HEMOPHILIA By: Sherrie D. Curtis Class: Special Topics In Science Mendelian and Human Genetics Date: August 1, 2005

Hemophilia A Disease Hemophilia is a genetic disease Hemophilia is passed down from generation to generation Hemophiliac do not bleed to death when they have a scratch or a wound Internal bleeding can cause serious problems

Who Gets Hemophilia? A little over half of the cases of hemophilia occur in families with no history of hemophilia. Females can carry this genetic disease but it is then more likely to be passed down to their sons. Males can not pass this genetic disease down to their sons.

Symptoms Lengthy bleeding after circumcision Excessive bruising Swollen, painful joints Swollen, tender muscles Excessive bleeding from the gums, tongue, or mouth following injury or operations Severe bleeding after tooth extractions or other dental procedures

Detection of Hemophilia Long process involved with detecting hemophilia Observation of the blood mixed with chemicals in a test tube that produces a blood clot Other extensive tests are performed

Treatment of Hemophilia Infusions of clotting factors at home are done Hemophiliacs can live a successful life

Cost of Treatment Clotting factor procedure at home can cost up to $40, a year. Complication may raise the cost up to $100, a year.

Prevention Prevention is not a possibility at this time Experimentation of vitro fertilization from embryos for implantation is a possibility in the future Important to check and see if hemophilia runs in the family so that a person can get tested for it.

The Royal Family

Great Britains Royal Family Hemophilia is known as the “Royal disease” because it spread to the royal families of Europe through Victoria’s descendants. Hemophilia first appeared in Victoria’s family in her eighth child, Prince Leopold, Duke of Albany. From there it spread through the Royal Houses of Europe as monarchs arranged marriages to consolidate political alliances. We can trace the appearance of hemophilia as it popped up in Spain, Russia, and Prussia by looking at the family tree.

More about the Royal Disease Fortunately, Leopold was the only one of Victoria’s sons who suffered from hemophilia. Now for the Spanish connection: Victoria’s youngest child, Beatrice, gave birth to one daughter, one normal son, and two hemophilic sons. Queen Victoria’s third child, Alice, passed hemophilia to the German and Russian imperial families. Of Alice’s six children, three were afflicted with hemophilia In 1995, a sixty-three year old man named Eugene Romanov, a resident of the former Soviet Union, turned up. He shared both the disease and his last name with the royal family of czarist Russia. He proclaimed himself a grandson of Nikolas II’s youngest daughter, Anastasia.

Royal Heritage cont. Prince Charles is the designated next king of England. His well publicized marriage to Princess Diana produced two sons before it ended in an acrimonious divorce. You learned that one of the two was a hemophiliac. Finally, our speculative natures compel us to mention that in 1995 two British brothers produced a new book (Queen Victoria’s Gene) with a breathtaking suggestion. Professors Malcom Potts, an embryologist at Berkeley, and William Potts, a zoologist at Britain’s Lancaster University, suggest that Queen Victoria might have been illegitimate. They point out that neither her father nor her husband was a hemophiliac. So either there was a spontaneous mutation—a one-in-50,000 chance—or Victoria is the daughter of someone other than the Duke of Kent. Think of the possible consequences to European history: no Victoria, and the current Prince of Hanover, Ernst (descendent of the brother of Victoria’s father), would be King of England today. More importantly, no Victoria would mean no hemophilic son of the Czar of Russia, no Rasputin, and no revolution? What are the chances of this scenario? (Aronova- Tiuntseva and Herreid, State University of New York, ).

References (2004). (printed; 2005). Vogin, Gary MD, (2001). mendel.htm, (printed; 2005). Aronova-Tiuntseva, Yelena and Herreid, Clyde Freeman, ( ).