Barron, Stephanie
The Flaw in the Blood
Bantam Dell, 2008, 289 pp.
ISBN 978-0-553-80524-6
A couple of weeks ago, I was in the local library, browsing around for something worth reading, while waiting for another book(which I'll review tomorrow). I came away with A Flaw in the Blood. Though I hadn't paid much attention, I noticed that she was a mystery writer who wrote a series of "period" mysteries featuring Jane Austen. For the record, I tend to stay away from mysteries centering around real people solving fictional mysteries. To me, there's something kind of "off-putting" about authors who do this kind of thing, that is, having someone like Elizabeth I(yes, I've seen mystery series with her as the detective; I have stayed away from them, too) solve a mystery. I think you would have to be a pretty diehard mystery fan to enjoy these.
This particular mystery doesn't really have Queen Victoria solving any mysterie; even Stephanie Barron apparently knows she couldn't quite pull this off. It just wouldn't have been in character for Victoria, from what I know of her. But she does(sort of) attempt to introduce what we understand as science into the story, as part of the solution.
It's pretty well known that Queen Victoria her husband Prince Albert, had a lot of children. The youngest son, Leopold, lived to grow up, but died fairly young. due, apparently, to a cerebral hemorrhage. He apparently inherited hemophilia from her. And she seems to have passed hemophilia on to two of her daughters. Unfortunately, they passed hemophilia on to some of their children, with disastrous results. The best known result of this genetic flaw was Tsarevich Alexei, of Russia(the child of the unfortunate Nicholas and Alexandra, the latter of whom was Victoria's granddaughter). Of course the Bolsheviks probably weren't fully aware of the genetic end of things when all of them were shot, but that's another story.
Which brings me to the problem I ended up having with The Flaw in the Blood. It worked reasonably well as a mystery, until the very end. The blurbs on the back of the book say Ms. Barron is good at historical detail, and they're right: she got the style in which Victoria wrote pretty much right, and the relationships between various "classes", and their various conditions in England, also right, as well as the difficulties women had breaking into any profession(one of the main characters is a rare woman doctor).
Unfortunately, Ms. Barron should also have reread something on basic biology and genetics. If she knew that Victoria passed the "hemophilia gene" to three of her children, and some grandchildren and great-grandchildren as well, she should have known that a man can't pass hemophilia directly to any sons he might have. Because Ms. Barron made the assumption(a) that Victoria herself was the product of her mother, but not the man her mother married(he died before they could produce any more children, which is how Victoria ended up being Queen Victoria). And she implied that the lover passed hemophilia on, because the mystery is "solved" when they find a hemophiliac descendant of the same man.
Unfortunately, genetically speaking, this is impossible. The presence of hemophilia in Victoria was probably something she mutated and passed on, or else her mother mutated it and passed it on to her. Because the gene itself exists on the X chromosome(it's sex linked). This is so well-known that every beginning biology class I've ever heard of --- even in high school --- mentions this --- and often mentions poor Victoria, as well. And unfortunately a man can't passit on himself, because he only has one X chromosome. Women don't get it, because even if they have it passed down to them, they are chromosomally XX. Since it acts as a "dominant", any man who inherits his mother's X chromosome, will have hemophilia, and most hemophiliac men probably won't reproduce. At least they didn't in the past; most of them died too young. So if a man is reproducing chldren, unless something very strange is going on, he won't be passing hemophilia to any of his children. In this case, the woman really is "to blam", though it's really not under anyone's control, even these days.
And that, dear readers, spoiled the book for me. If you can't get the basic science right, either don't write a book with that science in it, and for heaven's sake, don't write something so wrong that even a complete nongeneticist can spot it. And kindly don't ask anyone to read it.
Anne G
Redheaded Neanderlady
This is a photoshopped version of something I found in National Geographic about the time I started researching
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4 comments:
Was the haemophiliac male descendant of the supposed haemophiliac lover a son (impossible, as you say, unless his mother was also a haemophilia carrier as well and he got it from her), or a grandson (possible if rather unlikely in the period)? A man with haemophilia can pass the faulty gene on to his daughters, but not to his sons. A daughter gets one X chromosome from her mother and one from her father, so if her father has haemophilia she will get his faulty X chromosome plus one of her mother's X chromosomes. Assuming the mother is healthy (two normal X chromosomes), the daughter will be a carrier of haemophilia, and could pass the haemophilia gene on to her children if she has any. The son of a man with haemophilia will get the father's Y-chromosome, which has nothing to do with haemophilia, and one of his mother's X chromosomes. Assuming the mother has two normal X chromosomes, the son will be healthy and has no haemophilia gene to pass on to any of his children.
Median life expectancy for people with severe haemophilia was 11 years during the period between 1830 and 1920 (data from Sweden, but probably applies to most of Europe), so many men with the disease wouldn't have lived long enough to reproduce. (I assume that Victoria's haemophilia gene was the severe kind, given how seriously ill little Alexei seems to have been. Milder forms of haemophilia had longer life expectancy). But by definition half of them would have lived longer than the median, so it's theoretically possible that a man with severe haemophilia could have survived to adulthood and reproduced. Though rather unlikely, and even less likely that he could have been the secret lover of a royal lady!
Unfortunately the likely explanation for Victoria's haemophilia is the mundane one of a spontaneous mutation, either in her or in her father's sperm cells. Something like a third of haemophilia cases arise from a spontaneous mutation like this.
Carla:
The problem with the story was that the lover died in some stupid accident, after Victoria and the other child had been produced(and there was a widow who "told all"). It was implied that the lover had hemophilia and that was what killed him. Then there's this grandson who also has hemophilia. So that "clinches" everything. They had a daughter, and presumably the lover passed it to her, by which means the grandson had hemophilia. But that isn't the "usual" means, as I said, although I suppose it oculd have been possible, if it was a fairly mild form(I know there are several types). The trouble is, the author is implying that all these kids got their genes for hemophilia through the male line. I'm no geneticist, but that's not my understanding of the way hemophilia is paased.
Anne G
There's a good explanation of the genetics of haemophilia on the Canadian Haemophilia Society website here.
Carla:
Thanks for the link. I looked at it, and it pretty much covered the basics as I understand them(bear in mind, I'm not a geneticist; far from it). It did add stuff about genetic testing of women in "hemophiliac" families, but then, genetic testing essentially didn't exist when I was studying biology in high school and college.
Anne G
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