Tuesday, November 17, 2009

Panic in Level 4

I've begun reading Richard Preston's collection of science articles, "Panic in Level 4." Preston is a writer with a gift for affirming the axiom, "truth is stranger than fiction," or rather, more horrifying than you can possibly imagine. Consider the rare genetic disorder known as Lesch-Nyhan syndrome, which is caused by the mutation of a single nucleobase (C,G,A,T) in the victim's genetic code, and results in uric acid excretions, spastic limbs, and self-cannibalism.

That's right, self-cannibalism. Warning: graphic picture.

Victims rarely live beyond their 30's. By then, they've usually bitten off several fingers, torn away their own lips, and injured their eyes or nose. Developmentally, people with Lesh-Nyhan syndrome usually do not learn to walk and are confined to wheelchairs, but they are otherwise relatively lucid and aware. They suffer from a rare, self-sabotaging impulse, an internal logical reversal that is responsible for many of their injuries. The more a Lesh-Nyan likes you, the more cruel and violent he will act toward you. Befriend a victim of the disease and you should expect swearing, the occasional punch to the groin, and spitting followed by the sincerest apologies. They often report what feels like an evil phantom taking control of their limbs. Give them a sharp utensil and they're liable to gouge out their own eyes or rip off their nose. For these reasons, they feel most comfortable when they are bound or otherwise secured.

The disease is obviously tragic. But what makes it worse is that there is an element of humanity, of lucid consciousness, that is being tormented. These are not the sufferings of a brainless psychopath. These are the sufferings of men (the disease derives from the X chromosome, so it is extremely rare in women) who want control but are denied it by their very nature. Needless to say, this condition raises all sorts of questions about free will and morality. But continuing along this thread of humanity, I'd like to share a passage that illustrates the human power to find humor in tragedy.
Murphy had a record of making trouble in shopping malls. Malls put him in a bad mood, especially around Christmastime. "Too many people around. They make me Nervous," he explained to me.

One time, his assistants took him to a mall to do some Christmas shopping. A man dressed as Santa Claus was sitting in a snow scene that day, with children lining up to meet him. Murphy told his assistants that he would like to have his picture taken with Santa (one of them had a camera). They didn't see how they could refuse the client's request. They parked Murphy's wheelchair in the line of children, and Murphy cautioned the children to watch out for his arms and legs. (Neither Murphy or Elrod had been known to lash out at a child.)

Murphy got to the head of the line. The Santa asked Murphy if he'd like to sit on his lap.

Murphy said yes. The assistants placed him on the Santa's lap. The assistant with the camera, a young man named Dan Densley, got ready to take a picture.

"Ho, ho, ho! What do you want for Christmas?" Santa asked.

"A woman," Murphy answered, and delivered a punch to Santa's jaw. Santa's beard seemed to explode, and his eyeglasses went flying. The assistants grabbed Murphy and rolled him out of the mall at a dead run.

[Panic in Level 4, Pg. 173-174]


SuiginChou said...

I don't remember learning about self-mutilation (let alone self-cannibalism), but we discussed Lesch-Nyhan syndrome in my first year of medical school. I think someone even gave a presentation on it. But they must have forgotten to mention this, because your blog update is the first I've ever heard of this, and I can say that for a 99.9% fact.

Jay said...

Hmm, really? Seems like quite the oversight!

Jay said...

I found a video montage of Lesch-Nyhan victims played against the song "What a wonderful world."

The human spirit is baffling. Good for them for staying positive! But, gosh, a wonderful world and this syndrome, mashed together? I just don't know about that.