Tsarevich Alexei Nikolaevich's blood

 Tsarevich Alexei Nikolaevich's blood 

By Dr Suzie Edge

As Leopold, Duke of Albany stepped out onto a tiled floor in 1884, he slipped and fell. He hit the ground and the crash caused the vessels within his knee to break open. Worse, he also hit his head and the blood vessels in his head ruptured too. We've all slipped on a wet floor and had our pride bruised, but most of our blood vessels have a way of stemming the bleeding. But for Leopold this was bad - his blood would not stop flowing.

The Duke of Albany was Queen Victoria's son. When he was born in 1853 the Queen had famously used the exciting new anaesthetic chloroform, the agent developed by James Young Simpson. The use of chloroform at Leopold's birth, whilst an interesting side point, had no effect on the trouble in his veins.

Queen Victorias royal family had a problem. Their blood, blue as it was, was not clotting properly.

Normally, when the vessel wall is disrupted a chain of events is triggered. A cut of the skin or a disruption within the body signals that a clot is needed to act as a Band-Aid and prevent blood loss. Twelve proteins in the cascade, called factors, set each other off in turn, each catalysing the activation of the next, ultimately producing fibrin. Fibrin plugs the hole, recruits other factors and stops the bleeding. If anything interrupts this chain it is going to affect clotting to one degree or another.

Leopold had a defect in the gene that codes for the Factor IX protein within the clotting cascade. Without this clotting factor, his bleeding was not stopped. The smallest of injuries can lead to blood loss, which over time can be fatal. On this day there was no fibrin clot to shut Leopold's blood vessel's bleed off. Leopold had suffered with this throughout his life but this time, at age thirty, the bleed into his brain was the worst yet. The blood seeped in and pushed on the brain tissue. The Duke died a few hours later of a brain haemorrhage.

When Victoria was born in 1819 there were no obvious signs of the disease within the family. It has long been understood that the family's haemophilia started with Queen Victoria's own spontaneous mutation. Some have even said this is evidence that Victoria was not even legitimate. More recent evidence suggests that the disease could be seen in the ancestors of her mother.

Haemophilia was starting to be recognised as an inherited bleeding condition in the nineteenth century and although it is not restricted to royalty, it became known as the Royal Disease.

Haemophilia is not one irregularity but is a group of disorders characterised by clotting problems associated with a missing or broken part of the clotting cascade. Haemophilia A is the result of a break at Factor VIII and haemophilia B a break at Factor IX. These are X-linked recessive genetic disorders where the gene that encodes the proteins are found in the X chromosome. Males have one X chromosome and therefore are more susceptible to the disease. If the gene is present, there isn't another X chromosome to compete with it, as there is in females, and the likelihood is a haemophilia phenotype.

After the first year of life when children start to walk, bump into things and fall over, we start seeing the problems associated with haemophilia. Spontaneous haemarthroses are painful and inflamed and so the range of movement and function are greatly reduced. The disease can be debilitating and so young Leopold grew up with the threat of bleeding to death from the slightest injury hanging over him. In the winter of 1884, the Duke was suffering with painful joints and his doctor suggested that an environment more conducive to healing might be of help. England is cold and wet (he wasn't wrong) so the Duke should get himself to their house in Cannes in France where a spring warmth might do him good. It was there, away from his pregnant wife who he left at home in cold wet England, that he fell. This was a simple accident from which most would recover easily.

Queen Victoria passed on the trait to three of her nine children. As her children went far and wide across Europe, making important strategic marriages, they took haemophilia genes with them. Victoria was known as the grandmother of Europe because her family married into royal houses across the continent. She had a grandson, Friedrich, who also died of blood loss when he was only two years old. Another grandson, another Leopold, died at thirty-two, and Maurice at twenty-three. Leopold's daughter Alice inherited the gene and her son Rupert was born with it.
In 1904 The Tsarevich of Russia, Alexei Nikolaevich, inherited the gene from his mother Alexandra, granddaughter of Queen Victoria. When the boy was born, the umbilical cord bled and bled. It was alarming for his parents to witness. It was clear that the boy had the bleeding disease that ran in his family. As he grew he suffered from bruising, bleeding and joint pain but it was all kept quiet from the public. As ever, keeping secrets led to rumours that filled in the gaps. Alexei was murdered by the Bolsheviks in 1918 when he was just thirteen years old. The disease did not stop with the teenage Alexei.

Elsewhere in Europe, King Alfonso XIII of Spain married another granddaughter of Queen Victoria, Victoria Eugenie Battenburg of England. They had boys who carried the gene and it was not until they had a fourth son, Juan, that they had an unaffected heir. The law in Spain said that only a son without physical compromise could become the monarch. Can't think why they came up with that idea... were looking at you, Habsburgs.

Public opinion turned on Victoria Eugenie, blaming her for bringing bad blood to the throne, and the monarchy struggled to keep the confidence of the people.
With all families that pass on the genes that lead to haemo-philia there can be tragic consequences. With the royal families there could also be a profound political impact. That Alexei's parents spent considerable effort looking after the Tsarevich made them take their eye off the political intrigue that ended all their lives. How would Russia and the boy have fared had he lived and become Tsar?

It was a perilous existence with a condition hanging over him that meant a straightforward injury could result in death, just like it did for his uncle, Prince Leopold, Duke of Albany.

Source ~ ‘Vital Organs The History of The World’s Most Famous Body Parts ‘ book by Dr Suzie Edge