Hemophilia

H

Bleeding Disease Headline

Not the most original choice of topic, but it was the first thing that sprang to mind, particularly since there’s no letter H in Russian. And really, a lot of folks don’t seem to understand what exactly this disease is, and that people who have it aren’t 24/7 invalids, clumsy, bleeding all the time, and wilting lilies.

My Tsar Aleksey II, in my alternative history, defies expectations by surviving into adulthood with pretty decent health, and has four healthy children, two boys and two girls. In his real, too-short life, he constantly challenged the bounds of his disease, and was a very active boy doing his best to live a normal life. There are numerous photos of him doing things like standing on a cannon, going down a slide, balancing on a chair, riding a bicycle, and driving a car. He even had a toy Mercedes Benz which he loved driving. Many of his injuries came about precisely because he was so active, and refused to be defined by his disease.

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Hemophilia is inherited through the maternal line. The carrier has a normal X chromosome and a hemophiliac X chromosome. When a boy gets the wrong X chromosome, there’s no second X chromosome to cancel it out. When a hemophiliac has children with a non-carrier, all the girls will be automatic carriers, but the boys can’t have hemophilia themselves. However, just because a woman is a carrier doesn’t mean all her daughters will be automatic carriers, nor does it mean all her sons will have hemophilia.

Alexei_Nikolaievich_of_Russia_and_boatswain_Derevenko.jpeg

Attacks will often be more frequent and/or severe in childhood. Children don’t have the maturity and self-awareness to understand what it means to have a disease like this, nor how to avoid potential injuries. Aleksey had a lot of attacks as a boy, the most famous and severe suffered in 1912, but he got a lot stronger and healthier. He wasn’t clumsy and careless, but just trying to be a normal boy. He wasn’t some wilting lily staying inside reading and drawing all day.

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Until the 1960s, life expectancy was about 13 years, but there were always exceptions. A number of the hemophiliacs from Europe’s royal houses lived into adulthood; the longest-lived was Prince Waldemar of Prussia, who died at 56 in May 1945. A lot of people seem to have this false perception of the disease as constant, uncontrollable bleeding, from things as minor as a handshake or bumping an elbow. This disease is unpredictable. A child could appear to be fine after a nasty fall, only to develop a subcutaneous hemorrhage the next day, or slam his fingers in a door without incident.

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Sometimes an attack isn’t a cut that bleeds for days, but internal bleeding. A lot of Aleksey’s injuries were subcutaneous hemorrhages, bleeding under the skin that swelled up and took as long as months to be reabsorbed. The injury in 1912 was particularly life-threatening because it was a hemorrhage in the groin and stomach. One of his 1918 injuries was also a hemorrhage to the groin, caused by riding a sled down the staircase out of sheer boredom. These hemorrhages put pressure on his veins, nerves, capillaries, and joints. His left leg in particular was weakened after so many injuries.

Nicholas_II,_Alexandra_and_Alexei_during_the_tercentenary_in_Moscow

There were so many things Aleksey was forbidden to do, like play tennis, ride horses (all the photos of him on horseback were staged), climb trees, ride a bicycle, all sorts of fun things so-called normal children take for granted being able to do. His parents really seemed to have a contradictory attitude towards his disease, both being too overprotective and not vigilant enough. For example, one doctor was fired for telling him to wear calipers all the time, and another doctor was horrified to find Aleksey already out of bed and playing at least a week before his orders dictated.

In my alternative history, his uncle and Regent, Grand Duke Mikhail, institutes a lot of new rules, in the hopes this tough love strategy will enable him to regain somewhat normal health and live to adulthood. It does work, and he gets used to this quiet, interior life of the mind he’s been forced into. No more being carried in public well past babyhood or spending months in bed.

For more detailed information, please see my post on Writing a hemophiliac character.

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8 comments on “Hemophilia

  1. jazzfeathers says:

    This is something that doesn’t come up in stories very much. I remember it was a lot mor ecommon (at least in stories) when I was a child.

    @JazzFeathers
    The Old Shelter – Jazz Age Jazz

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  2. Tarkabarka says:

    Okay, so I want to read your book like, right now 🙂 It sounds like you turned him into a fascinating character 🙂

    @TarkabarkaHolgy from
    The Multicolored Diary
    MopDog

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  3. What a scary disease. I still don’t quite understand how the chromosome is passed. Excellent research.
    Mary at Play off the Page

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  4. Fascinating. And just because they have the disease doesn’t necessarily mean that’s what they died of, correct? It so reminds me of present-day cautions to folks like my dad who are on blood thinners or anticoagulants due to heart disease or risk of stroke. That’s the same warning speech we got from his doctors: avoid any activity that might cause a bleeding injury, especially internally or in the brain.

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  5. Nilanjana Bose says:

    Heredity is a wondrous and baffling thing. Both the actual character and the book character sound fascinating.

    Best wishes for the rest of the challenge,
    Nilanjana.
    Ninja Minion, A-Z 2016
    Madly-in-Verse

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  6. Akilah says:

    I learned a little about hemophilia in AP Bio when we discussed genetics but not this level of detail. The examples helped me understand it a lot more. Cool topic!

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  7. Arlee Bird says:

    I’m on coumadin and bleeding uncontrollably is something I’ve thought about. I don’t worry about it much, the idea has just crossed my mind now and then.

    Arlee Bird
    A to Z Challenge Co-host
    Tossing It Out

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  8. […] of God (19 views) The Grand Cathedral of the Winter Palace and the House of Gagarin (14 views) Hemophilia (19 views) Prince Igor Konstantinovich and the Iverskaya Chapel (20 views) The Jordan Staircase and […]

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