Monday, December 19, 2011

Miscellany

Between grading and the holidays, my blog has been sadly neglected for the past two weeks.  I'm hoping that will change in the next couple of weeks, when I have a little more time to think and a lot more time to write.

I've been busy getting the Christmas presents ready for the people, although I still have yet to get a tree.  And I have been cooking.  I found what looks like a wonderful recipe for pork loin wrapped in prosciutto, which I'm going to try to make tonight.

It's only "wonderful," of course, if you don't object to the idea of a slab of meat wrapped in meat and then stuffed with ... meat.  But it does use apples and kale, too.

I confess, I have a thing for prosciutto.  Whenever I see a recipe that uses it, I have to try it, come what may.

In terms of reading, it's mostly been student papers.  I'm actually taking a brief break from student papers right this very second, but I will return to them momentarily, I promise.

I did read an essay by Richard Preston about Lesch-Nyhan Syndrome, and I really wish I hadn't.

If you don't know what Lesch-Nyhan Syndrome is, don't Google it.  I'll tell you and then you'll know why I'm telling you not to Google it.

Children who suffer from Lesch-Nyhan Syndrome have a rare genetic disorder that causes them to be developmentally disabled and to exhibit bizarre and truly horrifying behaviors.

They mutilate their own faces.  Children with Lesch-Nyhan Syndrome used to have their upper teeth removed to keep them from biting off their own lower lip.

They are often afraid of their own hands.  They are occasionally missing fingers or joints of their fingers because they have bitten them off.

It is as if their hands have a mind of their own and attack their face, inflicting horrific injuries.  They will remove their own eyes.  They will remove their noses.  They bite their fingers off, trying to protect themselves from ... themselves.

Because they experience pain exactly the way the rest of us do, they will often scream for help while doing this to themselves.

There was a picture included with the essay.

I didn't sleep all night.  It took me two days to recover from reading about this.  I kept waking up and thinking about the children's parents, to say nothing of the children themselves and the individuals they grow up to be.

They often die in adolescence, although there are a couple of individuals with Lesch-Nyhan Syndrome who are in their 30's and 40's.

There is no cure for Lesch-Nyhan Syndrome, and no one is certain what causes the genetic disorder.  It is a recessive trait on the X-chromosome, so it only affects boys, although girls can be carriers of the mutation.

It apparently took Preston seven years to write the essay, because he was so disturbed by the subject.  He wanted to find a way to make something so bizarre and so horrifying human and understandable to the rest of us. 

He wanted us to see past the illness to the children themselves and the individuals that they become.

He did a good job.

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