'Why have they done this to her?': Mother grieved as bright, friendly girl died the frightening, degrading death that mad-cow disease brings

'Why have they done this to her?':
Mother grieved as bright, friendly girl died the frightening, degrading death that mad-cow disease brings

June 2, 2001 Ottawa Citizen by Mark Kennedy

Helen Jeffries couldn't hold back the fury in the pre-dawn darkness last October. For more than two years, the anger and frustration had welled up inside as she watched her daughter, Zoe, die from one of the most degrading, mysterious and frightening diseases known to man.

The vibrant, friendly girl first turned nasty. Then she became a crying, screaming child afraid of the shadows. Finally, Zoe was unable to walk, talk, feed herself or control her bowels.

The changes happened because Zoe's brain had become sponge-like, riddled with tiny holes. All because she had eaten British beef. And now, at 14, she was dead. After 28 months of lingering horror, it had come without warning. She just stopped breathing in her bed, set up in the living room of the family's rowhouse in Wigan, a working-class town west of Manchester.

Before long, the stillness was shattered by a bang on the door. The ambulance had arrived. So had the police. Helen swung open the door, slumped to the floor, and pulling on her hair, screamed: "You bastards! You've done this to her!"

Seven months later, she's still not sure exactly who she was shouting at, but she knows who's to blame. For at least 10 years, officials and cabinet ministers had assured Britons they could not contract a strange new disease that had first appeared in British cattle in 1984. Scientists called it bovine spongiform encephalopathy (BSE), but the tabloids dubbed it mad-cow disease. Cows that fell victim became skittish, staggered, drooled, and were aggressive.

In 1996, after 10 Britons began showing symptoms of a similar ailment, the government finally admitted there was a link. This, the scientists called variant Creutzfeldt-Jakob Disease (vCJD). The tabloids called it mad-cow disease, too.

By then, it was too late. Millions of Britons had eaten potentially contaminated beef. Parents like Helen had fed their children contaminated hamburgers, sausages and meat pies.

"I just kept saying, why have they done this to her?" Jeffries now says of her government. "A trust was abused. They've killed my child. What right have they got to do that? That's how I feel. They might as well have stabbed her. That would have been easier because she wouldn't have suffered so much."

Zoe Jeffries was the 81st and youngest victim to die from the disease, which tends to strike down people in their 20s and 30s. To date, experts have diagnosed 104 people with confirmed or suspected cases of vCJD - 100 in Britain, three in France, and one in Ireland. Most died within 18 months of first exhibiting symptoms. In the coming years, the human toll will rise, and its reach will likely extend to other countries. No one knows where, when or how many. There will be, at least, hundreds more. Likely thousands. Perhaps millions.

All victims who now live normal lives, unaware they are incubating a disease that confounds scientists and scares the hell out of many public-health officials.

Like the tip of an iceberg that has only just appeared on the horizon, it is a medical mystery that threatens to become a global epidemic. It is the AIDS of the new millennium.

Variant Creutzfeldt-Jakob Disease is part of a family of fatal neurological disorders known as transmissible spongiform encephalopathies (TSEs). They have been known to emerge spontaneously - almost as a genetic freak of nature - and hit various species. Sometimes the disease stays within a species. Other times it infects other animals.

Everything about the disease is terrifying. It transforms animals such as cows, deer and elk into slobbering, staggering beasts. In humans, it can cause memory loss, restlessness, aggressive behaviour, seizures and dementia.

The prevailing scientific view is that TSEs are caused by an abnormal mutation of protein molecules, known as prions (pronounced pree-ons), found in cells. Prions are found in many parts of the body, but are clustered in the brain and spinal cord. But, apparently when a single protein is folded into an abnormal shape, it creates a rogue prion that is replicated throughout the body. It eventually attacks the brain, leading to neurological breakdown and death.

The immune system cannot ward off the invading prions and they are virtually impossible to kill, even in laboratories. Scientists have tried freezing, boiling, burning, soaking in chemicals, and exposing them to devices that normally kill other infectious agents. Prions survive.

The disease was first recorded in the mid-1700s affecting European sheep. The animals initially scratched and bit their skin, lost weight, trembled, and scraped their hides against posts. The disease was dubbed scrapie, and it continues to emerge, striking herds of sheep but apparently not crossing the species barrier. About 50 years ago, a similar disease was diagnosed among mink at farms in Canada, the U.S. and Finland and became known as transmissible mink encephalopathy (TME).

By the 1960s, a new disorder, chronic wasting disease (CWD), appeared among deer and elk in Colorado and Wyoming. More recently, cases of CWD have been found among some animals in Saskatchewan.

In the 1920s, two German doctors, Hans Gerhard Creutzfeldt and Alfons Jakob, identified a human form of TSE that affects about one in a million every year. The disease, now christened sporadic (or classical) Creutzfeldt-Jakob Disease (CJD) carries a wide range of symptoms, and tends to hit people in their 50s and 60s.

A strange form of CJD, known as "kuru," was diagnosed in the early 1960s among a tribe in Papua New Guinea that practiced cannibalism. The Fore tribe ate the brains and muscles of deceased relatives as a tribute. Scientists believe that sometime before 1915, one of the tribe members developed CJD. The disease spread when the family cooked and ate the remains. It was passed down through the generations, as those family members died and were eaten at mortuary feasts.

In the 1980s, new variants of the disease emerged among British cows and beef-eating humans. Suddenly, an exotic, little-known disorder became front-page news. In his 1997 book, Deadly Feasts, Richard Rhodes summed up the challenge by comparing vCJD to another horrifying disease.

"Ebola is a terrorist," he wrote. "It sickens people quickly and spares at least one out of 10. The new disease is a stealth agent: it incubates silently for years and kills every last victim it infects."

- - -

Zoe Jeffries was born Nov. 15, 1985, the first of four children born to Helen and Derek Jeffries. Helen laughs about how she was a "bossy" little girl, especially to her brother Michael, just a year younger. "She was very friendly," Helen said. "Not shy at all. She made friends with everybody. Never short of talking. She was very bright. She could read and write just after her second birthday."

Zoe, a strawberry-blond with welcoming eyes, excelled at everything she did. She entered dance contests and won medals. She had a passion for sport and became an award-winning gymnast.

By age 9, acting was her first love. She joined the drama club and starred in school plays. People said she looked like Haley Mills, the child star from the 1960s. Zoe liked that.

Her ambition was to become a Hollywood actress and star in a movie with Leonardo DiCaprio. She watched the movie Titanic, over and over. The walls of her bedroom were covered with posters of Leonardo.

Since she was 2 years old, Zoe had been eating - at least three times a week - the types of beef products that scientists now believe were most heavily infected with BSE: hamburgers, sausages and shepherd's pie.

- - -

Later this month, scientists, government and industry officials, and consumer representatives will gather in Paris to grapple with the threats posed by BSE and vCJD. The conference is being hosted June 11 to 14 by three United Nations agencies: the World Health Organization (WHO), the Food and Agriculture Organization (FAO) and the Office International des Epizooties (OIE), which fosters better veterinary practices.

The agencies called the meeting after it became apparent mad-cow disease was not confined to the United Kingdom. Other European countries have reported their first cases in native cattle. Moreover, there are increasing fears that BSE could one day become a worldwide epidemic. Here's why: new evidence shows feed supplements containing the ground-up remains of infected British cattle were exported to dozens of countries in the '80s and '90s. That means more humans will eat infected beef and be exposed to vCJD.

The UN agencies want to know: How many animals are incubating mad-cow disease? Will it become an epidemic? If so, how can it be stalled and reversed?

Scientists are divided on many of the issues. There is a wide divergence over how many humans will contract vCJD. Estimates range from several hundred to several million.

"I don't think we have enough information to say what the upper limit is," says Neil Cashman, a University of Toronto medicine professor and one of Canada's leading experts on vCJD. "It makes me wake up sweating at night, because we don't know how bad this epidemic is going to be."

Cashman says governments and the scientific community need to confront vCJD just as they attacked AIDS nearly 20 years ago. "We have to face this disease the same way. I think the threat is just as serious."

Richard Lacey, a microbiologist in Leeds, England, was an early prophet in the mad-cow scandal. He warned that the disease could cross from cows to humans, but the then-Tory government called him a crank and offered assurances that British beef was safe.

Lacey says the British government of Tony Blair continues to deceive the public. Not only are an unknown number of people infected, he says, but many will become infected from eating beef that still contains BSE.

Lacey castigates countries such as Britain, Canada and the U.S. for refusing to conduct wide-scale tests of cattle. He notes only when tests began recently in countries such as Germany - which once declared itself to be "BSE-free" - were cases of BSE found. Lacey says governments are refusing to learn the basic lesson of the mad-cow scandal: take precautions, don't dismiss bad-news scenarios, and admit that the safety risk is unknown.

"But politicians are very short term. They only think of the next four of five years, of getting voted in again. This disease is so long term, no one can really cope with its concepts."

Just how significant is the threat? Three questions are central:

- How long is the incubation period for vCJD? If it's 10 to 15 years, the problem may not be so bad and we may already be witnessing the worst of a disease that will strike just a few hundred. But if, as some experts fear, the incubation period is 10 to 40 years the world is looking down the scope of a public-health nightmare. Many more people would then be incubating the disease and will eventually show symptoms. Furthermore, scientists fear that while the disease is lurking in victims, they could unknowingly pass it on if they donate blood or organs, or if they have surgery and the instruments are used on another patient.

- Does the disease strike everyone, or just those with a specific genetic type? So far, all the vCJD victims have shared a similar genetic make-up, one that occurs in about 40 per cent of the Caucasian population. If only they can get vCJD, this will limit the toll. However, scientists aren't yet confident that luck is on their side. It is possible - some think it is likely - that the genotype merely has an impact on the length of incubation, meaning some victims show symptoms sooner.

- What's the threshold for getting the disease? No one knows how much BSE-infected beef has to be eaten before a human gets vCJD - one hamburger or hundreds of meals over several months or years? Certain cuts of meat from a BSE-infected animal are probably more infective such as burgers, sausages and meat pies, which are ground up and more likely to contain fibres from the nerves, where the BSE prion is clustered. Meat attached to the bone, such as T-bone steaks, is considered more risky, while filet mignon less so.

Paul Brown, a senior American researcher at the National Institutes of Health near Washington, D.C., is one of the leading U.S. experts in the field. He is optimistic: "It is not now and never will be a global epidemic," Brown says flatly. "And that's because all of the countries in the Western world and other countries like Japan and Thailand have put in place regulations that simply wouldn't allow it to happen." Brown says he believes most vCJD victims will be British and the final tally could be about 600 people.

Others consider this too rosy a prediction, one that could lull governments into a false sense of security.

"He's wrong," says Stephen Dealler, a British microbiologist who, like Lacey, was among the first to warn of the dangers of eating British beef. "I hope he's right, but my figures are a maximum of about 5 million in the U.K. I expect to see a peak in the U.K. between 2010 and 2020, and the later (the peak) is, the bigger the numbers are going to be."

- - -

In February 1998, Zoe Jeffries's father, Derek, died of a heart attack. Helen was left with four children on a widow's allowance. She was an emotional wreck. In May, Zoe, then 11, began complaining of leg pains. Her joyful skip turned to a slight limp. Then the headaches started, followed by fits of crying. Then came depression.

Helen thought Zoe was upset by the death of her father and the doctors offered the same diagnosis. But Zoe got worse. "She went through a complete personality change," Helen recalled. "She just became a horrible little girl. She was aggressive, falling out with all her friends, fighting."

Zoe became an insomniac and one morning, she started crying and wouldn't stop. It continued for about three weeks. Soon, she could no longer attend school. She pulled violently on her own hair, yanking out handfuls. The hallucinations were particularly cruel. Gremlins, she called them - little men who climbed the curtains and came into her room at night.

Zoe couldn't keep still. At night, she'd thrash about, falling out of bed. The doctors continued to diagnose depression. Her face began twitching. One eye constantly winked. By Christmas, more than six months after it had begun, she was having difficulty directing food into her mouth with a fork or spoon. She was dribbling. And she was falling down. By the end of the year, she was crawling like a baby.

- - -

In December 1984 - 11 months before Zoe Jeffries was born - Peter Stent, a dairy farmer at Pitsham Farm in southern England was puzzled by one of his cows. It had lost a lot of weight, was waving its head from side to side and arching its back, drooling, and threatening the other cows.

Three days before Christmas, Stent received a visit from David Bee, a local veterinarian. He didn't know what to think, either. Perhaps it was mercury poisoning. Perhaps a kidney disease. The animal was a medical mystery. But soon Cow 133, as it was dubbed, became known as the first of thousands to exhibit mad-cow disease.

In September 1985, Carol Richardson, a pathologist at Britain's Central Veterinary Laboratory, was asked to study the brain of one of the mysteriously ill cattle that Bee had been seeing at Pitsham Farm. She noted the characteristics of a "spongiform encephalopathy," and sought a second opinion from a colleague who called it "bovine scrapie."

In April 1986, more cases were discovered at a farm in Kent. By November, the government's central veterinary lab officially recognized BSE as a disease, but the government's chief medical officer (responsible for human health) wasn't consulted by the Ministry of Agriculture, Fisheries and Food (MAFF) until much later.

The head of the central veterinary laboratory's pathology department, Ray Bradley, wrote an internal memo: "If the disease turned out to be bovine scrapie it would have severe repercussions to the export trade and possibly also for humans if, for example, it was discovered that humans with spongiform encephalopathies had close association with cattle. It is for these reasons that I have classified this document confidential. At present, I would recommend playing it low key."

On June 1, William Rees, the chief veterinary officer, wrote to Donald Thompson, the parliamentary secretary for MAFF: "Irresponsible or ill-informed publicity is likely to be unhelpful since it might lead to hysterical demands for immediate, draconian government measures and could lead to a rejection of British exports.

"It does not seem appropriate at this stage for MAFF to issue general information. The political implications are serious, particularly if not handled correctly."

By the end of October 1987, Britons remained largely unaware of the risks they faced. The number of reported cases of BSE had just begun to emerge - growing from 120 suspected cases and 29 confirmed cases at the end of October, to 370 suspects and 132 confirmed by the end of the year. Still, these were only reported cases of cows exhibiting symptoms. There were many more cows quietly incubating the disease.

Throughout the first half of 1988, officials dragged their feet. Then by the middle of June, they decided what to do. It had become clear how the disease was spreading. For decades, cattle had been fed the protein-rich remains of other cattle. It was a practice many consumers were unaware of, but which had become standard in Western nations, including Canada and the U.S.

Any part of the slaughtered animal not sold for human consumption was sent to a rendering plant, where it was boiled and mashed up. Part of the rendered product was turned into a dried powder called meat and bone meal (MBM), that could be added to animal feed. It improved the milk output of dairy cattle and added to their muscle mass. The danger, however, was that the pooled products could quickly spread disease.

In mid-June 1988, Britain banned the feeding of MBM made from ruminants (such as cows and sheep) to other ruminants. A five-week grace period gave farmers and feed companies until July 18 to use up much of their stock. The ban was a major safeguard, but it was not foolproof. Infected feed continued to be given to cattle, likely because of "cross-contamination" from feed prepared for pigs and poultry. (A ban on feeding MBM to non-ruminants wasn't implemented in Britain until 1996).

By 1988, BSE was escalating dramatically - infecting cattle at a rate of more than 10,000 a month.

In November 1989, the government ordered that brains and spinal cords of cows be kept from the food chain. Still, it maintained there was no risk to humans, insisting BSE was a form of scrapie.

Scientists now know BSE was, in fact, a new agent and one prevailing theory is that a single cow, perhaps in the 1960s or 1970s, experienced a genetic mutation of its proteins. The cow was slaughtered, sent to the rendering plant, and the disease quietly spread through generations of cattle.

In February 1990, the government acknowledged an experiment that showed BSE could be transmitted to mice that had been fed infected food. But the government insisted it was just an experiment, and there was no cause for alarm.

Then, in May, it was revealed a 5-year-old Siamese cat named Max had contracted BSE. This created a media firestorm with the government intent on dousing the flames.

Agriculture Minister John Gummer posed for a photo with his 4-year-old daughter, Cordelia, in which they were eating hamburgers.

By the mid-1990s, a strange new neurological disorder, similar to CJD, but somehow different, began hitting younger people. By the early spring of 1996, there were 10 cases and an advisory panel of scientists was now telling the government that there appeared to be a link between BSE and the new disease, "variant" CJD.

- - -

By early 1999, Zoe Jeffries, now in a wheelchair, was on the road to a proper diagnosis. At first, a CT-scan found nothing suspicious, but an MRI in the spring confirmed she had vCJD. Helen was given some pamphlets that explained the link to BSE. Helen and her children stopped eating beef.

Zoe came home from hospital and spent a year and a half slowly dying. She lost her ability to speak. Helen had to change her diapers. She responded to her mother's touch but she didn't really communicate. Helen still isn't sure, to this day, if Zoe went blind.

Zoe's bed was moved to the living room and the wall was plastered with posters of Leonardo DiCaprio. The musical soundtrack from Titanic was played again and again. She was fed through a tube inserted into her abdomen. From time to time, she stopped breathing.

- - -

Since the mad-cow scandal hit Britain, governments in Canada and the U.S. have insisted there is no cause for alarm here. The cattle and rendering industries offer the same assurances. Safeguards are in place to ensure vCJD does not gain a toe-hold on this continent, they say. But critics say those safeguards aren't enough. Among the points of disagreement:

- Contaminated feed: Canada says it hasn't imported meat and bone meal from Britain since 1982, and live cow imports from Britain stopped in 1989. But experts at the UN agencies note that much of the British by-products sent to Europe were repackaged and relabeled, and then sent elsewhere.

The UN agencies say this makes it hard to determine the place of origin of tons of products that were traded globally.

In 1997, Canada and the U.S. banned the feeding of domestically produced ruminant meat and bone meal back to ruminants. But both countries allow these products to be fed to pigs and poultry, which, once killed, can be rendered into protein and fed to cattle.

But the Canadian Food Inspection Agency (CFIA) says its annual inspections show Canada's rendering and feed industries are complying with rules. It says renderers are including the required statement "Do not feed to cattle, sheep, deer or other ruminants" on the labels of meat and bone-meal products, as well as invoices.

- Testing: Canada and the U.S. use "surveillance" systems to identify a cow with BSE. Any animal showing symptoms must be killed and the brain tested. Canada has 14.9 million cows. Between 1992 and 1999, 4,512 were tested for BSE. Only one has tested positive (a cow imported from Britain in 1987 and diagnosed in 1993. It was destroyed, along with the rest of its herd). On the basis of these results, the CFIA is confident Canada is BSE-free.

In Britain, Lacey says Canada is offering false reassurances, but he says he's not surprised. "They won't test because they don't want to find it. They won't know what to do. They would panic."

- - -

By October 2000, Zoe had been in medical limbo for more than a year. Her condition had not deteriorated much, nor had it improved. She slept much of the time.

Helen was approached by an intermediary for a vCJD family-support group. A newspaper and two TV networks - the BBC and ITN's Channel 4 - were aware that a 14-year-old had the disease.

Helen had always protected Zoe and no one in the community knew. But by now, Zoe would not understand why a camera crew was huddled into her small room. So Helen decided to go public.

One day in October, the camera crews arrived to interview Helen and record harrowing images of Zoe in her bed. A few weeks later, the nation was transfixed. It was the first time Britons had been shown what mad-cow disease does to a human.

- - -

Scientists and public-health officials caution that contaminated beef isn't all governments have to worry about to prevent an epidemic. They fear that BSE and vCJD possibly can be spread in other ways:

- Cosmetic creams and vaccines that used British beef ingredients during processing;

- Dietary supplements, largely unregulated in the U.S. and Canada, which could contain powdered cow brains and glands;

- Donated blood and organs from those unknowingly incubating vCJD;

- - -

Zoe Jeffries died on Saturday, Oct. 28, 2000 - just three days after her story was broadcast. The end came unexpectedly. Countless times before she had stopped breathing. There must have been a hundred during the final few months.

Whenever she had stopped breathing before, Zoe's face and hands had turned blue, as though she was fighting to stay alive.

This time, it was different. Her colour hadn't changed. She looked at peace.

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