UK Edition
Have you got the 'mad cow' gene? Why the disease could be about to strike again
20 years after the scandal over British beef, scientists are learning BSE can lay dormant for decades - so could Mad Cow Disease strike again?
This article has an estimated read time of seven minutes
Tommy Goodwin remembers the moment his son, Grant, started to change. It was 2008, and his normally vivacious, party-loving 29-year-old had become withdrawn and paranoid. In their regular phone calls, between Guernsey, where he lived, and his parents’ home in Hamilton, near Glasgow, Grant worried them with out-of-character reclusive behaviour and jealousy towards his girlfriend.
“We thought he was having a wee break down,” recalls Tommy Goodwin, now 58.
Grant received treatment for depression, but his symptoms worsened. After his joints became weak and he started collapsing without reason, he lost his job as an engineer. His parents worried he might have a brain tumour.
But these were in fact all signs of a serious, rare illness that would take more than a year to diagnose. Grant had Variant Creutzfeldt-Jakob disease (vCJD), the human form of Bovine Spongiform Encephalopathy (BSE), which was killing his brain cells one by one. It was going to kill him, too.
“The human form of mad cow disease… it’s a horrible thing to say,” says Goodwin. “Grant didn’t last six months. For the next four months, we took him home and just cared for him. He went through [the symptoms of] every illness you can think of, including dementia and schizophrenia. It was just horrendous.”
Grant died at home in January 2009 – and set the mad cow disease scandal on a new course. Before him, every person who had died from vCJD had been of the same genotype, called ‘MM’. Grant was the first person with ‘MV’ genotype, and so began a second generation of the outbreak.
Research in the 90s into a similar prion disease (those caused by an infectious protein), known as Kuru, had shown that people of MV genotype could incubate the illness without symptoms for as long as 50 years. Grant’s death was evidence that the same could be true for vCJD, and that a new wave of infections was on the horizon.
To date, 178 people in the UK have died from vCJD, which is the subject of tonight’s BBC Two documentary Mad Cow Disease: The Great British Beef Scandal. As it stands, there is no test or cure for vCJD, and no one has ever been held accountable for how it came to pass from cows to humans.
“Grant was 30 when he died, so where did he get this from?” Goodwin recalls asking Professor John Collinge, a vCJD researcher at University College London, when he was told the incubation times for MV genotypes. “He told me to look no further than Grant’s baby food. We didn’t realise it was poison.”
The origins of mad cow disease were known in the 1980s, almost two decades before the first human contracted vCJD, when it emerged that farmers had been feeding the remains of infected cattle back to cows through a rendered product called “meat and bone meal”. The Government banned the practice in 1988 after cows up and down the country developed BSE, but it was too late to halt the crisis. Children like Grant, who was born in 1979, had already been eating potentially contaminated meat for a decade.
It wasn’t until Stephen Churchill, 19, died from vCJD in 1996 that the Government accepted BSE could pass from cows to humans – and started to warn of the dangers of cheap meat.
“We were the first piece of the jigsaw that was falling into place slowly but surely,” says David Churchill, Stephen’s father. “It disappoints me that the disease should ever have occurred in the human population. And that there will be families whose loved ones are incubating the disease who will have to go through the trauma we experienced.”
Despite decades of research, a great deal remains unknown about vCJD. It is not clear exactly how it transferred to humans, why some people became infected when others didn’t, and how many people could yet become ill.
“We understand a lot more about the mechanisms than five years ago and a huge amount more than 1980,” says Professor Richard Knight, a researcher in clinical neurology at the CJD Surveillance Unit, citing the knowledge that it can be transferred through infected blood transfusions, and that it can affect parts of the body beyond the brain. But the information gaps mean there is currently no test for if someone is incubating vCJD – and no workable cures.
What is certain, though, is that more people are likely to die from the disease. “When there was a case in 2016, people were very alarmed,” says Prof Knight. “We have expected cases to occur. The big surprise to me would be if there weren’t any more cases.”
As with the initial outbreak, experts anticipate the scale will be small but painful for those it affects. “The number of cases from 1996 to today [178 in the UK] is likely to be greater than those we’d expect to find between now and 2050,” says Prof Knight. “In general, there have been fewer cases than one might have expected. But for those affected and their families, it’s very distressing and a major disaster.”
For the families of those who have died from vCJD, the agony is immeasurable – as is the feeling that no one has been held accountable. Every Christmas, one mother, Annie McVey, sends a picture of her daughter, Claire, 15, to a ‘hit list’ of those she blames for her death. With it, she attaches the message: “Your actions did this. When you sit down with a Christmas lunch, we sit down with an empty space.”
Families remember how John Selwyn Gummer, now Baron Deben and then Conservative minister for agriculture, fisheries and food, brazenly fed a burger to his four-year-old daughter, Cordelia, in 1990 to convince the public that British beef was safe to eat, despite mounting evidence that some of it might not be. And they recall that companies who sold infected meat have never apologised for harming their loved ones.
“I’ve contacted everyone from baby food companies to health ministers,” says Goodwin. “It’s totally outrageous what happened.”
The Goodwins received £120,000 in compensation before Grant died, which covered the cost of a downstairs extension for him to live while ill, and a short holiday to Glencoe. “We’ve got photos and memories of the trip, but he didn’t even know where he was,” says Goodwin. “That was in October and he passed away in January.”
On his deathbed, Grant begged his father: “Dad, please get the b******* who did this to me.”
“I’m sorry for swearing, but I’m being brutally honest,” says Goodwin. “I’m not going to go and do anything physically, because that would mean these people have won. But I will not rest until those with responsibility take some accountability for what they’ve done to my family.”
Churchill feels much the same sense of unfinished business: “I don’t feel there were any repercussions to the people involved in the decision making in the 80s. The public inquiry never found a smoking gun.” But, he adds, “I continue to wonder if, when official papers from that period are released in a few years, more could be revealed.”
As local authority budgets are cut and the UK plans its exit from the European Union, it is pertinent that the mistakes of the past aren’t repeated, says Professor Tim Lang, a food policy expert who followed the scandal from its start.
“There’s a lot we can learn from the mad cow disease outbreak,” says Prof Lang. “How not to handle food issues, how not to patronise the public, and how not to think cheap food can ever be the answer. If you cut public health and local authority inspection – as we’re doing now – there’s real danger to public health.”
After decades fighting for victims’ families, Churchill is trying to move on. But Stephen is always in his thoughts. “Certain things will trigger memories, like a photograph, or if I hear something about the Royal Air Force,” he says. “Days don’t pass without Stephen being remembered.”
Goodwin’s life fell apart after his son died. Before, he was a happily married father of two and production manager at Philips. Now, he works as a taxi driver and lives alone. “This thing has split up my whole family,” he says. “We were brokenhearted by what happened to my son. My daughter, Debbie, cannot take in that her brother died, and my wife still believes that Grant is alive.”
He visits Grant’s grave every day and reminds himself of the promise he made – to get justice for Grant, the other bereaved families, and victims yet to come. “We have watched our loved ones melt away and die in front of us,” he says. “And what’s happened? Nothing.”
Mad Cow Disease: The Great British Beef Scandal is on BBC Two on Thursday at 9pm