My family has the genetic version of CWD. My grandmother passed before I was born, my oldest aunt passed when I was about 12, and my third oldest aunt passed about a year ago. My grandmother had ten children so there will likely be more over the next decade.
I didnt realize there was a genetic one. Im so sorry for that and your family.
My great aunt had it. She went from walking to the casino 3 blocks away everyday to not being able to put her smoke to her lips in about 2 weeks. The hospital asked to study her body when she passed about a week after the smoke situation. Obviously we said yes. All they told us was that it was likely she ate infected cow as a child and it just decided to pop up, 50 years later.
Sorry to hear. We’re not allowed to give blood because of the potential genetic link, though they were almost certain my grandmother got it from eating while abroad.
It is a terrible thing to witness. A close friend of mine who was in his early 50's passed away from this two years ago. Two months from onset symptoms to passing away.
yeah, they also pointed out how it dangerously contaminates everything it comes in contact with from the meat grinders, to the surgical equipment used on someone who eats it and catches it, to the environment if you just leave it in the woods until it decays complely. prions are terrifying
You forgot a big one: prions can be absorbed and accumulated in plants if an infected animal dies and decays next to them. You could be a vegan and still get it, in theory anyway.
Can you elaborate? Prions replicate by damaging proteins similar to themselves. All known mammal prions affect brain and nerve tissues. How would plants have proteins similar to mammalian brains and nerves?
The CDC still maintains that it can’t spread to humans and claims there have been no cases (yes even after this thing). However, it’s been found to theoretically be able to transform human PrP in a lab environment, albeit very inefficiently. So I would say spread is real but would take a set of rare circumstances to occur. Either way do NOT touch CWD infected or untested deer meat.
Edit: This comment is not in defense of the CDC’s position, just pointing out what they said in response to these cases
The concerning thing is that cooking the meat can’t fix it, you need to denature the prions at about 170 C to destroy them and even then it’s not a given.
Their stability is what makes them so dangerous. Misfolded prions are too stable to be broken down by the body and they can "convince" non-misfolded prion proteins to be misfolded so they just... pile up forever
To conduct the study, the scientists prepared a paste of scrapie prion-infected brain tissue mixed with hot dogs. They then exposed the paste to temperatures of 120-135 degrees Celsius (250-275 degrees Fahrenheit) and short bursts of ultra high pressure, in excess of 100,000 pounds per square inch. The scientists found that they were able to retain the basic texture and flavor of the processed meat while reducing the prions to non-infective levels. This may have application in improving the safety of meat products.
This is like that mosquito shit that happened...a month or 2 ago? There was a post saying there were no mosquitos in Iceland, then a day later there was a post saying the first mosquito was spotted in Iceland. I like to think that someone read the first post, grabbed a mosquito and took it to Iceland to fix the non-issue..
There are lots of people that don't care about CWD and eat deer infected with it. People refuse to even test for it in highly infected areas. I think it is crazy, but people do a lot of dumb stuff. Similar vein to the anti vax movement. If you don't know anyone that has CWD/polio/measles/etc. then it's all a big conspiracy.
I used to volunteer for a hunting organization that tried to educate people on CWD. Whenever I went to game fairs, the most stubborn, ignorant people were always from areas that had the highest prevalence of CWD. I encountered more than a few who thought it was all a conspiracy by liberal DNR agents to eradicate deer to prevent people from hunting. It was bizarre.
Man, during my night shift last night I was researching about prions and CWD from that tainted deer meat someone was trying to get rid of on marketplace. Less than 8 hours ago I was reading how it had NEVER been transmitted to humans, and now here we are. Scary shit.
I saw that yesterday and was screaming into the void (my husband) about how INSANE that was. Then he sends me this not five minutes ago. I’m still screaming. 💀
In April 2024, a report was published regarding two hunters from the same lodge who, in 2022, were found to be afflicted with sporadic CJD after eating deer meat infected with chronic wasting disease (CWD), suggesting a potential link between CWD and CJD
This is why I refuse to kayak in orca zones, despite everyone chanting about wild ones never harming humans. I've lived through too many unprecedented events involving nature, thank you. I'm not rolling the dice on becoming a first with intelligent murder dolphins that torture their prey for amusement and skill building.
Hopefully I didn't just jinx the hell out of someone, the way things are going.
Was reading about this yesterday too since someone posted their infected deer meat that they were just giving away. Which made me think, yeah, someone could buy that for their dog for cheap meat. Which made me think... yeah, that means prions will pass through their dogs system and end up in their backyard and into their soil. A backyard food garden could theoretically be contaminated. And prions aren't living, so they can survive in the soil forever. So yeah, this all seems like really fucking bad news.
I remember comments in that post saying it's rare and can take 20-30 years to develop symptoms when people get infected. I've had questionable venison from hunters that were family friends in my teen years and now I'm in my 30s - wouldn't that be a wonderful surprise to develop symptoms suddenly in the next few years?
prions are damn near indestructible, they linger in the environment. the prevailing theory on how deer get infected is thru the environment (eating tainted grass or ticks) because deer arent eating each other which is normally how transmission occurs
in fact, recent studies show that prions in soil can effectively infect vegetation growing in the area
This is so strange, I work for a pet food company and I was learning about “mad cow disease” and in the end I thought “no moron would eat meat from a mad cow” so I thought no other way for us to get it…well shit.
There was someone selling deer meat that was contaminated with CWD on Facebook market place. Basically, "I wont eat it but you can." Im still flabbergasted by the post.
Giving it away for free. To whomever wanted to risk mad cow disease. AND, they only called it CWD positive, and did not elaborate on what CWD means. So, likely someone took the free meat, not understanding what it was infected with.
ETA: CWD is Mad Cow Disease, and it recently has been confirmed that it can be passed from deer/venison to humans. There is no way to make the meat safe for any consumption. Cooking, cleaning, disinfecting, sanitizing -> none of these destroy prions.
a small pedantic correction from a prion enthusiast: mad cow disease is BSE, bovine spongiform encephalopathy. CWD is chronic wasting disease, affecting cervids. they're both spongiform encephalopathies, but not the same disease. not just because of which animals are affected and how, but also because BSE jumps species – and infects humans, thereby transforming into variant Creutzfeld-Jacob disease (vCJD) – much more readily than CWD... for now, anyway.
I did have a patient once that was fine one day , on cruise ship, then literally bat shit crazy a few days later, like his mind was completely gone in few weeks. Docs were thinking it could be CJD. I think there are three types of prion disease. 2 that aren’t even related to eating meat.
There’s the sporadic one, which happens fast.
The genetic one that shows up later in life
And the mad cow one that can incubate for several years .
Scary as hell. Wash your fruits & vegetables too !
I saw a post I assumed was a joke about CWD Deer Meat for free on FB Marketplace.
Then I remembered people are fuxking stupid and use FB for 'research' and absolutely would eat that meat for free, even though the hunter and his buddy turned it down.
You get to do that human thing where we forcefully create patterns that don't exist and start to believe that perhaps you reading about it triggered the event to happen.
I remember finding a box in my attic that had various regular magazines, but also a few playboys, and audibly wondering if Hugh Hefner was still alive. The next morning the news was reporting that he was dead. I felt like I somehow killed the guy by mentioning him for the first time in years, regardless of how dumb that actually sounds.
This is completely false. Disingenuous misinformation.
2 hunters did develop CJD, however, there is no evidence it is related to CWD. This idea originates from an abstract for a poster that was presented at AAN last October (link). Poster abstracts can often be “sensationalized” to encourage people to come to the poster during the conference. This would not be the first AAN prion-related poster abstract that is a bit “click-baity”. Furthermore, poster abstracts are not peer-reviewed. The fact this research has not been written up as a peer reviewed paper 1.5 years later (not even a pre-print) is INCREDIBLY telling.
The biggest issue comes down to this: how do you know the hunters got infected with CWD (ie how do you know their CJD is related to CWD)? The most common type of prion disease is sporadic CJD (sCJD), specifically the subtype the hunters developed sCJDMM1. Sporadic means it happens with no known cause (ie not caused by infection or genetics). sCJD typically affects elderly (median age of onset 65 years), has a rapid decline (4 months). In comparison, variant CJD (vCJD) is caused when someone eats meat contaminated with mad cow disease (BSE). vCJD typically affects young people (median age of onset 26 years) and has a slower decline (14 months). Furthermore, sCJD is associated with motor and memory symptoms whereas vCJD is associated with psychiatric symptoms. The patient case in the abstract was a 72 year old male, with a rapid memory decline in a month. Does that seem more like sCJD or vCJD? I will make it very clear, the case presentation is 100% classical sCJD. Furthermore, it is hypothesized that if CWD does transmit to humans it would have an atypical phenotype (ie not present anything like sCJD). This is based on data from both NHPs and Tg Mice (here is the mouse paper).
Other considerations, the patient was originally diagnosed with sCJD, however, the authors hypothesize CWD because one of the patient’s friends also died recently of sCJD. The authors therefore argue this is a “cluster” of cases, likely showing infections. However, just because two rare independent events happen in close proximity does not mean they are linked. For example, 1 in 300 people get Parkinson’s Disease, but a TV show “Leo and Me” had 4 out of 125 people on set develop Parkinson’s Disease, including Micheal J. Fox. However, this “cluster” is not scientifically or statistically significant. Just like two people who happen to know each other both getting CJD is not significant.
Clusters of prion cases happen. Here is a spatial-temporal cluster of 5 cases (including 2 with similar occupations). It wasn’t big news because stuff like this happens. It gets written up in a paper and the general public never knows. The only reason the hunters story is widespread is because it fits the fear mongering narrative most people push about prions.
The second consideration is that just because someone is a hunter and eats deer does not mean that they have eaten CWD infected deer. Not every deer in a population has CWD. If you are going to claim the hunters got CWD from deer, you need to show that deer they have hunted are infected. The entire premise of this abstract hinges on the argument that they ate infected deer, despite providing no evidence for it. If these two patients who both got CJD that knew each other were not hunters, this would never of been reported. Correlation does not imply causation. That’s basic first year statistics.
This also ignores significant biochemical and cellular data suggesting that CWD is not transmissible to humans based on significant sequence differences between cervids and humans. Here is a pretty comprehensive review of the experiments show CWD does not transmit to humans (link).
Also the picture in the post is a picture a deer with CWD from a paper published 20 years ago. It is not show neuropathological change in the hunters brain. It is a deer that got a deer prion disease. (Link)
It isn’t just my opinion that the hunters had nothing more than sCJD, it is also the opinion of the CDC which investigated these cases. It is important to note the authors of the abstract say "causation remains unproven". That means the title of this post does not even fit the abstract that originates it.
I'm also a neurodegeneration scientist and neurologist. I've been posting similar rebuttals whenever this story gets posted.
They've never followed up this non-peer reviewed poster with the actual data and autopsy reports. While clusters should be investigated (and this one was) to see if there was something unusual, but the fact that two people who know each other got CJD is not unexpected. Humans tend to overinterpret cluster which is called the clustering illusion.
If I flipped a coin 100 times, chances are I'd get heads or tails 5-6 times in a row at some point. If the string of 5 heads in a row gets written up in article, it'd sound significant. But since we're tracking every flip (and every CJD case gets reported to state health agencies), we expect to see clusters.
While we have two experts in the room, let me ask something that's been bugging me for years:why don't humans contract CWD, or CJD from deer infected with CWD-associated prions?
Humans can get mad cow disease from infected cows, and deer can pass it to each other without (I assume) having to consume CNS tissues from other deer. If prions are just misfolded proteins, are human proteins sufficiently distinct from the affected deer proteins that it's a non-issue?
I legit sighed seeing this and sighed further having to scroll so far seeing an actual scientist responding to this particular case. I remember this coming out I was like ruh roh then literally did the squidward lawn chair meme when I read it was sporadic. There was a similar case in the early 2000s involving three much younger CJD victims that was initially very suspicious but was deemed unrelated to CWD.
Also, don’t vCJD and sCJD look quite different when you do PMCA on the gels? Like you get a very distinct banding pattern difference? I looked for the study and did not see any physical data like a PMCA. I’m not an actual prion researcher but I do work with prions infected animals. This case hit headlines right as I was doing my senior capstone which I chose vCJD to present on and I made a slide on my 45 minute presentation explaining why it isn’t a causal link between CWD.
I can't be the only one with that copypasta living rent free in my head, right?
Rabies is scary.
Rabies. It's exceptionally common, but people just don't run into the animals that carry it often. Skunks especially, and bats.
Let me paint you a picture.
You go camping, and at midday you decide to take a nap in a nice little hammock. While sleeping, a tiny brown bat, in the "rage" stages of infection is fidgeting in broad daylight, uncomfortable, and thirsty (due to the hydrophobia) and you snort, startling him. He goes into attack mode.
Except you're asleep, and he's a little brown bat, so weighs around 6 grams. You don't even feel him land on your bare knee, and he starts to bite. His teeth are tiny. Hardly enough to even break the skin, but he does manage to give you the equivalent of a tiny scrape that goes completely unnoticed.
Rabies does not travel in your blood. In fact, a blood test won't even tell you if you've got it. (Antibody tests may be done, but are useless if you've ever been vaccinated.)
You wake up, none the wiser. If you notice anything at the bite site at all, you assume you just lightly scraped it on something.
The bomb has been lit, and your nervous system is the wick. The rabies will multiply along your nervous system, doing virtually no damage, and completely undetectable. You literally have NO symptoms.
It may be four days, it may be a year, but the camping trip is most likely long forgotten. Then one day your back starts to ache... Or maybe you get a slight headache?
At this point, you're already dead. There is no cure.
(The sole caveat to this is the Milwaukee Protocol, which leaves most patients dead anyway, and the survivors mentally disabled, and is seldom done).
There's no treatment. It has a 100% kill rate.
Absorb that. Not a single other virus on the planet has a 100% kill rate. Only rabies. And once you're symptomatic, it's over. You're dead.
So what does that look like?
Your headache turns into a fever, and a general feeling of being unwell. You're fidgety. Uncomfortable. And scared. As the virus that has taken its time getting into your brain finds a vast network of nerve endings, it begins to rapidly reproduce, starting at the base of your brain... Where your "pons" is located. This is the part of the brain that controls communication between the rest of the brain and body, as well as sleep cycles.
Next you become anxious. You still think you have only a mild fever, but suddenly you find yourself becoming scared, even horrified, and it doesn't occur to you that you don't know why. This is because the rabies is chewing up your amygdala.
As your cerebellum becomes hot with the virus, you begin to lose muscle coordination, and balance. You think maybe it's a good idea to go to the doctor now, but assuming a doctor is smart enough to even run the tests necessary in the few days you have left on the planet, odds are they'll only be able to tell your loved ones what you died of later.
You're twitchy, shaking, and scared. You have the normal fear of not knowing what's going on, but with the virus really fucking the amygdala this is amplified a hundred fold. It's around this time the hydrophobia starts.
You're horribly thirsty, you just want water. But you can't drink. Every time you do, your throat clamps shut and you vomit. This has become a legitimate, active fear of water. You're thirsty, but looking at a glass of water begins to make you gag, and shy back in fear. The contradiction is hard for your hot brain to see at this point. By now, the doctors will have to put you on IVs to keep you hydrated, but even that's futile. You were dead the second you had a headache.
You begin hearing things, or not hearing at all as your thalamus goes. You taste sounds, you see smells, everything starts feeling like the most horrifying acid trip anyone has ever been on. With your hippocampus long under attack, you're having trouble remembering things, especially family.
You're alone, hallucinating, thirsty, confused, and absolutely, undeniably terrified. Everything scares the literal shit out of you at this point. These strange people in lab coats. These strange people standing around your bed crying, who keep trying to get you "drink something" and crying. And it's only been about a week since that little headache that you've completely forgotten. Time means nothing to you anymore. Funny enough, you now know how the bat felt when he bit you.
Eventually, you slip into the "dumb rabies" phase. Your brain has started the process of shutting down. Too much of it has been turned to liquid virus. Your face droops. You drool. You're all but unaware of what's around you. A sudden noise or light might startle you, but for the most part, it's all you can do to just stare at the ground. You haven't really slept for about 72 hours.
Then you die. Always, you die.
And there's not one... fucking... thing... anyone can do for you.
Then there's the question of what to do with your corpse. I mean, sure, burying it is the right thing to do. But the fucking virus can survive in a corpse for years. You could kill every rabid animal on the planet today, and if two years from now, some moist, preserved, rotten hunk of used-to-be brain gets eaten by an animal, it starts all over.
So yeah, rabies scares the shit out of me. And it's fucking EVERYWHERE. (Source: Spent a lot of time working with rabies. Would still get my vaccinations if I could afford them.)
I thought it was a liver or kidney but the corneas had also been donated so they had to remove them from the recipients. Did one of those people get it too?
I give people rabies shot pretty regularly. Both the regular vaccine to prevent it, and the igg if they have or think they have been in contact with an infected animal.
I don't feel nearly as concerned with it, although I get your point of "once you see the symptoms, you're already dead" kind of thing.
However, if I contact a potentially rabid animal, I'd go get the shots and feel very safe. If I ate meat and then later found it was prion contaminated, I think I'd exist in a state of dread for years, if not decades. Waiting for my brain to turn into swiss cheese. True horror.
WHAT. I THOUGHT YOU HAD TO CONSUME CONTAMINATED MEAT TO CONTRACT A PRION DISEASE. WELL, TIME TO START WORKING ON MY OCD AGAIN BECAYSE GODDAMN SHE IS GONNA LOVE THIS TIDBIT OF KNOWLEDGE.
Can I ask what is supposed to happen once you shoot the infected deer? Surely the prions remain infectious and eventually make their way into plant diet of later deer? Or other animals like vultures or things that hunt vultures or something?
Typically DNR would come and dispose of the animal carcass. In my area there is information posted online about proper disposal (double bagged in heavy garbage bags and drop off locations for incineration) as well as how to disinfect your equipment if used in processing an animal confirmed to have had CWD.
Reason it needs to be incinerated is as you mentioned, the prions persist and will potentially infect any other animal that eats from the carcass. I don’t recall if there is any steps required to disinfect ground that had blood spilled on it.
For hunters in my area, there is mandatory sample submission for harvested deer. I got my results back last week confirming my harvest was negative for CWD. Took a few weeks, but now I get it enjoy my venison worry free. There isn’t much for enforcement of the mandatory submission, but it’s free, relatively hassle free (take the whole head, or properly harvested biological samples to a drop off depot, fill out a form, get a free hat, and then wait), and leaves you with no doubt about the safety of your meat. If a hunter cares at all about conservation, it’s a no brainer to submit samples for testing as it only helps wildlife agencies to track and implement controls to help prevent the spread of CWD.
You’re very welcome. And thank you for my first ever reddit award.
And I would, but I actually don’t have a photo of it. Apparently there were a couple options based on what some friends have told me. But the one I received was actually a hunter safety blaze orange toque that says “Wildlife Safety Cooperator” on it. The other option was a camo baseball cap with the same words, but I was never given the choice to pick between them (I would have chosen the toque anyway).
Or just you know knowing this is a growing possibility in North America just stop eating hunted venison. Hunt them to cull populations if necessary but yea, I come from a deer hunting area and sorry to say but knowing this I'm never eating deer some random family member or acquaintance hunted ever again.
It freaks me out reading these articles, I'm from the UK and ate beef during the dreaded timeframe. Are they now allowing blood donations due to the risk being unlikely, or better screening, I wonder? Hopefully, due to an unlikely risk!
In 2024, doctors reported two hunters who hunted together at the same lodge both got a super-rare brain disease called sporadic Creutzfeldt-Jakob Disease (CJD), the human version of mad cow disease, after eating deer from areas riddled with Chronic Wasting Disease (CWD).
What are prion diseases? Think of prions as zombie proteins. Normal proteins in your brain fold one way to work properly. Prions are the same protein but folded wrong, they act like a virus that "infects" good proteins, turning them bad too. The bad proteins clump up, destroy brain cells, and turn your brain into Swiss cheese. No symptoms for years/decades, then sudden dementia, shaking, and death in months.
What happened to these hunters? Both developed sporadic Creutzfeldt-Jakob Disease (CJD), a prion disease that normally affects only 1-2 people per million each year. Two hunters from the same small group getting it is statistically practically impossible, unless linked. They had both consumed venison from areas where Chronic Wasting Disease (CWD), the deer version of mad cow disease, is endemic.
What makes this particularly concerning is that Chronic Wasting Disease now affects deer populations across North America, and the prions causing it remain infectious in soil for decades. For years, scientists believed a strong species barrier protected humans from CWD, but a 2022 study using humanized mice demonstrated that CWD can cross to human tissue, contradicting earlier assumptions. Even more alarming, research suggests CWD could present differently in humans than typical prion diseases, meaning cases might be misdiagnosed with current screening methods.
What's truly horrifying about prions is they contaminate everything they touch, permanently. Grind your CWD-infected deer meat in a shared grinder? It's contaminated forever. Have prions and need brain surgery? Those surgical tools must be incinerated at 1000°C or soaked in caustic chemical baths for hours, nothing else works. Dump the carcass in the woods? Prions bind to soil minerals and stay infectious for decades, reinfecting new deer. No bleach, no autoclave, no normal sterilization gets rid of them. Only cremation or extreme chemical exposure for hours destroys them.
Prions aren't alive, they don't have DNA/RNA that you can target or kill. This makes them insanely hard to destroy because normal sterilization works on living pathogens by disrupting their biology, but prions have no biology.
All prion diseases are 100% fatal and incurable. The incubation period could be decades, so if this cluster represents actual transmission, we won't know the true scope for years.
Miced humans haven't been successfully created. The "quiet as a mouse" part is where it always goes wrong. Humans are just slightly sophisticated apes; can't help constantly vocalizing nonsense. Source: Worked in cube farm for many years.
Here is the 2022 paper they refer to (Link). They use mice that have the mouse gene for the prion protein removed and the human gene for the prion protein added back in on an artificial chromosome. This paper was poorly done (that hurts me to say as I know the authors of this paper, but I have shared my opinion to them before). The conclusion from the paper should be, CWD is unlikely to transmit to humans.
Prions are so scary. I am a Biologist and when I first learned about them during my Bachelor's degree I was astonished about how long they survive on the soil.
I am a bit blurry on the details but I believe we were told about entire Scottish isles being prohibited from being used as grazing grounds for this exact reason
Another topic we discussed were certain cannibalistic cultures where mainly men were suffering from prion diseases. The reason? Men get to eat certain parts of the body the women don't and these parts tend to be riddled with prions.
They are so damn scary since you pretty much can't do anything about them
Cannibalizing the dead was respect among the Fore people in Papua New Guinea. Women and children were given the brain and spinal cord and thus eight times more of them got kuru compared to the men. Daniel Gadjusek got a Nobel prize for proving transmissibility of the condition.
Your mention of brains made me think of the gentleman I cared for as a nursing student here in Canada (1992 grad here) who had CJD, from a trip and had eaten sheep brains. Memorable to me as little old naive me did not comprehend how people ate that part of an animal (yes I know it can be cultural in some cases but for me personally would not do it, instant stomach turning/ick factor) and how dramatcally fast this gentleman deteriorated and died within a mere few days. 😕 many medical students involved with him as well as a case study, and our instructor said that likely may never see this again in our career, it was so rare.
I was at one of those Brazilian all you can eat grills where they serve endless meats. They came over once with organ meats, including brain, and I just remember thinking "wow, even after you cook it, it still looks just like brain..." I guess I thought it would transform at least a little.
I just remember reading it takes serious heat or some strong acids to break down the prions. Heat high enough for cremation essentially so normal cooking methods won’t be enough.
on the cannibal part does that mean that the human victims of the cannibalistic tribe already had prions they were infected with? Or does the digesting of certain parts cause misfolding to happen after the fact?
The first. Since the "victims" in this case we're deceased members of the tribe, the prions would just be passed along from generation to generation within it.
You can get endogenous dementia too, prions are just misfolded proteins and can happen later in life just like cancer. You would likely die before the concentration of them becomes large enough to actually cause dementia, but if you're eating the brains of other older folks that concentration increases dramatically
Look up “chronic wasting disease”. The deer are very much symptomatic. They are often described as “zombie deer”. They shake, they fall over a lot, they stumble around. Eventually they forget how to eat and drink or lack the muscle control to do so and then die.
No bleach, no autoclave, no normal sterilization gets rid of them.
What? How does an autoclave not nuke the protein? Seems unbelievable
Edit: I looked it up. "High-Temp/Prions: 134°C (273°F) for at least 18 mins." That makes way more sense. It seemed unbelievable that you could not destroy a protein with heat.
Yeah a lot of stuff like sterilising equipment at 1000 degrees is just adding a "we better be fucking sure" safety factor. It also depends on the protein, but I'd be surprised if any organic matter survived intact much higher than its own autoignition temperature.
The reason they're so heat-tolerant though is through being misfolded they're actually more stable, so don't denature like normal proteins do.
Just to add to the fun - some prions aren't destroyed by cooking or other conventional food preservation processes. Even if your "at risk" meat is "well done", dried, smoked or cured doesn't mean it is safe.
Not only that, prions shed by deer can get uptaken in plants and can infect someone that ate the plant. They are virtually indestructable. Chlorine will not even denature them. Not sure about sustained uv?
I believe that’s what happened to a cousin of mine. He was a lifelong hunter and fisherman for his hobbies. He especially enjoyed deer hunting.
He was the much-loved son of an uncle and his family didn’t want to say too much about it. But it was a neural degenerative disease specifically tied to deer and infected meat. His doctor has described it as the negative lottery.
From the little I heard, he exhibited severe short term memory loss. Sounded like a rough way to go but he was resigned to it.
Not dismissing the possible link to his lifestyle, but you can develop CJD spontaneously. We just had a close family friend die of it over the summer, literally 1 or 2 in a million. They were in their late 50s, over the course of about 6 weeks she suffered short term memory loss that looked like early onset dementia. Whilst awaiting investigation and diagnosis for that, their behaviour and personality altered rapidly, eventually resulting in two A&E incidents that were described as ‘psychotic breaks’. Shortly after they had a serious fall in the kitchen (in hindsight gross motor problems building). They were home for maybe a week at most when they collapsed and were taken to hospital, CJD diagnosis after 3 days on intensive care, and they passed something like 10-14 days later.
Whilst tragic and not pleasant at the time, especially for their partner, we all took solace in the fact it attacked very rapidly and there wasn’t a long drawn out decline, especially versus some losses we’ve had to dementia and cancer. Indeed, for the final 2 weeks they weren’t even conscious or aware.
Hmm… not sure where all he did his hunting but he was living in Georgia. I’m sure he would go hunting and fishing all over though but I know he did make it specifically to Florida, the Carolinas and Alabama.
He passed away about 2 years ago I think.
One of the crazy things I heard was he would eat something… and he’d forget he had eaten so he’d eat again or have to be reminded that he’d already eaten.
The X Files has an episode where a community of cannibals get CJD from eating one another. It absolutely terrified me, and made me scared of prion diseases.
I am curious if this effects the Amish community at all. We have a ton of deer where I am and they pretty much live off the meat over anything else.
that was sort of based off a true story. Tribes in Paua New Guinea practised cannibalism up until relatively recently. A disease called, IIRC, Kuru, killed many women. Turns out it was CJD and transmitted by the women eating the brains of fallen warriors from tribal skirmishes.
It was a cultural tradition of respect and mourning, so they were eating the remains of pretty much anyone, including those that died from the disease, over and over. Add to that, men ate the meat while women, who also had to prepare the feast, ate the remaining parts, including the brain.
I sat in on a presentation given this Fall by the VA department of game and I didn't come away reassured at all. Not only do they still not know whether or not it can be transmitted to humans (the OP's case so far is the only ones that imply it can be), it's almost certain you will harvest an infected deer in some parts of my state. In one county, over 40% of the tested deer were infected. It so happens that county is one I used to hunt in.
I gave up deer hunting a few years ago as a result. It helped that nobody in my family likes venison, so I ended up giving away much of what I harvested. Still, I liked all of the outdoors aspects of hunting deer; being in the woods, doing the prep work to identify their movements and where they were that season, watching the sunrise or sunset from my tree stand, and watching other animals while out hunting (such as sitting on a hillside enjoying a snack and watching a bear who was oblivious to my presence walk down the same hillside about 50yds away).
Hey my mom died from that ! Contamined by the cow meat pandemic from UK in the 1990's that shit can take up to 20years of incumbation in your body before waking up
Yep my coworkers dad died from it unexpectedly. Was in perfect health for his age then suddenly he was burning foods all the time without even realizing he had done it. Would just forget where or who people were. Few weeks later he was dead.
The doctors told the family they could get tested for cjd and they all outright refused. Who wants to know that there’s a ticking time bomb in their body that may or may never blow up? Plus my coworker didn’t want to have a panic attack everytime he forgot something.
My mum was a pathologist and there were only a few things that really worried her. The first and biggie was some of the germ warfare stuff the Soviets were working on in the 70s and 80s (she worked with the Royal Navy for 24months). The second was BSE and CJD prions. They cannot be killed, cannot be cleaned off of tools, and cannot be cured.
My wife's uncle died from CJD. If ever there was an argument for the legality of assisted suicide CJD is it. Nightmare does not even come close to describing what we witnessed him experiencing when we saw him shortly before his death.
As others have said, the only way to know is to have the animal tested. Here in VA there are dropboxes for this very thing. Unfortunately, it takes a few weeks to get the results back, so you have to have a way to store the carcass in the meantime.
I just saw a post of someone trying to get rid of CWD infected meat on facebook marketplace saying there’s no proof it can spread to humans.. wtf
Saw that as well. Glad I didn’t eat that much of it.
My great uncle passed from CJD, it's a terrible thing to witness
My grandmother in the early 80s. They barely knew what it was then. It’s tough.
My family has the genetic version of CWD. My grandmother passed before I was born, my oldest aunt passed when I was about 12, and my third oldest aunt passed about a year ago. My grandmother had ten children so there will likely be more over the next decade.
I didnt realize there was a genetic one. Im so sorry for that and your family.
My great aunt had it. She went from walking to the casino 3 blocks away everyday to not being able to put her smoke to her lips in about 2 weeks. The hospital asked to study her body when she passed about a week after the smoke situation. Obviously we said yes. All they told us was that it was likely she ate infected cow as a child and it just decided to pop up, 50 years later.
Omg that is horrifying. I’m sorry you and your family had to go through that.
Sorry to hear. We’re not allowed to give blood because of the potential genetic link, though they were almost certain my grandmother got it from eating while abroad.
A friend of mines husband has recently been diagnosed with this, its insane how quickly it has progressed. It truly seems awful.
Coworkers dad was diagnosed CJD positive and died within 3 weeks of symptom onset. Scary stuff.
It is a terrible thing to witness. A close friend of mine who was in his early 50's passed away from this two years ago. Two months from onset symptoms to passing away.
yeah, they also pointed out how it dangerously contaminates everything it comes in contact with from the meat grinders, to the surgical equipment used on someone who eats it and catches it, to the environment if you just leave it in the woods until it decays complely. prions are terrifying
You forgot a big one: prions can be absorbed and accumulated in plants if an infected animal dies and decays next to them. You could be a vegan and still get it, in theory anyway.
Okay now listen. This disease already was scary enough but now it sounds like you can hardly escape the damn thing :(
The real zombie apocalypse genesis story.
There is no part of prion disease that is not humanity-ending terrifying.
Can you elaborate? Prions replicate by damaging proteins similar to themselves. All known mammal prions affect brain and nerve tissues. How would plants have proteins similar to mammalian brains and nerves?
The short answer is we know it happens but don't understand the mechanism.
https://www.usgs.gov/centers/nwhc/news/plants-vectors-environmental-prion-transmission
Prions are extremely stable. The plants may just be a reservoir with no need for the Prions to replicate.
So basically every meat processor has been exposed so every person is exposed.
...and if you're infected by only a small amount, it can take years or decades before symptoms show.
OP also saw that post, as this was discussed there immediately
The CDC still maintains that it can’t spread to humans and claims there have been no cases (yes even after this thing). However, it’s been found to theoretically be able to transform human PrP in a lab environment, albeit very inefficiently. So I would say spread is real but would take a set of rare circumstances to occur. Either way do NOT touch CWD infected or untested deer meat.
Edit: This comment is not in defense of the CDC’s position, just pointing out what they said in response to these cases
You've already field dressed the deer before you get it tested though. Like what should you do get in full hazmat suit to dress a deer?
Ideally, you wouldn’t hunt in areas with CWD (which is publicly available info) and wouldn’t shoot any deer with obvious visual signs of CWD.
100% of my state has a theoretical possibility of CWD. Hunting is like one of our #1 “exports” or whatever it’s called.
The concerning thing is that cooking the meat can’t fix it, you need to denature the prions at about 170 C to destroy them and even then it’s not a given.
Their stability is what makes them so dangerous. Misfolded prions are too stable to be broken down by the body and they can "convince" non-misfolded prion proteins to be misfolded so they just... pile up forever
Wild that they just bust in to your body like "Hey guys, how about instead of what you're doing, you do this." Then you die horribly.
So they are the biological equivalent of private equity firms?
well, minus all the insufferable MBAs
I happen to know that if a person is infected with CJD, and have surgery, the instruments are incinerated instead of sterilized and reused.
Yes and in the uk patients are now cohorted for some equipment due to the CJD outbreak in the 90s!
170C won't even make the prions sweat.
You need to cook them at 900F for several hours, so about 500C for several hours to be safe.
Just have to pressurize it a bit.
https://www.infectioncontroltoday.com/view/pressure-combined-heat-reduces-prion-infectivity-processed-meats
Ah yes. Just short burts of slightly more than 6x the PSI at the bottom of Mariana's Trench. Just a bit of pressure.
“Welcome to Hydraulic Press Channel, today we are going to denaturize some prions. They are extremely dangerous, so we have to deal with them.”
Ah yes, the greater-than-zero but completely reassuring "non-infective levels."
The only way to guarantee you destroy any prions is by incinerating them. There's no other way
What if this is the start of the end? Since its has been processed probably every meat going through that will be infected and we all get infected /j
Saw that and was the first to reply to that post with a link to an article discussing these two cases.
I was literally looking up Cervine - Human prion transmission yesterday during a dull bit at work.
I had exactly 22 hours of peace of mind about CWD transmissions in my life - nice.
https://preview.redd.it/5mstlygt4l6g1.jpeg?width=1170&format=pjpg&auto=webp&s=db51066d0c6a1fc3074a977c395fdce42be1ba8f
https://preview.redd.it/aefhcitu4l6g1.jpeg?width=1170&format=pjpg&auto=webp&s=abb4a4a1265ba616f4b9e30def05a93ee4180198
Indeed
This is like that mosquito shit that happened...a month or 2 ago? There was a post saying there were no mosquitos in Iceland, then a day later there was a post saying the first mosquito was spotted in Iceland. I like to think that someone read the first post, grabbed a mosquito and took it to Iceland to fix the non-issue..
It’s not that they were found there it’s that they have successfully bred there for the first time in recorded history
Burn the country down I’ll live in the ash mosquito free, happily
FREE FOR THE TAKING??? The fuck?
“Oh, this meat is infected with a deadly disease.
…
……..
…………want it?”
Right??!! The only people you should be giving that away to is pathologists/infectious disease researchers..
You're not supposed to eat it. I have no idea why they would try to give it away. It's useless. It also stays in the ground for ages and ages.
Some of the comments implied that old people could eat it since CJD takes years to show up. But these hunters seemed to get rapid onset.
Free to good home:
Is visibly rabid raccoon.
There are lots of people that don't care about CWD and eat deer infected with it. People refuse to even test for it in highly infected areas. I think it is crazy, but people do a lot of dumb stuff. Similar vein to the anti vax movement. If you don't know anyone that has CWD/polio/measles/etc. then it's all a big conspiracy.
I used to volunteer for a hunting organization that tried to educate people on CWD. Whenever I went to game fairs, the most stubborn, ignorant people were always from areas that had the highest prevalence of CWD. I encountered more than a few who thought it was all a conspiracy by liberal DNR agents to eradicate deer to prevent people from hunting. It was bizarre.
There it is..
This should be criminal.
Man, during my night shift last night I was researching about prions and CWD from that tainted deer meat someone was trying to get rid of on marketplace. Less than 8 hours ago I was reading how it had NEVER been transmitted to humans, and now here we are. Scary shit.
Tell me it was the Wisconsin fb marketplace listing
Last year we had one listed in idaho. The raw milk crowd literally ate it up.
Knowingly consuming bad meat products is absolutely wild.
Knowingly consuming any potentially tainted food is wild to me. Feeding it to your kids because science is evil is just diabolical.
God. I wish I had never read that
I saw that yesterday and was screaming into the void (my husband) about how INSANE that was. Then he sends me this not five minutes ago. I’m still screaming. 💀
In April 2024, a report was published regarding two hunters from the same lodge who, in 2022, were found to be afflicted with sporadic CJD after eating deer meat infected with chronic wasting disease (CWD), suggesting a potential link between CWD and CJD
This is why I refuse to kayak in orca zones, despite everyone chanting about wild ones never harming humans. I've lived through too many unprecedented events involving nature, thank you. I'm not rolling the dice on becoming a first with intelligent murder dolphins that torture their prey for amusement and skill building.
Hopefully I didn't just jinx the hell out of someone, the way things are going.
Killing humans is the LAST stage of the training. Cute interactions with kayaks are just intelligence gathering for all we know.
Was reading about this yesterday too since someone posted their infected deer meat that they were just giving away. Which made me think, yeah, someone could buy that for their dog for cheap meat. Which made me think... yeah, that means prions will pass through their dogs system and end up in their backyard and into their soil. A backyard food garden could theoretically be contaminated. And prions aren't living, so they can survive in the soil forever. So yeah, this all seems like really fucking bad news.
I remember comments in that post saying it's rare and can take 20-30 years to develop symptoms when people get infected. I've had questionable venison from hunters that were family friends in my teen years and now I'm in my 30s - wouldn't that be a wonderful surprise to develop symptoms suddenly in the next few years?
Soil bacteria can break down proteins. Soil moisture and temperature are among the variables affecting bacterial activity.
The most common risk in backyard fecal matter are parasites. Deer stools are particularly dangerous for parasite risk.
prions are damn near indestructible, they linger in the environment. the prevailing theory on how deer get infected is thru the environment (eating tainted grass or ticks) because deer arent eating each other which is normally how transmission occurs
in fact, recent studies show that prions in soil can effectively infect vegetation growing in the area
Prions are proteins, however, they can be active in soils for several years.
This is so strange, I work for a pet food company and I was learning about “mad cow disease” and in the end I thought “no moron would eat meat from a mad cow” so I thought no other way for us to get it…well shit.
There was someone selling deer meat that was contaminated with CWD on Facebook market place. Basically, "I wont eat it but you can." Im still flabbergasted by the post.
Wait just a moment please….my brain needs to catch up here… So this person was knowingly selling diseased meat?
Giving it away for free. To whomever wanted to risk mad cow disease. AND, they only called it CWD positive, and did not elaborate on what CWD means. So, likely someone took the free meat, not understanding what it was infected with.
ETA: CWD is Mad Cow Disease, and it recently has been confirmed that it can be passed from deer/venison to humans. There is no way to make the meat safe for any consumption. Cooking, cleaning, disinfecting, sanitizing -> none of these destroy prions.
a small pedantic correction from a prion enthusiast: mad cow disease is BSE, bovine spongiform encephalopathy. CWD is chronic wasting disease, affecting cervids. they're both spongiform encephalopathies, but not the same disease. not just because of which animals are affected and how, but also because BSE jumps species – and infects humans, thereby transforming into variant Creutzfeld-Jacob disease (vCJD) – much more readily than CWD... for now, anyway.
See this is what I imagine the majority of the infections are caused by, knowingly purchasing non market meat
It can also lay dormant in your system for like 50 years as far as we know. Then one day it just goes HEYYYYY FUCK YOU IM GUNA EAT YOUR BRAIN NOW
I did have a patient once that was fine one day , on cruise ship, then literally bat shit crazy a few days later, like his mind was completely gone in few weeks. Docs were thinking it could be CJD. I think there are three types of prion disease. 2 that aren’t even related to eating meat. There’s the sporadic one, which happens fast. The genetic one that shows up later in life And the mad cow one that can incubate for several years .
Scary as hell. Wash your fruits & vegetables too !
That's why there are restrictions on donating blood if you were ever in the UK around the time thet had an outbreak there
Those restrictions have now been lifted. Source: I used to not be able to donate. Now I can.
you don't specifically choose what cow you eat when you buy a burger, there was no way to tell if it was contaminated or not
Add to that, your burger might be a mix of dozens of cows.
I saw a post I assumed was a joke about CWD Deer Meat for free on FB Marketplace.
Then I remembered people are fuxking stupid and use FB for 'research' and absolutely would eat that meat for free, even though the hunter and his buddy turned it down.
CWD meat with an ivermectin remoulade, you should be totally fine.
/s
I had a family member pass from mad cow about 10 years ago.
I also work for a pet food company and CWD is the reason why we don’t source venison from the US at all. Too risky & scary.
Oh goddd it’s a prion disease??? That’s the most terrifying disease!
Lost my father to sporadic CJD. I wouldn't wish that on my worst enemy.
You get to do that human thing where we forcefully create patterns that don't exist and start to believe that perhaps you reading about it triggered the event to happen.
I remember finding a box in my attic that had various regular magazines, but also a few playboys, and audibly wondering if Hugh Hefner was still alive. The next morning the news was reporting that he was dead. I felt like I somehow killed the guy by mentioning him for the first time in years, regardless of how dumb that actually sounds.
Someone I knew killed Roy Orbison the same way. Tragic
There was a Reddit post yesterday of someone trying to give away contaminated deer meat.
This is completely false. Disingenuous misinformation.
2 hunters did develop CJD, however, there is no evidence it is related to CWD. This idea originates from an abstract for a poster that was presented at AAN last October (link). Poster abstracts can often be “sensationalized” to encourage people to come to the poster during the conference. This would not be the first AAN prion-related poster abstract that is a bit “click-baity”. Furthermore, poster abstracts are not peer-reviewed. The fact this research has not been written up as a peer reviewed paper 1.5 years later (not even a pre-print) is INCREDIBLY telling.
The biggest issue comes down to this: how do you know the hunters got infected with CWD (ie how do you know their CJD is related to CWD)? The most common type of prion disease is sporadic CJD (sCJD), specifically the subtype the hunters developed sCJDMM1. Sporadic means it happens with no known cause (ie not caused by infection or genetics). sCJD typically affects elderly (median age of onset 65 years), has a rapid decline (4 months). In comparison, variant CJD (vCJD) is caused when someone eats meat contaminated with mad cow disease (BSE). vCJD typically affects young people (median age of onset 26 years) and has a slower decline (14 months). Furthermore, sCJD is associated with motor and memory symptoms whereas vCJD is associated with psychiatric symptoms. The patient case in the abstract was a 72 year old male, with a rapid memory decline in a month. Does that seem more like sCJD or vCJD? I will make it very clear, the case presentation is 100% classical sCJD. Furthermore, it is hypothesized that if CWD does transmit to humans it would have an atypical phenotype (ie not present anything like sCJD). This is based on data from both NHPs and Tg Mice (here is the mouse paper).
Other considerations, the patient was originally diagnosed with sCJD, however, the authors hypothesize CWD because one of the patient’s friends also died recently of sCJD. The authors therefore argue this is a “cluster” of cases, likely showing infections. However, just because two rare independent events happen in close proximity does not mean they are linked. For example, 1 in 300 people get Parkinson’s Disease, but a TV show “Leo and Me” had 4 out of 125 people on set develop Parkinson’s Disease, including Micheal J. Fox. However, this “cluster” is not scientifically or statistically significant. Just like two people who happen to know each other both getting CJD is not significant.
Clusters of prion cases happen. Here is a spatial-temporal cluster of 5 cases (including 2 with similar occupations). It wasn’t big news because stuff like this happens. It gets written up in a paper and the general public never knows. The only reason the hunters story is widespread is because it fits the fear mongering narrative most people push about prions.
The second consideration is that just because someone is a hunter and eats deer does not mean that they have eaten CWD infected deer. Not every deer in a population has CWD. If you are going to claim the hunters got CWD from deer, you need to show that deer they have hunted are infected. The entire premise of this abstract hinges on the argument that they ate infected deer, despite providing no evidence for it. If these two patients who both got CJD that knew each other were not hunters, this would never of been reported. Correlation does not imply causation. That’s basic first year statistics.
This also ignores significant biochemical and cellular data suggesting that CWD is not transmissible to humans based on significant sequence differences between cervids and humans. Here is a pretty comprehensive review of the experiments show CWD does not transmit to humans (link).
Also the picture in the post is a picture a deer with CWD from a paper published 20 years ago. It is not show neuropathological change in the hunters brain. It is a deer that got a deer prion disease. (Link)
It isn’t just my opinion that the hunters had nothing more than sCJD, it is also the opinion of the CDC which investigated these cases. It is important to note the authors of the abstract say "causation remains unproven". That means the title of this post does not even fit the abstract that originates it.
Sincerely,
One very annoyed prion scientist
I'm also a neurodegeneration scientist and neurologist. I've been posting similar rebuttals whenever this story gets posted.
They've never followed up this non-peer reviewed poster with the actual data and autopsy reports. While clusters should be investigated (and this one was) to see if there was something unusual, but the fact that two people who know each other got CJD is not unexpected. Humans tend to overinterpret cluster which is called the clustering illusion.
If I flipped a coin 100 times, chances are I'd get heads or tails 5-6 times in a row at some point. If the string of 5 heads in a row gets written up in article, it'd sound significant. But since we're tracking every flip (and every CJD case gets reported to state health agencies), we expect to see clusters.
While we have two experts in the room, let me ask something that's been bugging me for years: why don't humans contract CWD, or CJD from deer infected with CWD-associated prions?
Humans can get mad cow disease from infected cows, and deer can pass it to each other without (I assume) having to consume CNS tissues from other deer. If prions are just misfolded proteins, are human proteins sufficiently distinct from the affected deer proteins that it's a non-issue?
The complete lack of any detail regarding location or anything else in that “story” made me feel it was nothing more than that, a story.
I legit sighed seeing this and sighed further having to scroll so far seeing an actual scientist responding to this particular case. I remember this coming out I was like ruh roh then literally did the squidward lawn chair meme when I read it was sporadic. There was a similar case in the early 2000s involving three much younger CJD victims that was initially very suspicious but was deemed unrelated to CWD.
Also, don’t vCJD and sCJD look quite different when you do PMCA on the gels? Like you get a very distinct banding pattern difference? I looked for the study and did not see any physical data like a PMCA. I’m not an actual prion researcher but I do work with prions infected animals. This case hit headlines right as I was doing my senior capstone which I chose vCJD to present on and I made a slide on my 45 minute presentation explaining why it isn’t a causal link between CWD.
This needs to be higher up on this post
Clearly downvoted by someone
Obviously the downvotes are being coordinated by Big Prion.
I knew someone who died of CJD (not the mad cow one). It was horrific. Like 3-4 weeks from seeing the person at a party until their death.
as someone who grew up in the forest: KNOW WHAT CWD LOOKS LIKE. Make note of your location, and possible direction of travel.
If you have the shot lined up, take it.
Then call your local DNR office and relay that info.
Most states DNR will have a Hotspot map for areas hunters should avoid.
https://www.cdc.gov/chronic-wasting/animals/index.html
NOTE: it takes 18-24 months for symptoms to occur, this is why the Hotspot maps are so important for hunters.
So you're telling me nature invented something way worse than radioactive poisoning?
There are many things way worse than radioactive poisoning. Rabies is another good example.
I can't be the only one with that copypasta living rent free in my head, right?
Rabies is scary.
Rabies. It's exceptionally common, but people just don't run into the animals that carry it often. Skunks especially, and bats.
Let me paint you a picture.
You go camping, and at midday you decide to take a nap in a nice little hammock. While sleeping, a tiny brown bat, in the "rage" stages of infection is fidgeting in broad daylight, uncomfortable, and thirsty (due to the hydrophobia) and you snort, startling him. He goes into attack mode.
Except you're asleep, and he's a little brown bat, so weighs around 6 grams. You don't even feel him land on your bare knee, and he starts to bite. His teeth are tiny. Hardly enough to even break the skin, but he does manage to give you the equivalent of a tiny scrape that goes completely unnoticed.
Rabies does not travel in your blood. In fact, a blood test won't even tell you if you've got it. (Antibody tests may be done, but are useless if you've ever been vaccinated.)
You wake up, none the wiser. If you notice anything at the bite site at all, you assume you just lightly scraped it on something.
The bomb has been lit, and your nervous system is the wick. The rabies will multiply along your nervous system, doing virtually no damage, and completely undetectable. You literally have NO symptoms.
It may be four days, it may be a year, but the camping trip is most likely long forgotten. Then one day your back starts to ache... Or maybe you get a slight headache?
At this point, you're already dead. There is no cure.
(The sole caveat to this is the Milwaukee Protocol, which leaves most patients dead anyway, and the survivors mentally disabled, and is seldom done).
There's no treatment. It has a 100% kill rate.
Absorb that. Not a single other virus on the planet has a 100% kill rate. Only rabies. And once you're symptomatic, it's over. You're dead.
So what does that look like?
Your headache turns into a fever, and a general feeling of being unwell. You're fidgety. Uncomfortable. And scared. As the virus that has taken its time getting into your brain finds a vast network of nerve endings, it begins to rapidly reproduce, starting at the base of your brain... Where your "pons" is located. This is the part of the brain that controls communication between the rest of the brain and body, as well as sleep cycles.
Next you become anxious. You still think you have only a mild fever, but suddenly you find yourself becoming scared, even horrified, and it doesn't occur to you that you don't know why. This is because the rabies is chewing up your amygdala.
As your cerebellum becomes hot with the virus, you begin to lose muscle coordination, and balance. You think maybe it's a good idea to go to the doctor now, but assuming a doctor is smart enough to even run the tests necessary in the few days you have left on the planet, odds are they'll only be able to tell your loved ones what you died of later.
You're twitchy, shaking, and scared. You have the normal fear of not knowing what's going on, but with the virus really fucking the amygdala this is amplified a hundred fold. It's around this time the hydrophobia starts.
You're horribly thirsty, you just want water. But you can't drink. Every time you do, your throat clamps shut and you vomit. This has become a legitimate, active fear of water. You're thirsty, but looking at a glass of water begins to make you gag, and shy back in fear. The contradiction is hard for your hot brain to see at this point. By now, the doctors will have to put you on IVs to keep you hydrated, but even that's futile. You were dead the second you had a headache.
You begin hearing things, or not hearing at all as your thalamus goes. You taste sounds, you see smells, everything starts feeling like the most horrifying acid trip anyone has ever been on. With your hippocampus long under attack, you're having trouble remembering things, especially family.
You're alone, hallucinating, thirsty, confused, and absolutely, undeniably terrified. Everything scares the literal shit out of you at this point. These strange people in lab coats. These strange people standing around your bed crying, who keep trying to get you "drink something" and crying. And it's only been about a week since that little headache that you've completely forgotten. Time means nothing to you anymore. Funny enough, you now know how the bat felt when he bit you.
Eventually, you slip into the "dumb rabies" phase. Your brain has started the process of shutting down. Too much of it has been turned to liquid virus. Your face droops. You drool. You're all but unaware of what's around you. A sudden noise or light might startle you, but for the most part, it's all you can do to just stare at the ground. You haven't really slept for about 72 hours.
Then you die. Always, you die.
And there's not one... fucking... thing... anyone can do for you.
Then there's the question of what to do with your corpse. I mean, sure, burying it is the right thing to do. But the fucking virus can survive in a corpse for years. You could kill every rabid animal on the planet today, and if two years from now, some moist, preserved, rotten hunk of used-to-be brain gets eaten by an animal, it starts all over.
So yeah, rabies scares the shit out of me. And it's fucking EVERYWHERE. (Source: Spent a lot of time working with rabies. Would still get my vaccinations if I could afford them.)
You’re not alone. Every time I see the word rabies my mind immediately goes to that post. It’s going to haunt me forever lol.
[deleted]
Thats so unbelievably fucked. Imagine just getting some better vision and then you get fucking rabies from it
I thought it was a liver or kidney but the corneas had also been donated so they had to remove them from the recipients. Did one of those people get it too?
The person who died got something else. The cornea recipients had their corneas removed and are on anti rabies prophylaxis now.
Post it
I give people rabies shot pretty regularly. Both the regular vaccine to prevent it, and the igg if they have or think they have been in contact with an infected animal.
I don't feel nearly as concerned with it, although I get your point of "once you see the symptoms, you're already dead" kind of thing.
However, if I contact a potentially rabid animal, I'd go get the shots and feel very safe. If I ate meat and then later found it was prion contaminated, I think I'd exist in a state of dread for years, if not decades. Waiting for my brain to turn into swiss cheese. True horror.
The bad thing is, it’s not only the meat. Prion can be transmitted via meat, milk, feces, blood,
AND air.
Being close to an infected animal can be enough.
Source: PLoS Pathogens 2011 (via CORDIS)
WHAT. I THOUGHT YOU HAD TO CONSUME CONTAMINATED MEAT TO CONTRACT A PRION DISEASE. WELL, TIME TO START WORKING ON MY OCD AGAIN BECAYSE GODDAMN SHE IS GONNA LOVE THIS TIDBIT OF KNOWLEDGE.
It's 430am in Sydney, could you keep the yelling down, please?
ALL OF SYDNEY NEEDS TO BE AWARE OF THIS! BANG YOUR POTS AND HELP ME WAKE UP YOUR NEIGHBORS TO TELL THEM OF THE HORROR!
We still struggle to simulate a fraction of the horrors nature provides us with, daily.
Radiation is also something that exists in nature
Can I ask what is supposed to happen once you shoot the infected deer? Surely the prions remain infectious and eventually make their way into plant diet of later deer? Or other animals like vultures or things that hunt vultures or something?
Typically DNR would come and dispose of the animal carcass. In my area there is information posted online about proper disposal (double bagged in heavy garbage bags and drop off locations for incineration) as well as how to disinfect your equipment if used in processing an animal confirmed to have had CWD.
Reason it needs to be incinerated is as you mentioned, the prions persist and will potentially infect any other animal that eats from the carcass. I don’t recall if there is any steps required to disinfect ground that had blood spilled on it.
For hunters in my area, there is mandatory sample submission for harvested deer. I got my results back last week confirming my harvest was negative for CWD. Took a few weeks, but now I get it enjoy my venison worry free. There isn’t much for enforcement of the mandatory submission, but it’s free, relatively hassle free (take the whole head, or properly harvested biological samples to a drop off depot, fill out a form, get a free hat, and then wait), and leaves you with no doubt about the safety of your meat. If a hunter cares at all about conservation, it’s a no brainer to submit samples for testing as it only helps wildlife agencies to track and implement controls to help prevent the spread of CWD.
First, thank you so much for your informative reply!
Next…. Uh… 👉👈 can you share a photo of the free hat?
You’re very welcome. And thank you for my first ever reddit award.
And I would, but I actually don’t have a photo of it. Apparently there were a couple options based on what some friends have told me. But the one I received was actually a hunter safety blaze orange toque that says “Wildlife Safety Cooperator” on it. The other option was a camo baseball cap with the same words, but I was never given the choice to pick between them (I would have chosen the toque anyway).
Or just you know knowing this is a growing possibility in North America just stop eating hunted venison. Hunt them to cull populations if necessary but yea, I come from a deer hunting area and sorry to say but knowing this I'm never eating deer some random family member or acquaintance hunted ever again.
This brings back bad British memories.
Tell me about it! No T bone steaks for years!
All bad jokes aside though it was a genuinely scary time. My brothers mate actually died from CJD aged about 15 or 16. Really sad.
That's really young, and really scary. Do you have details about how he got it?
I've just found a newspaper report online. Apparently he was 17. He lived on a farm so I imagine there's a link there.
https://www.theguardian.com/uk/2002/aug/22/bse
Only was allowed to donate blood in Australia in the last couple of years. My terrible timing of living in the UK during that period.
It freaks me out reading these articles, I'm from the UK and ate beef during the dreaded timeframe. Are they now allowing blood donations due to the risk being unlikely, or better screening, I wonder? Hopefully, due to an unlikely risk!
They probably think anyone infected would show signs by now.
Except that cow populations are much easier to control than wild deer populations.
At least it’s easier to avoid eating venison though.
Two deer hunters ate CWD-Infected venison. Both later died of a “One-in-a-Million” brain disease.
In 2024, doctors reported two hunters who hunted together at the same lodge both got a super-rare brain disease called sporadic Creutzfeldt-Jakob Disease (CJD), the human version of mad cow disease, after eating deer from areas riddled with Chronic Wasting Disease (CWD).
What are prion diseases? Think of prions as zombie proteins. Normal proteins in your brain fold one way to work properly. Prions are the same protein but folded wrong, they act like a virus that "infects" good proteins, turning them bad too. The bad proteins clump up, destroy brain cells, and turn your brain into Swiss cheese. No symptoms for years/decades, then sudden dementia, shaking, and death in months.
What happened to these hunters? Both developed sporadic Creutzfeldt-Jakob Disease (CJD), a prion disease that normally affects only 1-2 people per million each year. Two hunters from the same small group getting it is statistically practically impossible, unless linked. They had both consumed venison from areas where Chronic Wasting Disease (CWD), the deer version of mad cow disease, is endemic.
What makes this particularly concerning is that Chronic Wasting Disease now affects deer populations across North America, and the prions causing it remain infectious in soil for decades. For years, scientists believed a strong species barrier protected humans from CWD, but a 2022 study using humanized mice demonstrated that CWD can cross to human tissue, contradicting earlier assumptions. Even more alarming, research suggests CWD could present differently in humans than typical prion diseases, meaning cases might be misdiagnosed with current screening methods.
What's truly horrifying about prions is they contaminate everything they touch, permanently. Grind your CWD-infected deer meat in a shared grinder? It's contaminated forever. Have prions and need brain surgery? Those surgical tools must be incinerated at 1000°C or soaked in caustic chemical baths for hours, nothing else works. Dump the carcass in the woods? Prions bind to soil minerals and stay infectious for decades, reinfecting new deer. No bleach, no autoclave, no normal sterilization gets rid of them. Only cremation or extreme chemical exposure for hours destroys them.
Prions aren't alive, they don't have DNA/RNA that you can target or kill. This makes them insanely hard to destroy because normal sterilization works on living pathogens by disrupting their biology, but prions have no biology.
All prion diseases are 100% fatal and incurable. The incubation period could be decades, so if this cluster represents actual transmission, we won't know the true scope for years.
What the fuck is humanised mice?
Mice made similar to humans genetically with respect to a system affected by a certain disease, in order to study that disease.
Don't forget they've also been given a SSN, 1040 form, and a mortgage.
And of course, you feel bad for their little mouse families because work at the lab barely covers rent, further humanizing them.
And crippling debt
My god thats true cruelty. Do they have a healthy family life or has inflation made them anxious for the future?
Wouldn't using miced humans give the same results??
Yes, but you'd need much bigger cages.
Miced humans haven't been successfully created. The "quiet as a mouse" part is where it always goes wrong. Humans are just slightly sophisticated apes; can't help constantly vocalizing nonsense. Source: Worked in cube farm for many years.
Howling laughing at the phrase “miced humans”
Little wigs and such
tophats, monacles, humanizing mice can get expensive but its mostly clothing/makeup.
Here is the 2022 paper they refer to (Link). They use mice that have the mouse gene for the prion protein removed and the human gene for the prion protein added back in on an artificial chromosome. This paper was poorly done (that hurts me to say as I know the authors of this paper, but I have shared my opinion to them before). The conclusion from the paper should be, CWD is unlikely to transmit to humans.
Prions are so scary. I am a Biologist and when I first learned about them during my Bachelor's degree I was astonished about how long they survive on the soil. I am a bit blurry on the details but I believe we were told about entire Scottish isles being prohibited from being used as grazing grounds for this exact reason
Another topic we discussed were certain cannibalistic cultures where mainly men were suffering from prion diseases. The reason? Men get to eat certain parts of the body the women don't and these parts tend to be riddled with prions.
They are so damn scary since you pretty much can't do anything about them
Cannibalizing the dead was respect among the Fore people in Papua New Guinea. Women and children were given the brain and spinal cord and thus eight times more of them got kuru compared to the men. Daniel Gadjusek got a Nobel prize for proving transmissibility of the condition.
I think you're thinking of Kuru from the Papua New Guinea cannabal tribe.
It was women and children mostly affected.
Men ate muscles from the corpses to give them the needed strength. Muscles had lower levels of prions.
Women and children got the leftovers, the brains and spinal cord, which contain much higher levels of infectious prions.
Your mention of brains made me think of the gentleman I cared for as a nursing student here in Canada (1992 grad here) who had CJD, from a trip and had eaten sheep brains. Memorable to me as little old naive me did not comprehend how people ate that part of an animal (yes I know it can be cultural in some cases but for me personally would not do it, instant stomach turning/ick factor) and how dramatcally fast this gentleman deteriorated and died within a mere few days. 😕 many medical students involved with him as well as a case study, and our instructor said that likely may never see this again in our career, it was so rare.
I was at one of those Brazilian all you can eat grills where they serve endless meats. They came over once with organ meats, including brain, and I just remember thinking "wow, even after you cook it, it still looks just like brain..." I guess I thought it would transform at least a little.
No thanks.
I just remember reading it takes serious heat or some strong acids to break down the prions. Heat high enough for cremation essentially so normal cooking methods won’t be enough.
Exactly. This is part of what makes them so freaking scary
on the cannibal part does that mean that the human victims of the cannibalistic tribe already had prions they were infected with? Or does the digesting of certain parts cause misfolding to happen after the fact?
The first. Since the "victims" in this case we're deceased members of the tribe, the prions would just be passed along from generation to generation within it.
You can get endogenous dementia too, prions are just misfolded proteins and can happen later in life just like cancer. You would likely die before the concentration of them becomes large enough to actually cause dementia, but if you're eating the brains of other older folks that concentration increases dramatically
Are the deer unaffected by these prions or are they visibly different/otherwise show signs of illness compared to normal or typical deer?
Look up “chronic wasting disease”. The deer are very much symptomatic. They are often described as “zombie deer”. They shake, they fall over a lot, they stumble around. Eventually they forget how to eat and drink or lack the muscle control to do so and then die.
They show neurological damage, but it takes a while from infection until they show any clear signs.
You have any clue where these people were hunting?
Seems to be some vital information that I can't find in the article.
I skin and process deer.
What? How does an autoclave not nuke the protein? Seems unbelievable
Edit: I looked it up. "High-Temp/Prions: 134°C (273°F) for at least 18 mins." That makes way more sense. It seemed unbelievable that you could not destroy a protein with heat.
Yeah a lot of stuff like sterilising equipment at 1000 degrees is just adding a "we better be fucking sure" safety factor. It also depends on the protein, but I'd be surprised if any organic matter survived intact much higher than its own autoignition temperature.
The reason they're so heat-tolerant though is through being misfolded they're actually more stable, so don't denature like normal proteins do.
Lucky I didn't end up picking up the CWD meat from that guy on marketplace
This is absolutely terrifying. 😨
The post directly under this on my feed:
https://preview.redd.it/w33l3rvmgk6g1.jpeg?width=1170&format=pjpg&auto=webp&s=f264358daaa4a317139e6914426c90cb7bdcb362
wtf
So cwd is cjd for deers? Prions?
Yep. If you’re old enough to remember BSE / mad cow disease being a thing in the UK then this is the cervine equivalent.
I would not eat venison from areas with CWD outbreaks. Prion disease is a dismal way to go.
Just to add to the fun - some prions aren't destroyed by cooking or other conventional food preservation processes. Even if your "at risk" meat is "well done", dried, smoked or cured doesn't mean it is safe.
I've never heard of any prion that is destroyed by cooking..
Several hours at 900F is the recommended method to destroy prions.
That's somewhat beyond well done.
Late edit: this is probably just to clear prions from the surface of items, I wouldn't rely on it to get every last one inside the cremated joint.
That's more like congratulations!
Not only that, prions shed by deer can get uptaken in plants and can infect someone that ate the plant. They are virtually indestructable. Chlorine will not even denature them. Not sure about sustained uv?
Probably not if it survives on the ground for decades
FYI prion isn't a classification, all prion diseases are caused by the same protein, major prion protein.
There was just a post of someone giving away meat with cpw 🤦🏾♂️
I believe that’s what happened to a cousin of mine. He was a lifelong hunter and fisherman for his hobbies. He especially enjoyed deer hunting.
He was the much-loved son of an uncle and his family didn’t want to say too much about it. But it was a neural degenerative disease specifically tied to deer and infected meat. His doctor has described it as the negative lottery.
From the little I heard, he exhibited severe short term memory loss. Sounded like a rough way to go but he was resigned to it.
Where was this?
I think the spread has been faitly recent. I think the upper midwest here had it before most of the rest of the country.
Sorry for your loss.
Not dismissing the possible link to his lifestyle, but you can develop CJD spontaneously. We just had a close family friend die of it over the summer, literally 1 or 2 in a million. They were in their late 50s, over the course of about 6 weeks she suffered short term memory loss that looked like early onset dementia. Whilst awaiting investigation and diagnosis for that, their behaviour and personality altered rapidly, eventually resulting in two A&E incidents that were described as ‘psychotic breaks’. Shortly after they had a serious fall in the kitchen (in hindsight gross motor problems building). They were home for maybe a week at most when they collapsed and were taken to hospital, CJD diagnosis after 3 days on intensive care, and they passed something like 10-14 days later. Whilst tragic and not pleasant at the time, especially for their partner, we all took solace in the fact it attacked very rapidly and there wasn’t a long drawn out decline, especially versus some losses we’ve had to dementia and cancer. Indeed, for the final 2 weeks they weren’t even conscious or aware.
Hmm… not sure where all he did his hunting but he was living in Georgia. I’m sure he would go hunting and fishing all over though but I know he did make it specifically to Florida, the Carolinas and Alabama.
He passed away about 2 years ago I think.
One of the crazy things I heard was he would eat something… and he’d forget he had eaten so he’d eat again or have to be reminded that he’d already eaten.
The victims of the prion meat post?
The X Files has an episode where a community of cannibals get CJD from eating one another. It absolutely terrified me, and made me scared of prion diseases.
I am curious if this effects the Amish community at all. We have a ton of deer where I am and they pretty much live off the meat over anything else.
that was sort of based off a true story. Tribes in Paua New Guinea practised cannibalism up until relatively recently. A disease called, IIRC, Kuru, killed many women. Turns out it was CJD and transmitted by the women eating the brains of fallen warriors from tribal skirmishes.
It wasn't just fallen warriors.
It was a cultural tradition of respect and mourning, so they were eating the remains of pretty much anyone, including those that died from the disease, over and over. Add to that, men ate the meat while women, who also had to prepare the feast, ate the remaining parts, including the brain.
Is this the zombie deer thing
Yeah, they'll lose weight, actions can become erratic, including losing their fear of people.
If you ever see any signs please call your DNR.
If they got CJD from eating CWD infected deer, then by definition it is not sporadic CJD, it is variant CJD.
Per CDC: “Once CWD is established in an area, the CWD proteins (prions) say in soil and water for years.”
New fear of bio warfare unlocked.
Nope, not for me
I sat in on a presentation given this Fall by the VA department of game and I didn't come away reassured at all. Not only do they still not know whether or not it can be transmitted to humans (the OP's case so far is the only ones that imply it can be), it's almost certain you will harvest an infected deer in some parts of my state. In one county, over 40% of the tested deer were infected. It so happens that county is one I used to hunt in.
I gave up deer hunting a few years ago as a result. It helped that nobody in my family likes venison, so I ended up giving away much of what I harvested. Still, I liked all of the outdoors aspects of hunting deer; being in the woods, doing the prep work to identify their movements and where they were that season, watching the sunrise or sunset from my tree stand, and watching other animals while out hunting (such as sitting on a hillside enjoying a snack and watching a bear who was oblivious to my presence walk down the same hillside about 50yds away).
Hey my mom died from that ! Contamined by the cow meat pandemic from UK in the 1990's that shit can take up to 20years of incumbation in your body before waking up
Yep my coworkers dad died from it unexpectedly. Was in perfect health for his age then suddenly he was burning foods all the time without even realizing he had done it. Would just forget where or who people were. Few weeks later he was dead.
The doctors told the family they could get tested for cjd and they all outright refused. Who wants to know that there’s a ticking time bomb in their body that may or may never blow up? Plus my coworker didn’t want to have a panic attack everytime he forgot something.
Great!
My mum was a pathologist and there were only a few things that really worried her. The first and biggie was some of the germ warfare stuff the Soviets were working on in the 70s and 80s (she worked with the Royal Navy for 24months). The second was BSE and CJD prions. They cannot be killed, cannot be cleaned off of tools, and cannot be cured.
My wife's uncle died from CJD. If ever there was an argument for the legality of assisted suicide CJD is it. Nightmare does not even come close to describing what we witnessed him experiencing when we saw him shortly before his death.
I'm a microbiologist, and the only things on this planet that truly terrify me are prions.
How does one know if the deer is infected? My dad does a lot of hunting and is always bringing me deer jerky and now I’m scared.
As others have said, the only way to know is to have the animal tested. Here in VA there are dropboxes for this very thing. Unfortunately, it takes a few weeks to get the results back, so you have to have a way to store the carcass in the meantime.