The disease first surfaced 15 years ago in Britain. The bizarre illness swept through cattle herds, and then, researchers say, started killing people who ate infected meat. Europe imposed strict laws to stop the spread of the disease. But this winter mad cow disease appeared in Spain and Germany. Now it has been reported in 15 countries in Europe and the Middle East. There's been one case reported in Canada.
Mad cow has never been found in the United States. But this country is not taking all precautions. Experts warn that America is risking too much.
"She was bright full of life, intelligent going places, my best friend," says Annie McVey of her daughter Claire, who died at 15.
Claire was one of the youngest victims in Britain. She suffered the human form of mad cow, a brain disorder called variant Creutzfeld-Jakob Disease, or CJD. The first sign that her mother noticed was when Claire suddenly insisted on eating alone.
"And I found later that she was eating on her own because sometimes the food went in her mouth, and sometimes it didn't," McVey says. "Sometimes it fell off the fork, and she couldn't control her movements."
Claire knew well before her mother did that there was something seriously wrong. It was a secret she could not keep for long.
"She would scream, ah, the terrible screaming that went on for hours and hours and she had no idea why she was screaming," McVey says.
Her mother first recognized symptoms in Claire only five months before she died.
The decline was much the same for another young English woman. Just a year ago, Sarah Roberts told her parents Sheila and Frank Roberts that her legs hurt. Doctors said it was stress.
"She took herself to the hospital," Sheila Roberts says. "The doctor said to her you need to go home....And if you don't stop what you're doing, you'll end up in the psychiatric block."
But Sarah Roberts wasn't losing her mind; she was losing her brain. She began to stagger and then to forget how to do things she did every day - like turning on her computer.
Her mother had to show her how to take a shower.
With more tests, doctors finally diagnosed variant CJD. Sarah Roberts rapidly descended from forgetfulness to insanity.
"She used to scream. It was terrible to hear her scream," recalls Sheila Roberts. "She saw things. She saw animals, she saw monsters."
Sarah Roberts became blind and helpless within nine weeks of the doctor's diagnosis of variant CJD.
"When he told me, I was so shocked," Sheila Roberts says. "And he just said there's nowhere in the world you can go, and no one, no amount of money can pay for treatment....There is no cure. There isn't any treatment at all. We just have to wait."
Researchers believe that variant CJD enterd the food chain in the English countryside. The problem stems from the way cattle have been fed in Western Europe and in the United States for decades. When cattle are slaughtered, part of the remains go into cattle feed.
The leftover bones, brains and blood help cows grow larger, faster. But in Britain diseased cows were mixed in the feed, and mad cow disease spread rapidly. In the final stages, animals suffer tremors and can't walk.
At one point it was thought mad cow wouldn't infect humans. Initially Britain's agriculture minister told consumers, "British beef is safe." But most researchers now believe varient CJD is transmitted by eating infected beef. In the United Kingdom that mostly likely included anything ranging from steaks to baby food. Victims never know exactly how they get it, but in Britain, so far 86 people have suffered the staggering gait, failing coordination and madness.
"It's a bit like a nightmare movie really," says Dr. Philip Monk, a British epidemiologist who is leading a new investigation into the spread of mad cow.
The disease is currently untreatable. Scientists believe it's a totally new kind of infection, not a bacterium or a virus.
A protein called a prion is found in all of our brains. Prions are harmless molecules unless they get twisted out of their normal shape.
"You would either chuck chemicals at them that kill infectious agents or you heat them to high temperatures and that kills the infectious agents," Monk says. "The problem with prions is that none of this seems to kill them and stop them being infectious."
There's nothing you can do in the slaughterhouse or kitchen to prevent the transmission of the prion, Monk says. "The only thing you can do is to make sure that you don't get exposed to it."
Europe has tried to curb exposure with very tough animal feeding regulations. Fifteen European countries have banned the use of animal meat and blood in animal feed. Farm animals there are vegetarians again.
But in the United States, the federal government has ot imposed the same strict standards. And some experts worry the United States has too many holes in its mad cow defense. In 1997 the government outlawed most cattle remains in cattle feed. But cows are still an important ingredient in other animal feeds in this country.
That feed is supposed to be labeled "Do not feed to cows." But an investigation by the FDA found hundreds of feed makers in this country are violating the law.
|The FDA's Stephen Sundlof|
"We did a massive campaign to educate all the people who handle these products," says Dr. Stephen Sundlof, who is in charge of policing animal feed as the head of veterinary medicine at the FDA. "But some of them missed the message. And in most cases when we went out and inspected and asked them why they weren't in compliance, the answer we got most often was 'I wasn't aware of the rule.'"
"Our intent was to build a firewall," he says. "Remember that we're the second line of defense. The first line of defense is to keep this disease out of the United States," he says. "We certainly recognize that within our firewall, as we're building it, there are still holes in the firewall."
One of those potential holes is perfectly legal: The FDA still allows the use of cow blood in cattle feed, a practice banned in Europe.
New laboratory research suggest blood can infect sheep with mad cow disease, although there's no evidence in cattle so far.
"We're looking at blood in light of some new evidence that shows that blood may in fact be able to transmit the disease," Sundlof says.
It can take anywhere from 18 months to two years for a regulation to become final, according to Sundlof.
Federal mad cow strategy is based on the premise that there is no disease in the United States. The government believes American cattle have been safe ever since it banned the import of meat, feed and animals from countries with mad cow. But critics of that strategy say we may not have found mad cow because we're not looking hard enough.
"Germany and these other countries didn't detect their first cases until they started to do much larger screening," says Michael Hansen, who studies prion diseases at the Consumer Policy Institute, which publishes Consumer Reports magazine.
He says France tests more than 40,000 cows a week while in the United States only a tiny fraction of the national herd undergoes such scrutiny.
"We're testing about 2,000 brains out of 36 million," Hansen says. "The test we're using is an old test where you actually physically take the brain out...and ou look at it under a microscope." That test takes 10 days.
In Europe, the test is done in just four hours. Europeans test 25 percent of the cattle and are finding prion disease in cows that still look healthy.
That concerns Dr. Michael Schwochert, who for seven years was a U.S. Department of Agriculture vet assigned to watch for sick animals in slaughterhouses.
The Department of Agriculture declined to comment on testing. The USDA did send 60 Minutes II a letter saying it will "ensure timely detection and swift response in the unlikely event that an introduction of (mad cow) were to occur" and that the agency will "continually improve the surveillance system."
But for now the United States says it doesn't need to adopt Europe's feed and testing precautions. The FDA is evaluating the latest research.
After Great Britain's big mad cow outbreak, France, Germany and Italy all implemented regulations that they were sure would protect them. But all three now have mad cow disease.
"It does worry me," Sundlof says about the European failure to contain the disease. "There was slippage in the enforcement."
"I think there's more that we can do, and I think we're committed to do that," Sundlof says.
Monk's advice to the United States would be adopt the measures of others. "That is the lesson from Europe. We had to learn the hard way," Monk says. "America should be quick on the draw and get on with this because the likelihood is it will come to America."
In the United Kingdom mad cow disease seems to be on the decline in cattle herds, but the number of people diagnosed with variant CJD is growing. The symptoms may take years to develop.
Only a few months after McVey buried her daughter, the specter of CJD returned to her home.
"This is a twist of fate we hadn't expected at all. About six months after Claire died I myself experienced some change and sensation in my legs and I thought psychological," says McVey. "And then we were trying to cross the road one day and I couldn't move. My legs wouldn't move at all....So I put people on dementia watch."
McVey may have multiple sclerosis but she worries she has CJD.
She watches herself everyday a slurred word or a lost memory exactly much like her daugher did.
"I don't want anybody else to go through this," McVey says. "It's preventable. When a child dies, it's terrible. When they die of variant CJD and all the things they're going to go through, that's horrendous. But when it's preventable, there's not enough containment in the world to hold the rage I feel at times."
The National Cattlemen's Beef Association says the American government is doing all the right things and in its view American beef is the "safest in the world."