This story is a couple of weeks old, but I've only just come across it. It reminds me that there may be some things worse than death, and this is one of them:
To see the face of 32-year-old Huang Chuancai is to witness a rare genetic condition in its most terrible form.
Chinese doctors say Huang, of China's southern Hunan province, suffers from a disease known as neurofibromatosis, a genetic disorder of the nervous system that primarily affects the development and growth of neural cell tissues.
For many of its sufferers, the disease means abnormal growth of these tissues and, as a result, facial disfigurement. But Huang's case could well be the most extreme case of such disfigurement in the world today.
Before surgical intervention, 50 pounds of tumor tissue had pulled and twisted Huang's face into a distorted form, nearly unrecognizable as human. According to wire reports, doctors found that the heavy mass that droops from his head and face had deformed his backbone, stunting his growth. The tumor had totally covered his left eye, while his left ear hung down to his shoulder, and his right ear and jaw were completely engulfed.
To see photos of just how bad neurofibromatosis effected Huang, click these links. But be warned. The images disturbed even me, a surgeon who's seen some pretty horrible things in person:
- Man With Tumor for Face Braves Surgery
- China's "Elephant Man" hopes to be normal after second surgery
- Surgery Hope For Tumour Face Man
I wasn't able to find much about how well Huang did after the surgery, which must have been a tour de force of surgical skill to get the tumor off without either killing the patient or causing serious complications and morbidity. Here's what this unfortunate man had to live with:
The "Elephant Man" from Hunan province could hardly speak before then because his tumour was so big.The crippling mass drooping from his head and face had totally covered his left eye, while his left ear hung down to his shoulder, and his right ear and jaw were engulfed by the tumour.
Mr Huang's condition has ostracised him from society - bullying from his classmates forced him to leave school when he was 10 years old.
He says he was even approached several years ago by someone who wished to buy him and display him as part of a circus freak show.
But hopes are high that Mr Huang will be a step closer to being cured when he undergoes the second operation today at the hospital in Guangzhou, the capital of prosperous Guangdong province.
"I hope that when my illness is cured, I can go back home and have a nice spring festival with my family," he said.
It's hard to imagine living with such a condition. It just goes to show that the worst diseases and conditions may not necessarily be the ones that kill us.
More like this
Huang appears to have neurofibromatosis type 1, which, with an incidence of 1:3000 to 1:3500 worldwide, is certainly not a rare disorder. A plexiform neurofibroma of such great size is unusual, of course, but then such tumors are thought to be congenital in about a third of people with NF1, and are typically removed sooner, since they can cause bone deformities and nerve damage. Huang is likely to have significant nerve damage, even if such a massive neurofibroma can be debulked successfully; neurofibromas are Schwann cell-derived and thus closely associated with nerve endings (the dermal or cutaneous neurofibromas) or nerve roots (the plexiform type). Even the dermal neurofibromas can be disfiguring, painful, and itchy.
More info about NF1 (and NF2 and schwannomatosis) can be found at the Children's Tumor Foundation website.
http://www.ctf.org
They don't disturb me, but then I've had the misfortune of seeing Huang's face now and then every day for the past month. The Yahoo News page has had Huang's face in the "most viewed" column on the right of their newsfeed almost every day, which I think tells you a lot about the human species. (Right now (Jan 27, 2007 11:00am or so) most of the pictures appear to be of Miss America, which also tells you something about our species.)
There are many different phenotypes to NF. I have a friend with NF, and you'd never know it if you weren't tuned into it. It causes more discomfort than is visible, however, as he has a few dozen of the buggers removed every year.
There are no pictures below the fold, right? I closed my eyes while I waited for this to load, in case my computer showed this part of the article instead. Sorry to bother, but I don't want to give myself nightmares.
skwee: no pictures below the fold.
Vorn
The reference to John Merrick(sic) AKA The Elephant Man isn't really valid as he actually had Proteus Syndrome, not NF.
Proteus syndrome(named after a shape-shifting Greek god) consists of lipolymphohemangiomas, hemihypertrophy, macrocephaly, partial gigantism of the feet, and nevi. The change in though regarding his dx was based on new DNA evidence using his remains a few years ago.
I visit a brainwashed housewife ... er, Christian helpmeet blog, and one of her post titles was, "Can the Clay Talk Back to the Sculptor?" In other words, can we humble humans, who have been created by a perfect, all-powerful, all-seeing, and all-knowing God, dare to question anything God did when making us?
I asked her what she made of a similarly terrible case (that of a young Haitian girl whose facial bones had grown to the point that her face resembled Uniqua on the Backyardigans. Or, in a less cutesy example, a moose with a tiny hole for a mouth.
If God made all of us, and He never makes mistakes, then surely he didn't make a mistake when creating that young girl, or when creating poor Huang -- how dare they "talk back" to their sculptor? How dare they change his design? They should just humbly accept their lot, since they were made by God, and He works in mysterious ways -- obviously He had a purpose in making them so distorted and miserable and in pain. Maybe, when they get to Heaven, He will tell them.
Curiously, I never got a response.
This was in China, the home of accupuncture? Didn't that work? I wonder if they even tried it.
@Linda:
It's painful to even think about, for a rational person, but if you want to wrench a religiotard's brain, you can ask them "what about spina bifida?" After all, since the great sky fairy is perfect and it's all part of the sky fairy plan, then the spina bifida must have been deliberately inflicted on the child in the womb. You can then ask the religiotard to explain what kind of loving supreme sky fairy would inflict something so appalling on an innocent life in order to teach its parents a lesson. It sure does make 'em squirm!!
And if they fall into the typical counter-dodge of saying "the great sky fairy's plan is mysterious and we shouldn't question it" then you can slam their toe in the door by pointing out that virtually all of *tianity is about purporting to know the intent of the great sky fairy. So either the plan is mysterious or it isn't - it can't be both.
Of course, rational argument doesn't work on the faithful, or they would already have healed themselves.
It would seem that many a person believes that this deformation is the fault of God. Refer to James 1:13 and draw your own conclusions.