Hemophilia is a disease in which the afflicted person has an impairment of his blood-clotting ability, so that he doesn't stop bleeding rapidly like the rest of us do. Sometimes he doesn't stop bleeding at all, and he dies. Often, this uncontrolled bleeding occurs in the person's joints, such as a knee if he falls down on it, but it can occur at whatever site the injury is located. Often, when the joint or other area finally fills up with blood and pressurizes, the bleeding will stop. This may take days of agony (if a joint) and is a living nightmare for the afflicted one. Of course, I am talking about the past - today we have coagulants that can be administered.
Women don't get hemophilia*, only males. Women, however, are the carriers of the recessive X-chromosome, which they can pass along to some of their sons. The daughters of hemophiliacs (should those hemophiliacs survive long enough to reproduce) may or may not become carriers. It is a genetic thing and therefore dependent upon the genetic odds.
Hemophilia has been called the disease of royals, due to its stubborn propensity to affect people of royal blood. This is not, I hasten to add, because they have different blood than commoners, but, rather, due to the fact that royals tend to intermarry with close relatives such as first cousins, and this makes their chances of passing on a defective (recessive) chromosome more likely. And, of course, the defect does not have to be hemophilia, but that is the one this post is about.
It would be a bit difficult to trace the incidence of hemophilia in ALL the royal houses of Europe, but since this series of posts are about Queen Victoria and her descendants, there are plenty enough incidents right there to keep us busy in this post.
*[I want to pause here and make a note, just to be technically correct. In reality it is possible for a female to have hemophilia, though ever so rare. A woman has two X chromosomes and a man has one X (female) and one Y (male). When the woman is a carrier, one of her X chromosomes is recessive but the other X chromosome compensates for her own blood clotting. So you can see that she would have to have both of her X chromosomes recessive in order for her to actually be a hemophiliac herself. Not bloody likely, if you'll forgive the unfortunate pun. And, with both chromosomes recessive, hemophilia would probably be the least of her problems. Of course, she would not have survived puberty in the old days anyway.]
In a link at the bottom of this post, I will put up a chart of Victoria's descendants which will show more clearly how the disease was spread throughout her family. When the first case of the disease showed up (in her son Leopold), the queen was shocked and was said to have protested, "This disease is simply not in our family!" Obviously it was.
Here are the cases of hemophilia in the three generations after Queen Victoria:
(Queen Victoria was a carrier)
1. Queen Victoria's daughter Alice was a carrier
2. Queen Victoria's son Leopold was a hemophiliac
3. Queen Victoria's daughter Beatrice was a carrier
4. Alice's daughter Irene was a carrier
5. Alice's son Friedrick was a hemophiliac
6. Alice's daughter Alix was a carrier
7. Leopold's daughter Alice was a carrier
8. Beatrice's daughter Eugenie was a carrier
9. Beatrice's son Leopold (Lord Mountbatten) was a hemophiliac
10. Beatrice's son Maurice was a hemophiliac
11. Irene's son Waldemar was a hemophiliac
12.Irene's son Henry was a hemophiliac
13. Alix's son Alexei was a hemophiliac
14. Alice's (daughter of Leopold) son Rupert was a hemophiliac
15. Eugenie's son Alfonso was a hemophiliac
16. Eugenie's son Gonzalo was a hemophiliac
This is fascinating. One of the arguments against Victoria having been illegitimate was that a haemophiliac would have been very ill and would have been unlikely to have an affair. Yet Leopold had two children in spite of it. All the same, I've read the recorded incidence of spontaneous mutation is 33% and that's quite a high percentage if it's true.
ReplyDeleteThere are female hemophiliacs, though exceedingly rare. And the coagulants, although making longer lives possible for hemophiliacs, aren't a cure-all. Those same factors, before we recognized the AIDS threat, endangered many hemophiliacs. I once saw a female hemophiliac on television who explained that she and her son had been infected via clotting agents (many of which weren't removed from stores even when the blood banks began screening). Her son and husband both died of AIDS.
ReplyDeleteThe daughter of a hemophiliac man is ALWAYS a carrier (or a hemophiliac herself if her mother's a carrier). Always. Men cannot, however, pass hemophilia on to their sons. Ironic, no?
By the way, "Beatrice's daughter Leopold" - you meant son, right?
It's a fascinating subject. My best friend in college, her brother was a hemophiliac and it was a real concern for her, being Catholic, wondering if she was a carrier. Fortunately, they can apparently test for that now and she wasn't. Even today, a hemophiliac's life is difficult.
What a sad chart! It's lucky indeed that only three of Victoria's children got that gene, given that, statistically speaking it should have been half. It was very sad to me, but many genetic disorders are sad.
ReplyDeleteYes, "son". I changed it. Thank you, S.
ReplyDeleteA sad disease even today.
@A. - Spontaneous mutation is what we are left with in Queen Victoria, unless you want to believe all of Eruope's royalty (sort of) stem from illegitimacy. Which, oddly, I find humorous. Sorry.
ReplyDelete