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Mad human disease?
January 5, 2007 2:58 PM   Subscribe

In the new book The Family That Couldn't Sleep, journalist D.T. Max investigates Fatal Familial Insomnia - a strange prion disease that has been linked to Variant Creutzfeld-Jakobs Disease (the human form of mad cow disease).
posted by grapefruitmoon (34 comments total) 6 users marked this as a favorite

 
The links between the diseases are fascinating, as are the implications for how disease can spread. Perhaps most fascinating is the reason why most of the world has NOT as of yet died of mad cow disease. (I'm not going to spoil it for anyone who wants to read the book.)
posted by grapefruitmoon at 2:59 PM on January 5, 2007


Sweet Jesus on a pogo stick that's nightmarish. Okay, poor word choice.
posted by tula at 3:08 PM on January 5, 2007


Wow. Absolutely amazing, and horrific. Thanks for that post.
posted by toma at 3:09 PM on January 5, 2007


This is so weird.. I heard about this on NPR a coule of weeks ago, and was talking about it last night to some friends. It is so creepy.
posted by splatta at 3:09 PM on January 5, 2007


(I'm not going to spoil it for anyone who wants to read the book.)

This isn't fiction, and you've piqued my curiousity. Please, spoil.
posted by cortex at 3:19 PM on January 5, 2007


I'll pass on all prion-related diseases, thanksverymuch.
posted by mosk at 3:24 PM on January 5, 2007


cortex: I'll try to explain as eloquently as I can, but IANAS. Genetics dictates that we have two copies of each chromosome - one from each parent. Prion diseases are more common in people who have different amino acids in the "prion space" on each copy of the chromosome that controls that sort of thing (20 or 21, I can't remember which). Thankfully, the reason we're not all dead of CJD is that the vast majority of the population has the same amino acid on both prion chromosomes.

Investigation into why evolution would have selected for this has speculated that mass cannibalism may have wiped out many of those prone to CJD around 800,000 BCE.

(CANNIBALISM! Doesn't get much creepier than that. Man, I love creepy stuff.)
posted by grapefruitmoon at 3:27 PM on January 5, 2007


I just crapped my pants.
posted by punishinglemur at 3:30 PM on January 5, 2007


And you're starting to smell. Go stand over there or I won't be able to sleep...

This is horrific - but great post, thanks!
posted by goo at 3:38 PM on January 5, 2007


so, here's an idea -- if you're genetically predisposed to a really fucked up disorder and you know it, based on your family's history DON'T BREED; unless you want to make your children suffer an agonizing life.
posted by wumpus at 3:58 PM on January 5, 2007


Great. . . another thing to worry about when I can't get to sleep.
posted by isopraxis at 4:08 PM on January 5, 2007


if you're genetically predisposed to a really fucked up disorder and you know it, based on your family's history DON'T BREED

A simple solution to a simple problem. Entirely acceptable and complete.
posted by Alex404 at 4:29 PM on January 5, 2007


wumpus,
thanks to the wonder of eugenics, your recommendation will be willfully misconstrued.

of course, there's also the argument that asking someone not to breed is akin to asking someone not to breathe (since, well, humans as a species are preprogrammed to have the urge to breed, hence the sex drive).

but, anyway.
posted by daq at 4:31 PM on January 5, 2007


i'd think it would be 20 since 21 is the sex chromosome.
posted by lisalisa123 at 4:38 PM on January 5, 2007


Arguably, humans are preprogrammed to have the urge to fuck (hence the propogation).
posted by cortex at 4:39 PM on January 5, 2007


20 or 21, I can't remember which
sorry, that's what i was responding to... hee hee
posted by lisalisa123 at 4:39 PM on January 5, 2007


Brief interview with Max on Salon.com here
posted by staggernation at 4:45 PM on January 5, 2007


When the zombie outbreak occurs, it will surely be in the form of a prion disease.

That being said, this sounds truly horrible.
posted by Telf at 4:49 PM on January 5, 2007


okay, let's get into responsible procreation a little. i actually agree with wumpus- alot. for several reasons. why would anyone want to have a child, knowing that the kid would be living a horror of a life? there are children who live horrible lives, but this particular condition is just torture and needless if it can be avoided. adoption might be a solution for people who are capable of managing such a disease. adoption solves 2 problems: it ceases the genetic anomoly, helps stop world overpopulation.

humans as a species are preprogrammed to have the urge to breed, hence the sex drive

and there is birth control to help prevent accidents. of course people are going to have sex and a sex drive! however, the need to procreate, although biologically is a necessity, is not logically a necessity for the survival of the species. edward o. wilson makes a point in an interview on the think tank about his bottleneck theory, and how our population explosion will become a regression in a few years.
posted by lisalisa123 at 4:54 PM on January 5, 2007


Ha! I gave this book to my sister for Christmas. Apparently there is a sheep disease called lickybutt or something like that.
posted by thirteenkiller at 4:56 PM on January 5, 2007


Also, wumpus, the people with this insomnia disease don't develop symptoms till late middle age or after, and then they die fairly quickly. So it's not like they're dooming their children to a lifetime of suffering.
posted by thirteenkiller at 4:59 PM on January 5, 2007


The sheep disease is called scrapie.
/pedant
posted by i_am_a_Jedi at 5:02 PM on January 5, 2007


Oh, actually, the disease is Licky End. Jedi, you know, there is more than one sheep disease.
posted by thirteenkiller at 5:16 PM on January 5, 2007


But Licky End may have been made up by Terry Pratchett and it probably isn't covered in the book in question.

/shifty look
posted by thirteenkiller at 5:18 PM on January 5, 2007


Sorry, thought we were talking prion diseases...
posted by i_am_a_Jedi at 5:28 PM on January 5, 2007


This is so terrifying I can't help myself from laughing maniacally. I go through some pretty severe bouts of insomnia, and I actually got to level two where my hallucinations and panic attacks started interfering with my life (winter in Inuvik fucked me up badly). Dying of insomnia, slowly going crazy, is my idea of hell. These poor people should be allowed euthanasia (if they want it) once the disease progresses to the batshitinsane level.
posted by arcticwoman at 5:40 PM on January 5, 2007


lisalisa123 writes "i'd think it would be 20 since 21 is the sex chromosome."

Not in humans, it isn't. 21 is the chromosome responsible for trisomy 21 (Down syndrome).
posted by mr_roboto at 6:08 PM on January 5, 2007


Great post, thanks! I find prion diseases fascinating.
posted by zia at 6:11 PM on January 5, 2007


why would anyone want to have a child, knowing that the kid would be living a horror of a life? there are children who live horrible lives, but this particular condition is just torture and needless if it can be avoided.

Well, the child wouldn't live a horror of a life at all. This is a horrible, godawful way to DIE, but the disease has little to no bearing on one's life up until that point.

Also, this disease has been haunting the family in question for centuries. Only recently have the mechanisms of disease transmission been understood well enough for families to make informed choices about adoption.

The author discusses the ethics of this, and the family has chosen to remain in the dark (so to speak) about who has the mutation that causes the disease so that it won't be a factor in their decisions to have children.

Yes, it's an awful way to die and I don't know if I would want to continue the disease, but it's extremely rare (it doesn't even occur in *all* members of the family) and life up until the onset of the disease (around age 50) is completely normal.
posted by grapefruitmoon at 7:20 PM on January 5, 2007


A comment regarding prions...

From the third (wikipedia) link:
"The mutation changes the shape of the protein so that it becomes a prion and makes other, normal protein molecules change to the abnormal shape."
(helpful image here).

This is know as the Prion Hypothesis, and as yet still remains a hypothesis. It's still somewhat controversial, and nobody has yet proven that it really occurs. The scientist who does prove it (assuming it's true), will probably get a Nobel Prize.
posted by kisch mokusch at 9:50 PM on January 5, 2007


So far, two Nobel prizes have already been given out based on prion research: Daniel Carleton Gajdusek and Stanley B. Prusiner.
posted by grapefruitmoon at 5:14 AM on January 6, 2007


Apparently scientists have already genetically-engineered cows that are supposedly not susceptible to BSE because they lack the protein that the prions fuck up.
posted by kindall at 5:53 PM on January 7, 2007


So...I'm confused. Are these people becoming ill from lack of sleep, or is lack of sleep one characteristic of an illness that causes many symptoms? This journal article makes it sound like a LOT is wrong with their bodies besides just not sleeping.
posted by etoile at 10:01 AM on January 11, 2007


The lack of sleep is indeed a symptom. Of holes in their brain.
posted by kindall at 8:59 PM on January 11, 2007


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