In the dying days of the 20th Century, a strange and terrifying plague began to carve a path through Britain. We’re not talking about HIV, the virus that caused such devastation across the globe in the 1980s and 1990s. We’re talking instead about something far more sinister – an epidemic that should never even have happened. Bovine Spongiform Encephalopathy, better known as BSE or by its nickname “Mad Cow Disease”, first appeared on a British farm around Christmas, 1984. In no time at all, it had spread first to herds across the country, and then on into the human food chain.

The results would be catastrophic.

Starting in 1996, nearly 240 people would be struck down by Variant Creutzfeldt–Jakob Disease, the human form of BSE. One by one, victims’ relatives were forced to watch as the plague devoured their loved one’s minds, eventually leaving only a tortured husk. Fatal in 100 percent of cases, its detection was a death sentence. Yet, rather than occurring naturally, this was a disease with a very human origin. One only made possible by a terrifying collision of corporate greed, government malfeasance, and sheer, callous stupidity. Today, Biographics is investigating the story of BSE – the deadly plague created by man.

A Tale of Two Sheep

Although it’s most-associated with the ‘90s, the story of BSE actually begins a long time ago. Nor does it begin with a cow. Instead, it starts on a British farm in 1732, with one unlucky sheep. Sadly, we don’t know enough about what happened to paint you a verbal picture. We can’t open this section with “it was a dark and stormy night…” or anything else suitably ominous.

But we can say how it must’ve looked to the farmer.

His long-forgotten sheep would’ve been anxious, delirious. It’s head would have jittered from side-to-side, making it appear possessed. Before long, it would’ve become unsteady on its feet. Started arching its back and scraping its body along stone walls, tearing out its wool.

Finally, a maximum of six months down the line, it would’ve died in agony.

Not long after, other sheep would’ve begun showing signs of the same awful disease. Known as scrapie, the blight eventually affected untold numbers of sheep – first across Britain, and then most of the rest of the world.

But, this being the early 18th Century, people were all like “hey, it’s still food,” and ate the dead, infected sheep. And it didn’t cause them any problems. To this day, there’s no evidence that scrapie can jump the species barrier.

Unfortunately, this would cause a major issue some 250 years down the line.

When the first cattle started showing signs of BSE in the mid-1980s, it would be because of humanity’s experience with scrapie that infected beef was declared safe to eat. But before we get to that part of our story, we have to make another pit stop. Cut to the 1920s. It’s the era of jazz, everyone’s dressed like they’re in the Great Gatsby, and a toothbrush moustache is still a legitimate fashion choice.

In the shadows, though, a new disease is growing.

Creutzfeldt–Jakob Disease (or CJD) is a degenerative brain disease that’s both super-deadly and super-rare, affecting only one in a million people. 

But what made it extra-fascinating for 1920s doctors was the way it worked.

See, the surface of our brains are covered in proteins called prions. What CJD does is fold these prions in a weird way. These weird, folded-proteins then latch together to form toxic chains. As these chains grow, break apart, and grow again, they spread across the brain, killing cells and leaving little sponge-like holes. But, because the proteins are a natural part of us, our immune systems don’t identify them as something worth attacking.

For someone infected with CJD, this means a steady decrease in mental functions and motor skills. There’s confusion, anxiety, and difficulty walking that soon progresses into serious disability.

Within a few months, you’re dead. 

And there’s no way to stop it.

Since its discovery in the 1920s, CJD has been with us pretty consistently. Most years, around 350 Americans die from it. But it would be events in the 1990s that brought CJD firmly into spotlight. The final piece of set-up we need for our story came around the same time that CJD was discovered.

At some point, farmers realized that they could get more profit from an animal by letting literally none of it go to waste. The grim bits like spinal cord and all the leftovers could be rendered down, mulched up, and fed back to other animals as feed.

Gross as this sounds, it was a major breakthrough.

Animals that consumed the shredded remains of their brethren tended to grow bigger and faster than others. By the 1980s, this barnyard cannibalism had spread across the planet, revolutionizing food production. 

Now it was about to cause the biggest food contamination crisis in British history.

A Very Un-Merry Christmas

Shortly before Christmas Day, 1984, David Bee got the call that would change his life. The local vet in his part of Sussex, Bee was famous among his neighbors for his pleasant singing voice.

Soon, though, he’d be known for a very different reason.

On a farm owned by Peter Stent, a cow was dangerously sick. What with, no-one knew. When Bee examined it, he found the cow had unsteady legs, an arched back, and a head that was wobbling from side to side. It wasn’t the first in the area to show such symptoms. Just recently, one of Bee’s veterinary friends had told of a cow that tried to chase him on legs so weak it had run on its knees.

But this was the first time Bee had seen this weird new illness up close.

And he didn’t like what he saw at all. The closest thing to the symptoms he could think of was mercury poisoning. But how the hell would a single cow get poisoned out on a random farm? So Bee kept an eye on the animal. And, when it died in February of 1985, he contacted the UK Central Veterinary Laboratory (CVL).

That September, the CVL examined the brain of Cow 133, and found the tell-tale sponge-like holes of Creutzfeldt–Jakob Disease.

They named this new, cattle version Bovine Spongiform Encephalopathy, or BSE. 

Barely had they identified the illness than it began cropping up everywhere. Over the next two years, BSE spread across the south of England. Six positive cases were confirmed, alongside 13 suspected ones. In the CVL, discussions turned to whether scrapie had jumped the species barrier from sheep to cows. There was even talk about getting more researchers involved.

But the powers that be put a stop to that. A governmental memo from the time read:

“If the disease turned out to be bovine scrapie it would have severe repercussions to the export trade (…) At present I would recommend playing it low key.”

In these two sentences, you can see the diseased heart of the BSE crisis.

In the 1980s, agriculture was a huge part of Britain’s economy. A health scare could cost the country billions. So officials were incentivised to keep quiet about a potential crisis. As 1987 dawned, no-one said a word.

They kept silent as the number of cases jumped to 46 that August. Then 73. Then 120.

By the end of October, the first reports were appearing in the farming press about a deadly new cattle blight.

But by then it was too late.

Remember how we said farmers in the mid-20th Century had started feeding unusable bits of dead animal back to their livestock? Well, they were now doing the same with BSE-infected cows. But no longer was the mulch just going to other cattle.

“Mechanically recovered meat” is a euphemism for all the gross bits of animal that no-one wants to eat. 

In the 1980s, someone had hit on he idea of putting it into human products. This being the Thatcher era, regulation was light. Whitehall was all like “you wanna feed spinal cord to children? Sure! Sounds like fun.”

And so this “mechanically recovered meat” – made from the most-infectious parts of BSE cattle – was recycled into sausages, into pies, into anything the industry could get away with.

By the time the press started reporting on BSE, the ship had already sailed.

Humans were eating infected meat by the millions.

And nobody had any idea what would happen next.

Anatomy of a Scandal

By 1988, 200 new cases of BSE were being reported every week, affecting 6,000 British farms.

To the British government’s credit, they did try to act. 

That same year, the practice of feeding animal remains to other animals was banned. The following year, the use of mechanically recovered meat in human foods was outlawed.

But that was where the positive action ended.

More than a million infected cattle were already in the human food chain. Burgers, steak, joints of roasting beef… all could be carrying this disease. The government had two clear choices. They either recalled the meat, told people to avoid beef, and watched the agriculture industry implode…

…or they just acted like nothing had happened and hoped for the best.

It was at this point that 1732 reached across the centuries to deliver the present a devastating kick to the testicles. BSE looked like scrapie, and since scrapie had been around since 1732 without infecting humans, officials assumed BSE would act in exactly the same way.

It was a decision based not on science, but assumption. One that would turn out to be catastrophically misjudged.

Not that the public would be told this.

In 1990, the food and agriculture minister, John Gummer, began a media blitz to get Brits eating beef again. A massive, full page advert was taken out in the major papers, declaring in bold text BEEF IS SAFE. On TV, Gummer repeated this message, saying British beef could be eaten “by adults and children” without worry.

At no point did he add “I mean, I guess? Seriously, guys, I’m just making wild assumptions at this stage”. 

No, the talking point was “beef is safe, anyone who says otherwise is a science-hating luddite”.

Not that science itself agreed. That same year, 1990, Doctor Stephen Dealler was conducting some worrying experiments. He’d shown that, unlike scrapie, BSE could be used to infect mice in laboratories. But when he tried to speak out, he was removed from his post, and reassigned to a lowly station where he couldn’t conduct further research.

Meanwhile, John Gummer took a break from photo ops eating steak tartar to go on TV and accuse worried scientists of scaremongering.

Looking back now, it’s weird just how Soviet this all sounds.

The official denials, the assumptions that politicians know better than scientists, the panicked coverups…  In a lot of ways, you could say the BSE scandal was really the West’s version of something like the Sverdlovsk anthrax leak, the accidental 1979 release of anthrax spores in Russia that killed 100 civilians.

In both cases, the government pretended nothing was wrong. In both cases, nobody ever faced any kind of prosecution.

But while the Sverdlovsk disaster would kill 100, BSE would kill many more.

By the end of the year, the John Gummer propaganda machine was in overdrive. In one uncomfortable instance, he was photographed feeding his four year old daughter a beef burger.

But by now the dangers of BSE were becoming all too apparent.

That May, 1990, a cat in Bristol had died from BSE after being fed infected beef. By the time the year ended, 18 more cats were dead from the disease. This wasn’t another scrapie. There was now clear evidence that BSE was not just jumping the species barrier, but leaping over it with gay abandon.

14 other species had now been infected, from household pets to animals in zoos. Everything that ate beef seemed to be coming down with this awful, fatal illness.

With one major exception.

As 1991 dawned, bringing with it a new Prime Minister, not a single case of BSE in humans had yet been recorded.

Sadly, that was about to change.

Crisis Time

Near the start of the crisis, in 1989, the British government had ordered the extermination of all affected cattle. However, they offered farmers only 50% compensation for each cow killed – in effect putting a heavy financial incentive in place for ignoring or misdiagnosing early signs of BSE.

On top of that, the mechanically recovered meat ban of 1989 had a woeful lack of oversight. As late as November, 1995, infected spinal column would still be making its way into the human food supply.

All of which may go some way toward explaining what happened next.

In May, 1995, BSE as a national crisis seemed to be on the wane. The peak days of 1992-93 – when three cattle in every 1,000 were infected – had already passed.

Then disaster struck.

The first symptoms 19-year old Steve Churchill showed were hallucinations and trouble walking. Initially, doctors diagnosed him with a psychiatric disorder. But as time went on and his condition deteriorated, it became clear that there was something badly wrong.

At 9am on May 21, 1995, Steve Churchill died. 

In the aftermath, his brain was sent to Edinburgh for analysis. There, they discovered the telltale tiny, sponge-like holes of Creutzfeldt-Jakob Disease.

In and of itself, this was not impossible. 

In the mid-90s, as now, natural CJD killed around 60 Britons a year. But rarely did it attack someone so young. By June, scientists working on the poor, dead boy’s brain were terrified they might have a new disease on their hands.

Their predictions would turn out to be painfully right.

That October, two teenagers came down with this new form of CJD. Then a cattle farmer. Come March, 1996, 8 young people had all died of CJD in a matter of months – a near statistical impossibility. 

On the 8th of the month, the government called a meeting with the nation’s top scientists. 

It was there that a horrifying announcement was made.

Despite all the years of assurances, despite John Gummer’s massive propaganda drive, BSE had jumped the species barrier. Mad Cow Disease was now killing humans, eating away at their brains. There was no way to stop the process or reverse it.

And no-one knew how many people were infected.

The next few days played out like a horror movie.  As the human form of BSE was declared Variant Creutzfeldt-Jakob Disease (or VCJD), panicked politicians asked how many people it might kill. The answer was anything from a few hundred… 

…to over half a million. 

Half a million Britons, killed by a disease that was entirely manmade. A disease that wouldn’t exist if the government hadn’t stifled scientists and made faulty assumptions. Yet, even as those in Whitehall faced down the barrel of a devastating pandemic, no-one bothered to warn the public. 

No-one told people about the dangers of eating beef for another twelve days.

Finally, on the morning of March 20, 1996, the tabloid Daily Mirror broke the story in bold text:

“BSE CAN KILL HUMANS.”

Just hours later, the Health Secretary stood up before Parliament and declared a new deadly brain disease caused by infected beef had been discovered.

By now, there were 10 cases in humans.

What happened next, no-one knew. They could, however, agree on one thing. It was going to be the scandal of the decade.

Years of Madness

In the aftermath of the government’s announcement, Britain’s beef industry collapsed. Billions of pounds were wiped out, alongside millions of cattle. In an effort to control the disease, 4.4 million cows were slaughtered and burned. 

Incinerators glowed on farms around the clock. Dead cattle piled up in mountains of corpses. Up to 60 farms went bankrupt.

Internationally, the EU immediately banned all beef imports from Briton. But it was already too late. Over the following years, 26 French people would die of VCJD, alongside 5 Spaniards, 4 Irish, 3 Dutch, 2 Italians, and 2 Portuguese.

But it was in Britain that the human cost would be at its highest.

By 1998, there were 18 cases a year, all of them fatal.

While that’s a relatively small number, it masks awful suffering. Almost all victims of VCJD were under 30. Most were teenagers. As the disease took hold, they lost their motor functions, the ability to speak. Became badly disabled, unable to do anything but wail in pain as their families looked on, helpless to stop their agony.

It was a nasty, painful way to die, one made worse by different approaches across the nation.

In some areas, patients were allowed to be with their families. In others, they were locked in isolation for the last months of their lives; left to die alone in agony by local authorities scared they might pass their sickness on.

That same year, the new government set up a research body to – basically – try and estimate how screwed they were.

The answer? Utterly screwed. 

VCJD was found to not just infect the brain and spine. It infected the entire body, even getting into the blood. And some of those who had already died of VCJD were known blood donors.

Over 6,000 people in Britain were given BSE infected blood. As the government spent £100m to filter the disease out the blood bank, most countries on Earth banned British blood donations – bans that still remain in effect.

As the new millennium dawned, it looked like the crisis would never end. 

The year 2000 saw a jump in annual deaths to 28. The next year, BSE appeared in Japanese cattle – the first country outside Britain to be sucked into the crisis. Two years after that, in 2003, Canadian cattle were infected. At least one entered the human food chain, leading to a total shutdown of Canadian exports that wiped $11m off the economy a day.

And then, just as it looked like the crisis had hit a tipping point… it stopped.

Although 2005 saw the first Japanese human death from VCJD, it wouldn’t be followed by another. In the UK, the cases were on a downward trend. By 2007, they had dropped to only five cases per annum. Although over 160 Britons had so far died, it seemed like the threat of death from Mad Cow Disease had at last faded.

Or had it?

The Second Wave

In the mid-1950s, scientists made a gruesome discovery in the highlands of New Guinea. An isolated tribe was being felled by something similar to Creutzfeldt-Jakob Disease. Children, adults, the elderly, all were succumbing; first losing their coordination, then feeling their minds slip away as the blight consumed them.

Given the rarity of CJD, it would’ve been remarkable if two members of the tribe had died of it. But dozens? 

Clearly something was going on.

So scientists investigated, and uncovered a shocking story.

The tribe were cannibals, whose death rituals involved eating the bodies of their loved ones. 

At some point, they’d consumed the brain of someone with CJD, allowing it to infect more tribe members, who’d in turn infected more tribe members when they’d died of the disease, and so-on. It was eerily similar to BSE in Britain. So much so that, when the Mad Cow Disease scare kicked off in the 1990s, London sent some people to investigate.

They would not return with reassuring news. 

Just like with VCJD, the New Guinea tribe had exhibited an onset of symptoms some five years after eating infected meat. 

But there hadn’t just been a single spate of deaths. 

There had been at least two distinct waves. In some cases, the incubation period had lasted up to fifty years. The proteins that CJD deforms – remember? The ones that turn into toxic chains that eat through your brain – always carry one of two variants, M or V.

Since they’re inherited from our parents, everyone on Earth has either MM genes in these proteins, or MV, or VV.

All the New Guinea tribe members to die in the first wave had had the MM genotype. Now, nearly half a century later, all those to die from VCJD in Britain and abroad had also been MM types.

This left a single, horrifying likelihood. 

BSE was going to return.

It started in late 2014. 

A 36-year old British man began showing signs of CJD. When he died in February, 2016, scientists checked his brain and discovered he’d been infected with the human form of BSE, likely from eating infected meat as a child.

But here’s the kicker. When scientists finally checked his genotype, they found he didn’t hold an MM combination, but MV. There had been a suspected case before, in 2009, but this was the first confirmed one.

Finally, the dormant disease was activating in a whole new slew of people.

And with that cliffhanger, our story has to end.

Since 2016, there have been no more confirmed deaths of MV geneholders due to VCJD. It may be that this manmade plague is over. That there will be no more deaths, and Mad Cow Disease will soon be seen as a relic of the 1990s on a par with the Spice Girls and tamagotchis. 

But that seems unlikely.

Everything we know about VCJD suggests there’s a second wave around the corner; a point in the near-future when it will cut a swathe through Britain’s population again, felling those with the VM genotype.

In the early 2010s, the UK government reported that as many as one in 2,000 Britons may be silent carriers of VCJD, with dozens more scattered across the globe.

When the second wave finally hits, it could kill any number of them.

To date, 178 people in the UK have died of human-varient BSE; with another 52 dead in other countries.  In a world shaken by CoVid-19, it’s tempting to hear such statistics and think “huh, that’s not so much,” but make no mistake.

VCJD isn’t a regular disease. It’s a manmade disaster and government coverup, one more comparable to a Soviet bioweapons leak, or the Tuskegee syphilis experiment than to a natural pandemic. Faced with knowledge that could have saved lives if published, but resulted in an economic hit, the British government chose to protect the economy.

Because of that choice, over 230 people died, with hundreds more possibly to come.

Usually when we think of these sorts of coverups, we think of incompetent dictatorships or nations in the distant past. But BSE – a disease caused by human greed – shows this isn’t always the case. You can live in a rich, western, functioning democracy and still be killed by government malfeasance.

The story of this horrific plague isn’t over. There may be another volume still waiting to be written. One that catalogues yet more deaths, yet more appalling, needless suffering.

All we can do is wait and see.

Sources:

YouTube link to an excellent BBC documentary: https://www.youtube.com/watch?v=meYnivLLl-A 

Link to the official BBC page: https://www.bbc.co.uk/programmes/m0006p49 

New cases still to come: https://www.bbc.com/news/uk-scotland-48947232 

Focus on the disease’s early days: https://www.theguardian.com/uk/2000/oct/29/bse.focus1 

The horrible future: https://www.newscientist.com/article/2118418-many-more-people-could-still-die-from-mad-cow-disease-in-the-uk/

Britannica: https://www.britannica.com/science/bovine-spongiform-encephalopathy 

Full timeline: https://www.theguardian.com/uk/2000/oct/26/bse3 

BSE History: https://www.newscientist.com/article/dn91-bse-disaster-the-history/ 

EU cases: https://www.ecdc.europa.eu/en/vcjd/facts 

Canada’s Mad Cow Crisis: https://edmontonjournal.com/news/local-news/a-brief-history-of-mad-cow-disease