Monday, January 23, 2012

Medical Mondays: Prions from Outer Space

A while ago, Krispy at A Nudge in the Right Direction asked me a question about picking out a slow disease to attack a character.

One of the diseases I considered were prion diseases. First off, the name "prion" sounds sci-fi, and as you'll see below, they are like no other disease you've ever encountered.

What's a prion? Prions are proteins that are folded in an abnormal state. They have no DNA or RNA, like most living things. They are difficult to destroy and are infectious. 

How do they multiply? Prions propagate themselves by causing nearby, normal proteins to fold abnormally like them. Once those proteins misfold, they too cause other nearby normal proteins to misfold, and on and on. (Kind of like the Borg and their assimilation).

How do you destroy prions? With a lot of effort. The normal way we disinfect things to destroy viruses and bacteria often don't work on prions. High, prolonged temperatures, high concentrations of formaldehyde or caustic solutions are needed.

Is it animal, vegetable, mineral, or alien? Prions are not technically alive, in that they do not need food, or respond to their environment. They defy the biological definition that living things have nucleic acids (the building blocks of DNA). However, they replicate. They spread. And their very existence appears to have one effect--to make more of itself. Sounds alien to me.

Where did they come from? Okay, okay. They probably didn't come from another planet. There is a gene in humans on chromosome 20 for a similar prion protein that is a normal protein in humans. Mutations in this gene have cause inherited diseases with prions, like Creutzfeld-Jacob disease (CJD), Gerstmann-Straüssler-Scheinker syndrome, and Familial Fatal Insomnia (Death by Insomnia).

Can human immune systems fight prions? In a word, no.

What other diseases in humans are caused by prions? Mad Cow Disease (or bovine spongiform encephalopathy) has been theorized to have been transmitted to humans and causing variant CJD. Kuru is found in a native population of New Guinea that practiced cannibalism. There are several diseases that affect animals too, such as scrapie and of course, mad cow disease.

Symptoms? All prion diseases are neurodegenerative. They are slow diseases that primarily effect the neural tissues (brain, spinal cord). The symptoms can range from progressive dementia, difficulty walking or standing up, jerking of the limbs and tremors. Familial Fatal Insomnia causes...well. The name says it all.

Is there a cure? No. They are uniformly fatal.

Why aren't we all dead from prions, if they are so hard to kill and there's no cure? When cows are infected, there are low levels of prion in milk and meat, and it's not easy to absorb by eating. You also can't get prions from other people through casual contact. You'd need to be exposed to brain or spinal fluid tissues, or though organ donation.

How common are these diseases? As of 2011, there have been a total of about 211 cases of variant CJD worldwide. As for random, non-inherited, non-Mad-Cow related cases of CJD? 1 in a million cases worldwide, per year. Basically, pretty rare.

Did I just hear a collective sigh of relief?

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50 comments:

Natalie Aguirre said...

That sounds like a terrible disease to get. We're pretty sure that one of my aunts who died got mad cow's disease and died. I did not get to visit her because she lived across the country but she experienced a lot of the symptoms you describe before she died. So sad that people have to suffer through things like this that they have no control over.

Thanks for sharing with us though.

Miranda Hardy said...

Still, very scary to think about. So glad it's not easily spread.

Laura Pauling said...

I will always think about the novel Going Bovine when I read about mad cow disease! Always!

Connie Keller said...

Really creepy!

Sarah said...

This post made me shudder. Great job, Lydia :)

B.E. Sanderson said...

Scary disease. Thank goodness it's so rare and hard to get. And now my inner hypochondriac wonders if my proteins are secretly folding.

SA Larsenッ said...

This does sound a bit sci-fi. It always amazes me how strong some of these viruses/disorders/diseases are.

Old Kitty said...

Now I'm petrified! And I love cows! Living ones! LOL! Take care
x

Dianne K. Salerni said...

They DO sound like some creepy disease that came out of asteroids, or something. I have heard of prion diseases before. I can't think of where -- but it was probably the TV show HOUSE! It doesn't matter how rare it is. They've seen it all!

Coleen Patrick said...

Scary, interesting and thankfully rare--yes I'm sighing in relief! :)

walk2write said...

The first time I learned about prions was in an A&P class years ago at a community college. The professor related that he was relieved of his duties for ratting on a former colleague at a medical school. The colleague was studying human brain tissue but ignoring simple sanitation and safety rules while conducting his research. Our teacher was a little bitter and upset about the whole thing and understandably so. The former colleague was putting people at risk with his sloppy habits. Anyway, those prions made quite an impression on me when I first heard about them. Scary!

Matthew MacNish said...

Wow. How utterly terrifying. Sounds like the makings of a great story, though.

Alex J. Cavanaugh said...

Glad it's not common. And I think Matthew meant to say it's 'udderly' terrifying. Yeah, bad pun, I know!

Emily Rose said...

These prions are bad news...

LTM said...

omg, Dr. K, that is so weird. And creepy and ... now I'm freakin out!!! :D

j/k. But that is interesting. Prions. I thought "it's like cancer" at first, but I guess the difference is... umm... well... LOL! I don't know.

But as always, interesting and informative! <3

K. Turley (Clutzattack) said...

Doesn't the character Thirteen(?) on House have some sort of slow terminal disease?

Except prions sound way more cool.

Jax said...

Omg, scary! I remember the whole Mad Cow disease scare. That stuff freaks me out!

Melissa Bradley said...

A very creepy and fascinating post! I may have some more questions for you, if you don't mind.

Justine Dell said...

Yes, HUGE sigh. Huge. I thought I was going to have to wrap myself up in a bubble.

~JD

Slamdunk said...

Scary yet informative Lydia. Glad to hear about the 1 in a million odds...

Amanda Borenstadt said...

Weird and horrible disease! But kind of fascinating too.

Bossy Betty said...

I love this stuff. You always give me facts I never knew! I am glad it is rare though.

Clarissa Draper said...

Scary disease. What a horrible way to die.

Linda Gray said...

I think you've just convinced me to become vegetarian. Prions, yikes. Reminded me of 'replicators' on an old sci-fi tv show, but at least they could shoot them!

Bryan Russell said...

If we come across a cannibal cow, we're really in trouble.

Meredith said...

Uniformly fatal? I don't care if it's rare, now I'm thoroughly freaked out! Fascinating post!

Em-Musing said...

I don't know what's scarier...enemies outside the body...or inside.

Tamara Narayan said...

Okay, so here's the big question: What happens to zombies who eat prion-infested brains? Hmmm?

Bathwater said...

That does sound like a case for Dr. House although it sucks when he doesn't cure the patient.

Emily R. King said...

Scary stuff! I'm glad it's rare. Thanks for the interesting read. I learned a lot!

Roland D. Yeomans said...

O.K. You have just officially freaked out me and Victor! I am so happy they are rare. Wow. No more hamburgers for me for awhile. I know. It can't be transmitted by eating cow -- but there's always Mad Cow disease! LOL. Roland

Krispy said...

Ok, reading this info again is freaking me out again. Haha. Thanks for the suggestions, Lydia! You're awesome! :)

mooderino said...

Now that'sa scary disease. everything about it feels like it was made up by a sci-fi writer. This is why I don't share brain fluid with anyone.

mood

Carrie Butler said...

That gave me cold chills, just reading it! Creepy stuff. :|

Great post, though! :)

Colene Murphy said...

AHHH!!! That's scary!! Wow. I never knew such a thing existed. Very creepy. Screw ax murderers, this is scarier.

Deb Salisbury said...

Wow! Fascinating!

I seem to be pretty weird, though. It didn't occur to me to be scared until I read the comments.

justjoe said...

Oh man, those are some scary diseases. Especially kuru. But I'm glad they're not so common!

The Golden Eagle said...

It's interesting that they're not technically alive; they sound similar to viruses in that respect.

Abby Minard said...

Ew! Sounds like it could be some kind of scary pandemic outbreak or something (if people start touching brain matter all over the place). Great for a book or movie!

JEFritz said...

Woo! Those things are scary. I've heard of them but didn't understand what they were. It's good to know they aren't easily transmitted. Great explanation.

alexia said...

Scary! And morbidly fascinating...

Angela Brown said...

I can not tell a lie. Mine was probably the loudest in that collective sigh you heard :-)

This sounds like something totally Outbreak-like. Morbid and scary.

Sarah Pearson said...

Wow, I'd never even heard of this. Glad it's so rare.

Cynthia Chapman Willis said...

How scary and creepy. And yet, interesting.

Carol Riggs said...

Wow. No kidding we're relieved this is rare. It does sound sci-fi-like! Scary stuff...

Nancy Thompson said...

WOW! I see a horror movie in the making!

lbdiamond said...

Scary!!!!!!

February Grace said...

they misdiagnosed my grandfather with CJD back in the early 80's. Know what he actually died of?

Lead poisoning- which he got through his skin by dyeing shoes for a living for decades and decades.

No I am not making that up...I'm sure if that had happened now with much better diagnostic techniques the mistake would not have been made.

Fascinating! (I say, eyebrow arching a'la Spock...) hey, one good Trek reference deserves another!

Suze said...

Okay, yeah, they're rare but good night!

Shallee said...

I always thought prions were like the horror-movie version of any kind of illness...shiver. Thanks for the detailed information, though-- good story fodder. :)

 
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