Hemophilia Among the Russia’s Royal Family

Hemophilia Among the Russia’s Royal Family

            Hemophilia is a rare disease which is characterized by the inability to form blood clots. It is called “the royal disease” because it cropped up in the children of Queen Victoria of Great Britain. Queen Victoria was assumed to be the original carrier of the recessive X-linked hemophilia gene (Ochap, 2004). The clotting gene is carried by the X chromosome, which makes males more prone to hemophilia (Aronova-Tiuntseva and Herried, 2003). Males have only one X chromosome whereas females have two, one of which can compensate if the other is found defective.

Hemophilia was discovered in Spain, Prussia and Russia because of inbreeding. Inbreeding has contributed to the spread of the disease among the royal family as it increases the chance of receiving a harmful recessive allele (like hemophilia) from a common ancestor. As descendants marry their relatives to merge in order to strengthen political alliances and “to preserve royal blood lines” (Ochap, 2004), the chances of passing on hemophilia to offsprings increased.

            The royal family of Russia was not spared from the disease. Tsar Nikolas II of the Russian Imperial family married Alexandra, Queen Victoria’s granddaughter, who was a carrier of the disease. Alexis, the youngest and the only son, inherited hemophilia among the five children. Signs that Alexis inherited hemophilia appeared when his navel bled unexpectedly after birth. Moreover, dark swellings appeared every time Alexis bumped his leg or arm. The worst about his disease was that even his joints bled. This means that the affected limbs become crippled, and he experienced agonizing pain (Aronova-Tiuntseva & Herried, 2003). His condition prevented him from enjoying his childhood as it required him to stay in bed for weeks, and a small cut or wound would always bleed profusely. With this situation, Alexis’ parents became worried and afraid from the possibility that their son might bleed to death. A pedigree of the royal family is perhaps helpful in showing who carries the disease and which of the children inherits it.

References

Aronova-Tiuntseva, Y. & C.F. Herried. (2003). Hemophilia: ‘The royal disease.’ National Center for Case Study Teaching in Science. Retrieved November 19, 2008, from http://www.sciencecases.org/hemo/hemo.asp

Ochap, E. (2004). Inbreeding in humans. Retrieved November 19, 2008, from http://www.as.wvu.edu/~kgarbutt/QuantGen/Gen535_2_2004/Inbreeding_Humans.htm