Tay-Sachs disease

[conuly]

I read a book once, a murder mystery, where the key to the murder was that a woman who'd had an affair with a Jewish man gave up her legitimate son because somebody carried the Tay-Sachs gene, and she didn't want to be caught out. The reality was that it's not only Ashkenazi Jews who are prone to this genetic problem, other groups also are highly effected.

In a minute of boredom, I decided to check this out for myself. Apparently, Cajuns have rates of the Tay-Sachs gene as high as the Ashkenazi (1 in 27). No doubt, if you're Cajun, you already knew something about this. Irish Americans half half that, 1 in 50 carry the gene. The rate in the rest of the population is around 1 in 250. There's a good chance that a lot of Irish Americans don't know this. I certainly didn't, until just now.

Also, apparently, Tay-Sachs isn't just cute little babies who die. That's the most common form of the disease, but onset can be as late as adulthood. Very interesting. Not good news, but interesting.

So, if you're anywhere near "at-risk", didn't know it, and plan to have kids, do yourself a favor beforehand and get tested. As near as I can tell, this is always fatal, and most of the time it starts in infancy, and the child is dead by the age of four or five. Juvenile Tay-Sachs is fatal by the mid-teens.

And now I wash dishes. Djusk' a.

Edit: Incidentally, one of those sites links to a page about a similar disease which is also more common in Ashkenazi Jews. No idea how common it is in the rest of the population, though.

Comments

[georgiapeachy.livejournal.com]

Cajuns not only have tay-sachs to worry about, but a whole bunch of other genetic diseases. I believe Cajuns are 250% more likely to have a genetic disease than the general populace. I only am 1/8 Cajun and mixed enough with various ethnic groups that I don't really have to worry that much, but one of my cousins has Friedreich's Ataxia, which is a disease primarily associated with Cajuns, but that also occurs in non-cajuns. I think the common link between Jews and Cajuns is that in both cases people are very much encouraged to marry within their ethnic groups, which leads to inevitable inbreeding.

[conuly]

I think the common link between Jews and Cajuns is that in both cases people are very much encouraged to marry within their ethnic groups, which leads to inevitable inbreeding.

That explains why both groups have high risks of genetic diseases, but not why this specific gene is so overrepresented in both communities.

[georgiapeachy.livejournal.com]

The only thign that could account for that I guess is that Cajuns and Jews are somehow related or that this gene is present in all populations, but brought out by inbreeding (I admit my knowledge of genetics is not too extensive).

[conuly]

Well, that's one thing people are researching.

Please note that not all Jews are likely carriers. Only the Ashkenazi have this high rate of Tay-Sachs. Sephardic Jews (the other large group) have the same rate as the general population. I imagine that Ethopian Jews also have a low rate of Tay-Sachs, and that other African Jewish group does as well.