wdcsmwscaa, I don’t recall specifically what happens to them. I would assume that they would suffer whatever the usual effects are of constantly being exposed to the air. If you look hard enough, floating around somewhere online is a short video clip of a woman bottle-feeding a baby with harlequin ichthyosis. The baby is just able to get its lips around the nipple, but it obviously is having a hard time of it 
Thank goodness that disease is so rare.
Does anybody remember that guy that was on Larry King Live (I think) once? It was quite a while ago. I don’t know if it was a diease or an infection. Either way, I THINK what had happend was that he had gone into surgery for one reason or another, and by the time they were done they had to remove a large section of his face. From just above his eyes to below his nose, ear to ear, and about to the back of his throat. Basically he had a hole the size of a baseball in his face. He wore a mask that did a halfway decent job of disguising it, but when he took it off, it was slightly disturbing. I’m sure someone else here knows who I’m talking about and can provide a link or a picture.
When I worked at a pediatric nursing home, we had a patient with Epidermis bullosa.
His skin was at best peeling, at worst covered with weeping sores and blisters. We could barely touch him for fear of tearing his skin. When we lifted him, it was in a mesh sling and his bed was lined with sheepskin. He could only be bathed with running water/antibacterial solution.
It used to break my heart because he wanted to be touched so badly. He’d reach out and grab your hand and place it on his.
I think I saw that guy on another talk show, with a host with a British accent. Was it a big overweight guy with dark brown or black hair?
If it was, what happened was he got some kind of flesh eating fungus in his nasal passages while he slept, and it spread and basically liquefied most of the inside of his face. The hole in his face (from the bottom of his forehead to his lower jaw) is because the doctors removed all the effected tissue to keep it from spreading. They said it was miraculous that he survived.
Something like that. I also want to say that I belive he that he was going under for something more minor (ie taking a little chunk out) and woke up with like that.
Progeria (?) The one where kids take on the appearance of a very, very old person. Truly horrible.
Undine’s Curse.
WARNING: CHRONIC HYPOCHONDRIACS SHOULD READ NO FURTHER.
SERIOUSLY.
YOU HAVE BEEN WARNED.
Old German legend:
Once there was a prince who fell in love with a water-nymph named Undine. When he dumped her for someone else, she put the whammy on him: she took away his ability to breathe automatically. He couldn’t take a breath without willing himself to do so. After suffering many exhausting, sleepless, terror-filled days and nights, he finally drifted off to sleep and died.
Fantasy? Hell, no. This is exactly what happens to the worst sufferers of primary alveolar hypoventilation, or “Undine’s Curse.”
And what’s worse, many, perhaps most. cases of Undine’s Curse are psychosomatic–a particularly sensitive person HEARS about the malady and suffers anxiety attacks, making it difficult to breathe unless s/he’s concentrating on it at all times.
Another story:
I was in the ENT clinic back in med school, and I walked out of one exam room only to have an attending wave for me to join him in another one. He didn’t say anything about the patient. (This is never a good sign.)
The patient was an sweet older woman who reminded me of my grandmother, with her daughter and two young grandkids there as well. She had a large bandage over the middle of her face as if she had just had a nose job. She and the attending talked for a minute before she removed the bandage to reveal…
…nothing. Where her nose used to be, that is. It was as if someone had taken a magic marker and drawn a circle around her nose, and just cut on the line and yanked. (I imagine that is almost exactly what happened.) She had some sort of skin tumor in her nose, and had to get the whole thing removed.
I tried my best not to recoil in horror. It wouldn’t have been nearly so disturbing if she hadn’t been such a sweet old lady, who was talking to me just like any sweet old lady would, except she DIDN’T HAVE A NOSE!
The discussion that followed was even more bizarre–she was there to start talking about getting a prosthesis. In other words, they were going to make her a nose. Not a permanently attached one, mind you–they said that they could make it attach with clips or even little magnets.
(My first thought–if I could take my nose on and off at will, I would. Frequently. In public. “Ah…ah…ah…CHOO!” “Bless y–GAH!”)
Dr. J
If you think diseases of the brain are the worst…I’ve always thought Huntington’s Disease sounds absolutely horrible. Basically, the cells of the basal ganglia in your brain deteriorate. As a result, you lose your ability to walk, talk, and even think. By the end, you’re totally dependant on others for your care. A significant number of Huntington’s sufferers die as a result of choking on food, because they are unable to swallow. To top it off, the disease is genetic, so if you’ve got it, you’re stuck with it.
Okay, so it’s not as shocking to the eye as harlequin icthyosis, but I have this horrible fear of being trapped in a body completely without function.
global aphasia.
where a stroke or other brain damage leaves you unable to understand language.
not written, spoken, nothing. and nobody understands you either because you can’t speak or write coherently.
horrible.
Syphilis.
It’s easily treated now, if caught early. But several years ago, it was often left untreated because the early stages are almost without symptoms.
My Mom had a friend who was diagnosed 20 years after she got it, by then it had eaten away half her face, and she was going blind.
Oh my god. DoctorJ your account engaged my morbid curiosity. I found some photos of a baby who got Fournier’s after a circumciscion (and no, I’m not starting this debate again). He had to have a lot of skin removed. Unbelievable.
This could be the aforementioned “flesh-eating fungus,” since this patient also had a prosthetic face (although it’s probably not Larry King’s guest):
http://www.courier-journal.com/localnews/2002/02/05/ke020502s150465.htm
I’d just like to take a short time-out from the festivities to remind everyone that the supposedly perfect design of the human body is frequently trotted out as evidence of creation by a favorite deity.
Carry on.
Wouldn’t have happend to be in Cincinati would it? That happened to my Grandmother. Started as a squamous growth under her nose. Spread to the whole nose. The Doctors kept cutting, a bit at a time, trying to win the race against the cancer. Eventually it got to her eye too, and it had to be removed. She got a prosthesis, but it wasn’t very convincing. Better than the bandages though, I guess. Eventually it got to her brain and she died. Skin cancer sounds like nothing, it’s just skin, right? but it can spread just like other cancers, and not hidden inside, but out in the open where you can see it.
Scary stuff… but those harlequin babies rip my heart out. That’s gott be the worst…
Not really the most hideous disease, but Hyperelastic’s post reminded me of a pretty nasty-looking one–severe whipworm infections that result in a prolapsed rectum. Pictures I’ve seen are interesting; the prolapsed bit varies in color from bright red to deep maroon, but it’s always covered in little white curlicues–the whipworms.
I’d give the “Harlequin Fetus” my vote. That is terrible, terrible, terrible stuff.
Parasitism has got to be the worst.
I one saw a pic of a guy with a bad whipworm infestation, with a prolapsed anus - his guts were hanging out his ass, writhing with worms. It just can’t get worse than that.
Just as terrifying is that thin, eel-like fish in the Amazon which attempts to swim up into your bladder - and has backward-pointing spines, so you can’t pull it out. Ugggg.
I don’t know if you guys took a look at the rest of the pics on Mad Matt’s site, but if you didn’t, do so. That ulcer is nothing to laugh about either is that syphilitic woman’s face.
I took a vertebrate/invertebrate biology class (it was thorough). The nematode unit was…interesting. One picture I remember in particular: a girl, about thirteen years old or so, was lying naked on her stomach. She’d just been given a pill to expel all the worms that had been living in her guts. There was a pile of white worms from her crotch to her knees. Thousands of them.
Or then there’s the one that lives just under your skin. You notice that you have a small sore that just won’t heal. This is because every night the worm extends its anus end through that sore and lays eggs. Removing it is the worst part. You can’t cut it out because of the danger that you’ll cut into it, which would cause blood poisoning. Similarly, you can’t yank it out all at once, for the same reason. So you have to grab it when it’s laying eggs, and pull about a centimeter of it out, then tape it to your skin so it doesn’t go back in. Repeat daily until the whole thing is finally out. In the meantime, you have a chunk of living worm hanging out of your arm.