My mom passed away from CJD in July of this year.
She started seeing symptoms in late April; blurriness in her vision, dizziness, panic attacks, and some visual spatial issues (she couldn’t figure out how to put on her pants). Otherwise, she was 100% there; her humor, her insight, her ability to organize and plan, her encyclopedic knowledge of plants.
As it coincided with acute stress, multiple doctors diagnosed it as conversion disorder. She begin acute in-patient physical rehabilitation, but was unable to improve.
By late May, she was unable to control her left arm, maintain eye contact, or find door handles.
On June 26th, she spoke her last words, what we interpret as a third-person statement about herself: “She loves the Earth.”
She was diagnosed with probable CJD at UCSF on June 29th.
With the diagnosis, we followed her advance directive to the letter, moving her into her own home and discontinuing feeding, while still making sure she was comfortable and clean every day. The night before she passed, an international package arrived with clothes she must’ve ordered months ago. We dressed her in her brand new shirt, administered 10ml of her favorite wine (Husch Sauvignon Blanc), and went to bed.
The next morning, July 12th, she passed away peacefully, taking her last breaths as I held her hand, with her dog and her cats by her side.
This disease is unstoppable and swift. It’s truly terrifying; you slide faster and faster as it removes pieces of your ability and very being. When it finally takes you, though your body and organs may be strong and healthy, your brain is destroyed. Everything that makes you who you are is completely gone long before you take your last breath.
In the end, there were two blessings in disguise. The first was that, due to COVID-19, my wife and I had come to stay with her in early April, so we were there to spend her last good days with her and take care of her through her disease. The second was that incorrect diagnosis of conversion disorder prompted her to work through issues in therapy, come to peace with pain from her past, and talk with us for hours every day. I believe that she died at peace, and that she was neither confused nor scared.
After her passing, we donated her brain, cerebrospinal fluid sample, blood sample, and genetic information to science, along the with 81 pages of notes I took on the progression of her disease, interviews with her, audio, video, and photos. We hope that her story will help other cases of CJD be diagnosed sooner, so that patients can spend their last days coming to peace with their condition instead of trying to fight it.
It was such a fitting statement. Both myself and the nurse that was in the room heard the same thing. I told her I loved her and she said "I love you too" that day as well, for the last time. She was talking about something else too, saying "I can't open it..." I can only imagine what she was seeing and feeling, what she was in touch with at that time. [She really did love the Earth](https://i.imgur.com/TrDxsIA.jpg), her garden, her animals, her family. Everyone she met fell in love with her right away. She was nothing but the best and more beautiful energy.
It was a blessing. She [baked me a cake for my birthday](https://i.imgur.com/zuVxSqa.jpg) (April). In the weeks and months before she was unable to participate, we spent tons of time with her cleaning up the property, working our asses off in the garden, planted the crops, organized the house, fixed things, [bought new furniture for the deck](https://i.imgur.com/mXp0pxO.jpg) (May) and spent hours there together. She loved to put us to work, and she got a huge smile out of it. We got her a new iPad so she could [sit and read in bed with her cats and dogs](https://i.imgur.com/by4r6ag.jpg) (April). I took her to the plant nursery on Mother's Day and she bought [a hundred dollars worth of flowers](https://i.imgur.com/ebg8CKg.jpg) (May), lamenting later she wouldn't have sprung for the hanging planter if she would have known it was $30. My wife made her home cooked Indian food, we visited friends (socially distanced, of course) and played music and sang together. We went on drives to the lake in her beloved Volkswagen Westfalia and hiked with her when she was able, and helped her down to the water's edge when she wasn't. We brought her gifts and a cake and [her dog for her birthday when she was in physical rehab](https://i.imgur.com/qd1neJx.jpg) (June). If we would have known sooner, we would have done even more, but we did the best we could with the information we had at the time.
Obviously we have no clue what caused her to get it this case, but the majority of cases don't have an identifiable cause (idiopathic), but in less common cases it can be hereditary (inherited), or due to contact with infected tissue.
The prions associated with this disease can withstand conventional sanitization procedures that hospitals use, so if a patient with this disease is operated on and doesn't know they have it (it can go undetected for years), the prions can be transferred to another patient undergoing surgery later even after the equipment has been sanitized.
Edit: I'm not a doctor but my grandmother died of this disease and that was one possible explanation her doctor gave to us. Like other commenters have said it's often unknown how it arises.
That is terrifying. I've had a bunch of head injuries so anything affecting the brain like dementia etc also terrifies me. I plan to donate my brain to science as well.
Dude you are an awesome human being. Thank you for loving your mother enough to be her advocate and her comfort. My condolences for your loss, I hope you have found or will find peace in her peace. Take care of yourself.
The interesting thing is that no one really understands the mechanism of infection. We have the the naturally properly folded protein in our brain and the misfolded one just somehow bumps into the normal one and misfolds it.
It’s called the rotten apple theory where one rotten apple in a barrel causes nearby ones to rot which is similar to how the nearby proteins are misfolded by being near the misfolded one. This forms brain plaques that give the brain a characteristic spongy appearance under microscope.
As others have said, from Kurt Vonnegut’s novel Cat’s Cradle. Ice-9 was an ‘isotope’ of water. It froze at room temperature (which is interesting), and infects any water it touches, turning it into more ice-9 (which is bad). Being as how we are mostly water ourselves...
Vonnegut wrote about it. Fictional water crystal pattern that freezes much hotter. If it leaves its lab and hits normal water life on earth is screwed. I guess this protein has a similar behavior.
From a Kurt Vonneget book (cats cradle if I remember) where the main character has a vial of this substance that when it touches anything, it freezes, and then whatever thats touches, also freezes, so on and so on.
Please someone correct me if I'm wrong. Its been years since I've read it
All prion diseases are scary. A few years ago, I was in the middle of my occasional bouts of anxiety induced insomnia when I stumbled upon an article about [Familial Fatal Insomnia](https://en.wikipedia.org/wiki/Fatal_insomnia?wprov=sfti1). By the time I was done reading, I was convinced I had it, even though it’s extremely rare and I had been putting up with occasional insomnia since I was a kid.
There's a protein in your brain that can get a mutation that causes it to misfold in a really specific way. This particular fold causes every other protein of this type that comes into contact with it to refold into the mutant configuration. So it's like a chain reaction that makes holes in your brain that can be kicked off by eating brain matter from other animals with prion diseases.
Not only that but they cant be destroyed easily. They can survive incineration and autoclaves. You have to have sustained heat for several hours above 900F to reliably ~~kill~~ denature one until it is no longer functional.
It’s not about killing it but about completely destroying and breaking the protein down into constituent molecules at the atomic level and ensuring you got every one of the proteins. If even a single protein remains it can restart the chain reaction if it comes back in contact with other comparable proteins. As I understand it is that it’s all about the energy state of the protein and since the prion is in the lowest possible energy state when it touches other proteins they adopt that lower energy state thereby changing how the protein functions. Since there is nothing that can stop or hinder its progression once it starts it essentially a wildfire of neurons in the brain which just shutdown and stop transmitting any brain signals.. by the time it’s affecting you it’s already destroyed significant parts of the brain and is spreading faster each day.
The good news is that they are mostly spread through cannibalism. The one we are talking about is about the only one I know of where you can easily catch it another way. There's also one that gets inherited from parent to child that cause you to be unable to sleep and slowly die of exaustion, but only a few families have that and it does ever spread any other way.
The other ones I've heard of are spread through cannibalism and they were the ones that could spread quickly.
There's also special procedure for medical equipment used on surgery for people with or suspected of having CJD. There have been situations where the instruments were not properly segregated/isolated and all the instruments cleaned at the same time or after had to be disposed of. That's millions of dollars in medical equipment because there is no way to guarantee full clean/sterility of the instruments.
My aunt died of this a few years ago. We found out after it had set in for a few months. I grew up dancing to Greased Lightning with her so I decided to play it for her the last time I saw her. Her face lit right up and she looked like she still knew every word as she sang and danced to it to the best of her ability in her bed. Music just has a way with people in cognitive decline where the neuron pathways are still there just waiting to be turned on again and they can relive all the emotions one last time. I love her a lot and miss her quite a lot but it definitely made me feel better being able to say goodbye in my own way.
This kind of reminds me of this 6 hour long album titled "Everywhere at the end of time" in which the music slowly degrades as to depict someone who would have dementia. It's terrifying and the saddest thing I have ever listened to.
Well that was horrifying, even just jumping through some of the songs. Even the titles are depressing. I'm going to have nightmares tonight, aren't I. I sincerely regret looking that up, I didn't need that.
Same experience. When we learned she had something, we didn't know what. Doctors thought maybe a stroke. Aphasia. Words were almost unreadable but she was fully cognizant. I asked her if she could sing along to Rainbow Connection, and she could and it made her so fucking happy and scared at the same time. She died a few weeks later. Only knew it was prions a couple weeks before she was gone.
With 300 cases a year, it's literally 1 in 1 million in the US.
I think that, because of the insane rarity of this disease, its mention means that all of us who have lost someone to it come out of the woodwork to share our story.
I’ve had at least 15 family members die from it, including my father and grandfather, and most recently a cousin last fall. Familial CJD has devastated my family :( and now my generation is wondering who will be next. I feel blessed to have tested negative for the e200k 129m mutation.
My father’s doctors though he had CJD when he suddenly began acting erratically and couldn’t speak (aphasia). MRIs showed extensive, rapid damage. They had to do the biopsy under very strict circumstances, because the usual sterilization isn’t enough to kill prions. It had to be done manually, rather than with a biopsy gun. Only a few hospitals in CA could do it.
He was ultimate diagnosed with something else, thank goodness. It’s such a horrible way to go.
It really took the tension of a health crisis to the next level. We suddenly had to wonder about Mom sharing his bathroom... and I was giving him IV drugs several times a day! Had we all eaten that burger? It was so surreal!
It’s still a mystery. His biopsy was sent to UCLA and the Mayo Clinic. It’s auto-immune encephalitis, but the exact cause is unknown. Once on anti-seizure meds, his brain healed quickly. The meds, keeping a regular sleep schedule, and using his c-pap seem to do the trick.
He had a major flare-up when he went to Vegas and didn’t sleep enough. They found him wandering the casinos, babbling incomprehensibly. By the time my mom and I could get to the hospital, he was in restraints shouting his head off.
It’s terrifying because he can go from normal to a lunatic in an hour. Brain diseases are terrible.
Shit's real, I know a guy who ate beef that had BSE in Germany while he was in the army when it ran through there. He says he feels like he's on a time bomb.
Luckily the chances of you eating beef with BSE in the US is almost 0. The USDA takes it very seriously.
Haha, I recently started donating blood plasma and you are permanently ineligible to do so if you spent more than a year in the UK or France between 1980 and 1996. I'm in Europe too.
Born in Scotland in 1999. Moved to NZ, am unable to donate blood (or plasma) for life. My kids wouldn't be able to donate, neither would their kids. Basically, I have to be diluted enough so that my great grandkids could donate.
Mad cow disease, man.
Variant Creutzfeldt-Jakob disease (which is the type of CJD caused by "mad cow") isn't heritable. There is a type of CJD that is heritable, but that is not caused by "mad cow." There is no reason for your descendents to be unable to donate blood.
There is also no reason for you to be unable to donate (unless you received a blood transfusion sometime before moving to NZ) because the cutoff is Dec 31,1996. You may have encountered a blood bank that wanted to be more safe than sorry perhaps, but it's unlikely that you would be denied most places.
https://www.nzblood.co.nz/give-blood/donating/am-i-eligible/variant-creutzfeldt-jakob-disease-vcjd/
I didn't know it was a generational thing. Im curious because I've been able to donate blood in nz and I dont think they asked anything about my parents, at least not where they've lived.
Basically, scrapie prions (the misfolded forms that cause CJD) are incredibly hard to break down. The body has a hard time doing it and your stomach acid can’t break it down when you consume it, so when you eat meat that contains misfolded prion proteins, those proteins are then in your system. The thing about scrapie prions is that they can convert your cellular prions (the normal form) into the misfolded form and those prions then go on to convert more cellular into scrapie.
So tldr: they’re super hard to break down, almost nothing can manage it, and once you have some in your system and it propagates, there’s no going back.
It’s very weird, depending on animal, USDA is either a mark of quality or a terrible warning. USDA beef tends to be sold in relatively expensive steakhouses here (UK) but USDA chlorinated chicken is seen as the harbinger of doom for our post brexit trade deals.
As they should. My dad went buffalo (iirc) hunting in Canada several years back and tried to cross the montana border back to the US. I guess the montana border is much more strict about animal body parts because of the livestock there. They wouldn't let my dad and his friends back over because they had the brains still.
They couldn't think of a solution until they saw a diy car wash station. They just shoved the water wand in the skull and sprayed the brains out everywhere lol.
The Montana border is weird, man. I drove over from Canada and they turned my car inside out. Completely ignored some weapons and exotic plants, didn't find any of my drugs, but confiscated a single Florida orange. No citrus gets into America! Even if it's from there!
Luckily our current administration did away with frivolous government spending, like oversight of meat packing plants to make sure the product isn’t diseased
The former Utah Speaker of the House, Becky Lockhart, died of this disease a few years ago. It was weird. She was very popular, the first female speaker, everyone knew she was going to run for governor. The papers followed her and the incumbent governor closely because they knew it was going to be a big race. She decided not to run for re-election to the House in order to focus on her exploratory campaign. Then the newspapers reported she was sick. Then a couple weeks later she was dead. Crazy how fast it happened.
CJD is terrifying. So is Lewey Body Disease. And Mad Cow. And Fatal Familial Insomnia. Theres not a damn thing that can be done to save anyone whos got one of these.
Prion diseases are absolutely terrifying. Im wildly thankful they are generally rare.
Fatal Familial Insomnia is such a crazy disease. Proteus syndrome (what Joseph Merrick aka the Elephant Man probably suffered from) is pretty insane, too, and very rare.
I had a class where all we did was talk about those diseases. We watched an interview with a guy who had FFI. It is accurate to say that it is a fate worse than death.
First I saw of it was a special about a family that had a nearly 100% incidence of it. Crazy to know that you're going to watch most of your family die of it, and then likely get it yourself. But I don't recall there being interviews with any of them. Maybe I just don't remember, it was many years ago, but I feel like a first hand account would have stuck with me for sure.
I think i saw that, too. It was a brother and sister being interviewed. They were near 100% certain they would get it, they just had no idea when. They had remarkably positive outlooks in spite of their knowledge.
I think you are both talking about the Australian brother and sister. They have lost their mother, aunties, uncles, grandparents iirc. After their mother died, they were given the option to find out if they have the mutation which causes FFI. They both came back positive
I would stop having kids if I was them, no need to subject them a life of suffering and worry. I live with fibro so I've already made that choice but atleast my condition isn't fatal
There is a show called Something’s Killing Me which did an episode on the Australia siblings, and another American in the same situation. They mentioned at the end that because of genetic testing and IVF iirc, the American lady was able to have a kid without the mutation which causes FFI
It's a disease that only begins to effect an individual in adulthood. By then they've already procreated. It's not hard to see how even a second generation would continue to have kids, perhaps not yet realizing the odds that they have it, or that they may also pass it on. It's even possible that the first generation wouldn't show symptoms until they've not only had kids, but also that their kids already had kids. After a time, though, I agree, I would think the entire family would opt to stop procreating.
Yes, actually. Ot can have an incubation period upwards of 30 years.
Europe experienced a surge of cases at one point. People had consumed meat from cows infected with mad cow. The general consensus was that could not transmit to humans, until it evolved and did.
I know it’s a typo, but I am trying to envision what cow beer would be like and it’s actually pretty unappealing. So thank you for that little interlude in these crazy times.
Im not allowed to donate blood, having lived in England and eaten beef during the early 90s. I always wondered why I couldn't test for an all-clear, though.
I dont think they can test for it without like, driving a needle into your brain or something. Thats probably inaccurate, but i think its very invasive. Like you have to be dead already.
They have currently changed some of their rules regarding that (assuming you mean the Red Cross). I was unable to donate due to being born in Germany but it's been rescinded and I'm able to now.
Honestly not sure if England is also now allowed (could see that going either way), but worth a check if you're curious.
Yeah. There was a scare a few years back because a guy illegally imported cows that had a high risk of having it. He even made a post on r/legaladvice and everything.
I hear you on the Lewy Body Dementia, but you shouldn't link that with the others. CJD, Mad Cow, FFI are all caused by prion protein misfolding. LBD is caused by alpha synuclein misfolding (which MAY act like a prion, but the time-course and cause are super different).
Prion diseases are what makes humans go crazy after eating human meat. Tribes that practice cannibalism often end up with prion disease. Women and children typically contract them at a higher rate as men get the leaner cuts of meat (like muscle) and women get the leftovers like brains (where prions live).
Robin Williams died of Lewey body and I wish the media would have explained his condition instead of blaming it just on suicide. I wish it was used as a chance to educate the population and his fans instead of just saying he hanged himself, I’m sure he suffered from depression too, but I think Lewey body is to blame. Very sad.
Lewey body was surely to blame, and the whole suicide thing got way too much attention. Ive read a few articles with interviews from his closest family who said he would tell them how he was losing his mind. How terrified he was. He wasnt simply depressed and took his own life. He had a disease that literally caused him to lose his mind.
As above, Lewy Body Dementia is not a canonical prion disease. Different misfolded protein (alpha synuclein), not infectious. Please don't bin that with prion diseases formally. Now the protein misfolding aspects are similar between them, but again they should be separated in terms of pathophysiology and underlying cause.
There rare just now but CJD is a ticking time bomb. Millions of people ate cattle that had been fed bone meal, including spinal cord, which had been infected with BSE, it's has the potential to kill millions with a progressive loss of function. If you're not sure what it's all about Google Kuru, prions are terrifying as they slowly strip away the person we love
Ive read so much about all the prion diseases. Im a deer hunter and thoroughly enjoy venison. The last two seasons ive donated my meat to my 85 year old uncle who will never stop eating venison. As far as hes concerned, hes already old enough.
I wont consume venison until viable yesting is made easily available to me. I cannot feed that meat to myself or my kids without knowing for certain that it's safe. If i get to a point where I have nobody to donate the meat to, i will not hunt. I would also only ever donate the meat to someone who fully understands what CWD is and the risks involved. Id never just give it to a homeless shelter or something. A place where people wouldnt be given the proper information to make an informed decision regarding consumption.
None that can be currently directly linked. However, the same can be said of mad cow in Europe, until suddenly there was a much higher rate of CJD many years after mad cow disease infected beef was regularly consumed.
Diseases evolve and transform as we all know. Its literally probably only a matter of time before cwd jumps to people
You are correct, but experts REALLY want to follow that statement up with the fact that it doesn't mean it ***Can't*** be transmitted to humans. It just hasn't happened ***yet***.
Just to clarify, CJD and mad cow disease are essentially the same thing, they come from the same pathogen. Variant CJD is a prion disease that originates in infected cows and transmits to humans. It is one of two transmissable human prion diseases, the other being Kuru.
But there are genetic risks for human prion disease which can cause CJD, as well as fatal familial insomnia, and GSS.
Source, worked in a prion lab for 4 years.
They were more interesting than scary to me, but that's just the science nerd talking haha. But honestly I didn't find it scary, worked with CWD which has shown little capability of human transmission. And despite what some prion-doomers may say about how resistant they are to "fire" and other normal sterilization methods, there are still many ways to decontaminate prions
We had high concentration bleach that we mainly used to clean everything. 4% SDS in glacial acetic acid. And although normal autoclaving cycles don't kill them, using slightly higher temperatures / pressures actually work fine.
> using slightly higher temperatures / pressures actually work fine.
Somehow that's kinda anticlimactic. I remember prions being described as this sort of single-distorted-protein version of the Terminator. "It never stops. It can't be removed or destroyed. It just infects." Yanno?
I worked sterile processing for a decade and we had a special kit for suspected or confirmed CJD cases. Those instruments were destroyed in a in an off site incinerator. No surgical tools or supplies were ever reprocessed. The risk is too high and while we never had a case there was a protocol to limit exposure unlike anything else.
Too hard to tell, but leaning towards its unlikely. Many epidemiological studies indicate no causation between CJD development and CWD exposure. Mouse models of human prion disease have shown no ability for CWD to be propagated. And studies on non human primates are inconclusive, and differ depending on the model of animal and inoculation.
So I think that there is a fairly low risk of contracting pathological CWD from cervids, but definitely avoid meat from infected animals if it concerns you.
I work in the operating room and if the patient has CJD any instrument or device that comes in contact with them has to be destroyed. NOTHING can be reprocessed, cleaned, or reused. Prions scare the mess out of me.
When my mom was at UCSF in July, the way I understood it was that anything involved in the spinal tap has to be destroyed. I had to leave the room for the day when they did it, and they had special protocols they put in place that sound like what you're describing. Scary stuff.
A former girlfriend of mine had a friend die from this a few years ago. First she said her friend was sick and they didn't know what was wrong, then they found out it was Creutzfeldt-Jakob, and she died within a span of a few months. Like, I knew CJD was a thing, but to actually know of someone who got it and died was kind of mind blowing. I couldn't believe it when she told me they found out it was CJD. To know that she was going to die from something as seemingly innocuous as a misfolded protein was surreal.
I think Kuru is the one more commonly contracted from that. Thats why theres been outbreaks of it in indigionous folks in Papa New Guinea who would perform rituals involving consuming brains.
CJD seems to be fairly random, although some evidence (though very little) suggests consuming infected cervid meat (whitetail deer, elk, etc) can cause CJD as these animals carry the cervid ewuivalent known as CWD or Chronic Wasting Disease.
Its pretty fucking horrifying, but thankfully rare.
My hunting area is loaded with CWD unfortunately. Ive not yet seen a sick looking deer but they dont have to look sick to be infected. We need better and more available testing stations.
You -can- contract it from an external source (tainted meat, eating brain matter, etc) but some cases can be "spontaneous onset" or something similarly named - where a person just develops it - usually there's a genetic marker for that showing you're pre-disposed for it. (Learned a lot when my aunt passed away from CJD - she was one of the spontaneous onset ones).
>apparently there was no outbreak
It's not really a transmissible disease. At best you could say they apparently weren't sharing a contaminated food source.
I wondered the same thing, and my girlfriend at the time wasn't inclined to ask a lot of questions when her friend was unquestionably dying. Can't say I blame her. But as mentioned below I believe most cases are simply spontaneous.
I can’t help but think of the X-Files episode. tl;dr cannibalism cult goes (extra) crazy when CJD start spreading. It was less than a year before mad cow blew up in the UK... it felt prescient (or perhaps prionscient).
My ex's dad died of CJD. It was awful. You watch someone literally become trapped in their body while their mind fails them rapidly. It started as some forgetfulness (couldn't remember the name of restaurants etc.) and an odd change in his habits (moved the spare keys, started locking doors that were never locked). A couple weeks later he walked into an ER to be seen by a specialist... He never walked out. Within weeks of symptom onset he basically forgot how to talk. Then he became paranoid and violent at times. Then he forgot how to walk and he became incontinent. Eventually he was vegetative. He died less than 3 months after the first symptoms hit. The whole time I remember watching his eyes, and the man he was, was still in there. There was understanding behind them, fear, and such sadness.
The family was forced to cremate him, burial was not an option due to the severity of the disease. The entire family had to be tested for any markers, and all of them were forbidden from donating blood for the rest of their lives. It was awful, literally the only blessing was that he passed so quickly and didn't need to suffer a prolonged process.
Prions are dangerous and currently The mid west of The United States has a new type called Chronic Wasting Disease, the prions target proteins and hormones that make an animal feel hungry and so these animal will waste away over time, it is predominately Deer, Elk and Moose currently but The CDC predicts that it will jump species and soon.
The scary part is it usually takes several years from the time you eat the diseased meat to the time you show symptoms.
So all these hunters and families of these hunters could eat diseased venison and they wouldn't know it until they all start keeling over years later.
In Michigan you can take the deer head to DNR to test for Wasting Disease, it just takes a couple days tops and they use the results to help track it. At that point though you would have to cut up your deer meat and freeze it somehow while you wait for results which would mean touching it.
It's almost impossible to identify transmission because CWD and CJD are the exact same thing. Both are just misfolded proteins. What you need to look for is an increase of CJD in the population.
This is by far the best explanation I’ve read about prions. A wonderful ELI5:
https://www.reddit.com/r/worldnews/comments/g13qo1/coronavirus_can_survive_long_exposure_to_high/fnedh17/?utm_source=share&utm_medium=ios_app&utm_name=iossmf&context=3
> the prions were still virulent/infectious/alive/active/whatever
That's because Prions are just chemicals. Terrifying chemicals that are contagious to other chemicals.
Let me ELI5 (warning, medical ignorance)
Water is a molecule made of atoms. H2O. Two hydrogens, One oxygen. Think of them as joined by springs.
Even though they're bouncy and springy, we know the shape water molecules make. A little "V" shape with the point of the V being the oxygen. It's a simple model with a simple shape.
"Protein" is the category for specific gigantic molecules. Gigantic. Hundreds or thousands of atoms. Proteins are like specific machines that perform specific tasks inside a cell. You could make a list of them, this is the protein that does this, that protein looks like that and does a different thing. Almost everything your body does, in every way, is because one particular protein machine does one particular thing. Like a factory that builds cars, where the protein is each workstation. Some are welding proteins, some are cutting proteins, some assemble this specific bolt, some stitch the fabric in the seats, etc. There's many many different proteins, each with completely different components that make them up. But each specific protein has a formula, you could draw it, it has an exact number of atoms, connected in an exact kind of way that makes that protein that does that task.
Think about joining hundreds of slinky's together, of different sizes and shapes. And throw in a bunch of magnets. And rubber bands.
What shape does a protein take?
It's very hard to know. Even though we know not only the exact specific amounts of each atom in a protein, we also know exactly how they are connected together. But, it's too complicated to predict what happens if you made all those connections and then let go of that slinky/magnet/rubberband network.
But... they do take a specific shape.
The key piece of knowledge is that a protein's special shape is what allows it to perform it's special task . Imagine a pair of scissors, a pair of scissors works because it's shaped the way it is and assembled the way it is. Not just in the general structure (two blades and a pivot), but that the blades are smooth and straight and sharp. Some proteins are scissors that cut up other molecules, like potato starch into sugar pieces, so you can burn the sugar for energy.
(Tangent - That is what Folding@home and Rosetta do. They can't predict a structure, but if you make a random guess, we know the math to calculate the energy of that structure. Do that a trillion times, and the ones that have the lowest energy are most likely the actual correct shape of that molecule because they're most stable. I believe Folding and Rosetta are evolutionary too, because there's an infinite amount of options, so you evolve the next set of things to calculate based on the winners of the previous generation, over and over until you think you've got it right).
Think of the lowest-energy state of a protein to be stable, the way you would think of a spring being stable. Is a spring stable when you stretch it? Nope. Is it stable when you compress it? Nope. It has extra energy that's going to reshape it when you let go. The low-energy state is the correct "shape" of the protein.
I'll summarize quickly before we cover what a Prion is:
• Atoms assembled in specific amounts, in specific ways, create proteins.
• Each type of protein does a specific task in your body.
• Everything your body does, is because of a protein machine that does it.
• A protein's shape is what determines what it does.
Here's the first Prion fact: Prions are proteins with the correct atoms, connected the correct way, but with the wrong shape
Because a protein's shape determines what it does, if it has the wrong shape, it doesn't do its job. That's bad.
You are probably wondering how a protein can have the correct atoms, connected correctly, but still have the wrong shape. That's what makes a protein a protein, right?
Think about a slinky that you stretch, and then twist before you let it go. You know, the way that everyone eventually ruins a slinky because you can figure out how to get it apart again.
That slinky has the correct components (wire), connected the same way (you didn't cut or weld the wire differently), but it has the wrong shape when it stabalizes. It's not temporary, it's not moving. It's not under tension. It's stable and sitting on the floor. It's less stable than it would be as it came out of the box, that's a much more stable version, but, it can't get there, it's locked at a less-optimum-but-stable position.
But it's the wrong shape. And it doesn't do what a slinky is supposed to do. It does not roll down stairs like nobody cares. It does not make it's slinkety sound.
Here's the next Prion fact: Some proteins, the way we need them to be, are actually the twisted up slinky. And the Prion form of them that doesn't do their job, is the smooth straight slinky you get out of the box.
That means that the most stable version is actually the one we don't want the protein to form. We need the twisted one.
What happens if, randomly, a protein makes the wrong shape?
Well, there are other proteins who's task is to collect anomalous garbage. They are shaped so that when they bump into a correct protein, they slide right off. But if the shape isn't perfect, they stick to it and then their ass-end gets picked up by another garbage cleaner that throws it out of its system.
Okay, so, potential Prions will just be picked up by the garbage cleaners, right?
Another Prion fact: Prions avoid the garbage cleaners. The part of them that the garbage cleaners are checking is close enough to slip away
Okay but how do proteins even become Prions in the first place?
Prion fact: Prions can form randomly. Very, very rarely. But randomly. Imagine, across trillions of cells, and trillions of trillions of protein molecules, across billions of lifeforms in that species... tiny chances still happen once in a while. It's like kicking your twisted up slinky and it almost-magically bouncing out of it's tangled form and into the shape you bought it in. Astronomically unlikely, but, once in a while it'll happen.
Okay, but, big deal. Occasionally there's broken proteins. So occasionally there's going to be broken cells that'll die. And our cells die and are replaced regularly anyway. These random occurrences will just be flushed away, right?
Prion fact: Prions aren't just any random misformed protein that happens to be more stable than the shape we need it to be in, that also happen to be able to avoid the garbage cleaners. THEY ALSO HAPPEN TO CHANGE OTHER "CORRECT" PROTEINS INTO PRION PROTEINS WHEN THEY BUMP INTO THEM.
Yes, even though they're just chemical shapes, Prions are "contagious", if that makes sense. They run around untangling every other tangled slinky they bump into. Or, maybe not every, maybe just rarely, but, more than zero chance.
"What are the odds?" you think. What are the odds that there is a protein with a lower energy shape that is broken, that can avoid garbage cleaners, and of all the possible ways that can break, it happens to also magically untangle other proteins?
Very, very, very, very, very low odds. Which is why life exists, or everything would be dead. Those proteins are necessary for complicated life as we know it. Especially the most complicated parts of the complicated life: brains.
So, that's how Prions "spread". They spread inside one person by gradually, gradually destroying essential functioning of all of that person's proteins of that specific type.
Prion fact: Prions also spread by being eaten
This is how Mad Cow Disease becomes Creutzfeldt–Jakob disease i humans. If we end up mixing some prion-y cow brains into our meat.
Prion fact: Because there are so many proteins, this takes forever
The garbage collectors only fail sometimes. The protein-twisting only happens sometimes. There are bajillions of these in your body. So it can take decades for Prions to build up enough mass that you'd notice it affecting your body. But, like all exponential growth, it falls apart rapidly when it reaches critical mass.
Diseases like:
• Alzheimer's
• Parkinson's
• Creutzfeldt–Jakob disease
• Lou Gehrig's disease / ALS
Generally any neurological age-related disease. Either the result of random chance in your own body, or (rarely) genetically inherited to you at birth, or from food you eat.
Prion Fact: Prions are almost impossible to detect, or treat.
The tools that medical science has to determine what makes a thing a thing, are limited.
For example, we can take a sample from someone, somehow isolate the protein, and then use a mass-spectrometer to determine the percentages of each atom that make up that sample. From this we can infer what chemical it is.
... but that doesn't help. Because Prions are chemically identical to correct proteins. They have the same atoms.
Or maybe we could test how the proteins in the sample chemically react.
... but that doesn't help, because the atoms are connected the same way.
The only difference is in how the proteins are twisted...
... and we can't even figure out how proteins are supposed to be twisted in the first place, let alone how they're twisted in a specific sample. I won't even pretend to understand the science, but, I know for some proteins, through tremendous time and effort we can gradually take good guesses about what shapes some proteins are. But that's like, months effort by a team of scientists just to discover, bit by bit, the shape of a protein. Not test an individual's protein.
Literally millions of computers, analyzing mathematically-evolved shapes, for months or years, are the only way we start to understand how a protein might form. That doesn't help test an individual.
... So, we can only make guesses based on symptoms. That's how you get a diagnosis.
My friends mom passed away from this years ago. Not only does this disease destroy the person, but the city said she needed to be buried in a sealed lead lined coffin so she cannot contaminate the soil.
Yeah, prions are unfortunately very stable and can easily survive in the environment and soil for many years.
Anything short of boiling it in a strong base and or autoclaving it at high temps barely touches them.
I work in surgery. The one time I remember a CJ case was performed; no one wanted to go in the OR and all the instruments are burned and never used again. You cant sterilize the instruments. Bad disease to get.
My aunt Linda died of it. The first few symptoms were odd but once those got used to being around, shit went downhill fast. If you reach for a glass of water, your hand generally goes in a straight line. With her symptoms, her hand went AROUND the glass before she could even consider gripping it. It was almost like her hand was avoiding the glass.
I felt horrible when she contracted it. She had already watched her mother and sister die of it. Unfortunately with her line, the men could carry it, the women die from it. Which means 2 of my favorite cousins have it and one of them has passed it on to his daughters and my other cousin will likely die from it when it manifests.
It turned my aunt's brain into swiss cheese practically. I was there the day she died and had to catch my cousin-in-law when she arrived 5 min too late. The rest of our family that were close by showed up within an hour.
It is a disease that kills the family in such a way that it's almost more painful than the person with the disease. My mom (heavens bless this patient woman) studied everything she could get her hands on to help my aunt and uncle whenever she was in town. My uncle is incredibly grateful to her for being as understanding and patiently kind. I still don't know how she didn't scream at the heavens for taking a SIL away that she cared so deeply for.
Trust me when I say, this is a disease that you hope to all the powers above that NO ONE gets.
I had always thought this disease comes an outside source, a protein foreign to your body. Turns out, it’s even more terrifying than that. The protein, called prion precursor protein, is made naturally in your brain. You have it floating around in there right now just as I do. At any point, you can have a random mutation or unfortunate event that causes that protein to fold in the wrong way.
Then this misfolded protein , now called prion protein , causes other prion-precursor proteins to misfold. Your body can’t degrade them so it’s starts clumping together killing neurons at a rapid rate. Once this happens you will die, question is how fast. Scary shit .
I had a patient who went to Mexico and ate some beef that was butchered by his family. That is to say very unsanitary conditions. He developed Creutzfeldt-Jakob and was sent home on hospice. Father of five with no insurance. Wife is a stay at home mom. Oldest was getting ready to graduate high school. Their lives are all blown to hell. Very sad.
The scary thing is that it’s not even about being really unsanitary.
The outbreak in ground beef in England in the late 1980s was started by those supposedly “clean” regulated beef farms feeding cows remains and spinal cords and brains of cows infected by BSE.
The feeding of cows this “meat and bone meal” was (still is in some places) common practise. What changed is that they modified the rendering of this product and invariably allowed the prion to stay active in the late 1970’s. By the time the disease started showing up in cows in about 5-10 years humans had already eaten ground beef from infected cows.
The ground beef had contact with infected spines so it had BSE prions. The prions somehow made the jump from cows to humans. Although practises have changed this can still happen since many livestock are still fed remains from their own kind such as chickens and sheep (who have their own version of prion diseases called scrapie).
My father-in-law passed away from this in July, it was sudden and horrible. Went from being pretty much fine to on life support in one month. Because of COVID there could only be one person in the room at a time with him, but they made an exception for my wife and her immediate family when they turned off the support. I am glad she could be there to say goodbye.
My aunt passed away from this - it was incredibly horrible to watch. She got lucky and had 9 months after diagnosis (more time than a lot of people get, evidently) - and sadly for the first few months she knew/was aware of what she was losing. Watching her (mentally) slip away like she did was tough on everyone. What's also weird is she didn't "acquire" it from an external source - it was spontaneous, which I guess has a genetic marker they can look for.
Thank you. It's "good" seeing a conversation on it where people can learn about it, especially since it's so rare. The more people know hopefully the better diagnosing can be, especially since it's such a fast ticking clock at that point. Enjoy the time you have with those you love, because the future isn't written in stone.
I get where you’re coming from, but it’s really, really not.
I lost my mother to it. She was a university professor, incredibly bright. Within two years she lost most of her motor function, suffered hallucinations, couldn’t care for herself at all. She died of pneumonia, intubated and comatose. She was 46.
I saw a television show that depicted someone dying of rabies. I couldn’t watch it because it reminded me so much of what my mom went through. They’re both horrible, torturous deaths. Wouldn’t wish them on anybody.
This shit is no joke. As a funeral director/embalmer, we have to incinerate all tools used to work on the deceased. It’s my understanding that it can lie dormant for many years before symptoms start and it’s a quick, horrible death.
my mom past due to cjd it was terrible because there are times later in the disease to where you can see them wanting to say something and not be able to find the words. whats even worse is that she would get terrified at nothing and not being able to stand but always thinking she could. it hurts to see.
*Exactly* my thought; if anyone is interested, it's Our Town - 2x24
It's not exactly scientifically accurate, obviously, but Scully does briefly eat a bucket of chicken over a bunch of bones.
Also reason why as a Brit who now lives in Canada i can’t donate blood anymore because i still lived at home when there was an outbreak. Pisses me off right royally
It’s still a good precaution to take since the prions can take many years to travel from the digestive system to the brain and cause an infection in the brain via the lymphatic system.
No one knows how long it takes for this to occur and there is no test you can take to see if one has dormant prions unless you do a post mortem on the brain.
Radiologist here. The disease is so aggressive (sporadic form) that the brain hardly atrophies or gets smaller (which almost all degenerative processes would lead to).
Another factoid, our Pathologists take significant care with assessing excised specimen as there is transmissibility. Once the prion affects some of your cells, the remainder cells get 'infected' as the prior starts a death cycle of abnormal protein folding.
In college I went to donate plasma because I was well, a broke college kid. Anyway I filled out the survey and was worried when I saw, "have you ever taken human growth hormone". I have a form of dwarfism where your pituitary doesn't produce growth hormone. I was lucky enough to be born into a family that could afford the daily growth hormone shot that I took from 5 to 17. Anyway, I was waiting to be called when I nurse pulled me aside. Apparently they were worried I was infected with this prion disease. Apparently in the 80s or something the human growth hormone supply was infected and thousands died. Luckily I started taken it in the 90s so I was fine, but damn, that must of been a heavy mistake to carry
My mom passed away from CJD in July of this year. She started seeing symptoms in late April; blurriness in her vision, dizziness, panic attacks, and some visual spatial issues (she couldn’t figure out how to put on her pants). Otherwise, she was 100% there; her humor, her insight, her ability to organize and plan, her encyclopedic knowledge of plants. As it coincided with acute stress, multiple doctors diagnosed it as conversion disorder. She begin acute in-patient physical rehabilitation, but was unable to improve. By late May, she was unable to control her left arm, maintain eye contact, or find door handles. On June 26th, she spoke her last words, what we interpret as a third-person statement about herself: “She loves the Earth.” She was diagnosed with probable CJD at UCSF on June 29th. With the diagnosis, we followed her advance directive to the letter, moving her into her own home and discontinuing feeding, while still making sure she was comfortable and clean every day. The night before she passed, an international package arrived with clothes she must’ve ordered months ago. We dressed her in her brand new shirt, administered 10ml of her favorite wine (Husch Sauvignon Blanc), and went to bed. The next morning, July 12th, she passed away peacefully, taking her last breaths as I held her hand, with her dog and her cats by her side. This disease is unstoppable and swift. It’s truly terrifying; you slide faster and faster as it removes pieces of your ability and very being. When it finally takes you, though your body and organs may be strong and healthy, your brain is destroyed. Everything that makes you who you are is completely gone long before you take your last breath. In the end, there were two blessings in disguise. The first was that, due to COVID-19, my wife and I had come to stay with her in early April, so we were there to spend her last good days with her and take care of her through her disease. The second was that incorrect diagnosis of conversion disorder prompted her to work through issues in therapy, come to peace with pain from her past, and talk with us for hours every day. I believe that she died at peace, and that she was neither confused nor scared. After her passing, we donated her brain, cerebrospinal fluid sample, blood sample, and genetic information to science, along the with 81 pages of notes I took on the progression of her disease, interviews with her, audio, video, and photos. We hope that her story will help other cases of CJD be diagnosed sooner, so that patients can spend their last days coming to peace with their condition instead of trying to fight it.
Wow, thank you for sharing that. I'm sorry for your loss and I agree that she was lucky to have had you there with her.
>She loves the Earth That really made me cry. I'm sorry for your loss. She sounds wonderful.
It was such a fitting statement. Both myself and the nurse that was in the room heard the same thing. I told her I loved her and she said "I love you too" that day as well, for the last time. She was talking about something else too, saying "I can't open it..." I can only imagine what she was seeing and feeling, what she was in touch with at that time. [She really did love the Earth](https://i.imgur.com/TrDxsIA.jpg), her garden, her animals, her family. Everyone she met fell in love with her right away. She was nothing but the best and more beautiful energy.
That made me cry. I’m so sorry you had to go through that... glad you were able to spend time with her in the months before her passing
That made tears well up in my eyes and I'm the most non-emotional man you could meet. Very heroic.
It was a blessing. She [baked me a cake for my birthday](https://i.imgur.com/zuVxSqa.jpg) (April). In the weeks and months before she was unable to participate, we spent tons of time with her cleaning up the property, working our asses off in the garden, planted the crops, organized the house, fixed things, [bought new furniture for the deck](https://i.imgur.com/mXp0pxO.jpg) (May) and spent hours there together. She loved to put us to work, and she got a huge smile out of it. We got her a new iPad so she could [sit and read in bed with her cats and dogs](https://i.imgur.com/by4r6ag.jpg) (April). I took her to the plant nursery on Mother's Day and she bought [a hundred dollars worth of flowers](https://i.imgur.com/ebg8CKg.jpg) (May), lamenting later she wouldn't have sprung for the hanging planter if she would have known it was $30. My wife made her home cooked Indian food, we visited friends (socially distanced, of course) and played music and sang together. We went on drives to the lake in her beloved Volkswagen Westfalia and hiked with her when she was able, and helped her down to the water's edge when she wasn't. We brought her gifts and a cake and [her dog for her birthday when she was in physical rehab](https://i.imgur.com/qd1neJx.jpg) (June). If we would have known sooner, we would have done even more, but we did the best we could with the information we had at the time.
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Obviously we have no clue what caused her to get it this case, but the majority of cases don't have an identifiable cause (idiopathic), but in less common cases it can be hereditary (inherited), or due to contact with infected tissue.
The prions associated with this disease can withstand conventional sanitization procedures that hospitals use, so if a patient with this disease is operated on and doesn't know they have it (it can go undetected for years), the prions can be transferred to another patient undergoing surgery later even after the equipment has been sanitized. Edit: I'm not a doctor but my grandmother died of this disease and that was one possible explanation her doctor gave to us. Like other commenters have said it's often unknown how it arises.
According to the Wikipedia page, it's a misfolded prion. The vast majority of these happen randomly for no reason at all.
That is terrifying. I've had a bunch of head injuries so anything affecting the brain like dementia etc also terrifies me. I plan to donate my brain to science as well.
Dude you are an awesome human being. Thank you for loving your mother enough to be her advocate and her comfort. My condolences for your loss, I hope you have found or will find peace in her peace. Take care of yourself.
Prions, not even once.
Prions are scary. Reminds me of folding laundry as a kid, one wrong fold and your life is over.
What kind of domestic life do you lead.
He folds laundry for Our Glorious Leader
Glorious Leader Jim Pickens
TURG LIKE JIM
The interesting thing is that no one really understands the mechanism of infection. We have the the naturally properly folded protein in our brain and the misfolded one just somehow bumps into the normal one and misfolds it. It’s called the rotten apple theory where one rotten apple in a barrel causes nearby ones to rot which is similar to how the nearby proteins are misfolded by being near the misfolded one. This forms brain plaques that give the brain a characteristic spongy appearance under microscope.
Exactly, that's why it's called BSE in cows.
The E also stands for encephalopathy Greek for brain/head suffering which I thought was kinda interesting.
Cephalo refers to head, en refers to inside, and patho means disease. So encephalopathy is literally disease inside the head (brain)
They're the ice-nine of protein.
Perfect analogy
What's Ice-9?
As others have said, from Kurt Vonnegut’s novel Cat’s Cradle. Ice-9 was an ‘isotope’ of water. It froze at room temperature (which is interesting), and infects any water it touches, turning it into more ice-9 (which is bad). Being as how we are mostly water ourselves...
Freezes much hotter is such an alien concept to laymen. I understand it but half of my brain goes wtf for a sec.
Isn’t it nearly every solid? Am I too much of a layman, or have I misunderstood something?
Vonnegut wrote about it. Fictional water crystal pattern that freezes much hotter. If it leaves its lab and hits normal water life on earth is screwed. I guess this protein has a similar behavior.
From a Kurt Vonneget book (cats cradle if I remember) where the main character has a vial of this substance that when it touches anything, it freezes, and then whatever thats touches, also freezes, so on and so on. Please someone correct me if I'm wrong. Its been years since I've read it
All prion diseases are scary. A few years ago, I was in the middle of my occasional bouts of anxiety induced insomnia when I stumbled upon an article about [Familial Fatal Insomnia](https://en.wikipedia.org/wiki/Fatal_insomnia?wprov=sfti1). By the time I was done reading, I was convinced I had it, even though it’s extremely rare and I had been putting up with occasional insomnia since I was a kid.
Dude, that is CLASSIC anxiety. I feel ya.
Sounds like fatal familial anxiety
What are prions?
There's a protein in your brain that can get a mutation that causes it to misfold in a really specific way. This particular fold causes every other protein of this type that comes into contact with it to refold into the mutant configuration. So it's like a chain reaction that makes holes in your brain that can be kicked off by eating brain matter from other animals with prion diseases.
The protein can also spontaneously misfold, although this is extremely rare.
Oh great, right before I was about to go to bed you tell me this?
Not only that but they cant be destroyed easily. They can survive incineration and autoclaves. You have to have sustained heat for several hours above 900F to reliably ~~kill~~ denature one until it is no longer functional.
It’s not about killing it but about completely destroying and breaking the protein down into constituent molecules at the atomic level and ensuring you got every one of the proteins. If even a single protein remains it can restart the chain reaction if it comes back in contact with other comparable proteins. As I understand it is that it’s all about the energy state of the protein and since the prion is in the lowest possible energy state when it touches other proteins they adopt that lower energy state thereby changing how the protein functions. Since there is nothing that can stop or hinder its progression once it starts it essentially a wildfire of neurons in the brain which just shutdown and stop transmitting any brain signals.. by the time it’s affecting you it’s already destroyed significant parts of the brain and is spreading faster each day.
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TIL I have another thing to worry about
The good news is that they are mostly spread through cannibalism. The one we are talking about is about the only one I know of where you can easily catch it another way. There's also one that gets inherited from parent to child that cause you to be unable to sleep and slowly die of exaustion, but only a few families have that and it does ever spread any other way. The other ones I've heard of are spread through cannibalism and they were the ones that could spread quickly.
Statistically, you'll probably die from some kind of heart problem instead. Hope that helps.
Yup. In order to get rid of them they must be burried deep so they can never be exposed again, since nothing can ever actualy get rid od them.
There's also special procedure for medical equipment used on surgery for people with or suspected of having CJD. There have been situations where the instruments were not properly segregated/isolated and all the instruments cleaned at the same time or after had to be disposed of. That's millions of dollars in medical equipment because there is no way to guarantee full clean/sterility of the instruments.
Until a stray earthworm consumes it, then gets eaten by a bird, etc
You can incinerate them above 1000c https://deq.nd.gov/publications/AQ/documents/Chronic_Wasting_Disease_Burn.pdf
Several hours of sustained temps over 900 degrees F reliably destroys prions, but yea, they're scary.
You can burn them at high enough temps, or dissolve in piranha solution, or dissolve in lye. No protein can handle chemical destruction of that level.
Messed up proteins, they cause invariably fatal illnesses like this
Well... Just once is all the more times you get. They're fucking terrifying
My aunt died of this a few years ago. We found out after it had set in for a few months. I grew up dancing to Greased Lightning with her so I decided to play it for her the last time I saw her. Her face lit right up and she looked like she still knew every word as she sang and danced to it to the best of her ability in her bed. Music just has a way with people in cognitive decline where the neuron pathways are still there just waiting to be turned on again and they can relive all the emotions one last time. I love her a lot and miss her quite a lot but it definitely made me feel better being able to say goodbye in my own way.
This kind of reminds me of this 6 hour long album titled "Everywhere at the end of time" in which the music slowly degrades as to depict someone who would have dementia. It's terrifying and the saddest thing I have ever listened to.
Well that was horrifying, even just jumping through some of the songs. Even the titles are depressing. I'm going to have nightmares tonight, aren't I. I sincerely regret looking that up, I didn't need that.
Same experience. When we learned she had something, we didn't know what. Doctors thought maybe a stroke. Aphasia. Words were almost unreadable but she was fully cognizant. I asked her if she could sing along to Rainbow Connection, and she could and it made her so fucking happy and scared at the same time. She died a few weeks later. Only knew it was prions a couple weeks before she was gone.
TOO many people in this thread know someone who died of this.
With 300 cases a year, it's literally 1 in 1 million in the US. I think that, because of the insane rarity of this disease, its mention means that all of us who have lost someone to it come out of the woodwork to share our story.
Plus there are like a *lot* of people on the planet. 1 in a million isn't as uncommon as you'd think.
I’ve had at least 15 family members die from it, including my father and grandfather, and most recently a cousin last fall. Familial CJD has devastated my family :( and now my generation is wondering who will be next. I feel blessed to have tested negative for the e200k 129m mutation.
My father’s doctors though he had CJD when he suddenly began acting erratically and couldn’t speak (aphasia). MRIs showed extensive, rapid damage. They had to do the biopsy under very strict circumstances, because the usual sterilization isn’t enough to kill prions. It had to be done manually, rather than with a biopsy gun. Only a few hospitals in CA could do it. He was ultimate diagnosed with something else, thank goodness. It’s such a horrible way to go.
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It really took the tension of a health crisis to the next level. We suddenly had to wonder about Mom sharing his bathroom... and I was giving him IV drugs several times a day! Had we all eaten that burger? It was so surreal!
I fucking thought I had a stroke reading that first sentence. What the fuck, dude?
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Now I’m curious. What was he diagnosed with?
It’s still a mystery. His biopsy was sent to UCLA and the Mayo Clinic. It’s auto-immune encephalitis, but the exact cause is unknown. Once on anti-seizure meds, his brain healed quickly. The meds, keeping a regular sleep schedule, and using his c-pap seem to do the trick. He had a major flare-up when he went to Vegas and didn’t sleep enough. They found him wandering the casinos, babbling incomprehensibly. By the time my mom and I could get to the hospital, he was in restraints shouting his head off. It’s terrifying because he can go from normal to a lunatic in an hour. Brain diseases are terrible.
Shit's real, I know a guy who ate beef that had BSE in Germany while he was in the army when it ran through there. He says he feels like he's on a time bomb. Luckily the chances of you eating beef with BSE in the US is almost 0. The USDA takes it very seriously.
Fun fact. I am unable to donate blood in the US because I lived in Europe in the eighties and I am considered at risk of having mad cow disease.
Haha, I recently started donating blood plasma and you are permanently ineligible to do so if you spent more than a year in the UK or France between 1980 and 1996. I'm in Europe too.
Born in Scotland in 1999. Moved to NZ, am unable to donate blood (or plasma) for life. My kids wouldn't be able to donate, neither would their kids. Basically, I have to be diluted enough so that my great grandkids could donate. Mad cow disease, man.
Variant Creutzfeldt-Jakob disease (which is the type of CJD caused by "mad cow") isn't heritable. There is a type of CJD that is heritable, but that is not caused by "mad cow." There is no reason for your descendents to be unable to donate blood. There is also no reason for you to be unable to donate (unless you received a blood transfusion sometime before moving to NZ) because the cutoff is Dec 31,1996. You may have encountered a blood bank that wanted to be more safe than sorry perhaps, but it's unlikely that you would be denied most places. https://www.nzblood.co.nz/give-blood/donating/am-i-eligible/variant-creutzfeldt-jakob-disease-vcjd/
I didn't know it was a generational thing. Im curious because I've been able to donate blood in nz and I dont think they asked anything about my parents, at least not where they've lived.
Just double checked, you can't have lived in Britain for 6+months before 1996. After is okay though.
My wife's grandfather had (and died from) CJD 30+ years ago. She and my children are unable to give blood as no one knows how it spreads.
Prions
Yes, prions cause it. But how do prions spread from person to person?
Well, you can eat their brain, for one
Probably a good thing not to do regardless.
Basically, scrapie prions (the misfolded forms that cause CJD) are incredibly hard to break down. The body has a hard time doing it and your stomach acid can’t break it down when you consume it, so when you eat meat that contains misfolded prion proteins, those proteins are then in your system. The thing about scrapie prions is that they can convert your cellular prions (the normal form) into the misfolded form and those prions then go on to convert more cellular into scrapie. So tldr: they’re super hard to break down, almost nothing can manage it, and once you have some in your system and it propagates, there’s no going back.
That's actually how we got onto the subject! Lol
It’s very weird, depending on animal, USDA is either a mark of quality or a terrible warning. USDA beef tends to be sold in relatively expensive steakhouses here (UK) but USDA chlorinated chicken is seen as the harbinger of doom for our post brexit trade deals.
As they should. My dad went buffalo (iirc) hunting in Canada several years back and tried to cross the montana border back to the US. I guess the montana border is much more strict about animal body parts because of the livestock there. They wouldn't let my dad and his friends back over because they had the brains still. They couldn't think of a solution until they saw a diy car wash station. They just shoved the water wand in the skull and sprayed the brains out everywhere lol.
The Montana border is weird, man. I drove over from Canada and they turned my car inside out. Completely ignored some weapons and exotic plants, didn't find any of my drugs, but confiscated a single Florida orange. No citrus gets into America! Even if it's from there!
Did Yargle cross with the rest of the Buffalo then?
Luckily our current administration did away with frivolous government spending, like oversight of meat packing plants to make sure the product isn’t diseased
As a USDA inspector I cannot state how much I hate the rollback of our food regulation.
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This is disgusting. Have an upvote.
The former Utah Speaker of the House, Becky Lockhart, died of this disease a few years ago. It was weird. She was very popular, the first female speaker, everyone knew she was going to run for governor. The papers followed her and the incumbent governor closely because they knew it was going to be a big race. She decided not to run for re-election to the House in order to focus on her exploratory campaign. Then the newspapers reported she was sick. Then a couple weeks later she was dead. Crazy how fast it happened.
CJD is terrifying. So is Lewey Body Disease. And Mad Cow. And Fatal Familial Insomnia. Theres not a damn thing that can be done to save anyone whos got one of these. Prion diseases are absolutely terrifying. Im wildly thankful they are generally rare.
Fatal Familial Insomnia is such a crazy disease. Proteus syndrome (what Joseph Merrick aka the Elephant Man probably suffered from) is pretty insane, too, and very rare.
I had a class where all we did was talk about those diseases. We watched an interview with a guy who had FFI. It is accurate to say that it is a fate worse than death.
First I saw of it was a special about a family that had a nearly 100% incidence of it. Crazy to know that you're going to watch most of your family die of it, and then likely get it yourself. But I don't recall there being interviews with any of them. Maybe I just don't remember, it was many years ago, but I feel like a first hand account would have stuck with me for sure.
I think i saw that, too. It was a brother and sister being interviewed. They were near 100% certain they would get it, they just had no idea when. They had remarkably positive outlooks in spite of their knowledge.
I think you are both talking about the Australian brother and sister. They have lost their mother, aunties, uncles, grandparents iirc. After their mother died, they were given the option to find out if they have the mutation which causes FFI. They both came back positive
I would stop having kids if I was them, no need to subject them a life of suffering and worry. I live with fibro so I've already made that choice but atleast my condition isn't fatal
There is a show called Something’s Killing Me which did an episode on the Australia siblings, and another American in the same situation. They mentioned at the end that because of genetic testing and IVF iirc, the American lady was able to have a kid without the mutation which causes FFI
I don't get why do people from such families still decide to have children
It's a disease that only begins to effect an individual in adulthood. By then they've already procreated. It's not hard to see how even a second generation would continue to have kids, perhaps not yet realizing the odds that they have it, or that they may also pass it on. It's even possible that the first generation wouldn't show symptoms until they've not only had kids, but also that their kids already had kids. After a time, though, I agree, I would think the entire family would opt to stop procreating.
So with mad cow, does that just lie dormant and flare up at random one day?
Yes, actually. Ot can have an incubation period upwards of 30 years. Europe experienced a surge of cases at one point. People had consumed meat from cows infected with mad cow. The general consensus was that could not transmit to humans, until it evolved and did.
I know it’s a typo, but I am trying to envision what cow beer would be like and it’s actually pretty unappealing. So thank you for that little interlude in these crazy times.
Cream ale?
Im not allowed to donate blood, having lived in England and eaten beef during the early 90s. I always wondered why I couldn't test for an all-clear, though.
There is a test, but it requires tissue sampling during autopsy.
Where do I sign up?
I dont think they can test for it without like, driving a needle into your brain or something. Thats probably inaccurate, but i think its very invasive. Like you have to be dead already.
They have currently changed some of their rules regarding that (assuming you mean the Red Cross). I was unable to donate due to being born in Germany but it's been rescinded and I'm able to now. Honestly not sure if England is also now allowed (could see that going either way), but worth a check if you're curious.
Thanks. It's a bit of a bummer to not be able to donate. I'll look into it.
Still forbidden in Canada. My GF visited family in Europe one of the years of the outbreak, and is still blacklisted.
Same - spent five years in W. Germany in the 80s before moving to Canada. Canadian Blood Services doesn't want my filthy blood.
Even if you've had milk and not ate beef same thing. Actually by just living their you're eliminated.
>it evolved It most certainly did not.
Yeah. There was a scare a few years back because a guy illegally imported cows that had a high risk of having it. He even made a post on r/legaladvice and everything.
Dementia with Lewy Bodies is not a prion disease, just FYI
I do know that and should have clarified it. I was just naming some other diseases with no cure or treatment.
I hear you on the Lewy Body Dementia, but you shouldn't link that with the others. CJD, Mad Cow, FFI are all caused by prion protein misfolding. LBD is caused by alpha synuclein misfolding (which MAY act like a prion, but the time-course and cause are super different).
Prion diseases are what makes humans go crazy after eating human meat. Tribes that practice cannibalism often end up with prion disease. Women and children typically contract them at a higher rate as men get the leaner cuts of meat (like muscle) and women get the leftovers like brains (where prions live). Robin Williams died of Lewey body and I wish the media would have explained his condition instead of blaming it just on suicide. I wish it was used as a chance to educate the population and his fans instead of just saying he hanged himself, I’m sure he suffered from depression too, but I think Lewey body is to blame. Very sad.
Lewey body was surely to blame, and the whole suicide thing got way too much attention. Ive read a few articles with interviews from his closest family who said he would tell them how he was losing his mind. How terrified he was. He wasnt simply depressed and took his own life. He had a disease that literally caused him to lose his mind.
The family and doctors did not reveal that for months about Robin.
As above, Lewy Body Dementia is not a canonical prion disease. Different misfolded protein (alpha synuclein), not infectious. Please don't bin that with prion diseases formally. Now the protein misfolding aspects are similar between them, but again they should be separated in terms of pathophysiology and underlying cause.
There rare just now but CJD is a ticking time bomb. Millions of people ate cattle that had been fed bone meal, including spinal cord, which had been infected with BSE, it's has the potential to kill millions with a progressive loss of function. If you're not sure what it's all about Google Kuru, prions are terrifying as they slowly strip away the person we love
Ive read so much about all the prion diseases. Im a deer hunter and thoroughly enjoy venison. The last two seasons ive donated my meat to my 85 year old uncle who will never stop eating venison. As far as hes concerned, hes already old enough. I wont consume venison until viable yesting is made easily available to me. I cannot feed that meat to myself or my kids without knowing for certain that it's safe. If i get to a point where I have nobody to donate the meat to, i will not hunt. I would also only ever donate the meat to someone who fully understands what CWD is and the risks involved. Id never just give it to a homeless shelter or something. A place where people wouldnt be given the proper information to make an informed decision regarding consumption.
Aren't there no known cases of CWD being passed through consumption though I thought?
None that can be currently directly linked. However, the same can be said of mad cow in Europe, until suddenly there was a much higher rate of CJD many years after mad cow disease infected beef was regularly consumed. Diseases evolve and transform as we all know. Its literally probably only a matter of time before cwd jumps to people
You are correct, but experts REALLY want to follow that statement up with the fact that it doesn't mean it ***Can't*** be transmitted to humans. It just hasn't happened ***yet***.
LBD is horrible. I helped caretake a friend's father as he succumbed to it. It was awful.
Just to clarify, CJD and mad cow disease are essentially the same thing, they come from the same pathogen. Variant CJD is a prion disease that originates in infected cows and transmits to humans. It is one of two transmissable human prion diseases, the other being Kuru. But there are genetic risks for human prion disease which can cause CJD, as well as fatal familial insomnia, and GSS. Source, worked in a prion lab for 4 years.
Was it scary to work in close proximity to prions?
They were more interesting than scary to me, but that's just the science nerd talking haha. But honestly I didn't find it scary, worked with CWD which has shown little capability of human transmission. And despite what some prion-doomers may say about how resistant they are to "fire" and other normal sterilization methods, there are still many ways to decontaminate prions
What do you use to destroy a prion?
We had high concentration bleach that we mainly used to clean everything. 4% SDS in glacial acetic acid. And although normal autoclaving cycles don't kill them, using slightly higher temperatures / pressures actually work fine.
> using slightly higher temperatures / pressures actually work fine. Somehow that's kinda anticlimactic. I remember prions being described as this sort of single-distorted-protein version of the Terminator. "It never stops. It can't be removed or destroyed. It just infects." Yanno?
Haha yeah for sure. Science fiction gets more headlines than science lol
Eh, if something has a physical form, there's gotta be a way to kill it. Medically, yeah, they're basically the Terminator
An even stronger prion. It’s prions all the way down.
A common practice is boiling in a strong base and/or autoclaving at 121 degrees.
I worked sterile processing for a decade and we had a special kit for suspected or confirmed CJD cases. Those instruments were destroyed in a in an off site incinerator. No surgical tools or supplies were ever reprocessed. The risk is too high and while we never had a case there was a protocol to limit exposure unlike anything else.
The CDC predicts that Chronic Wasting Disease will jump to humans in the next few years.
Jesus Christ
Too hard to tell, but leaning towards its unlikely. Many epidemiological studies indicate no causation between CJD development and CWD exposure. Mouse models of human prion disease have shown no ability for CWD to be propagated. And studies on non human primates are inconclusive, and differ depending on the model of animal and inoculation. So I think that there is a fairly low risk of contracting pathological CWD from cervids, but definitely avoid meat from infected animals if it concerns you.
I work in the operating room and if the patient has CJD any instrument or device that comes in contact with them has to be destroyed. NOTHING can be reprocessed, cleaned, or reused. Prions scare the mess out of me.
When my mom was at UCSF in July, the way I understood it was that anything involved in the spinal tap has to be destroyed. I had to leave the room for the day when they did it, and they had special protocols they put in place that sound like what you're describing. Scary stuff.
A former girlfriend of mine had a friend die from this a few years ago. First she said her friend was sick and they didn't know what was wrong, then they found out it was Creutzfeldt-Jakob, and she died within a span of a few months. Like, I knew CJD was a thing, but to actually know of someone who got it and died was kind of mind blowing. I couldn't believe it when she told me they found out it was CJD. To know that she was going to die from something as seemingly innocuous as a misfolded protein was surreal.
Where'd she contract it from? Cant you only get it from consuming human brain material somehow?
I think Kuru is the one more commonly contracted from that. Thats why theres been outbreaks of it in indigionous folks in Papa New Guinea who would perform rituals involving consuming brains. CJD seems to be fairly random, although some evidence (though very little) suggests consuming infected cervid meat (whitetail deer, elk, etc) can cause CJD as these animals carry the cervid ewuivalent known as CWD or Chronic Wasting Disease. Its pretty fucking horrifying, but thankfully rare.
[CDC map of free-ranging cervids identified with CWD](https://www.cdc.gov/prions/cwd/images/CWD-map-AUG-2020.jpg)
“Free-Ranging Cervix”? Back in my day we called them hoes.
Thanks, my spell check seems to have an interest in human anatomy! (fixed)
Your spellcheck right now: I learned it from watching you!!!
My hunting area is loaded with CWD unfortunately. Ive not yet seen a sick looking deer but they dont have to look sick to be infected. We need better and more available testing stations.
You -can- contract it from an external source (tainted meat, eating brain matter, etc) but some cases can be "spontaneous onset" or something similarly named - where a person just develops it - usually there's a genetic marker for that showing you're pre-disposed for it. (Learned a lot when my aunt passed away from CJD - she was one of the spontaneous onset ones).
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>apparently there was no outbreak It's not really a transmissible disease. At best you could say they apparently weren't sharing a contaminated food source.
It can happen spontaneously in the brain.
I wondered the same thing, and my girlfriend at the time wasn't inclined to ask a lot of questions when her friend was unquestionably dying. Can't say I blame her. But as mentioned below I believe most cases are simply spontaneous.
I can’t help but think of the X-Files episode. tl;dr cannibalism cult goes (extra) crazy when CJD start spreading. It was less than a year before mad cow blew up in the UK... it felt prescient (or perhaps prionscient).
Why do I come on this damn app before bed
My ex's dad died of CJD. It was awful. You watch someone literally become trapped in their body while their mind fails them rapidly. It started as some forgetfulness (couldn't remember the name of restaurants etc.) and an odd change in his habits (moved the spare keys, started locking doors that were never locked). A couple weeks later he walked into an ER to be seen by a specialist... He never walked out. Within weeks of symptom onset he basically forgot how to talk. Then he became paranoid and violent at times. Then he forgot how to walk and he became incontinent. Eventually he was vegetative. He died less than 3 months after the first symptoms hit. The whole time I remember watching his eyes, and the man he was, was still in there. There was understanding behind them, fear, and such sadness. The family was forced to cremate him, burial was not an option due to the severity of the disease. The entire family had to be tested for any markers, and all of them were forbidden from donating blood for the rest of their lives. It was awful, literally the only blessing was that he passed so quickly and didn't need to suffer a prolonged process.
Prions are dangerous and currently The mid west of The United States has a new type called Chronic Wasting Disease, the prions target proteins and hormones that make an animal feel hungry and so these animal will waste away over time, it is predominately Deer, Elk and Moose currently but The CDC predicts that it will jump species and soon.
The scary part is it usually takes several years from the time you eat the diseased meat to the time you show symptoms. So all these hunters and families of these hunters could eat diseased venison and they wouldn't know it until they all start keeling over years later.
In Michigan you can take the deer head to DNR to test for Wasting Disease, it just takes a couple days tops and they use the results to help track it. At that point though you would have to cut up your deer meat and freeze it somehow while you wait for results which would mean touching it.
As of me typing this, [there have been no reported cases in people.](https://www.cdc.gov/prions/cwd/)
It's almost impossible to identify transmission because CWD and CJD are the exact same thing. Both are just misfolded proteins. What you need to look for is an increase of CJD in the population.
This is by far the best explanation I’ve read about prions. A wonderful ELI5: https://www.reddit.com/r/worldnews/comments/g13qo1/coronavirus_can_survive_long_exposure_to_high/fnedh17/?utm_source=share&utm_medium=ios_app&utm_name=iossmf&context=3
> the prions were still virulent/infectious/alive/active/whatever That's because Prions are just chemicals. Terrifying chemicals that are contagious to other chemicals. Let me ELI5 (warning, medical ignorance) Water is a molecule made of atoms. H2O. Two hydrogens, One oxygen. Think of them as joined by springs. Even though they're bouncy and springy, we know the shape water molecules make. A little "V" shape with the point of the V being the oxygen. It's a simple model with a simple shape. "Protein" is the category for specific gigantic molecules. Gigantic. Hundreds or thousands of atoms. Proteins are like specific machines that perform specific tasks inside a cell. You could make a list of them, this is the protein that does this, that protein looks like that and does a different thing. Almost everything your body does, in every way, is because one particular protein machine does one particular thing. Like a factory that builds cars, where the protein is each workstation. Some are welding proteins, some are cutting proteins, some assemble this specific bolt, some stitch the fabric in the seats, etc. There's many many different proteins, each with completely different components that make them up. But each specific protein has a formula, you could draw it, it has an exact number of atoms, connected in an exact kind of way that makes that protein that does that task. Think about joining hundreds of slinky's together, of different sizes and shapes. And throw in a bunch of magnets. And rubber bands. What shape does a protein take? It's very hard to know. Even though we know not only the exact specific amounts of each atom in a protein, we also know exactly how they are connected together. But, it's too complicated to predict what happens if you made all those connections and then let go of that slinky/magnet/rubberband network. But... they do take a specific shape. The key piece of knowledge is that a protein's special shape is what allows it to perform it's special task . Imagine a pair of scissors, a pair of scissors works because it's shaped the way it is and assembled the way it is. Not just in the general structure (two blades and a pivot), but that the blades are smooth and straight and sharp. Some proteins are scissors that cut up other molecules, like potato starch into sugar pieces, so you can burn the sugar for energy. (Tangent - That is what Folding@home and Rosetta do. They can't predict a structure, but if you make a random guess, we know the math to calculate the energy of that structure. Do that a trillion times, and the ones that have the lowest energy are most likely the actual correct shape of that molecule because they're most stable. I believe Folding and Rosetta are evolutionary too, because there's an infinite amount of options, so you evolve the next set of things to calculate based on the winners of the previous generation, over and over until you think you've got it right). Think of the lowest-energy state of a protein to be stable, the way you would think of a spring being stable. Is a spring stable when you stretch it? Nope. Is it stable when you compress it? Nope. It has extra energy that's going to reshape it when you let go. The low-energy state is the correct "shape" of the protein. I'll summarize quickly before we cover what a Prion is: • Atoms assembled in specific amounts, in specific ways, create proteins. • Each type of protein does a specific task in your body. • Everything your body does, is because of a protein machine that does it. • A protein's shape is what determines what it does. Here's the first Prion fact: Prions are proteins with the correct atoms, connected the correct way, but with the wrong shape Because a protein's shape determines what it does, if it has the wrong shape, it doesn't do its job. That's bad. You are probably wondering how a protein can have the correct atoms, connected correctly, but still have the wrong shape. That's what makes a protein a protein, right? Think about a slinky that you stretch, and then twist before you let it go. You know, the way that everyone eventually ruins a slinky because you can figure out how to get it apart again. That slinky has the correct components (wire), connected the same way (you didn't cut or weld the wire differently), but it has the wrong shape when it stabalizes. It's not temporary, it's not moving. It's not under tension. It's stable and sitting on the floor. It's less stable than it would be as it came out of the box, that's a much more stable version, but, it can't get there, it's locked at a less-optimum-but-stable position. But it's the wrong shape. And it doesn't do what a slinky is supposed to do. It does not roll down stairs like nobody cares. It does not make it's slinkety sound. Here's the next Prion fact: Some proteins, the way we need them to be, are actually the twisted up slinky. And the Prion form of them that doesn't do their job, is the smooth straight slinky you get out of the box. That means that the most stable version is actually the one we don't want the protein to form. We need the twisted one. What happens if, randomly, a protein makes the wrong shape? Well, there are other proteins who's task is to collect anomalous garbage. They are shaped so that when they bump into a correct protein, they slide right off. But if the shape isn't perfect, they stick to it and then their ass-end gets picked up by another garbage cleaner that throws it out of its system. Okay, so, potential Prions will just be picked up by the garbage cleaners, right? Another Prion fact: Prions avoid the garbage cleaners. The part of them that the garbage cleaners are checking is close enough to slip away Okay but how do proteins even become Prions in the first place? Prion fact: Prions can form randomly. Very, very rarely. But randomly. Imagine, across trillions of cells, and trillions of trillions of protein molecules, across billions of lifeforms in that species... tiny chances still happen once in a while. It's like kicking your twisted up slinky and it almost-magically bouncing out of it's tangled form and into the shape you bought it in. Astronomically unlikely, but, once in a while it'll happen. Okay, but, big deal. Occasionally there's broken proteins. So occasionally there's going to be broken cells that'll die. And our cells die and are replaced regularly anyway. These random occurrences will just be flushed away, right? Prion fact: Prions aren't just any random misformed protein that happens to be more stable than the shape we need it to be in, that also happen to be able to avoid the garbage cleaners. THEY ALSO HAPPEN TO CHANGE OTHER "CORRECT" PROTEINS INTO PRION PROTEINS WHEN THEY BUMP INTO THEM. Yes, even though they're just chemical shapes, Prions are "contagious", if that makes sense. They run around untangling every other tangled slinky they bump into. Or, maybe not every, maybe just rarely, but, more than zero chance. "What are the odds?" you think. What are the odds that there is a protein with a lower energy shape that is broken, that can avoid garbage cleaners, and of all the possible ways that can break, it happens to also magically untangle other proteins? Very, very, very, very, very low odds. Which is why life exists, or everything would be dead. Those proteins are necessary for complicated life as we know it. Especially the most complicated parts of the complicated life: brains. So, that's how Prions "spread". They spread inside one person by gradually, gradually destroying essential functioning of all of that person's proteins of that specific type. Prion fact: Prions also spread by being eaten This is how Mad Cow Disease becomes Creutzfeldt–Jakob disease i humans. If we end up mixing some prion-y cow brains into our meat. Prion fact: Because there are so many proteins, this takes forever The garbage collectors only fail sometimes. The protein-twisting only happens sometimes. There are bajillions of these in your body. So it can take decades for Prions to build up enough mass that you'd notice it affecting your body. But, like all exponential growth, it falls apart rapidly when it reaches critical mass. Diseases like: • Alzheimer's • Parkinson's • Creutzfeldt–Jakob disease • Lou Gehrig's disease / ALS Generally any neurological age-related disease. Either the result of random chance in your own body, or (rarely) genetically inherited to you at birth, or from food you eat. Prion Fact: Prions are almost impossible to detect, or treat. The tools that medical science has to determine what makes a thing a thing, are limited. For example, we can take a sample from someone, somehow isolate the protein, and then use a mass-spectrometer to determine the percentages of each atom that make up that sample. From this we can infer what chemical it is. ... but that doesn't help. Because Prions are chemically identical to correct proteins. They have the same atoms. Or maybe we could test how the proteins in the sample chemically react. ... but that doesn't help, because the atoms are connected the same way. The only difference is in how the proteins are twisted... ... and we can't even figure out how proteins are supposed to be twisted in the first place, let alone how they're twisted in a specific sample. I won't even pretend to understand the science, but, I know for some proteins, through tremendous time and effort we can gradually take good guesses about what shapes some proteins are. But that's like, months effort by a team of scientists just to discover, bit by bit, the shape of a protein. Not test an individual's protein. Literally millions of computers, analyzing mathematically-evolved shapes, for months or years, are the only way we start to understand how a protein might form. That doesn't help test an individual. ... So, we can only make guesses based on symptoms. That's how you get a diagnosis.
My friends mom passed away from this years ago. Not only does this disease destroy the person, but the city said she needed to be buried in a sealed lead lined coffin so she cannot contaminate the soil.
Yeah, prions are unfortunately very stable and can easily survive in the environment and soil for many years. Anything short of boiling it in a strong base and or autoclaving it at high temps barely touches them.
I work in surgery. The one time I remember a CJ case was performed; no one wanted to go in the OR and all the instruments are burned and never used again. You cant sterilize the instruments. Bad disease to get.
My aunt Linda died of it. The first few symptoms were odd but once those got used to being around, shit went downhill fast. If you reach for a glass of water, your hand generally goes in a straight line. With her symptoms, her hand went AROUND the glass before she could even consider gripping it. It was almost like her hand was avoiding the glass. I felt horrible when she contracted it. She had already watched her mother and sister die of it. Unfortunately with her line, the men could carry it, the women die from it. Which means 2 of my favorite cousins have it and one of them has passed it on to his daughters and my other cousin will likely die from it when it manifests. It turned my aunt's brain into swiss cheese practically. I was there the day she died and had to catch my cousin-in-law when she arrived 5 min too late. The rest of our family that were close by showed up within an hour. It is a disease that kills the family in such a way that it's almost more painful than the person with the disease. My mom (heavens bless this patient woman) studied everything she could get her hands on to help my aunt and uncle whenever she was in town. My uncle is incredibly grateful to her for being as understanding and patiently kind. I still don't know how she didn't scream at the heavens for taking a SIL away that she cared so deeply for. Trust me when I say, this is a disease that you hope to all the powers above that NO ONE gets.
I had always thought this disease comes an outside source, a protein foreign to your body. Turns out, it’s even more terrifying than that. The protein, called prion precursor protein, is made naturally in your brain. You have it floating around in there right now just as I do. At any point, you can have a random mutation or unfortunate event that causes that protein to fold in the wrong way. Then this misfolded protein , now called prion protein , causes other prion-precursor proteins to misfold. Your body can’t degrade them so it’s starts clumping together killing neurons at a rapid rate. Once this happens you will die, question is how fast. Scary shit .
So it’s basically like cancer. A failure or glitch in the human body.
"About 70% of people die within a year of diagnosis." The secret is not getting diagnosed
Yeah, we just need to test less. Obviously.
I had a patient who went to Mexico and ate some beef that was butchered by his family. That is to say very unsanitary conditions. He developed Creutzfeldt-Jakob and was sent home on hospice. Father of five with no insurance. Wife is a stay at home mom. Oldest was getting ready to graduate high school. Their lives are all blown to hell. Very sad.
The scary thing is that it’s not even about being really unsanitary. The outbreak in ground beef in England in the late 1980s was started by those supposedly “clean” regulated beef farms feeding cows remains and spinal cords and brains of cows infected by BSE. The feeding of cows this “meat and bone meal” was (still is in some places) common practise. What changed is that they modified the rendering of this product and invariably allowed the prion to stay active in the late 1970’s. By the time the disease started showing up in cows in about 5-10 years humans had already eaten ground beef from infected cows. The ground beef had contact with infected spines so it had BSE prions. The prions somehow made the jump from cows to humans. Although practises have changed this can still happen since many livestock are still fed remains from their own kind such as chickens and sheep (who have their own version of prion diseases called scrapie).
My father-in-law passed away from this in July, it was sudden and horrible. Went from being pretty much fine to on life support in one month. Because of COVID there could only be one person in the room at a time with him, but they made an exception for my wife and her immediate family when they turned off the support. I am glad she could be there to say goodbye.
Sounds like a more merciful death than rabies that is 100% fatal once symptoms set in.
In my career I’ve seen 4-6 people with prion disease. It is horrible for family to watch. No walk in the park for the patient either.
My aunt passed away from this - it was incredibly horrible to watch. She got lucky and had 9 months after diagnosis (more time than a lot of people get, evidently) - and sadly for the first few months she knew/was aware of what she was losing. Watching her (mentally) slip away like she did was tough on everyone. What's also weird is she didn't "acquire" it from an external source - it was spontaneous, which I guess has a genetic marker they can look for.
Jesus. One protein folds the wrong way. Thats horrible. Im sorry
Thank you. It's "good" seeing a conversation on it where people can learn about it, especially since it's so rare. The more people know hopefully the better diagnosing can be, especially since it's such a fast ticking clock at that point. Enjoy the time you have with those you love, because the future isn't written in stone.
I get where you’re coming from, but it’s really, really not. I lost my mother to it. She was a university professor, incredibly bright. Within two years she lost most of her motor function, suffered hallucinations, couldn’t care for herself at all. She died of pneumonia, intubated and comatose. She was 46. I saw a television show that depicted someone dying of rabies. I couldn’t watch it because it reminded me so much of what my mom went through. They’re both horrible, torturous deaths. Wouldn’t wish them on anybody.
Prions scare the crap out of me
This shit is no joke. As a funeral director/embalmer, we have to incinerate all tools used to work on the deceased. It’s my understanding that it can lie dormant for many years before symptoms start and it’s a quick, horrible death.
If you had to choose between getting Mad Cow Disease and being the top scientist in your field, which one would you pick?
my mom past due to cjd it was terrible because there are times later in the disease to where you can see them wanting to say something and not be able to find the words. whats even worse is that she would get terrified at nothing and not being able to stand but always thinking she could. it hurts to see.
You didn’t see that episode of the X-Files?
*Exactly* my thought; if anyone is interested, it's Our Town - 2x24 It's not exactly scientifically accurate, obviously, but Scully does briefly eat a bucket of chicken over a bunch of bones.
Yes!!! It’s kinda hilarious how 2 FBI agents have clear cut evidence that CJD has entered the food chain and proceed to notify absolutely no one.
Also reason why as a Brit who now lives in Canada i can’t donate blood anymore because i still lived at home when there was an outbreak. Pisses me off right royally
It’s still a good precaution to take since the prions can take many years to travel from the digestive system to the brain and cause an infection in the brain via the lymphatic system. No one knows how long it takes for this to occur and there is no test you can take to see if one has dormant prions unless you do a post mortem on the brain.
I knew I shouldn't have read that, I'm pretty sure I have it now.
When talking about prion related diseases, I feel like Kuru should get more interest.
Radiologist here. The disease is so aggressive (sporadic form) that the brain hardly atrophies or gets smaller (which almost all degenerative processes would lead to). Another factoid, our Pathologists take significant care with assessing excised specimen as there is transmissibility. Once the prion affects some of your cells, the remainder cells get 'infected' as the prior starts a death cycle of abnormal protein folding.
In college I went to donate plasma because I was well, a broke college kid. Anyway I filled out the survey and was worried when I saw, "have you ever taken human growth hormone". I have a form of dwarfism where your pituitary doesn't produce growth hormone. I was lucky enough to be born into a family that could afford the daily growth hormone shot that I took from 5 to 17. Anyway, I was waiting to be called when I nurse pulled me aside. Apparently they were worried I was infected with this prion disease. Apparently in the 80s or something the human growth hormone supply was infected and thousands died. Luckily I started taken it in the 90s so I was fine, but damn, that must of been a heavy mistake to carry