Hemophilia: The Royal Disease
	By Aaron Nazarian and Idan Ivri What is hemophilia? Why is hemophilia called The Royal Disease? How did hemophilia enter the royal lineages of Europe and which ones did it enter? Pedigree of Queen Victoria's Descendants Pedigrees of Queen Victoria's Affected Children Hemophilia in the royal families of today What is hemophilia? Hemophilia is a hereditary (X-linked, recessive) blood disorder that affects the proper clotting of blood. It is a disease that affects males much more frequently (1 in 10,000) than females (1 in 100,000,000). This occurs because a critical blood-clotting gene is carried on the X chromosome. So because males carry only one X chromosome, if it is defective he will have hemophilia. Females, on the other hand, carry two X chromosomes. If only one is defective, the other normal X chromosome can compensate. The woman will have normal blood clotting, but she will still be a carrier of the recessive defective gene. A woman will know if she is a carrier sooner through genetic testing or later if any of her children are hemophiliacs. Naturally, female hemophiliacs are rare because it takes two defective X chromosomes in order for the condition to occur. Women with the recessive gene that codes for hemophilia simply pass it on to their offspring. Why is hemophilia called The Royal Disease? Hemophilia has often been referred to as The Royal Disease. The marriage of England's Queen Victoria and Prince Albert marked the beginning of hemophilia in the British royal line. Queen Victoria, Queen of England from 1837 to 1901, was a carrier of the hemophilia gene. She had nine children and as English royal family members married into royalty of other countries, the disease eventually infected most of the royal houses of Europe. So hemophilia gained its title of The Royal Disease because it spread through the royal families of Europe through Victoria's descendants. How did hemophilia enter the royal lineages of Europe and which ones did it enter? There is no certainty of how hemophilia originated in the British royal lineage. The traditional view is that there was a mutation in either Victoria's genes or in the sperm of her father, Edward Augustus, Duke of Kent. The earliest documentation of hemophilia in the royal houses of Europe was in the children of Queen Victoria. From the time it suddenly appeared in the children of Queen Victoria, hemophilia has played an important role in Europe's history. From Queen Victoria and the British royal family it spread through the royal houses of Europe as monarchs arranged marriages to consolidate political alliances. Queen Victoria and Prince Albert's marriage began the trail of hemophilia in the British royal lineage and became the link for the eventual spread into the Spanish, German (Prussian), and Russian royal families. It is also believed that Victoria's children spread the disease through the royal lineages of Denmark and Austria, but without solid records it is difficult to tell for sure. Pedigree of Queen Victoria's Descendants The appearance of hemophilia as it emerged in Spain, Russia, and Prussia (Germany) can be traced by following the family tree of Queen Victoria's descendants. Pedigrees of Queen Victoria's Affected Children Queen Victoria's eighth child, Leopold, was her only hemophiliac child. Two of Queen Victoria's daughters, Beatrice and Alice, were also carriers of hemophilia. The daughter's were the links that passed the disease onto the Spanish, German and Russian royal families. Below is a pedigree of Leopold's family. Leopold suffered from frequent hemorrhages and died of one at the age of 31. He did have two children before he died, however. His daughter, Alice of Athlone, was a carrier and had one hemophilic son (Rupert) and two other children, a boy and a girl. It is unknown whether or not the other two children were also affected, but in 1928 the boy died of a brain hemorrhage. Above is a pedigree of Beatrice's family. Beatrice°Øs lineage is the link between Queen Victoria and the Spanish connection to hemophilia. Beatrice was Queen Victoria's youngest child and gave birth to one daughter, one normal son, and two hemophilic sons. Below is a pedigree of Alice's family. Queen Victoria's third child, Alice, passed hemophilia to the German and Russian royal families. Of Alice's six children, one had hemophilia and two were carriers. Alice's daughter Irene, a carrier, married her first cousin, Prince Henry of Prussia, and gave birth to two hemophilic sons. Attempts were made to conceal the fact that the disease had shown itself in the German royal family, but, at the age of four, Waldemar, the youngest of the princes, bled to death. Alice's other daughter, Alix, was also a carrier. Had she accepted the offer of marriage from Prince Eddy, or his brother George, hemophilia would have been re-introduced into the reigning branch of the British royal family. But Alix married Tsar Nikolas II instead and carried the disease into the Russian imperial family. She had four daughters, Olga, Tatiana, Marie, and Anastasia, before giving birth to the long-awaited son, Alexis, heir to the Russian throne. These children, along with their parents, were eventually murdered during the Bolshevik Revolution. The existence of hemophilia in the Russian Royal family has historical significance because of its role in leading to the Bolshevik Revolution. Hemophilia in the royal families today Fortunately, Leopold was the only one of Queen Victoria's sons who suffered from hemophilia. Her other three sons, Edward, Alfred, and Arthur, were unaffected. Since the present royal family of England descended from Edward VII, the first son, it is free from hemophilia. In addition, the other European royal families from the late 1800's and early 1900's with hemophilia have long ago died and no present-day royal family members are known to have hemophilia. Sources—Canadian Hemophilia Society, www.yahoo.com, http://ublib.buffalo.edu/libraries/projects/cases/hemo.htm