiquity. In the Talmud, a collection of Jewish Rabbinical writings from the 2nd century, it was written that male babies did not have to be circumcised if two brothers had already died from the procedure. In the 12th century, an Arabian physician from Cordoba named Albucasis wrote of males in a particular village who had died of uncontrollable bleeding. Occasional references to bleeding can be found in the scientific literature of following centuries.
In the U.S., transmission of hemophilia from mothers to sons was first described in the early 19th century. In 1803, Philadelphia physician Dr. John Conrad Otto wrote an account of “a hemorrhagic disposition existing in certain families.” He recognized a particular bleeding condition was hereditary and predominately affected males. He traced the disease back through three generations to a woman who had settled near Plymouth, New Hampshire in 1720. The word “hemophilia” first appeared in a description of a bleeding disorder condition at the University of Zurich in 1828.
Having hemophilia means you may bleed for a longer time than others after an injury. You also may bleed internally, especially in your knees, ankles and elbows. This bleeding can damage your organs and tissues and may be life threatening.
Hemophilia is usually inherited and passed from parents to children through genes. Those born with hemophilia have little or no clotting factor. Clotting factor is a protein needed for normal blood clotting. There are several types of clotting factors. These proteins work with platelets to help the blood clot.
Platelets are small blood cell fragments that form in the bone marrow — a sponge-like tissue in the bones. Platelets play a major role in blood clotting. When blood vessels are injured, clotting factors help platelets stick together to plug cuts and breaks on the vessels and stop bleeding.
The two main types of hemophilia are A and B. If you have hemophilia A, you’re missing or have low levels of clotting factor VIII (8). About nine out of 10 people who have hemophilia have type A. If you have hemophilia B, you’re missing or have low levels of clotting factor IX (9).
Rarely, hemophilia can be acquired. “Acquired” means you aren’t born with the disorder, but you develop it during your lifetime. This can happen if your body forms antibodies (proteins) that attack the clotting factors in your bloodstream. The antibodies can prevent the clotting factors from working.
Hemophilia can be mild, moderate or severe, depending on how much clotting factor is in your blood. About seven out of 10 people who have hemophilia A have the severe form of the disorder.
People who don’t have hemophilia have a factor VIII activity of 100 percent. People who have severe hemophilia A have a factor VIII activity of less than 1 percent.
Hemophilia usually occurs in males and only about 1 in 5,000 males are born with hemophilia each year.
(A Wellness Update is a magazine devoted to up-to-the minute information on health issues from physicians, major hospitals and clinics, universities and health care agencies across the U.S. Online at www.awellnessupdate.com.)
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