An almost biblical curse

There are a couple of small towns on the border between Utah and Arizona that are basically feudal theocracies, ruled by a particularly nasty splinter sect of polygamist Mormons. It’s got some truly ugly social consequences — daughters are prizes given away to church leaders, while sons are competitors who are driven away — but now it turns out that there also some biological consequences. The community is deeply inbred, and their prize is the possession of the highest rate of fumarase deficiency in the world, with at least 20 afflicted children in the last 15 years. Fumarase is an enzyme in the Krebs cycle; deleterious mutations in these genes cause a metabolic disorder called fumarase deficiency.

It’s not a nice innocuous disease. It’s variable in its severity, but bad cases lead to debilitating mental retardation, frequent seizures, characteristic appearance (a large head, coarse features), and death. With care, affected individuals can live for many years, but they’ll never be self-sufficient and they do require near-constant attention.

And because it is widespread in a small isolated community, that for various reasons (including religion) is neither a desirable destination for new residents nor will its population try to integrate with the outside world, it’s going to get worse. Half the children born to a pair of carriers will be carriers of the disease themselves, and those nice big Mormon families produce lots of children. Not only are the young girls in this community forced to marry and start spawning baby after baby, but compound the horror with the idea that some will be having children who will need to have their diapers changed for 20 years…

But Isabella is such a lovely name!

Apparently, you shouldn’t name your daughter “Barbie” unless you want her to grow up to be an airhead. A study, reported in the Guardian, claims that names have a powerful influence on social expectations — they report a significant effect in lowering exam scores based on whether the student’s name is classifiable as coming from a distinct ethnic/socioeconomic class, and further claim that the femininity of a name has a negative correlation with performance in math and science.

It’s somewhat odd. For one thing, they calculated a femininity score for various names based on letter and sound combinations—”Isabella” is the most feminine name, while “Abigail” comes out near the bottom. I don’t know any male Abigails, and Grace and Ashley, two other names in the list with low femininity scores, don’t sound particularly macho to me.

On the whole, people judged to have more traditional names such as Rachel and Robert did extremely well. More alternative names scored badly. Breeze, for example, was given 16 out of 100, while Christopher received full marks. ‘A name is part of an impression package,’ said Mehrabian. ‘Parents who make up bizarre names for their children are ignorant, arrogant or just foolish.’

Eh. It sounds like traditional conservative bigotry to me. If it holds up, though, it’s an interesting example of the way cultural biases can affect performance on supposedly bias-free examinations. I wouldn’t be too surprised if it’s valid, though: I know that when I grade exams and papers, I consciously avoid looking at the name on the exam until it’s time to record the scores in the gradebook, and I often grade from back to front to make that easier to do. That’s not to avoid bias from the femininity of the names, though, but because I know the students and want to avoid preconceptions.

I do have to note, though, that a) we named our daughter Skatje, and b) the author of the report is named “Anushka” (which sounds nice to me), and one of the teachers interviewed is named “Edyta” (an unfamiliar name, and now I suppose I’m going to imagine every Edyta I meet is a schoolteacher. Or not.)

(via Unhindered by talent)

Saint, monster…they’re the same thing

A name I’d like to see banished to oblivion is that of Paul Hill, the religious fanatic and murderer who gunned down Dr John Britton and James Barrett at an abortion clinic. I don’t care whether you are pro-choice or anti-woman, only the most wretched, insane god-walloper can possibly approve of assassinating health-care providers to protect fetuses. And there can’t be that many of them, can there? And they are going to face universal public censure, right? Cruelly, archy tells me otherwise.

Behold, Paul Hill Days.

A couple of deranged Christian organizations are planning to send their members to Milwaukee, Wisconsin at the end of July to thump about like a gang of gumbies, bellowing about what a good man Paul Hill was. God’s man. A hero. A martyr.

And to crown this exercise in lunacy and poor taste, they’re planning a reenactment of the murders.

I guess the Rev. Phelps and his merry band of haters aren’t quite as uniquely bizarre as I’d thought.

Ick — now the Rev. Donald Spitz has sent McKay a letter, blaming him and people like us for AIDS, rape, murder and robbery, and all kinds of such nastiness, because we don’t believe in Jeeezus. These people are crazy evil; I’m beginning to get a sinister vibe from just the title “Reverend” anymore.

Council of Europe’s anti-creationism recommendation

The Council of Europe has put up a wonderful motion for a recommendation. Can anyone imagine this being discussed in the American senate or house of representatives? The Republicans would howl in fury against it, and the Democrats would rush to bury it, lest they offend the Republicans and annoy the ignorant members of their electorate. (The Council of Europe, by the way, is not the same thing as the Council of the European Union or the European Parliament, so it’s not really comparable to our congress. Europe says some very sensible things, but Europe is also very confusing.)

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Idiots with too much money

I don’t normally mind people their hobbies and obsessions, even when I don’t share any interest in their object of desire at all, but I did feel some intense schadenfreude at this story about people smashing their over-priced, over-powered sports cars. There is just something excessive about spending $1.5 million on a car. The other troubling part of the article, though, is the mention that more and more people are buying these extravagant luxuries — the number has tripled since 2003 — which is why we’re seeing more of them involved in accidents. It’s another example of how the rich-poor divide is widening in this country. So, yeah, rich pampered scumbags, wreck those cars.

And then you read stories like this one: expensive sports car goes out of control at a charity event, bodies are flying through the air, and 6 spectators end up dead and a score are in the hospital. The driver, of course, is mostly uninjured.

(via Hillary Rettig)

You sickos!

Matt asks a weird question: he’s wondering who is the target of the sickest web searches. I should recuse myself, because I thought I got no perverts searching for me: after all, I think searching for “sex with a spider” or “penis tentacles” is perfectly normal. But I took a look at the search terms anyway, and I was appalled—there actually are several very common phrases people use to find their way here that I find objectionable.

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How desperate can they get?

Bill Dembski is touting some strange ID-positive blog as a sign that there is a “growing number of non-religious ID proponents” — alas for poor Bill, when you glance at the blog, it’s some random guy making a post about once a month, whose background is as a musician and professional crackpot. His sole qualification as a “scientist” seems to be that he signed up to post on that ID web forum, ISCID. You should read more on Stranger Fruit, and Afarensis reveals that rather than touting his non-religious credentials, his unique claim to fame is as an “ID Pleasurian,” believe it or not. How will we ever deal with the growing number of sex-positive, porn-friendly ID proponents?

Oh, and he’s resentful that I actually have a blog category titled “Kooks.” Probably because he presciently knew that if ever I commented on William Brookfield, that’s the category I’d pick.

Sometimes, fence-sitting is the domain of the crazy, too

The fundamentalist nuts in this country leave us goggling aghast at the lunacy they propagate, but man, some of the in-betweeners are almost as creepy—and I get to pick on somewhere other than America! This page on the “noble lie” brings up the Straussian hypocrisy that many confused pro-religion people are supporting in the UK — we have to support faith to keep the masses placid.

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