Hemophilia: A Royal Disease

By Kimberly Shen
Edited by Arianna Winchester
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On August 12, 1904, three hundred rounds of cannons were set off to announce the birth of the first son born to the current royal family in St. Petersburg, Russia. This birth was particularly special for both the Russian royal family and Russian people since they finally had a new heir to the throne. Russian law dictated that only a male could inherit the throne, so Alexei Nikolaevich became the only child who could rule over Russia after his father, Nicholas II, died.

However, the joy that Nicholas II and his wife experienced soon turned into horror when they found out that their only son was born with Hemophilia B. Those born with hemophilia lack clotting factors in their blood, so even a small injury can lead to severe bleeding or death. Hemophilia B is a recessive sex-linked disorder that is more frequent s in males because the gene for this disorder is only found on the X chromosome. Males only have one X chromosome, so to be diseased a boy would need to inherit only one mutated X chromosome with the gene for hemophilia from their mother. Hemophilia B is much less common in females because a girl would have to inherit two mutated X-chromosomes to be diseased. The disease is recessive, so if a girl is born with one mutated X chromosome and one normal X, she will only use her healthy X and therefore will not demonstrate symptoms of hemophilia. Women who have one healthy X and one mutated X are referred to as “carriers” because they have the capability to pass on the disease to offspring.

Alexei’s condition was a constant source of anguish for his parents, especially his mother, Alexandra, who felt extremely guilty for passing on the condition to her only son. Alexandra’s grandmother, Queen Victoria of England, was also a carrier who passed the trait on to a few of her children. Several other members of Alexandra’s family died due to complications from this disease. Knowing that even the smallest injury could potentially lead to death for Alexei, both Nicholas and Alexandra became painfully aware that Russia’s future was uncertain. Nicholas and Alexandra chose to keep his condition a secret from most of their subjects.

When he was three years old, Alexei suffered an injury that left him on the brink of death. In desperation, his mother asked a close confidante to secure the help of a mystic named Grigori Rasputin. What followed seemed to be nothing short of a miracle. At a time when even the best doctors in Russia were unable to Alexei’s condition, Rasputin was able to make his bleeding stop. Even up now, the nature of Rasputin’s healing methods remain a mystery. Some historians believe that Rasputin hypnotized Alexei. Others believe that Rasputin stopped administering anti-clotting drugs that may have been aggravating Alexei’s condition.

Regardless, Alexandra saw Rasputin as a holy savior for her son. As a result, Rasputin was given legal privileges and easy access to the royal family. He even advised the Emperor and Empress on political affairs and filled government posts with his own supporters. This often led to disastrous political results, but the Empress remained blind to Rasputin’s faults since she saw him as the only person who could save her son.

Ultimately, Rasputin’s influence over the royal family helped lead to their downfall. Because very few people knew of Alexei’s condition, most people could not understand why a commoner like Rasputin had such influence over the royal family. Rasputin’s power in court angered nobles and commoners alike.

Rasputin was ultimately assassinated at the hands of a group of Russian nobles, two of whom were relatives of Nicholas II. Less than a month before his death in 1916, Rasputin wrote a last letter to Nicholas II. In this letter, Rasputin seemed to foresee his own death, writing:

“If I am murdered by boyars, nobles, and if they shed my blood, their hands will remain  soiled with  my  blood… Tsar of the land of Russia, if you hear the sound of the bell which will tell you that Grigori  has been killed, you must know this: if it was your relations who have  wrought my  death then no one of your family, that is to say, none of your children or relations will remain  alive  for  more  than two  years.  They will be killed by the Russian people.”

Eerily enough, Rasputin’s predictions came true. A little over a year later, Nicholas and his family were imprisoned in Yekaterinburg and executed by Russian revolutionaries. For many years, it was rumored that thirteen-year old Alexei may have survived the execution. Still, many people considered his survival highly unlikely due to his medical problems. The truth was finally revealed in 2007, when human remains were discovered in a forest in Yekaterinburg. After extensive DNA testing, it was ultimately confirmed that they were the remains of Alexei, the last heir to the throne of Russia.