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cJD - We're All Doomed!

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 Rob Exile Ward 10 Sep 2015
At least, that's what we were told 20 years ago. Mad cow disease was going to spread to humans and there were 100,000's of cases just waiting to happen.

Literally billions has been spent defending against this appalling threat - we just can't be too careful.

Well all those precautions must have worked, because on average there is just 1 fatality a year from the disease. 1. Has there ever been so much hype expended on such a miniscule threat?
1
 Ridge 10 Sep 2015
In reply to Rob Exile Ward:

Moooo!
 The New NickB 10 Sep 2015
In reply to Rob Exile Ward:

Barely ever posts these days
 JJL 10 Sep 2015
In reply to Rob Exile Ward:

> Well all those precautions must have worked, because on average there is just 1 fatality a year from the disease. 1. Has there ever been so much hype expended on such a miniscule threat?

Year 2K bug?
 JayPee630 10 Sep 2015
In reply to Rob Exile Ward:

I think there was some gap between what the experts said might happen, and then what the tabloids said was likely to happen.

But yes, generally, what a palaver! Although agree the Y2K might have been a bigger, longer build-up to nothing.
 timjones 10 Sep 2015
In reply to Rob Exile Ward:

> At least, that's what we were told 20 years ago. Mad cow disease was going to spread to humans and there were 100,000's of cases just waiting to happen.

> Literally billions has been spent defending against this appalling threat - we just can't be too careful.

> Well all those precautions must have worked, because on average there is just 1 fatality a year from the disease. 1. Has there ever been so much hype expended on such a miniscule threat?

It's the ongoing damage to our export trade that is my concern at the moment.

Some bright spark proposed that sheep could possibly get BSE so controls were put in place to minimise the hypothetical risk. 20 years down the line a highly risk averse FSA still refuses to relax those controls and it's the devils own job persuading the likes of China that there is no risk when you still have regulations in place to control it
 timjones 10 Sep 2015
In reply to JayPee630:

> I think there was some gap between what the experts said might happen, and then what the tabloids said was likely to happen.

> But yes, generally, what a palaver! Although agree the Y2K might have been a bigger, longer build-up to nothing.

Good old Hugh Pennington. It's amazing that anyone who made such a massive and public error of judgement is still regarded as employable ;(
 Timmd 10 Sep 2015
In reply to Rob Exile Ward:
I'm not worried, I'm a helicopter
Post edited at 15:41
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 Luke90 10 Sep 2015
In reply to JJL:
> Year 2K bug?

You might well be better informed than me but people I know who worked in IT in the period running up to the Millenium get pretty aggravated when the Y2K thing gets dismissed as needless hysteria. In the company concerned, a lot of people worked an obscene amount of overtime to make sure that a wide variety of software got updated to remain functional.

In that company, at least, ignoring the situation would have had dire implications for their business and those only failed to materialise because of the effort put in to avoid it. Perhaps this company was an unusual outlier but I was left with the impression that the bug was a genuine issue that was only resolved by considerable effort by a vast number of people.
1
 skog 10 Sep 2015
In reply to JayPee630:

> Although agree the Y2K might have been a bigger, longer build-up to nothing.

I did a fair bit of work sorting out some systems to deal with the Y2K bug.

It was, on the whole, a very easy bug to fix, but it was quite real - lots of programs and reports would have failed, and many others would not have worked as intended.

It's kind of funny how the success of all the (mundane, but time-consuming) work somehow becomes 'the problem never existed, because nothing went wrong'.

So, back on topic, maybe the precautions really did work against CJD. Or maybe it wasn't as dangerous as feared, but the potential consequences were so horrific it wasn't worth chancing ignoring it.

Or maybe it did happen, and it's mostly being misdiagnosed as Alzheimers or something...
 Offwidth 10 Sep 2015
In reply to Rob Exile Ward:

Shark attacks?

Anyway CJD is more serious than you imply.

http://www.cjd.ed.ac.uk/
cb294 10 Sep 2015
In reply to Rob Exile Ward:

The threat was not minuscule at all, we were just lucky that the worst case scenarios did not materialize.

To strongly simplify, there are two main versions of the human prion protein (PrP, the protein that clumps in your brain when exposed to the misfolded BSE or CJD versions). One of these variants is much more likely to form these clumps than the other, more common one, especially when transmission occurs via food. As it fortunately turned out, all (AFAIK) human victims that contracted BSE from cows had two copies of this rarer, more sensitive version.

However, both versions are in principle able to be converted into these clumps, either experimentally or by contaminated surgery equipment, when higher protein levels are transmitted (and directly to the brain!).

Since for prion diseases incubation time is strictly dependent on the starting PrP load, discovering that a) both human PrP versions can in principle be converted, and b) the first cases started appearing was pretty dramatic.

As it happened, the average time to conversion for people with at least one less sensitive variant turned out to be on the order of our life span. Any (rare) cases of human BSE will just disappear in the background noise of age related dementia.

However, there was no way of knowing, and had the species barrier only been a little lower, our health systems could have be overwhelmed by now.

CB
 Dave Garnett 10 Sep 2015
In reply to Rob Exile Ward:

> Well all those precautions must have worked, because on average there is just 1 fatality a year from the disease. 1. Has there ever been so much hype expended on such a miniscule threat?

Yes, but 28 people died at the peak of the risk in 200 and 176 people died between 1995 and 2011. All this agrees pretty closely with one of the epidemiological models. It's also still possible that there will be another peak, possibly larger and broader, yet to come, although it now looks as though for those of us with a less vulnerable genetic background will die of something else before vCJD gets us. It's also undoubtedly true that the measures taken back then saved many lives. If we hadn't changed the rules on rendered bovine material getting into the food chain then, at the very least, we'd be continuing to have about 30 young people a year die from vCJD.

Maybe there were one or two alarmists, but the level of concern amongst those who understood the threat back in the 1980s was appropriate. This was not just a new disease but new biology. Given a choice between Hugh Pennington and John Gummer, I know who I'd sooner believe. I remember arguing with the then chief veterinary officer about the general level of political complacency and lack of understanding of the science at the time.

I had a research interest in prion diseases back then and I didn't eat beef between 1986 and 2005. There are still things I wouldn't eat now. Squirrel brains, admittedly, but the risk isn't entirely hypothetical.
 Shani 10 Sep 2015
In reply to Rob Exile Ward:
"Has there ever been so much hype expended on such a miniscule threat?"

Global Warming!
Post edited at 16:06
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 Dave Garnett 10 Sep 2015
In reply to timjones:

> Some bright spark proposed that sheep could possibly get BSE so controls were put in place to minimise the hypothetical risk.

But we know that sheep get TSEs because we've known about the nature of scrapie for decades. The interesting thing is that, as far as we can tell, humans don't seem to contract a vCJD from eating them.
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 Greasy Prusiks 10 Sep 2015
In reply to Rob Exile Ward:
Does anyone want to hear my mad cow disease joke?....
In reply to Rob Exile Ward:

How long have you got?

Interestingly, this is a bit topical at the moment: recently, a paper was published in which the author noted that there were similar structural changes in seven (out of eight) brains retained from CJD cases, to *one of several* changes seen in the brains of Altzheimer cases. Obviously, the press aren't going to leave that one alone, although the Beeb showed greater restraint than most:

http://www.bbc.co.uk/news/health-34184470

I have a friend who's a proper boffin in brain research, and I've had this conversation with him a few times - I'm convinced that Altzheimers will be demonstrated to be at least in part a prion infection (I'm not sitting on a giant pile of evidence, but some of the aetiology seems to fit). He doesn't invest in this view, but says it has been popular in the past.

Interestingly, many of the predictions in the early days of BSE came from one man: a chap called Richard Lacey, who was extremely accommodating to the press and who was always happy to predict doom on demand. Depending on one's viewpoint, he was either an eminent, extraordinarily gifted and prescient scientist, or else an attention-seeking, rather petulant little man who would walk out of giving a lecture if the audience asked awkward questions about the validity of his conclusions (having met him a couple of times, and having had to escort him to the door as he stormed out on two occasions, I know which side I'm on).

The truth of the BSE debacle is a lot worse than is publicly known, but mostly covers inconveniences like accountability, politics and empirical decision making. It really was the most squalid of times and I know a couple of farmers who took their own lives as a result of what was done to them.

Sheep have been getting a transmissible spongiform encephalopathy for at least a few hundred years - it's called scrapie.
 Chris the Tall 10 Sep 2015
In reply to skog:

> It's kind of funny how the success of all the (mundane, but time-consuming) work somehow becomes 'the problem never existed, because nothing went wrong'.

Yep, planes didn't fall out of the sky, the banking systems didn't fail and toasters didn't explode, so some people now think Y2K was all a hoax. Things is, we are going to see similar problems in 2035 and 2038, and they'll be far more lines of code and computerized devices by then - I wonder if we'll see the same effort.
cb294 10 Sep 2015
In reply to Shani:

Seriously?

CB
cb294 10 Sep 2015
In reply to Dave Garnett:
We also know that Scrapie prions are much less effective in converting human PrP to the pathogenic form than BSE prions. These experiments used to be very tricky, but are almost trivial (relatively..) since transgenic mice expressing the human protein instead of their own PrP have become available.

The epidemiology simply shows that sheep to man is a rather large species barrier.

Sheep infected with BSE are different: Such prion proteins indeed are as able as BSE prions to convert the human protein.

CB
Post edited at 16:39
 CJD 10 Sep 2015
In reply to The New NickB:

Morning! (or rather, afternoon)
 Neil Williams 10 Sep 2015
In reply to Rob Exile Ward:

Did Num Num eat a mad cow? Or is that just Mrs Num Num?
 Dave Garnett 10 Sep 2015
In reply to cb294:

> Sheep infected with BSE are different: Such prion proteins indeed are as able as BSE prions to convert the human protein.

Yes, I recall the early papers showing this but I moved on before they figured out why. I can imagine how it might work but it's about time I caught up with about 20 years of research. Know a good review paper?

 Dave Garnett 10 Sep 2015
In reply to Martin not maisie:

> Interestingly, many of the predictions in the early days of BSE came from one man: a chap called Richard Lacey, who was extremely accommodating to the press and who was always happy to predict doom on demand. Depending on one's viewpoint, he was either an eminent, extraordinarily gifted and prescient scientist, or else an attention-seeking, rather petulant little man who would walk out of giving a lecture if the audience asked awkward questions about the validity of his conclusions (having met him a couple of times, and having had to escort him to the door as he stormed out on two occasions, I know which side I'm on).

Ah yes, Richard Lacey. I'd almost managed to forget about him. It was a field that attracted both the very talented and the very eccentric.

I once had lunch with Stan Prusiner and he said he spent years worrying that he'd lose his tenure and end up sweeping the floors of the labs at UCSF if the results didn't stack up. He ended up with a Nobel Prize, as it happens.
abseil 10 Sep 2015
In reply to Greasy Prusiks:

> Does anyone want to hear my mad cow disease joke?....

Yes please.
 Greasy Prusiks 10 Sep 2015
In reply to abseil:

At last! You'll have to imagine the west country accent that all cows have.

Ok so there's these two cows standing in their field chewing the cud and one cow turns to the other and says "So have you heard about this mad cow disease then?". The second cow replys "Yeah very scary that, what a way to go" then pauses thinks for a few seconds and adds "but of course, I'm not worried because I'm a squirrel!"
Princess Bobina 10 Sep 2015
In reply to Rob Exile Ward:

It was all silly bull.
 bpmclimb 10 Sep 2015
In reply to Rob Exile Ward:

Has there ever been so much hype expended on such a miniscule threat?

Uh? Not logical. You can't assess the degree of the threat that way. Perhaps we were very, very lucky. You might similarly conclude that driving at 100 mph in dense fog is almost completely safe, on the basis that you've been doing it regularly and aren't dead (yet).
 Jon Stewart 10 Sep 2015
In reply to Shani:

> "Has there ever been so much hype expended on such a miniscule threat?"

> Global Warming!

Terrorism.
1
abseil 10 Sep 2015
In reply to Greasy Prusiks:

> At last! You'll have to imagine the west country accent that all cows have..... Ok so there's these two cows standing in their field chewing the cud and one cow turns to the other...

Thank you! Worth the wait!
 Ridge 10 Sep 2015
In reply to CJD:

> Morning! (or rather, afternoon)

We said her name three times!
cb294 10 Sep 2015
In reply to Dave Garnett:

Same here, Pubmed would have to be my friend. I did a degree in virology when the prion hypothesis was winning, but have moved on to different bits of biology since. Nowadays I just follow immunology and virology our of a certain sentimental interest.

Sorry,

CB
 wercat 10 Sep 2015
In reply to Chris the Tall:
I was personally involved in making sure aircraft didn't fall out of the sky, along with lots of other people at BA and the Indian contract companies who fixed some of the programs for us. Prior to that was the "bug" that involved maintenance systems being unable to record more than 999999 flying hours on aircraft and every subassembly and component such that the systems that flag preventative maintenance wouldn't work properly on that number of hours. And yes, at that time there were aircraft approaching the million hour mark! (This is many years ago)
Anyone who claims the Y2K thing was a hoax IS an idiot because they ignore the evidence and history of the projects to prevent failures or because they are talking from a position of wilful ignorance
Post edited at 22:57
 Dax H 10 Sep 2015
In reply to Rob Exile Ward:

Was it last year or the year before that we were all going to die because of swine flu?
Loads spent on vaccination and face masks etc and all came to very little in the end.
Had nothing been done and there had been an epidemic the survivors would say the government didn't do enough.
Damned if they do and Damned if they don't.
 Morgan Woods 10 Sep 2015
In reply to The New NickB:

> Barely ever posts these days

I got it
 The New NickB 10 Sep 2015
In reply to Dax H:

Swine Flu was 6 years ago, time flies. I remember because I was tasked with finding a couple of buildings to use if we had to isolate people locally. 12 years since SARS.
In reply to Greasy Prusiks: One of the funniest ever Private Eye cartoons came out at the time of the cjd crisis, which coincidentally was at the same time a Van Gogh was sold at auction for £10 million.

It was set in the auction room, the painting was on display, and there was a cow on the front row with a silly smile on its face and its hoof in the air, making the winning bid.

 Tony the Blade 11 Sep 2015
In reply to Rob Exile Ward:

> Has there ever been so much hype expended on such a miniscule threat?

How about the fabled Weapons of Mass Destruction?
 RockAngel 11 Sep 2015
In reply to Rob Exile Ward: swine flu, bird flu epidemics. Everyone seemed to get it, except me! Not that I wanted it but it made me think that most people just had a regular cold. I've had the flu so know first hand how ill it makes you and the descriptions from my friends of their swine or bird flu wasn't even close.

 Andy Say 11 Sep 2015
In reply to Rob Exile Ward:

I assumed that once CJD had changed her username to TallClare we were all safe?

Was I wrong?

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