Royal Disease

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.

Why Harry Doesn’t Have Hemophilia

The randy royal Harry has been usurping the news lately, after his infamous interview with Megan on Oprah. But the royal who truly deserves some attention is Prince Phillip, Queen Elizabeth II’s spouse, who died last week at age 99. Hemophilia is known as the “royal disease,” primarily due to Phillip’s and Elizabeth’s joint family tree: Phillip’s great-great-grandmother was Queen Victoria, a known carrier of factor IX deficiency, also called hemophilia B. Let’s look at his family tree, to answer the question, why doesn’t Harry (or William, or Charles, or Phillip) have hemophilia?

Prince Phillip

Prince Phillip was born a prince (unlike Diana, Camilla, Kate and Megan, 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). These are very good odds!

Alice married Louis IV, the Grand Duke of Hesse. Alice introduced hemophilia into the House of Hesse and this German lineage. There were 7 Hesse children, and like their grandmum, 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 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.

Queen Elizabeth? Not so much drama with hemophilia. Why? She is not a carrier of hemophilia. She is a direct descendant of King Edward VII, a son of Queen Victoria who did not have hemophilia. Now, Queen Elizabeth and Prince Phillip are related as third cousins. But since Phillip 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), and since Queen Elizabeth had no hemophilia in her direct line, neither Charles nor his sons, William and Harry, have hemophilia.

Harry’s got other problems, but hemophilia isn’t one of them, thankfully!

The Royal Disease

Hemophilia Myth #1: The Royal Disease

Queen Victoria: the most famous
carrier of hemophilia

Perhaps because hemophilia is so rare, it has generated many myths. Myths are stories, sometimes created by people in an attempt to make something understandable when scientific information is unavailable. Remember the Greek tale of Pandora’s Box? Pandora was the first woman on earth. She was given a wedding gift from the gods, a beautiful container. But she was warned never to open it. Driven by curiosity, she disobeyed the gods and opened it. Inside were evils— hate, disease, pain—that flew out of the container and escaped into the world. This story was invented by the ancient Greeks to explain sickness and suffering, because they didn’t know about bacteria and viruses. Some myths develop from a nugget of fact or experience, but then take on a life of their own within certain cultural settings.

Becoming familiar with the most widely held myths about hemophilia and their sources will help you explain the facts of the disorder to others. During this month, which is Hemophilia Awareness Month, we’ll discuss some common myths about hemophilia.

Myth #1! Hemophilia is a royal disease.

The Truth? Anyone can get hemophilia—rich or poor, famous or unknown. Hemophilia was dubbed the royal disease because in the 1800s, hemophilia affected the family of Queen Victoria of England, who was a carrier of the hemophilia gene. Hemophilia was transmitted to three other royal families when Victoria’s daughters and granddaughters, also carriers, married into the Russian, German, and Spanish royal families. Though it’s no longer known to be present in any European royal family, hemophilia is still often associated with royalty.

Question 1: What type of hemophilia did the English royal family have, A or B?

Question 2: Why does the present royal family no longer have hemophilia?

Order a copy of Alexis: The Prince Who Had Hemophilia to learn more!

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