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Monday, October 03, 2016

The Most Famous Hemophilia Carrier

I’m preparing for a visit to Russia next week, a country I have not been to since 1999. So I’ve been reading two excellent books to prepare: Peter the Great and Nicolas and Alexandra, both by Pulitzer-prize winning author Robert K. Massie.

These books, and last week’s post on hemophilia myths, got me thinking: Do you know who the most famous carrier of hemophilia was? Hint: It’s why hemophilia is dubbed “The Royal Disease.”

Queen Victoria of England, one of England’s longest reigning monarch. Her 63-year reign became known as the Victorian era, and was the longest reign until September 9, 2015, when Queen Elizabeth II surpassed her. Her era saw British power at its zenith across the globe; Victoria believed that the British Empire existed to civilize people in less developed countries and to protect them from their own rulers and the aggression of neighboring rulers. Others saw the purely commercial aspect of this world domination of lands and trade routes.

Victoria took the throne at age 18, and later married her first cousin, Prince Albert of Saxe-Coburg and Gotha, in 1840. Over the next seventeen years, she and Albert had nine children: Albert Edward, (b. 1841), Alice (b. 1843), Alfred (b. 1844), Helena (b. 1846), Louise (b. 1848), Arthur (b. 1850), Leopold (b. 1853) and Beatrice (b. 1857). The last child, Beatrice, was delivered under the care of Dr. John Snow, who later became the founder of modern public health by discovering the transmission mechanism of cholera (infected water). Snow used anesthesia on Victoria, thus giving her pain-free childbirth for the first time! While Snow did not invent anesthesia, he created a more convenient and safer way to administer it.

When their nine children married into royal and noble families throughout Europe (for mostly political reasons), Victoria was called "the grandmother of Europe," and indeed was called “Granny” by all her grandchildren and their spouses. 

Hemophilia was not known to exist in the royal family before, but Victoria carried the gene for hemophilia B. Only Leopold had hemophilia; two daughters, Beatrice and Alice, were carriers. They later transmitted hemophilia to the Spanish and Russian royal families. 

Leopold grew up to be a tall, intelligent, affectionate yet stubborn prince, whose willfulness often led to injuries and bleeds, according to author Massie. The Queen was unusually attached to her son, and worried over him incessantly. Victoria reported in one letter that Leopold had been at death’s door four or five times. Eventually Victoria tried to keep him confined to the upper floors of Buckingham Palace for his own protection, even as a man! But he managed to get away to Paris for two weeks. Eventually at age 29, he fell in love with a German princess, Helen of Waldeck. They had a daughter. When Helen was pregnant a second time, Leopold fell, suffered a brain hemorrhage and died at age 31.

 There was no treatment for hemophilia then, of course. Victoria was informed by telegram that her youngest son had died in Cannes. He was "the dearest of my dear sons," she lamented.

According to one of her biographers, Victoria wrote an average of 2,500 words a day during her adult life, and kept a detailed journal, which eventually encompassed 122 volumes. From this we learned a bit about hemophilia in those days.  The Queen didn't know what type of hemophilia her son had or if there even were types; only much later, through genetic testing, was the family found to have hemophilia B. While the mechanisms weren’t entirely understood, the royal family knew that hemophilia could be passed down from generation to generation. And it was; when it hit the Russian royal family, it would change the world.

For more information, read Robert K. Massie's excellent books, or view "Nicolas and Alexandra," a major motion picture and excellent portrayal of the last years of the Russian monarchy. Available on  Robert K. Massie also has an adult son, Robert K. Massie, Jr., who was later cured of his hemophilia through a liver transplant. His life is exquisitely portrayed in the book Journey.

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