Welcome to the rabbit hole — say hello to The Good, the bad, and the ugly.
The Good News: You Probably Don’t Have Prion Disease!
Prion disease is super, super, super uncommon. The most common type of prion disease, Creutzfeldt-Jakob Disease (CJD) occurs sporadically in 1 out of 1,000,000 humans. Dr. Michael Geschwind from UCSF, one of the world experts in prion disease, says that 98% of the cases he reviews are not prion disease.
Genetic prion diseases make up just 10-15% of all prion disease cases. Which makes your odds of having a genetic mutation for prion disease even lower, like 1 in 10 million.
⚡️ Statistically speaking, you’re more likely to get hit by lightning than to ever get prion disease. ⚡️
SO! If you’re on here doomscrolling bc you’ve had insomnia for a few weeks and are freaking out that you secretly have Fatal Familial Insomnia (FFI), try your best to take a deep breath and don’t freak out. You most likely don’t have it.
And guess what? FFI is generally a genetic prion disease, so unless other people in your family have mysteriously died and been diagnosed with FFI, you probably don’t have it.
Yes, sporadic FFI has been known to occur once in a freaky while, but there have only ever been a handful of cases in the last hundred years, so, for some perspective: the chances of having genetic FFI are about 1 in 30 million (ask me how I know.) The chances of having sporadic FFI are waaaaay lower — like 1 in 300 million at least. So you’d have a higher chance of winning the Powerball lottery than ever spontaneously dying of a freaky disease you read about once in a Top 25 Horrors List.
So, YAY! Basically, go buy yourself a lottery ticket and relax because you probably don’t have prion disease! And you can still nerd out on all my info, y’know, just because knowledge.
The Maybe Bad News?
If you or a loved one is showing symptoms of prion disease and all your doctors have been stumped in terms of a diagnosis, get this ruled out ASAP! Prion disease is known as “the great mimicker” because it seems like so many other diseases, and is so rare that health practitioners hardly ever recognize it. In the extremely unlikely case that it turns out to be prion disease, it does move rather quickly, so the sooner you know, the better.
My doctor friends tell me that in med school, prion disease was described as “this is something you’ll likely never come across in your career.” My dad’s neurologist only figured it out because he happened to have two MRI’s that were done one month apart, and his brain looked significantly different in each MRI — hello, literal rapidly progressive dementia (RPD), a class of diseases to which prion disease belongs.
Now, —repeat after me — just because your doctors are stumped, and you happen to be showing some RPD symptoms, which could also be representative of a ton of other diseases, 👏 DOES 👏 NOT 👏 MEAN 👏 YOU 👏 HAVE 👏 PRION 👏 DISEASE. Don’t jump to conclusions! Get all the bloodwork and neurological tests done so that you can conclusively see.
OK, Fine, The Oogly…
OK, doctors have concluded without a doubt that you or a loved one has prion disease. What now?
First and foremost, I’m so emphatically sorry. Your first step should absolutely be to check out the CJD Foundation website. They’ve been supporting CJD patients and caregivers for a long time, and they know what they’re doing! They have a treasure trove of information, supportive material, resources, and a wide network. Give them a call. I promise you’ll be glad you did.
Everything I include on this site is information I found useful during my own journey with prion disease. I’m going to try my best not to repeat too much information from other websites. Instead, I’ll link all useful information from other websites. Think of this as your one stop shop for prion disease dummies.
FAQs
WTF are prions?
Think of prions as proteins gone bad.
Everyone has “prion protein” in their body which helps the nervous system function via coordination and sensations.
Prions are NOT the same thing as prion proteins!
The word “prion” actually stands for “proteinaceous infectious particle.” Basically, prion protein gone bad and turned infectious, turning other good prion protein into prions. Those prions go on to turn other good prion protein into prions, resulting in exponential growth of prions.
WTF is prion disease, then?
Prion disease is when prions (remember those prion proteins gone bad up above??) start spreading to other healthy prion proteins, turning them bad too. These baddies clump together, attach to a brain cell, and turn it off (aka kill it).
As the patient begins losing brain cells that control various parts of the body, symptoms begin to appear, such as, imbalance and incoordination, memory loss and impaired thinking, and psychiatric symptoms such as anxiety or depression.
Imagine that your brain is your body’s main operating computer, and that it starts malfunctioning one tiny way at a time. That’s how prion disease works.
These prions continue to multiply and kill brain cells at an exponential rate until the patient ultimately dies.
WTF, that sounds terrifying.
Yes, prion disease is every bit as scary as it sounds, if not scarier.
Prion disease is one big motherfucker that I wouldn’t wish on my worst enemy.
If you or someone you know is currently dealing with prion disease, I STRONGLY recommend therapy during and after. I — 10/10 — do not recommend going through this without professional mental health support.
I had a great therapist then, I have a great therapist now, and still, I will say right now that I honestly don’t think I could go thru this again.
Who gets prion disease?
The answer is somewhat complicated. Bear with me.
MOST (~85%) HUMAN prion disease happens in the form of sporadic CJD. Most sporadic CJD patients are in their 60s. If you hear about someone who has prion disease, they most likely have sporadic CJD, and they are most likely in their 50s or 60s.
Exceptions in human prion diseases generally refer to other types of prion disease, such as genetic forms, iatrogenic, and variant CJD.
Mammals other than humans can also get prion disease. The most popular example being bovine spongiform encephalopathy aka mad cow disease. Other examples of mammalian prion disease occur in deer (aka chronic wasting disease), cats, monkeys, sheep (aka scrapie), but — thankfully! — not dogs!
Wait, Is human prion disease the same thing as mad cow disease??
Technically and scientifically, no. BUT, for our intents and purposes, I’m going to say yes. Here’s why:
Prion disease operates via the same mechanism in all mammals. Healthy prion proteins turn into bad prions and spread, and then they attack brain cells. The various names given to each mammalian prion disease specify the mammal that it affects. So technically, a human can’t have mad cow disease, but both humans and cows can have prion disease. Make sense?
Not everyone will agree, but I personally described my father’s illness by saying he had the human version of mad cow disease, just bc way more people have heard of the mad cow epidemic than of any human prion diseases. Plus, all the symptoms are the same: you start losing your mind until you turn into a zombie and then you die.
So if anyone corrects you from now on while you’re talking about prion disease, you’ll know what to say!
Holy shit, I love beef. What are my chances of contracting prion disease from a mad cow?
The statistics of prion disease cases varies from country to country.
Not to be that American asshole who champions American exceptionalism, but…. if you’re in the US, you’re probably going to be fine.
Here’s why.
(Insert international rates of vCJD)
Wait, you said “other types of prion diseases like genetic, iatrogenic, & variant. Wtf are those?
Can I get prion disease from another person? i want to be supportive of a friend but i’m freaked out & don’t want to act like a douchebag.
How thoughtful of you, thank you for asking!
Explain how to be a considerate friend and not an insensitive selfish dickbag.
So how exactly does one get prion disease from another person?
Why do people use all these terms (CJD, FFI, GSS, mad cow disease, scrapie) when they talk about prion disease? I'm confused.
Prion disease is when prions (remember those prion proteins gone bad?) start spreading to other healthy prion proteins, turning them bad too. These baddies clump together, attach to a brain cell, and turn it off (aka kill it).
As the patient begins losing brain cells that control various parts of the body, symptoms begin to appear, such as, imbalance and incoordination, memory loss and impaired thinking, and psychiatric symptoms such as anxiety or depression.The prions continue to multiply and kill brain cells at an exponential rate until the patient ultimately dies.
Why should prion disease matter to anyone who’s not a patient or a caregiver?
MOST (~85%) prion disease happens in the form of sporadic CJD. Most sporadic CJD patients are in their 60s. If you hear about someone who has prion disease, they most likely have sporadic CJD, and they are most likely in their 60s.
Exceptions generally refer to other types of prion disease, such as genetic forms, iatrogenic, and variant CJD.
how can i be a good ally for prion disease prevention?
It all begins with an idea. Maybe you want to launch a business. Maybe you want to turn a hobby into something more. Or maybe you have a creative project to share with the world. Whatever it is, the way you tell your story online can make all the difference.
Tips & Tricks I HONESTLY WISH YOU DIDn’t need
…yet here we are. Sigh, life.