Are we still living in the shadow of Mad Cow Disease?

In the years before Covid, there were a host of emerging health problems which threatened to change our way of life and very existence. But perhaps none so as frightening as variant Creutzfeldt-Jakob Disease (vCJD). And Kent appeared to find itself right at the centre of it; with a cluster of deaths in the county.

Millions of cows were slaughtered in order to try and eradicate BSE in the UK's cattle herds Millions of cows were slaughtered in order to try and eradicate BSE in the UK’s cattle herds

Caused by the consumption of cattle infected with bovine spongiform encephalopathy (BSE), it was quickly dubbed Mad Cow Disease.

In the mid-90s it emerged as a major threat to public health – and one which may take years to fully emerge. In short, the public were warned that if they had inadvertently eaten infected meat, they were at risk. It attacked the brain and there was – and remains – no cure.

Little wonder it caused considerable concern and dominated the headlines at the time.

Yet after 139 recorded deaths in the UK between 1995 and 2003, rates have steadily declined. In fact, over the last 10 years, there have been just two recorded deaths across the nation.

Scientists have long researched the causes of vCJD Scientists have long researched the causes of vCJD

But there are fears many may be carrying the disease. In 2014, MPs on the science and technology committee warned tens of thousands of people could be “silent” carriers of a disease which, it is feared, could have an incubation period “of decades”.

The year before, a report published in the British Medical Journal suggested one in 2,000 people in the UK could have the prions (misfolded proteins) which cause the disease.

Just two years ago, Richard Knight, a senior neurologist working at the University of Edinburgh’s National CJD Research and Surveillance Unit said: “Every prediction that we have suggests that there are going to be further cases.” Which begs the question, could Mad Cow Disease come back and haunt us all these years later?

Mick Tuite, emeritus professor of molecular biology at the University of Kent's School of Biosciences Mick Tuite, emeritus professor of molecular biology at the University of Kent’s School of Biosciences

“My gut feeling is it won’t,” says Mick Tuite, emeritus professor of molecular biology at the University of Kent’s School of Biosciences. “It’s now 20 years later and there’s been a decline to almost zero.

I cannot believe they take that long to emerge. “There was a lot of sensational press coverage at the time which said there could be tens of thousands of cases by the year 2000 or 2010 and that’s never happened.” It’s a cruel and horrific disease too.

“Once disease manifests itself,” he explains, “vCJD starts with depression, anxiety, hallucinations, and as something I once read, described it as a ‘journey into hell’. Within six months to two years, it’s fatal.

“There was a lot of sensational press coverage at the time which said there could be tens of thousands of cases by the year 2000 or 2010 and that’s never happened…”

“I would be shocked if it emerged, unless, of course, BSE was to return.” Which, earlier this month, it did – albeit, hopefully, fleetingly.

A single case of BSE in a cow in Somerset was identified according to the Animal and Plant Health Agency, which was quick to add there was “no risk to food safety”. It added “precautionary movement restrictions have been put in place on the farm while further investigations continue to identify the origin of the disease”. It will have sent a shiver down the spine of many.

There remains no cure for those suffering from vCJD - or the more common sporadic CJD There remains no cure for those suffering from vCJD – or the more common sporadic CJD

The first case of BSE in a cow had been detected in 1985 but it would take 10 years for it to leap into the public consciousness.

It was thought the illness which started to infect hundreds of cattle would, like other animal illnesses before it, not make the leap from animal to human. The cause was believed to have been due to reprocessed, infected, meat by-products being turned into animal feed – in effect, feeding BSE infected food to healthy cattle. By 1989, amid mounting concerns, the government banned the likes of offal being sold for human consumption.

But confidence in British beef was starting to fade among consumers. The following year, most of Europe banned British beef imports – an enormous blow to the industry both in terms of hard-fought-for reputation and financially.

Beef exports were banned by Europe during the crisis Beef exports were banned by Europe during the crisis

In 1996, the government admitted there was a link between BSE and vCJD – an announcement which saw British beef subjected to a worldwide ban. It ordered a mass cull of all cattle over 30 months old.

It is estimated some four million cattle were slaughtered as a result. Thruxted Mill in Godmersham, near Canterbury, was one of only four sites authorised to dispose of carcasses infected with BSE. Many remember the truck loads of animals being transported to the site.

British beef, one of the nation’s most successful exports, had become persona non grata on the supermarket shelves or restaurant menus around the world. There were a flurry of cases in Kent – seemingly centred around those who had lived around the Ashford area.

Thruxted Mill in Godmersham disposed of the infected cattle Thruxted Mill in Godmersham disposed of the infected cattle

Speculation started to mount as to the cause – had infected cattle waste entered the water supply, were rendering plants somehow responsible? Kent had four cases within 50km of two rendering plants – which given that up until 1998 only 26 cases of vCJD in England, Scotland and Wales had been identified – prompted fears it was down to more than just eating contaminated beef products.

In 1999, a report published in medical journal The Lancet, concluded “there is no evidence that people with variant CJD tended to live closer than the population as a whole to rendering plants in the 1980s. The reported cluster of variant CJD cases in Kent is most probably a chance finding”. “The steps taken to tackle the issue did the job,” says molecular scientist and prion expert Mick Tuite. “I think we acted appropriately.

“By 2000, BSE was basically ruled as having been eradicated from the British cow population. There have been only two cases of BSE in the last decade too.” But while the issue seemed to disappear – Europe finally relented and allowed British beef exports to resume by 2002 – there remained fears of an incubation period which could still claim lives.

Adds Mick Tuite: “The worry was the disease was still in a large part of the population and some people still talk about the fact there could be as many as one in 2,000 people have got a silent form of this infectious agent. But given we’re now talking 20 years, that’s still an extremely long incubation time. It would be unprecedented.

“As diseases go it’s extraordinarily rare and you have to be extremely unlucky to get it.” By 2019, there have been around 230 cases of vCJD – 178 of which were in the UK. Variant CJD – which is a different strain to sporadic CJD – claimed victims who were predominantly young.

“By 2000, BSE was basically ruled as having been eradicated from the British cow population…”

Explains the molecular scientist: “What happens is the protein takes up a new shape – it literally unravels and then rewinds into a new structure which is somehow toxic.

We don’t yet understand how it’s toxic. But it’s called a protein misfolding disease. “Alzheimer’s and Parkinson’s are protein misfolding diseases.

So there’s a big group of diseases of which prions are in an extraordinarily rare class of as they are an infectious entity, in other words you can take this misfolding protein, inject it into an animal, and the animal goes down with the disease within 200 days. “That’s the big question mark – how long do these prions take to manifest into a disease and if a large proportion of our population are carrying this newly emerging misfolding protein, will they all go down with this disease? “I would be shocked if that was to happen now.

“It’s a bit like when Covid first emerged, there was a lot of money thrown into research in that area, and there still is. It was the same with BSE – a lot of funding went into research but as the issue drops away, then the funding agencies shift their focus to whatever else comes along. “There’s no progress I’m aware of developing drugs in the industry but still a lot of interest amid academia to understand how these things behave and are transmitted.”

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