The Royal Disease and Russia

Both the Royals and the Russians have been making the news this past year, not much of it good. For the Royals, it’s mostly just the Markles (on their “Privacy Tour”). For Russia…. well, let’s not go there.

While Russia is making a ruinous name for itself these past two years, it’s famous for its hemophilia history. Which originated from the English Royals.

We noted last week that it’s Bleeding Disorder Awareness Month, and we shared some popular myths about hemophilia. One was that hemophilia has been dubbed “The Royal Disease.” I shared in detail how this happened, and who it affected in my blog here.

But the most famous outcome of a genetic link in the English Royal family happened when Princess Alix, whose grandmother was Queen Victoria, a carrier of hemophilia B, married Tsar Nicholas II of Russia. Queen Victoria had nine children, of whom two were carriers (Alice and Beatrice) and one had hemophilia (Leopold). It was well known that hemophilia was now running in the family.

Alix, nicknamed “Sunny” by Nicolas, gave birth to Alexis (or Alexei), after already having four girls. They got their heir to the throne. But Alexis had hemophilia.

Alexis had no access to clotting factor of course; this was 1904, after all. The royal family came rely on a person of ill repute: Rasputin, the mad monk. He had a lascivious reputation but also a track record of helping people in pain, probably through hypnosis. Rasputin became ingratiated into the royal family and helped also to bring down the Russian monarchy. It’s been proposed that Nicholas II was so distracted by his son’s suffering due to hemophilia, that eventually he lost his grip on the monarchy at a time when the Bolshevik Revolution was poised to strike. And it did. It has been proposed that hemophilia changed the course of World War I, and changed the course of history. The Cold War, the Soviet Empire… all find their roots in the royal palace of the Tsar and a little boy with hemophilia.

Order Alexis: the Prince Who Had Hemophilia here for your child with hemophilia.

The Queen and Hemophilia

Queen Elizabeth II

The Queen is dead; long live the King! This phrase is being used around the world as England mourns the death of Queen Elizabeth II. Her eldest son Charles, once married to Diana, has been pronounced King Charles III.

Queen Elizabeth II’s death renewed my curiosity about why the current royals—Charles (now Charles III) and Diana, Princess Anne, Andrew and Edward—and all their assorted children and grandchildren, don’t have hemophilia? After all, both Elizabeth II and Prince Phillip, her husband, were both direct descendants of the most famous carrier of hemophilia in the world—Queen Victoria. They are third cousins.

Hemophilia became the “royal disease” with Queen Victoria. She was, until Queen Elizabeth II, who just passed away at age 96, surpassed her, the longest ruling monarch in history, at 63 years and 216 days. Queen Victoria, a known carrier of factor IX deficiency, also called hemophilia B, was Prince Phillip’s great-great-grandmother.

The Paternal Lineage

Prince Leopold

Prince Phillip was born a prince (unlike Diana, Camilla, Kate and Meghan, who married into the family). His mother, Princess Alice, was a great-granddaughter of Queen Victoria. Phillip was descended from the third child of Queen Victoria, also called Alice, who like her mother, was a carrier for factor IX deficiency, or hemophilia B. In fact, Queen Victoria had nine children, of whom two were carriers (Alice and Beatrice) and one had hemophilia (Leopold).

Alice married Louis IV, the Grand Duke of Hesse. Alice introduced hemophilia into the House of Hesse and this German lineage. There were seven Hesse children: one had hemophilia (Frederick) and two were carriers (Victoria, Phillip’s grandmother, and Alix). Alix married the Tsar of Russia, Nicholas II, and gave birth to Alexei (Alexis), who had hemophilia. So, Prince Phillip has a long and illustrious side to his family tree regarding hemophilia! It’s been proposed that Nicholas II was so distracted by his son’s suffering due to hemophilia, that eventually he lost his grip on the monarchy at a time when the Bolshevik Revolution was poised to strike. And it did strike.

Leopold, the only one of Queen Victoria’s nine children to have hemophilia, passed the carrier gene to his daughter Alice. Descendants of Victoria would married into the royal families of Russia, Spain, and Germany, and have children with hemophilia. And that’s where you get the moniker “Royal Disease.”

The Maternal Lineage

Queen Elizabeth’s side does not carry hemophilia. She is a direct descendant of King Edward VII, a son of Queen Victoria who did not have hemophilia. Phillip is her great-great-grandson but did not inherit hemophilia from his mother (not a carrier) or grandmother (also not a carrier but with a flip of the genetic coin might have been).

King Charles III, who does not have “The Royal Disease.”

Since Queen Elizabeth II had no hemophilia in her direct line, neither Charles nor his sons, William and Harry, have hemophilia or were ever at risk of inheriting it. We are happy none of them have hemophilia, but even if they did, with the excellent treatments we have today, it would not interfere much with their royal duties.

There as a time when I knew very little about the royals. I do recall in the 1980s playing the game Trivial Pursuit with friends, and pulling up the card that asked, “What disease is called the royal disease?” I knew the answer, but couldn’t recall why; did I read about Russian history? Or medical history? I don’t know; I just knew it. What I didn’t know is that a few years later I would give birth to a baby with hemophilia.

Our condolences to England for the loss of their lovely queen; and all best wishes to Charles as King Charles III.

Anniversary of Tsar’s Death

Today, 89 years ago, Tsar Nicholas II of Russia and his entire family, including Alexis, the prince who had hemophilia, were assassinated in Siberia. This event shocked Russia and the world, and the elimination of the Tsar and the monarchy fueled a bloody civil war in which tens of millions of Russians died. The deaths of the Tsar, Empress Alexandra and their five young children have been the subject of many books and movies. One recent book is “The Kitchen Boy,” by Robert Alexander, which I just happened to read last night, not even aware that I was reading it on the eve of the anniversary.

The book is fiction, but draws on certain facts, and is narrated by the “kitchen boy” who served at Ipatiev House, where the royal family was imprisoned. As a servant, he had access to watch all that happened at the house, including the family’s death. He becomes close to the royals, and details their personalities, flaws, loving manner toward one another, and even their brutal deaths. The “kitchen boy,” now 94, lives in Illinois and wants to share some details he has never revealed before to his granddaughter. He records his memoirs, which becomes the narration of past events in the book. He eventually reveals who killed the Tsar and his family, and what became of the two missing bodies, believed to be Alexis and Anastasia (about whom there are also many movies and books). He reveals that actually Alexis and Maria were missing, and he tells how and why, and how that impacts his life, even now.

The book is easy to read–I read it in one sitting–and the language and style is a bit too easy. It reminded me of the kind of book we had to read for junior high over the summer. Interesting but light. You can almost believe the conversations and events took place, and I think that’s the best part of the book–the way Alexander makes the royal family come to life. But the book loses credibility towards the end. It tries to read like a da Vinci code a la Dan Brown, but it’s much too simplistic a plot for that. The plot takes a sudden radical twist at the end, which kind of left me head-scratching. I just didn’t get it.

Part of the ending included reference to someone with hemophilia (no spoilers) who had mild hemophilia, apparently had no manifestations of hemophilia his whole life, then got into a car accident, hit his head on the steering wheel and died at the scene. I am not a doctor, but this just didn’t sound accurate to me. There were plenty of accurate references to Alexis’s hemophilia, and how the poor boy suffered.

All in all, I would recommend this as a read, but don’t expect a great historical book or a great mystery. It’s light, not perfect, but a great summer beach book that can be read fast, and you may come away with a new appreciation of the Russian royal family. Hemophilia is a pervasive theme throughout the book, and plays a part in solving the mystery of what happened to the royals, and the two missing bodies. (Two stars out of four)

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