Compounding USDA's lax practices has been its refusal to allow beef processors to independently test cattle for mad cow disease. In 2004, Creekstone Farms, a Kansas processor of black Angus beef with a large Japanese clientele, asked for permission to test its 300,000 cattle for BSE using a $500,000 testing site it had built to USDA specifications.
But the agency ruled that the BSE test was licensed only for "surveillance" of animal health, and rejected Creekstone's request because it implied "a consumer safety aspect" that was "not scientifically warranted."
This is an incomplete description of the USDA's rationale. Although I agree that there shouldn't be a ban on private testing, the USDA's position is that BSE testing is ineffective at the time most cows are slaughtered, and would therefore provide an unwarranted impression of safety.
> NOT A FOOD SAFETY TEST
> BSE tests are not conducted on cuts of meat, but involve taking samples from the brain of a dead animal to see if the infectious agent is present. We know that the earliest point at which current tests can accurately detect BSE is 2-to-3 months before the animal begins to show symptoms. The time between initial infection and the appearance of symptoms is about 5 years. Since most cattle that go to slaughter in the United States are both young and clinically normal, testing all slaughter cattle for BSE might offer misleading assurances of safety to the public.
> ...
> Why doesn't USDA test every animal at slaughter?
> There is currently no test to detect the disease in a live animal. BSE is confirmed by taking samples from the brain of an animal and testing to see if the infectious agent - the abnormal form of the prion protein - is present. The earliest point at which current tests can accurately detect BSE is 2 to 3 months before the animal begins to show symptoms, and the time between initial infection and the appearance of symptoms is about 5 years. Therefore, there is a long period of time during which current tests would not be able to detect the disease in an infected animal.
> Since most cattle are slaughtered in the United States at a young age, they are in that period where tests would not be able to detect the disease if present. Testing all slaughter cattle for BSE could produce an exceedingly high rate of false negative test results and offer misleading assurances of the presence or absence of disease.
> Simply put, the most effective way to detect BSE is not to test all animals, which could lead to false security, but to test those animals most likely to have the disease, which is the basis of USDA's current program.
The rational middle ground would be to allow the private testing, but regulate any advertising or marketing of the testing and/or results on the basis it would likely confuse the consumers. If the interest in private testing magically disappears, then the intent is clear and might be a valuable factor for the consumers who might reasonably conclude the private company only had interest in performing testing they knew or should have known was immaterial for the purposes of marketing the testing to consumers as though it were material.
This is the same error the CDC & FDA made in the early stages of COVID, insisting on fussy rationed tests based on hand-wavy ideas about public misinterpretation. That error likely contributed to hundreds of thousands of preventable deaths by hiding initial community spread for a month or more.
Mass testing, even testing that's mostly at a stage unlikely to detect anything, and only occasionally on less-typical older animals, will provide new useful info. Waiting to test until an a old symptomatic cow "most likely to have the disease" shows up is closing the barn door after the mad cows have already escaped.
The USDA's logic is dangerous like the bureaucratic rationing of COVID tests, early on. At crucial times in early 2020, no matter how suspicious a case/death, and how well it fit COVID symptoms, if the patient hadn't returned from Wuhan in the last few weeks, a test was prohibited. That's a "we don't want to know" policy.
> But the agency ruled that the BSE test was licensed only for "surveillance" of animal health, and rejected Creekstone's request because it implied "a consumer safety aspect" that was "not scientifically warranted."
I read this as "you can test your animals if you want to, but you can't then use the results to make any claim or statement about them". I suspect they're free to test their animals, but not free to stamp "100% tested BSE-free beef!" on their steaks. I suspect this is what's meant by "surveillance". If that's the case, I agree with the gov't; private testing shouldn't be used to make claims. But it could be used for internal product safety, and if anything comes back positive, the USDA could be brought in to verify and take action.
The “fun” question is whether prions are present in milk and other dairy products. One might argue that they are not, by virtue of them being largely localized to neural tissue.
Of course, this raises the question of how CWD is being transmitted in elk, since elk don’t eat elk.
“Scientists believe CWD proteins (prions) likely spread between animals through body fluids like feces, saliva, blood, or urine, either through direct contact or indirectly through environmental contamination of soil, food or water.”
I stopped eating US beef many years ago for this very reason. The Japanese are not stupid and it's quite obvious that something fishy is going on.
Knowing what we know about the practices of most US beef farms and the countless USDA failures in both detection and enforcement of rules/regulations meant to protect the consumer, I think it's the wise path to take for everyone living here.
A. BSE tests are not conducted on cuts of meat, but involve taking samples from the brain of a dead animal to see if the infectious agent is present. We know that the earliest point at which current tests can accurately detect BSE is 2-to-3 months before the animal begins to show symptoms. The time between initial infection and the appearance of symptoms is about 5 years. Since most cattle that go to slaughter in the United States are both young and clinically normal, testing all slaughter cattle for BSE might offer misleading assurances of safety to the public.
The BSE surveillance program is not for the purposes of determining food safety. Rather, it is an animal health surveillance program. USDA's BSE surveillance program allows USDA to detect the disease if it exists at very low levels in the U.S. cattle population and provides assurances to consumers and our international trading partners that the interlocking system of safeguards in place to prevent BSE are working.
The risk of cow->human BSE transmission is infinitesimal. Species barriers in prions are surprisingly strong and, for practical purposes, nearly impermeable [0]. In fact, the Prion Protein (PrP) in humans causes multiple distinct diseases including CJD, Kuru, and FFI. Infectious prion confirmations are probably highly influenced by glycosylation [1,2]. Even if this testing wasn't god awfully slow, it's unclear one learns anything from it.
What you are saying is dangerous. We had a serious set of exactly these transmission events in Britain only a few decades ago. Clearly the risk is not infinitesimal.
>The risk of cow->human BSE transmission is infinitesimal.
I don't think your sources support this conclusion.
bovine spongiform encephalopathy (BSE) rarely passes the species barrier, but it is difficult, if not impossible to estimate what the rate is in the absence of mitigations.
I think there's a pretty fundamental difference between "allowing farms to police themselves" and actually prohibiting them from doing voluntary testing for diseases. I would compare it to the difference between eliminating a need for prescriptions and prohibiting people from getting screened for skin cancer on the regular.
Is there a better reason to prohibit voluntary testing than what is included in the article?
That isn't what is happening. The USDA is prohibiting suplemental testing. The allegation is that this is because that might reveal cases of infection that would show the USDA testing policies are not sufficient.
Thus the USDA is placing their reputation over the health of US citizens.
In other Prion news, there's a prion disease, Chronic Wasting Disease (CWD), affecting populations of wild Deer, Elk, etc. in North America and other chunks of the world. It's not yet known to infect people, but please be careful when eating wild game. [1]
My mind first went to this issue when I read the headline, though upon reading the article it's probably something else, as CWD isn't really a thing in that region [it's more of a Mountain state kind of thing thing]. And, of course, I'm not a prion expert, so I only vaguely know what I'm writing about.
The species barrier in prion transmission appear very strong [0]. Transmitting a prion from a hamster into a mouse often takes multiple passages of direct in brain inoculation. In these closely related species, perhaps it's less strong. It's doubtful that CWD can transmit to humans at typical exposure levels. In vitro, conversion of human PrP is possible, but exceedingly difficult.
Prions terrify me as well because it seems like there's no stopping them..
> In 2015, researchers at The University of Texas Health Science Center at Houston found that plants can be a vector for prions. When researchers fed hamsters grass that grew on ground where a deer that died with chronic wasting disease (CWD) was buried, the hamsters became ill with CWD, suggesting that prions can bind to plants, which then take them up into the leaf and stem structure, where they can be eaten by herbivores, thus completing the cycle. It is thus possible that there is a progressively accumulating number of prions in the environment
I had read this before. It's horrifying. How do you stop the chain reaction of proteins folding into lower energy states? It's not like a bacteria or virus; it's more like ice-nine, except instead of water it's your brain.
There is also a study from 2014 that suggests that prions were not detected in stems even though the roots of wheat plants were exposed to them. I don't know if there is conclusive evidence yet on getting sick by eating a plant based diet.
"This suggests wheat was unable to transport sufficient PrP(TSE) from the roots to the stem to be detectable by the methods employed."
Maybe this is a naive question, but your immune system works by identifying foreign proteins and killing those cells. Is there no way for your immune system to target cells contaminated with prions?
Whenever a topic about something that could potentially destroy humanity comes up (say, climate issues), there is always some comment that makes a reasonable argument of why things are not as bad as they seem.
The simplest counterpoint to prion cataclysm fear is that it hasn't happened yet. That implies a low chance that it'll happen anytime in the near future.
There's no evidence that any significant human population has ever been wiped out by prions at any point in the history of the species. Heck, we haven't even observed widespread (>10% infected) prion disease in any species.
At least with something like climate change, there's the fact that post-industrial activity is doing something different than pre-industrial history. But this reasoning doesn't hold for prion cataclysm. It's more like asteroid apocalypse. Yes it could happen, but statistically it's not something we have to worry about on a civilizational timeframe.
I don't see any justification for why humanity in 2021 is courting prion disaster to a greater degree than it was in 2000 BC. Animal husbandry has existed for fifteen thousand years. Maybe globalization would spread a prion outbreak faster. But the point is we've never even observed localized prion collapse. If prions were that dangerous, there should already be regional areas that have transformed into total no-go zones.
Actually, there is some good news. Many types of prions diseases do not spread from animals to humans (so far). There have been some studies around this with CWD, but also Scapie has been around for hundreds or even thousands of years and never made the jump. Even in areas with CWD, we see that herd numbers do typically rebound. It's believed that there is some genetic factor that prevents the onset of the symptoms, like the body's proteins are less likely to fold in that way, so you only accumulate what you eat, or that the body can clean up the misfolded proteins. So it's not like it would wipe out everyone. I think I remember reading that some people are also researching prion treatments with some limited sucess. I would imagine they would come up with something even more quickly if there was a epidemic type of situation, similar to how medical researchers around the world focused on covid.
Actually there is a positive anecdote often cited. The Fore people of New Guinea engaged in ritual cannibalism which lead to the propagation of prions across generations. When they stopped, the generations of disease also stopped.
You jinxed it, so I'll be the "some comment": prions won't spread everywhere all at once, so humanity will probably self-isolate in at least a few prion-free clusters.
Prion caused disease is indeed terrifying on individual level but, from its impact over the species' survival, it isn't that menacing. The decay takes decades, which is enough for breeding the next generation. Human life expectancy may get shorter, which sucks, of course, but humans lived shorter lives for most of their history. So no, it's not that bad. Even assuming that the entire human population will get infected from young age with no way to avoid it, it won't be that bad. It would come with a bit of adjustment, like having to raise kids sooner because the luxury to postpone (for life enjoyment or other pursuits) won't be there, but things will go on.
Well, prion diseases are very rare in humans (or at least their transmission is rare), and there is no evolutive pressure to create a super-prion that can infect more people, like happens with other pathogens. Thus, the most likely situation is that it will continue to be very rare until we are able to change it into virtually non-existent by technology.
They are a really scary thing, but it's a meteor strike style of scary, nobody really expects it to happen before we can deal with it.
I lived through it. I mean, it was scary, but the risks were low.
I remember my landlord and his girlfriend at the time saying they were going to give up beef due to the risks. They said this whilst smoking cigarettes.
People are so good at ignoring large daily risks and freaking out about tiny unknown risks.
Just look how many people are scared of the covid19 vaccines, but take bigger risks in their morning commute. Actually if you just stick to the AstraZenica vaccine with the risk of blood clots - you take a bigger risk just getting out of bed in the morning, even if you never leave the house.
I lost someone very close to me to sporadic CJD. This was some time back, but the episode is one of the most troubling moments in my life because of the suffering it inflicts on the patient - the hallucinations with no way to communicate or to stop it.
Many countries would not allow people who lived in the UK during the 80s and 90s to give blood. I ate so many burgers back then too. Our kids after they were born weren't allowed beef for years.
Yep I'm one of them. Lived in the UK in the early 80's as a child. Gave blood for the first time when I was in college in the 90's to the Red Cross here in the US, and they mailed me back saying to never give blood again but didn't specify a reason. It wasn't until a few years later that I found out why. And the fact I might have a prion disease lurking in my blood sometimes terrifies me.
They can’t evolve independently but biological systems that produce proteins can evolve new proteins and new ways of manipulating proteins. A nightmare scenario would be a bacterial or viral mutation that produced a deadly prion.
I'm not sure why this is the case. They replicate. There might be an error in replication, no? Or is that just really hard? Is it that their existence is in a sweet spot, and nothing even marginally different would work the same way?
If you took affected neural tissue and burned it to ash on a wood fire, there remains (although low) infective potential but probably only if you were trying in a lab. It takes 1000 C incineration to defeat infectivity and even then they’re still detectable.
Composting so the proteins are broken down biologically is also an alternative.
No amount of cooking prions that leave anything you want to eat makes infected material safe.
`Prion aggregates are stable, and this structural stability means that prions are resistant to denaturation by chemical and physical agents: they cannot be destroyed by ordinary disinfection or cooking.`
Yes they do. From wikipedia : Prion aggregates are stable, and this structural stability means that prions are resistant to denaturation by chemical and physical agents: they cannot be destroyed by ordinary disinfection or cooking. This makes disposal and containment of these particles difficult.
Yes they do, unfortunately. And there are recently discovered cases of chronic wasting disease (CWD) in elk, deer and moose worldwide. I hope it doesn't pass to humans. For the time being I'll refrain from eating game.
Apparently the only options for diagnostic testing are brain biopsy and pulling CSF samples, both of which are probably a non-starter for screening purposes across a population.
Clinical Laboratory Tests Used To Aid in Diagnosis of Human Prion Disease
Allyson Connor, Han Wang, Brian S. Appleby, Daniel D. Rhoads
Journal of Clinical Microbiology Sep 2019, 57 (10) e00769-19; DOI: 10.1128/JCM.00769-19
That was the going assumption as far as I understood (I'm from the area). They're hasn't been direct health advise on areas to avoid either, so it's slightly disconcerting.
I would generally agree, except in wonder what the aquifers/groundwater looks like in that region and also the sea water along that coast (likely more of a biological source than an industrial one in that case, I think).
There was some research which suggested that a not insignificant number of dementia cases were actually misdiagnosed mad cow disease. Because CJD can stick around for a while without taking affect, I wouldn't be surprised.
I know someone in their early 40s experiencing dementia-type symptoms (consistent with frontotemporal dementia). It is early but is quickly getting worse. It's heartbreaking to see how this is affecting his spouse and children. So far, they don't have a diagnosis. They are still running tests. It has to be quite frustrating not to be able to point to a root cause. So little can be known without direct access to the brain.
I have read a couple of times that an HSV infection (super common) can get into the brain and cause dementia. Just trying to help here, but maybe they could try an HSV antiviral?
Not this again. I really hope that the incubation period is not around the corner with another wave of vCJD 'variant Creutzfeldt-Jakob disease' aka (Mad cow disease).
Interesting. The symptoms they list are entirely typical of FND, which accounts more than 1 in 7 referrals to a neurology department and is at least as common as MS [1]. They list 48 active cases, but there would presumably be a lot more FND cases than that in NB. Also, it seems they only uncovered this new syndrome when they actively started looking for them via the Creutzfeldt-Jakob Disease Surveillance System. Could it just be that many doctors and neurologists are unaware of FND, even though it is by far the most common neurological disorder they see?
Readit News
But the agency ruled that the BSE test was licensed only for "surveillance" of animal health, and rejected Creekstone's request because it implied "a consumer safety aspect" that was "not scientifically warranted."
https://www.eurekalert.org/pub_releases/2006-05/uoia-ftf0515...
> NOT A FOOD SAFETY TEST
> BSE tests are not conducted on cuts of meat, but involve taking samples from the brain of a dead animal to see if the infectious agent is present. We know that the earliest point at which current tests can accurately detect BSE is 2-to-3 months before the animal begins to show symptoms. The time between initial infection and the appearance of symptoms is about 5 years. Since most cattle that go to slaughter in the United States are both young and clinically normal, testing all slaughter cattle for BSE might offer misleading assurances of safety to the public.
> ...
> Why doesn't USDA test every animal at slaughter?
> There is currently no test to detect the disease in a live animal. BSE is confirmed by taking samples from the brain of an animal and testing to see if the infectious agent - the abnormal form of the prion protein - is present. The earliest point at which current tests can accurately detect BSE is 2 to 3 months before the animal begins to show symptoms, and the time between initial infection and the appearance of symptoms is about 5 years. Therefore, there is a long period of time during which current tests would not be able to detect the disease in an infected animal.
> Since most cattle are slaughtered in the United States at a young age, they are in that period where tests would not be able to detect the disease if present. Testing all slaughter cattle for BSE could produce an exceedingly high rate of false negative test results and offer misleading assurances of the presence or absence of disease.
> Simply put, the most effective way to detect BSE is not to test all animals, which could lead to false security, but to test those animals most likely to have the disease, which is the basis of USDA's current program.
https://www.usda.gov/topics/animals/bse-surveillance-informa...
Mass testing, even testing that's mostly at a stage unlikely to detect anything, and only occasionally on less-typical older animals, will provide new useful info. Waiting to test until an a old symptomatic cow "most likely to have the disease" shows up is closing the barn door after the mad cows have already escaped.
The USDA's logic is dangerous like the bureaucratic rationing of COVID tests, early on. At crucial times in early 2020, no matter how suspicious a case/death, and how well it fit COVID symptoms, if the patient hadn't returned from Wuhan in the last few weeks, a test was prohibited. That's a "we don't want to know" policy.
I really try to stay away from politics, but sounds as something made by some bribed political appointment.
I read this as "you can test your animals if you want to, but you can't then use the results to make any claim or statement about them". I suspect they're free to test their animals, but not free to stamp "100% tested BSE-free beef!" on their steaks. I suspect this is what's meant by "surveillance". If that's the case, I agree with the gov't; private testing shouldn't be used to make claims. But it could be used for internal product safety, and if anything comes back positive, the USDA could be brought in to verify and take action.
It's not entirely clear to me from the article.
Of course, this raises the question of how CWD is being transmitted in elk, since elk don’t eat elk.
“Scientists believe CWD proteins (prions) likely spread between animals through body fluids like feces, saliva, blood, or urine, either through direct contact or indirectly through environmental contamination of soil, food or water.”
https://www.cdc.gov/prions/cwd/transmission.html
Milk is clearly a body fluid, though is curiously absent from the above statement.
And as other comments about the difficulty of sterilizing prions make clear, pasteurization will have no effect.
Knowing what we know about the practices of most US beef farms and the countless USDA failures in both detection and enforcement of rules/regulations meant to protect the consumer, I think it's the wise path to take for everyone living here.
A. BSE tests are not conducted on cuts of meat, but involve taking samples from the brain of a dead animal to see if the infectious agent is present. We know that the earliest point at which current tests can accurately detect BSE is 2-to-3 months before the animal begins to show symptoms. The time between initial infection and the appearance of symptoms is about 5 years. Since most cattle that go to slaughter in the United States are both young and clinically normal, testing all slaughter cattle for BSE might offer misleading assurances of safety to the public.
The BSE surveillance program is not for the purposes of determining food safety. Rather, it is an animal health surveillance program. USDA's BSE surveillance program allows USDA to detect the disease if it exists at very low levels in the U.S. cattle population and provides assurances to consumers and our international trading partners that the interlocking system of safeguards in place to prevent BSE are working.
[0] https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2736662/ [1] https://www.cureffi.org/2013/05/05/prion-protein-n-linked-gl... [2] https://www.biorxiv.org/content/10.1101/2021.02.14.431014v2
I don't think your sources support this conclusion. bovine spongiform encephalopathy (BSE) rarely passes the species barrier, but it is difficult, if not impossible to estimate what the rate is in the absence of mitigations.
Is there a better reason to prohibit voluntary testing than what is included in the article?
Thus the USDA is placing their reputation over the health of US citizens.
My mind first went to this issue when I read the headline, though upon reading the article it's probably something else, as CWD isn't really a thing in that region [it's more of a Mountain state kind of thing thing]. And, of course, I'm not a prion expert, so I only vaguely know what I'm writing about.
[1] https://www.canada.ca/en/health-canada/services/food-nutriti...
[0] https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2736662/ [1] https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5667731/
https://www.cdc.gov/prions/cwd/occurrence.html
I wonder if there have been studies on the scale of potential latent vCJD/Prion disease in the population?
> In 2015, researchers at The University of Texas Health Science Center at Houston found that plants can be a vector for prions. When researchers fed hamsters grass that grew on ground where a deer that died with chronic wasting disease (CWD) was buried, the hamsters became ill with CWD, suggesting that prions can bind to plants, which then take them up into the leaf and stem structure, where they can be eaten by herbivores, thus completing the cycle. It is thus possible that there is a progressively accumulating number of prions in the environment
"This suggests wheat was unable to transport sufficient PrP(TSE) from the roots to the stem to be detectable by the methods employed."
Source: https://pubmed.ncbi.nlm.nih.gov/24509640/
I really need to grow my own food.
This never happens when prions are the topic.
There's no evidence that any significant human population has ever been wiped out by prions at any point in the history of the species. Heck, we haven't even observed widespread (>10% infected) prion disease in any species.
At least with something like climate change, there's the fact that post-industrial activity is doing something different than pre-industrial history. But this reasoning doesn't hold for prion cataclysm. It's more like asteroid apocalypse. Yes it could happen, but statistically it's not something we have to worry about on a civilizational timeframe.
I don't see any justification for why humanity in 2021 is courting prion disaster to a greater degree than it was in 2000 BC. Animal husbandry has existed for fifteen thousand years. Maybe globalization would spread a prion outbreak faster. But the point is we've never even observed localized prion collapse. If prions were that dangerous, there should already be regional areas that have transformed into total no-go zones.
https://en.wikipedia.org/wiki/Kuru_(disease)
They are a really scary thing, but it's a meteor strike style of scary, nobody really expects it to happen before we can deal with it.
I remember my landlord and his girlfriend at the time saying they were going to give up beef due to the risks. They said this whilst smoking cigarettes.
Just look how many people are scared of the covid19 vaccines, but take bigger risks in their morning commute. Actually if you just stick to the AstraZenica vaccine with the risk of blood clots - you take a bigger risk just getting out of bed in the morning, even if you never leave the house.
Composting so the proteins are broken down biologically is also an alternative.
No amount of cooking prions that leave anything you want to eat makes infected material safe.
To quote from Wikipedia:
`Prion aggregates are stable, and this structural stability means that prions are resistant to denaturation by chemical and physical agents: they cannot be destroyed by ordinary disinfection or cooking.`
> 134 °C (273 °F) for 18 minutes in a pressurized steam autoclave has been found to be somewhat effective in deactivating the agent of disease
https://en.wikipedia.org/wiki/Prion#Sterilization
https://www.scientificamerican.com/article/surgical-exposure...
Clinical Laboratory Tests Used To Aid in Diagnosis of Human Prion Disease Allyson Connor, Han Wang, Brian S. Appleby, Daniel D. Rhoads Journal of Clinical Microbiology Sep 2019, 57 (10) e00769-19; DOI: 10.1128/JCM.00769-19
https://jcm.asm.org/content/57/10/e00769-19
Deleted Comment
[1] http://www.rcpe.ac.uk/journal/issue/journal_41_1/stone.pdf