I’ve been reading And the Band Played On (a few decades late, I know) and ran into the statistic that some 90% of American hemophiliacs who received transfusions or blood products in the early 80’s (which was almost all of them) got AIDS and, one assumes given the time period, died.
Now I realize that hemophilia is a recessive sex-linked thing and therefore that there are more people who are carriers for the gene than there are who actually express it, in the same way that since my dad is colorblind I have a chance of passing it on to any children I might have even though I’m not colorblind. I also realize that the book is decades old, research-wise, but that doesn’t seem like the sort of thing that would be seen in a new light these days.
So did the untimely death of all those poor people (who albeit had a shorter lifespan anyway because of their disorder) decrease the prevalence of the trait at all?
Just because AIDS treatment was in its infancy in the 80s doesn’t mean that most people who contracted HIV in the 80s died. Remember, AIDS can have a decades-long incubation period, which means that many of them lived long enough for effective medications to become available.
It seems reasonable to suppose that some hemophiliac genes were eliminated from the population as a result of HIV/AIDS, but you’d really need to quantify it to see what impact it had. Specifically, you’d need to know how many people in the US had hemophilia at the time, how many of them died of AIDS, how many died of other hemophilia-related causes, and how many kids all of them had. Just off the top of my head. That would let you figure out a rough selection coefficient - how much pressure HIV added to the natural selection against the hemophilia allele.
Remember that hemophilia A and B are X linked recessive genes. That means the women out there carrying one copy of the gene but who are not hemophiliacs were not affected in any way by the AIDS epidemic. Of course, I have no idea how many hemophiliacs reproduce.
Men with the disorder rarely lived long enough to do so until Factor VIII came along, so they couldn’t pass the gene on to their daughters.
I’ve seen stories about hemophilia camps from the 1970s with pictures of several dozen boys, and the commentator said things like, “Within 10 years, all but 4 of these boys were dead from AIDS by 1990.”
BTW, hemophiliacs, being male with a lifelong chronic disease, also tend to engage in self-destructive behaviors like drug addiction and extreme sports.
It was (and by all accounts, still is) not uncommon for their parents to get so fed up with their behavior (the main thing being making themselves have bleeds for attention) that they simply take the child to the hospital and leave him there. :eek:
There’s a book called “Journey”, by Robert and Suzanne Massie, that was a big best-seller in the early 1970s and is a near-ubiquitous presence at used book sales. It’s the story of their hemophiliac son, and if you think people battle health insurance companies and “the system” now, things were even worse back then. I wondered what ever became of the son, and it turned out he’s an Episcopal priest who has an additional gene that makes him immune from AIDS even though he’s long been HIV positive. He does have hepatitis C, from all the blood transfusions he’s had over the years, and last time I checked was on a waiting list for a liver transplant.
ISTM the offspring of a hemophiliac male and normal female would be either a normal male or a carrier female, neither of which would themselves have problems (although the daughter would risk having children with the gene). So unlike Tay-Sachs or Huntigndons, there is not as great an incentive to refrain from having children or (today) get prenatal testing.
But doesn’t that depend on whether the female is also a carrier? (Which, admittedly, is more of a problem if you’re an inbred royal family, as it isn’t exactly a common mutation like color blindness.)
This reminds me of the only hemophiliac I’ve known (that I was aware of). It was back in high school, and everybody knew about his condition. And he was one of the biggest assholes I’ve ever met. I heard several other guys comment on how they’d love to punch him in the face, but they didn’t dare because it might kill him. It makes me wonder if growing up with everybody knowing to not dare hurt him, because it could kill him, led to a “license to offend”.
Once again, any woman whose father is a hemophiliac will be a carrier, no exceptions.
And it’s actually not true that hemophilia was caused by inbreeding. It just caused so much devastation because royalty usually married royalty. I’d go so far as to say that it was partly responsible for the fall of the Russian monarchy, if not THE cause.
I THINK there might be family left that still carries the gene, but it’s an obscure branch of the British royals, and they’re really not likely to inherit the throne any time soon.
(Just a little interesting fact – today is the anniversary of the day the Russian Imperial family were killed.)
Decrease it compared to what? For most of history – including well into the 20th century – it was rare for people with hemophilia to live long enough to hit puberty. This meant that hardly anyone inherited hemophilia from a parent who actually had hemophilia. It was nearly always inherited from a carrier mother or developed as the result of a genetic mutation.
If the information on Wikipedia is accurate, it wasn’t until the 1980s that advances in medicine allowed people with hemophilia to live well into adulthood. Unfortunately for them, this happened at about the same time as the AIDS crisis. So while AIDS related deaths presumably mean there are fewer people with hemophilia having children than if AIDS had never existed, it seems unlikely that AIDS has resulted in fewer people with hemophilia having children than had actually been the case previously.
No, she could have hemophilia herself rather than just being a carrier if her father has hemophilia AND her mother is a carrier.
Robert Massie became interested in the Russian royal family, which had a son with hemophilia, because of his own child’s condition. He wrote a biography of them called Nicholas and Alexandra, which is interesting.
