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.
It’s Bleeding Disorder Awareness Month! True: Designated in 1986 as Hemphilia Awareness Month this is the month to educate the public—and ourselves—about bleeding disorders.
I usually start with what hemophilia is not. 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. Here are some common myths about hemophilia.
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.
Myth: A small cut will cause blood to rush out and a child to bleed to death.
Truth: People with hemophilia do not bleed faster than anyone else. But they will bleed longer because their blood doesn’t clot properly. Still, not every cut will continue to bleed just because a person has hemophilia. Some cuts, especially small ones, stop bleeding on their own.
Myth: Children with hemophilia cannot play sports.
Truth: Children with hemophilia enjoy a wide variety of sports, including swimming, baseball, tennis, running, and martial arts. You’ll realize this someday when you’re chauffeuring your sports fan to various afterschool activities! High-impact contact sports such as football, hockey, and boxing are not advised. Physical activity is always encouraged for children with hemophilia.
Myth: Children with hemophilia must wear helmets and protective gear.
Truth: Although some parents place protective helmets or knee pads on their toddlers, it’s uncommon for a child with hemophilia to wear protective gear for normal activities. Medical treatments today are excellent and easy, offering a normal lifestyle. Of course, all children must wear protective gear when participating in risky activities: helmets should always be worn when riding bikes or motorcycles, ores, or when skiing or snowboarding. Oh yes, your child will be able to do these things one day!
Myth: Children with hemophilia must attend a special needs school.
Truth: Except for having a defective blood protein for blood clotting, our children have normal health and intelligence. They can be enrolled in any school. Overall, your child is normal and will be treated as such by the school system and the public.
Myth: Children with hemophilia need to be placed in special schools or classes.
Truth: Except for having a defective blood protein for blood clotting, our children have normal health and intelligence. They can be enrolled in any school. Overall, your child is normal and will be treated as such by the school system and the public.
Myth: Children with hemophilia will grow out of it.
Truth: Hemophilia is a lifelong condition. Your child does not have a disease that will get better or go into remission. Your child will not grow out of hemophilia: the mechanism for producing clotting factor is defective. Hemophilia is part of his genetic makeup, just like traits for hair or eye color, which can’t be outgrown. However, there is now a genetic therapy available for hemophilia B that will give, with one shot, protection against uncontrollable bleeds for a long time.
Myth: Hemophilia is caused by something you did during pregnancy.
Truth: Nothing you did while pregnant gave your baby hemophilia. Women who have perfect pregnancies and follow every safety precaution can give birth to children with hemophilia. There’s nothing you could have done to prevent hemophilia; nor could you have changed your child’s hair or eye color. Hemophilia is simply part of his genetic makeup.
Don’t feel guilty; get educated, and educate others. Now’s the month especially to do this!
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.
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!
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?
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