This month of March, Hemophilia Awareness Month, we’re exploring myths about hemophilia. Myths are stories, sometimes created by people in an attempt to make something understandable when scientific information is unavailable. The ancient Greeks had many myths that we still reference. Remember the myth of the demigod warrior Achilles, son of Theta, a sea goddess, and Peleus, a mortal? His mother dipped the infant in the river Styx, which was said to have protective powers, so that Achilles could be kept invulnerable in battle. But his mother held the infant by his heel, which did not get wet, and so his heel was his weak spot. Today, we say that someone with a weakness has an “Achilles heel.”
Myth: Children with hemophilia will grow out of it.
This is a myth. Someone who says this to you does not understand the science behind hemophilia.
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.
But someday–maybe someday soon–we will have gene therapy, and your child may be cured.
Myths evolved in history when people lacked scientific information to explain natural phenomenon. During March, Hemophilia Awareness Month, we’re looking at hemophilia myths!
Last week, Myth #1: The Royal Disease. Here’s Myth #2. A small cut will cause blood to rush out, and the child will 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.
Do you know the three steps in coagulation? 1) Vasconstriction 2) Platelet plug 3) Fibrin net.
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?
What does a typical family in the bleeding disorder community look like? I don’t know. There’s so much variability in family composition that the range of normal seems boundless.
Novelists may base their fictional characters on people they know, or use their imagination to create characters. Some novelists want to make a bleeding disorder an important part of their novel’s plot, yet choose another family member as the leading character. I’m curious about these protagonists—wife, mother, sister of someone with a bleeding disorder—in the following novels.
A Place for Kathy
Henry Denker William Morrow, 1997
Tessa Taylor, 40, has a type-A personality and runs her own organic makeup company. She is married with three kids. Living on the Florida panhandle coast, Tessa hunkers down alone for a Category 4 hurricane, with her family elsewhere. Curious, she snoops on her architect husband Ethan’s iPad and discovers his affair with Lindsey, a 26-year-old nursing student. Tessa tricks Lindsey into coming to her home, even though she has no power and no gas for the generator. The two women fight, verbally and physically, over Ethan—only to discover that he has additional mistresses. During the storm, and in the middle of this fight, Tessa’s eldest child, Colt, who is 18 years old, 6’6″ tall, and 200 pounds, returns from Florida State University because he runs out of meds for his hemophilia. Colt knows that his mother always keeps some in the refrigerator. Colt arrives bleeding profusely from his leg. Both women stop their fighting to care for Colt by hanging blood-clotting factor bags on an IV pole, which I found to be a puzzling way to treat hemophilia. Fortunately, mothers always seem to be prepared, regardless of the age of their child with a bleeding disorder.
Courtney Cole. Gallery Books, 2019
This melodramatic novel is sure to produce misty eyes, if you overlook some of the contrived storyline. In 1994, Grace Cameron becomes a widow when her husband, Kip, dies unexpectedly from a brain hemorrhage caused by a fall while hiking. Kip taught literature at a midwestern university, but had no life insurance because of his pre-existing condition, hemophilia. Grace learned of Kip’s hemophilia when they first dated, and she knew that he had tested negative for HIV. So in 1996, she is shocked to learn that she is HIV positive. As her medical symptoms worsen over 18 months, despite numerous antiviral drugs (AZT, DDC, DDI), Grace is overwhelmed by the prospect of dying. She is desperate to find a home for her 13-year-old daughter, Kathy. For Grace, few people meet her strict criteria as caregivers for Kathy. As I read the book, I wondered why Grace never hired a lawyer to plan her daughter’s guardianship, and never joined an AIDS support group. Or why Kip never attended an HTC. Still, spouses of HIV-infected people with hemophilia need more recognition for their important caregiving roles.
The Key to Finding Jack
Ewa Jozeflowicz. Zepher, 2020
Felicity Chesterford, who goes by “Flick,” is enrolled in secondary school in London. She assists her older brother, Jack, in his quest to solve puzzles. Jack finishes his A-level exams in June and plans to spend a gap year traveling in South America. Their father, a barrister, pressures Jack to become a lawyer. Their mother, who runs a social media company, is overprotective of Jack because he has hemophilia. Jack is well prepared with a special medical kit and a doctor’s letter for his hemophilia. Unexpectedly, in January an earthquake hits Lima, Peru, where Jack is headed. Communication is impossible. Flick and her best friend, Kiera, decide to act like detectives to uncover clues that will locate her missing brother, who might be in a medical emergency. Flick is successful, while the foreign office and local police are not, in finding Jack. In this novel for young readers, the plot emphasizes finding clues to solve puzzles, while the often neglected role of sibling bonding, when bleeding disorders are involved, receives recognition.
As seen in these novels, family members without bleeding disorders are just as important as the ones with bleeding disorders. The novelists realize that, while the bleeding disorder provides the drama, other family members can play a leading role. All this creates a dynamic fictional situation, one that reflects reality.