Paper:
Cadaveric Human Growth Hormone–Associated Creutzfeldt-Jakob Disease with Long Latency Period, United States - https://wwwnc.cdc.gov/eid/article/31/6/24-1519_article | https://doi.org/10.3201/eid3106.241519
The paper makes it clear that the analysis of the prions showed it was iatrogenic (meaning it was caused by a medical treatment) and not something that she acquired by some other pathway such as eating meat or brain from an infected animal).
I guess maybe the immune system protects a bit against the disease but weakens as you get older?
I remember when Mad Cow hit in the 90s. It seemed like not much happened, but one of the unmentioned little details was that the time frame of the disease matched the lifespan of the animal.
I realized then that we wouldn't know for decades how many humans caught it.
Not much happened to people but mad cow(prion diseases in general) completely changed US beef/cattle production.
For example, it used to be common practice to feed dead cattle to the remaining cattle. That changed. Some ranchers still do it, though. We also changed the breeding programs and import/export. A lot of countries, because of prion diseases, basically stopped importing US beef.
https://www.nytimes.com/2004/01/05/us/mad-cow-forces-beef-in...
The US is also dealing with a chronic wasting disease issue in deer and other wild animals right now. It's one of those "about to explode" problems.
https://www.aphis.usda.gov/livestock-poultry-disease/cervid/...
What the fuck they made cows eat other cows? Dear God.
If you drive through farm country you see "fat" advertisements for finishing cattle or increasing milk production. IE: https://energyfeeds.com/magnafat/
Normal feed/grazing isn't optimal or cost effective compared to other options, for the animal's full lifecycle.
It used to be common place to grind it up and put it in the feed for animals.
Ranchers do a ton of shit that is pretty disgusting and messed up. If you call them out for it they'll get pretty upset and call you a commie or unamerican.
A lot of ranchers have "dead piles" where they just let the bodies rot. Dairy ops also routinely do the same stuff, along with just dumping milk on the ground or in "manure pits" which often leak into the groundwater.
I grew up in this world. Small town, ranching/farming, conservative. The stuff ranchers and dairy ops do is mindblowingly fucked up. They call it tradition and if you call them out you will be attacked.
A while back the governor of Colorado was like "Yo let's designate one day where people don't eat beef. It'll be good for the environment." Colorado ranchers lost their fucking minds. They threw huge hissy fits and ran campaigns to get people to consume more meat on that day. So dumb. Find some videos of the ranchers basically crying and throwing fits, they're pretty funny https://www.koaa.com/news/covering-colorado/communities-gath...
You've got a very ignorant view of dairy farming. Now I'm sure there's some operations in those western desert states that behave that way but it is absolutely not the norm east of the Mississippi.
I've got a friend who owns a dairy farm. He doesn't keep dead cows around because the risk of introducing disease isn't worth it.
Manure gets treated in a "lagoon" (which looks like an open pit to you) wherein the water evaporated and bacteria breaks it down to become fertilizer for the next guy. The system is specifically designed to keep it from running off. Good dirt work is cheaper than having you people up his ass over pollution.
If he dumps milk it's like 20gal from purging equipment or something, not enough to matter. Milk is money so he tries to avoid dumping any. If he did want to dump a bunch of it, he could dump it straight into the lagoons because a liquid designed to be nutritious for growing mammals will be a walk in the park for bacteria that feed on what the mammals leave behind.
And 40yr ago when he dad owned the farm all of this was the same. The technology has changed a little but they weren't piling bodies then either.
Your friend might be able to get incentives to construct an electric producing bioreactor for their lagoon pit. Something to perhaps mention if they aren't aware.
> A lot of countries, because of prion diseases, basically stopped importing US beef.
In many ways this is their loss. Yes, there is a risk, but US beef is the best in the world, with no small thanks to the rich multicultural society we live in.
Lol no it's not. Each country has specific tastes then breeds for it. American beef is not the same as Brazilian beef which isn't the same as European beef which isnt the same as Australian beef.
It's just preference. There is no "better".
I grew up ranching and have visited operations in several countries. It's all just preference. A lot of people think US beef isn't very good.
I will buy grass-fed beef from NZ and Australia, but not from the US because of how often it contains toxins (probably mostly heavy metals, hard to say because I never had any analyzed, but I am unusually sensitive to heavy metals) in my personal beef-buying experience.
Grain-fed US beef doesn't have the problem.
My guess is that there has just been much more industrial activity in the US than in Aus and NZ.
Australian beef is way better, and doesn’t come loaded with prions.
I happened to pick up How the Cows Turned Mad by Maxine Schwartz a couple weeks ago. I'm not finished with it yet, but in the section about Kuru there was a comment about how cases happened at least 4 decades after the cannibalism ended. I picked it up just to flip through but it is a pretty engrossing history of the various forms of prions disease.
> I realized then that we wouldn't know for decades how many humans caught it.
Considering that the 90s is about 20 years ago, the timeline seems to match up given the social climate today.
This is one reason why there are limits around the world on who can donate blood. I received a blood transfusion as a child in the UK in the 90s, and specifically because of it being that country/period, I will never be able to donate blood here in Australia.
I thought the time limit in Australia expired recently (as in: within the last two to three years).
As of July 25, 2022, the Australian blood donation rules that previously barred individuals who lived in the UK for more than six months between 1980 and 1996 from donating blood have been lifted.
Maybe worth double checking at your local blood donor centre. There always seems to be a shortage of blood.
It recently expired in the US as well
> likely contracted through contaminated human growth hormone (HGH) treatments she received as a child, they determined.
Important context for those unlikely to click through
prion diseases is one of the nightmare fuels. Alzheimer is one such disease, with no known cure, and as in this case, can lay dormant for years (thank god, i guess?)
Yeah, they are the only diseases I really fear. There are few situations where a single molecule is enough to fuck up your entire brain in a way it can't even detect, and certainly not fight against.
Don't eat animal brains. (And while we're at it: be cautious about livers, since some contain toxic levels of vitamin A...)
Alzheimer’s isn’t a prion disease, but it does show prion-like behavior. Misfolded proteins like amyloid-beta and tau can spread within the brain similarly to prions, but unlike true prion diseases (e.g. CJD), Alzheimer’s isn’t infectious and isn’t caused by prions. It's a distinct neurodegenerative disorder with overlapping mechanisms, not classification.
I recently read “The Family That Couldn’t Sleep” which is the story of the history and discovery of prion diseases.
It’s from the mid-00’s so it’s missing the latest research but it’s still an extremely interesting (and terrifying) read.
Sporadic (spontaneous) CJD is the most common form. It doesn't require contaminated food or medical products. Proteins can misfold all on their own. The risk is about 2 micromorts / year.
That is such a long latency period.
I wonder if it slowly progresses over time, or if it develops opportunistically once some other bodily system that keeps it in check breaks down with age.
You have one misfolded protein that “teaches” other proteins to misfold in the same way[1]. It’s very slow initially because you’re talking about individual proteins, and it takes a while for each prion to bump into a normal protein of the correct type. The immune system can’t do anything about them though, so once a protein is converted there’s permanently another prion. The conversion rate is very slow, but it never ever ratchets back - the number only increases. It’s a geometric progression.
Once symptoms show up there’s a TON of them, and it goes downhill fairly quickly. That’s why there’s such a long latency period.
[1]It’s like Ice-9, if you’ve read Cat’s Cradle.
Why mine for Bitcoin, when you can fold some prions at home?
For people who prefer vaccines to nightmare fuels, the following might offer some hope:
(2014)
https://nyulangone.org/news/first-successful-vaccination-aga...
Considering how hard it has been to clense against prions, this is very encouraging. I find it interesting that we struggle to decontaminate materials because of their resistance, but we might be able to train the body to deal with them.
PF?? Intuition says our immune system isn't equipped to detect them (note how odourless/tasteless) but you make me wonder if we might evolve bacteria/archae to sense them, as a first milestone..
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so everyone with dimentia that dies soon after diagnosis could be victim of mad cow disease?
I guess that means it could be made domant again maybe.
That doesn’t seem very likely from my reading. It sounds like it’s not unheard of for prion-diseases like Creutzfeldt-Jakob to take a while to destroy a person. The poor lady fta apparently had a genetic component that contributed to the latency. Brutal. Inducing dormancy in this and similar cases would effectively present a treatment, if not a cure, to an hereto untreatable, uncurable disease.
> … the woman first visited doctors with tremors and trouble balancing. As is often the case, once symptoms started, her condition rapidly worsened. She was hospitalized … and several days into her stay, she fell into a coma that she would never awaken from.
In other news, apparently hgh used to be extracted from cadavers. Wtlf.
They still get other stuff from cadavers.
Like for gum transplant, they offer cadaver gum vs autologous gum taken from the patient's upper palate...
I'd advise avoiding the cadaver gum. I assume they think it's safe or they wouldn't use it, but there could be unknown risks...
To be honest, it's a bit comforting to know that it happened quickly. Better in some ways than a long, slow degradation of the mind from which there's no recovery.
> In other news, apparently hgh used to be extracted from cadavers.
Creatine too.
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