Breaking down hemophilia (the “royal disease”) and its influence on the downfall of the Russian Romanov dynasty

It is widely known that many of the European royalty have a blood-clotting disorder called hemophilia, which gained itself a nickname, the “royal disease”. It has been considered as a mystery, or even a curse to the royal family, since all of them who suffered from this were their male descendants, while nothing appeared on the female counterparts. You can tell that this is hardly a coincidence, so lots of conspiracy theories prevailed. Many researches have revealed the biology behind it, but did you know that hemophilia has also led to the downfall of the Russian Romanov dynasty? Here is why.

What is hemophilia?

Group 6 515 A And B (Hema And Hemb) Joseph Alma - Lessons - Tes Teach

Hemophilia is a rare disorder where your blood doesn’t clot normally. It is a genetic disease that inhibits the formation of blood clotting factors.

If you have hemophilia, even minor injuries can be fatal as the bleeding can last for days or even weeks. 

In human DNA, we have a pair of sex chromosomes that determines gender. Males have an XY pair while females have an XX pair. Obviously, boys (with an XY pair) have his X chromosome inherited from his mother and the Y chromosome from his father. In contrast, girls inherit an X chromosome from each of her parents.

Hemophilia is a recessive disease and is carried on the X chromosome. Women who are carriers of hemophilia have one of her X chromosomes with hemophilia and the other one without. Due to the recessive nature of the disease, they do not appear to have the disease but the affected chromosome can be passed on to her offspring. 

In order to inherit the disease, a female must receive two affected X chromosomes, one from each parent, while a male receiving one affected X chromosome from his mother is sufficient to express the disorder. In short, males have a higher chance of suffering from hemophilia compared to females.

Hemophilia Inheritance Patterns | Indiana Hemophilia & Thrombosis ...

In the example above, there is a 50% chance that a female carrier’s son (XY) of having hemophilia, while the daughter (XX) is never going to express hemophilia. There is a 50% chance that the daughter is a carrier just like the mother.

Queen Victoria’s cursed descents

Victoria (1819-1901) was Queen of the United Kingdom of Great Britain and Ireland (1837–1901) and Empress of India (1876–1901). She was believed to be a carrier of hemophilia with the affected gene from a spontaneous mutation. Of her children, one son, Leopold, had hemophilia, and two daughters, Alice and Beatrice, were carriers. The mutation was passed on to various royal families through her descendants’ marriage across the continent, such as Spain, Germany and Russia. Putting it in another, less glamorous way: they were inbreeding.

ISSUES - Genetics isu : hemophilia

The royal disease and the royal collapse

Alexei Nikolaevich, who is the only son of Russian Emperor Nicholas II and Empress Alexandra Feodorovna (granddaughter of Queen Victoria), was born with hemophilia. Since there was very little knowledge about hemophilia at the time, no effective treatment could be done to alleviate his pain. The desperate Queen turned to Grigori Rasputin, a Russian mystic who claimed to wield healing magic. 

Alexandra and Nicholas II believed in Rasputin, and so he gained unbelievable political influence. His political decisions were based on his self-interest causing Russia on a brink of collapse. The loss of respect to the royal family plays a critical part leading to the Russian revolutions afterwards. He was in essence, a fraud. 

The tragic end

The disease did not kill the little prince Alexei. Ironically, he was murdered at age 13 in 1918 along with the rest of the Russian royal family following the Russian Revolution. But don’t fret, we can still find their genes in modern monarchies of today as a result of centuries of such marital practices. 

Tags: biology, academic lifestyle, history

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