CBS 60 Minutes--Will Mad Cow Disease Ravage the USA

[Note: Claire McVey's mother now apparently has vCJD. Article about
Claire's death last year is attached.]

Reprinted from www.mad-cow.org <http://www.mad-cow.org>

21 Mar 01 - CJD - Mad Cow In The U.S.?
Scott Pelley

CBS News <http://cbsnews.com/> --Wednesday 21 March 2001
Sixty Minuites

Will Disease Strike American Cattle?
Some Poke Holes In U.S. Testing And Rules
Should Officials Adopt European Precautions?
(CBS) Mad Cow disease has spread throughout Europe. Could it come to the
United States?

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 entered 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.

At Your Table How have reports about Mad Cow disease changed your eating
habits? Share your views on 60 Minutes II's bulletin boards.

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 not 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.

CBS: 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 you 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."

From: "B. Elswood" <belswood@hotmail.com>
Date: Sat, 20 Jan 2001 18:14:48 -0700
To: "Blaine Elswood" <Blaine.Elswood@snow.edu>
Subject: *MUST* Reading! the death of Claire McVey

An unthinkable death

Claire McVey, 15, died within months of being diagnosed with the human form
of mad cow disease. Allison Pearson talks to her mother about her
unimaginable loss

IT was the shoes that did it. Annie McVey had known something was wrong with
her daughter. Claire was stroppy, she had stopped eating with the family and
was so clumsy it was like having a puppy in the house; knocking over coffee
cups, smashing phones. Things kept getting broken. For God's sake, they
said, be more careful, can't you? Her mood swings were so wild that no one
was too surprised when she stabbed the coffee table with a pen. 'I hate
you,' she said, with a venom that scared her mother. But Claire was 15 and
this was a bad case of fifteenitis, Annie thought grimly. Trust her feisty,
opinionated girl to have the Krakatoa of all adolescences.

A portrait of Claire taken in October last year: she died three months later
And then that morning Claire came down and announced that she wasn't going
to wear her new shoes to school; she thought she might wear trainers
instead. And Annie knew. The flutter of mother's instinct suddenly like a
flock of seagulls rising. There had been so many battles in shoe shops down
the years - Claire holding out for Himalayan wedges, Annie arguing for
something a little more down to earth. She knew her daughter wouldn't
surrender her footwear lightly. 'Why don't you want to wear your shoes?' 'I
just don't feel safe coming down the stairs, that's all.'

Annie McVey was a nursing manager with the Northern Devon Healthcare NHS
Trust, so she knew who to ring. Come down to the ward, they said, and we'll
squeeze you in. Because she was a nurse she knew right away. The mother
watched as the paediatrician began his neurological examination. 'Claire,
can you rub your heel against your shin?' She couldn't on one side. ('That's
deficit,' Annie thought.) 'Close your eyes,' he said, 'and touch your nose.'
She couldn't. ('That's abnormal.') Nor could she stand upright with her eyes
shut without wobbling. ('That's not right.') And then Claire refused to
co-operate: 'Cos I can't. Cos I can't.' Like a toddler stuck in mid-tantrum.

'Are we thinking brain tumour?' Annie asked when Claire was out of earshot,
and the doctor said, 'Yes, I think so.' Mother told daughter they would have
to drive to Bristol for a scan because the doctor thought she had something
on her brain or her back. 'Do you mean a brain tumour?' Claire asked, and
Annie said that was probably what they were looking for. Claire seemed
almost relieved. 'It was as if she thought, "Oh, there's a reason why I'm
feeling like this." '

They stopped at home on the way to ring Claire's stepfather and to break the
news to James, Claire's brother, and Wayne Lee, Annie's new boyfriend, and
to pick up a nightbag, just in case. Claire asked her mother if they would
have to operate and she agreed it was a possibility. They made the first of
their deals. 'If Claire had to have her head shaved, I'd have mine shaved as
well.' It was August 3, 1999. Two days later, a brain scan at Frenchay
Hospital revealed no tumour, but showed some subtle changes. 'I thought
there can be three reasons for those changes,' Annie recalls. 'It's
infection or metabolism or something genetic. Well, genetic was unlikely by
Claire's age, she had no other signs of infection, so it had to be something
metabolic.'

Claire had walked into Frenchay, but a fortnight later she left in a
wheelchair. It was as though being admitted to hospital had finally allowed
her to admit the truth to herself. Annie had always known her daughter was
intensely private and proud; now, she began to grasp the lengths she had
gone to to hide her deterioration. All those jagged shifts in behaviour made
sense. Only recently, a relative who phoned to ask if Claire could babysit
had hung up in disgust because he said she was drunk (her speech was already
slurring).

Annie, who had tentatively asked her daughter about anorexia and been
rebuffed with a stout, 'You must be joking', discovered that Claire had
actually been avoiding family meals because she was having trouble
swallowing. And Claire's solitary evening runs, it turned out, were an
attempt to build up strength in the legs which were so unaccountably failing
her. As for that spooked look she got in the eye of her tantrums: 'I thought
it was rage; now I could see it was terror.'

The night they got back from the hospital, Annie and Wayne, an ambulance
paramedic, trawled the internet looking for neurological disorders that
fitted Claire's symptoms. One kept coming up: new variant Creutzfeldt-Jakob
Disease (nvCJD). Believed to be caused by an abnormal form of brain protein,
nvCJD was increasingly seen by scientists as being the human form of bovine
spongiform encephalopathy (BSE). At least, Annie McVey thought, it's not
that. Annie's job at the Trust was risk adviser, for heaven's sake; if
anyone knew about medical odds it was her. Statistically, the chance of her
child contracting mad cow disease - well, it was practically science
fiction, wasn't it?

As the tests on Claire continued, specialists ruled out some of the nastiest
and grimiest spanners in the human works. 'I told her, whatever you've got
you are really rare, and she quite liked that,' Annie says. 'She'd always
felt different and special - well, she was different and special - but with
being rare comes all the stuff like, "we can't give you a treatment".' Not
long after, Annie dived into a hospital loo and opened the doctor's note she
had been given to deliver to another consultant. Inside was an incredulous
scrawled query: 'McVey: nvCJD!!???'

Mother and daughter made another deal: 'I told her I would always be
truthful and that I would never ever leave her.' And she never did. (Even at
the graveside in January, Annie remembers thinking she had to take Claire
home. You can forgive her confusion: it had taken her daughter less than
five months to die.) It was as though someone had put a tape of Claire
McVey's childhood in the video and held down the rewind button. The years
ripped past, from skiing holidays and blooming sophistication all the way
back to nappies and broken nights. 'When Claire was little, every day she
could do something more; with CJD, every day she could do something less.'

Only two months earlier, in May, just before Claire turned 15, she had stood
in the kitchen as her mother made dinner and said, 'When I'm 16, Mum, I'll
be able to have sex.' 'Well, you don't just become 16 and have sex,' snorted
Annie, and Claire laughed and laughed; she enjoyed teasing her mother. In
fact, she had pretty firm views about boys - you didn't want to be getting
yourself into that kind of thing too young. Claire was everyone's mate, but
there was still no boyfriend. Wayne reckoned the lads were intimidated by
the smart, funny girl: 'She would torture them. Couldn't help herself coming
out with these one-liners.'

And her beauty must have been daunting too. Recently, she had shot up to 5ft
10in and was on course for a supermodel six foot. Blonde with blue eyes,
milky skin and a generous mouth, Claire McVey was a rambling English rose.
So, when it came to the moment for facing facts - for saying what Claire's
real future would be, not thinking of all her possible ones - her mother dug
in her heels. 'The consultant and the ward sister took me into the office
and I said, "I am not grieving for her until she's dead." And they said,
"Don't you understand what we're saying, Mrs McVey? This is a progressive
neurological disorder." I understood perfectly. Yes, she was going to die.
Yes, it's going to happen in months not years. Yes, there was nothing they
could do about it. But I'm not grieving for her while she's alive.'

After that, the family got a new rule: no tears in front of Claire. If you
wanted to cry, you left the room.

Almost any other disease, and mother and child would have had a fighting
chance. But the enemy was no longer around to be engaged: it had planted its
bomb maybe a decade ago, lit the slow-burning fuse and quit the scene.

All that remained was to manage the surrender. Annie got hold of a voice box
so that Claire, whose speech was failing every day, could record some
favourite phrases for use later on. They included 'Bugger off' and 'I love
you'. By the end, that pretty much covered what any human being needs to say
to another.

Seventy-five people have died so far of variant CJD (the 'new' prefix no
longer applies). As epidemics go, it's been pretty quiet. After the doomy
headlines of the mid-Nineties, when three million cattle were burnt
<http://www.telegraph.co.uk/et?ac=000142527164265&amp;rtmo=lnAwzvlt&amp;atmo
=kkkkkkku&amp;pg=/et/96/6/18/nbse18.html> in a bovine Valhalla and humans
were said to be heading in their thousands for the same fate, the BSE crisis
has slowed to a trickle of scare stories about school food (could this have
been a key source of infection in the late Eighties?), the so-called
Queniborough cluster in Leicestershire and, most recently, the safety of
dental instruments.

Driving on a balmy summer's evening from Barnstaple to Kentisbury Ford to
meet Annie McVey, I try to imagine what I would feel if it were my child who
was one of the 75 dead - or only 75 dead, as the living tend to refer to
them. Around the Devon hamlet where the McVeys live, England is at her most
voluptuously come-hither. The hedges are high and creamy and in the fields
the cows swish, dreamy with contentment. On one five-bar gate, I spot a torn
Union Jack poster with a slogan: 'Support British Beef!'

The BSE crisis had the distinction - some would say the disastrous drawback
- of being bound up with a product that was not just made in Britain, but at
some gut level was felt to be the stuff of which Britain was made. By
extension, variant CJD - a beastly disease in every sense - became an
unpatriotic outrage almost before it registered as a source of human
suffering. To stand up against it, to proclaim your disbelief in it - and to
mock the joyless health inspectors who tried to monitor what went in our
fridges - was to fly the flag. To succumb to it, on the other hand, was a
source of secret shame. 'Best not mention it,' a mother on Merseyside, whose
son was among the first to die, was told.

Another family held out for their boy to be buried after the authorities
insisted on cremation - as if wanting to erase all trace of him, like a
Plague victim or, indeed, a mad cow. The family's priest had to intervene
when they were told that the police would come and impound the body. 'I'll
bring this man to the church,' the priest said, 'and keep him in the
church.' Eventually, a compromise was reached: the grave was lined with lime
and the ceremony monitored by two council officials in white coats. 'It's
just as if they punished him for something,' the boy's mother said. He had
died of a disease that wasn't meant to exist.

'I don't have any problem with the farmers, except when they go round saying
BSE has nothing to do with vCJD,' sighs Annie McVey. 'I know a lot have
killed themselves because of money worries and I don't want to get into a
debate about whether my daughter's life is worth more than a farmer's life -
that's just hideous.'

We are talking in the sitting-room of the McVeys' 200-year-old cottage. It
is a pretty room - cream sofas, coral cushions, stripped pine - in a pretty
house, but the place has a spectral, unlived-in air. It was only a few days
after they moved in that Annie drove Claire to the hospital and since then
womanly touches, you guess, have been reserved for a child's brow rather
than soft furnishings.

The hush of the countryside is broken occasionally by the arthritic creak of
a door as James, Annie's 19-year-old son, moves between the kitchen and the
television room. A tall, gentle hangdog figure, James, according to his
mother, has burrowed even further into himself since Claire died. 'The other
day, he told me, "When I'm out I tell myself she's at home and when I'm in I
tell myself she's out with her mates." ' James loathes the freakish circus
that surrounds a vCJD death - reporters at midnight, a public inquest - and
cringes at the letters from research groups around the world begging tissue
samples from mother and son. 'There'd be nothing left of us if we gave a bit
to everyone,' says Annie. 'This time last year, I felt I had everything; I
had two children I'd brought up who were not on drugs, not smoking, not
pregnant, doing well in their education, a new home, a loving partner and
then.'

Claire McVey was about as unfortunate as you can get in this life, but she
had one great piece of luck - her mother. Variant CJD is hard to identify in
the early stages and the majority of sufferers have been misdiagnosed and
treated for depression. Petrified youngsters have ended up in mental
hospitals or on wards for the senile. 'Nobody can believe Claire's case was
spotted so quickly,' says Wayne, who has just come in with a tray of coffee.
'That's because of Annie.' Wayne - a droll Devonian Tom Cruise - sets the
tray down on the table in front of us, which still bears the livid scar that
Claire made in it with a pen. 'She would become hugely aggressive,' says
Annie, 'but the eyes gave her away. They had this look that said, help me,
Mummy, I don't know what's going on here.' Annie didn't have time to be
terrified herself. 'I suppose it was scary knowing what the average mother
wouldn't know. But it also made it much easier, because I was able to warn
Claire prior to everything. I had to prepare myself so that I was prepared
to prepare her.'

By the time Claire had returned home at the end of August, her balance had
completely gone, leaving her with that cruel, drunken vCJD gait. To try and
get across the sitting-room, she would grab the oak beams that run the
length of the ceiling. 'She's even taller than me,' explains Annie, 'so she
can hold on to those.' (I notice Annie often starts talking about Claire in
the present tense; then comes an almost audible click as the brain updates
itself and she remembers to put her daughter in the past.)

Annie McVey cries like someone who has had a lot of practice. She weeps
fast, efficiently, palming the tears to the side of her cheeks with a single
downward sweep of the hands. When I say Claire sounds like an incredibly
proud person, she nods so fervently that her tears spatter like rain against
my face. 'Dignity was the thing with Claire all the way through. The first
time she crawled in here I thought my heart would break because I knew if
she was crawling in front of anyone she had to be desperate.'

The girl who had loved drama at school now put on a show at home. When her
friends came to visit, Claire would be carried upstairs and arranged to
receive them. There was always a pile of sandwiches, but Claire's dribbling
had got too bad to eat in public. Mother and daughter had codewords for
these occasions. 'There was one to tell me she needed to go to the loo and I
had to discreetly get the girls out so I could carry her to the commode
hidden next door.' Another signal let Annie know Claire was tired. 'I would
come the heavy-handed parent and say, "Time you all went!" And Claire would
protest, "Oh, Mum, not yet." She really ran things just so. I think she
didn't want to frighten them.' And, perhaps, by being the Claire they knew
for just a little longer, not frighten herself.

Mother and daughter - now nurse and patient - talked a lot about pain, at
least the kind you could dose from a bottle. When Claire confessed she was
worried what would happen when she couldn't swallow any longer, Annie gave
her the options: a tube into the stomach or one up the nose. ' "Oh, I
couldn't bear one up my nose," she said, so at least I knew what she
wanted.'

They often say of men that they had a good war; well, Annie McVey had a
stirring defeat. She managed her daughter's dying with a combination of high
professional skill and low cunning. When they told her she couldn't have an
electric bed, she asked for one on trial, knowing full well that Claire
would be dead before it was time to hand it back.

The disease was so rapid that officialdom panted to keep up. Overnight,
Annie McVey had gone from healthcare provider to consumer and was dismayed
by what she found. 'Certain individuals were wonderful, but there was a
total lack of initiative from departments, they couldn't extend beyond known
procedures. I'd ask for a suction machine and they'd say, she doesn't need
one yet and I'd say, well, if she starts choking in the middle of the night
she will.' Annie is defiant about the bruised toes she left in her wake.
What concerns her is the other vCJD families who have no chance of working,
let alone beating, the system. I ask Annie how she views the handling of the
BSE crisis: 'Economics first, politics second, humanity third,' she snaps.

Isn't that the way of the world?

'You know, Allison, I'm not some Mad Cow Mum. I may be full of rage some
days, but I absolutely understand the desire to preserve the status quo. But
they made a bad mistake. They saw that BSE could jump the species barrier
and they decided to take the risk that it wouldn't jump into humans. People
complain about the Nanny State telling you not to smoke, not to drink, but
what about the Nanny State that takes a decision not to give the children a
piece of information about risk because they're not to be trusted with it?
They'll only go and panic. Personally, as a parent I reserve the right to
panic if there's something in the food chain that can kill my child.'

I tell Annie that a civil servant friend of mine maintains that the business
of government is heartlessness and she makes a stung sound, like a wasp
hitting a window.

If they'd said there was a tiny risk, they would have collapsed the beef
industry, wouldn't they?

'It collapsed anyway and look at the cost.'

>From a table by her side, Annie produces an album of portraits taken last
October when she and Claire went up to London, paid for by the people of
Ilfracombe. To me, the pair look like an advert for radiance, but Annie
knows better: 'She can't sit up straight there, I'm helping her balance. And
this is three months after we know she's ill. Her eye is a bit droopy
already and that smile's not natural. She's having to make that smile
because her face isn't working.' I say Claire looks dangerously minxy and
Annie laughs, the first time I have heard that sound. 'I said to her, "I
want to know where you learnt to give that naughty over-the-shoulder look,
my girl!" '

It is the older woman in the pictures that I find it hard to stop staring at
- a fine-boned Cherie Lunghi type with glossy hair and hazel eyes. Eight
months on, the woman beside me is the ghost of her former self. The huge
eyes are buried in mauve sockets, the frame skeletal - she looks like one of
those Sienese Madonnas, gaunt with the burden of love.

You look lovely in those pictures, Annie.

'A vaguely healthy human there,' she laughs again, but without pleasure this
time.

What was Claire like when she was little?

'Oh, big reader, total chatterbox, show-off. Opinionated, independent to the
nth degree.' Annie moves her head as though trying to dislodge an idea that
buzzes in there. 'I look back at photographs of her fifth birthday and I
think, did she have it in her brain then? When she's smiling at the camera,
did she have something that would kill her in 10 years' time? They think it
was probably controlled by 1989/90, so up to the age of six she could have
caught it. And I look at the pictures of her at parties back then and I
think, did she eat a sausage roll? Is it then? Is it then?'

Claire Louise McVey was born on May 29, 1984, in Liverpool. When she was
three months old, her mother became a single parent after the McVeys'
marriage broke down and Claire's father walked out. Annie McVey herself came
from the kind of home where it paid to read the signs, and she has never
lost that habit of looking out for trouble and trying to head it off. She
prepared almost fanatically for her own children's upbringing, reading all
the books and even buying New Scientist. James and Claire both had natural
pureed fruit and vegetables, no salt or sugar. Sweets only at the weekends.
'Neither of them has fillings.' Click. 'Had fillings.'

Claire informed her mother that when she grew up she would get married and
have a big house with a little house for her mummy down the bottom of the
garden and a husband who was going to stay home and look after the children
who, by the way, would be twin girls. 'Your husband might not want to stay
home and look after the children,' cautioned her mother. 'I wouldn't have
married him if he wasn't willing.' Not surprisingly, this natural
rationalist intended to become a lawyer - in America, because she reckoned
they got to argue more in that system and she was game for a fight was
Claire.

When we are first born, our mother is our whole world; as vCJD claimed
Claire McVey, her world shrank until, once again, Annie's was the only face
in it. Over the weeks, her tastes grew more childlike. The teenager began
watching the video of Anastasia - bloody Anastasia, Annie calls it - over
and over. When Annie and Claire travelled to Disneyland in Florida, paid for
by local wellwishers, the 15-year-old would suddenly get agitated and her
mother would take her into a toy shop where she could hold the fluffy
animals, and that would soothe her. One morning, Claire woke and said,
'Mummy, if I'm in a coma and they come to you and say there's a cure but
it's really painful, no matter what it is and if they ask your permission I
want you to say yes. Because I want to live.' Annie pauses. 'And she did.
She desperately wanted to live. I'm sorry.'

In the final stages of vCJD, Claire McVey became profoundly disinhibited - a
polite word for rude. On Christmas Day, her skin was so sensitive she kept
trying to take her clothes off. Annie undressed her, laid her on the sofa
where we are sitting, and covered her with a sheet.

'I said, "Right, I'll bring your dinner in here," and she said, "I don't
want any ffffff-fucking dinner." And as that f-word was coming out, I was so
shocked because Claire never ever swore. She'd tell off James if he said
"crap". I said, "For goodness sake, just say you don't want it." But in a
way I was glad, it was a moment of normality; for a second she was just a
stroppy teenager again.'

On the morning of January 10 this year, Claire woke her mother, who was
sleeping in the bed next to her, by patting at her face and forming the
sounds for yogurt. By this stage Claire had been moved to Little Bridge
House hospice, near Barnstaple. 'We were lucky,' says Annie. 'I've talked to
some of the other families and their children stopped being able to
recognise them. That must be hard to bear. She knew me until the end.' The
next day Claire died. 'I knew she was dying in the afternoon, and I just sat
with her and sang and rubbed her hair and she knew I was there and that I
loved her.'

Claire McVey was buried in the prom dress that she and her mother had picked
out in Florida, the one she wore to London for the photographs. At her
memorial service, her teacher said, 'You always heard Claire before you saw
her - there was a giggle or a squeal.'

The regulations for dealing with a vCJD body are almost as brutal and
incomprehensible as the disease itself. Annie McVey starts trembling when
she explains that undertakers are told parents can have superficial contact,
but instructions for the biopsy and post-mortem say that the dead must be
sealed. Annie was told that Claire had to be zipped inside a double body
bag, but then the hospice came up with the idea of making her a sealed glass
coffin. 'Up to the moment of death, I was doing the most intimate procedures
with her. She spat in my face two or three times, she weed on my hand, she
dribbled when she went to kiss me. And suddenly she's dead and I mustn't
touch her.'

Annie has asked questions about this through her MP; she is also agitated
about the state of surgical instruments which, she says, cannot be said to
be sterile if there is a chance they have come into contact with someone
incubating vCJD. These are not trivial concerns, but when Claire was alive
Annie hid from her pain in practicalities, and now she is gone her mind -
anguished, restless - is still seeking a diversion.

In his epic poem of loss, In Memoriam, Tennyson wrote, 'For tho' my lips may
breathe adieu, I cannot think the thing farewell.'

That paralysingly sad evening in Kentisbury Ford, I realised that the woman
talking to me perfectly rationally had not begun to think the thing
farewell; neither was she wholly among the living.

'It's the realising I can't touch her and I can feel her hand and I want it.
The urge to have her physically present is so devastating. The last couple
of months it's got worse because it's hitting in. It's for ever. This will
sound mad, but I think at 15 she's too young to be wherever she is without
me.'

The night has drawn in, leaving us talking in a silvery gloom. Annie invites
me to stay for supper and disappears upstairs. When she doesn't join us,
Wayne explains that since Claire's death, Annie has stopped eating.

What, no food at all?

'I make her Horlicks every night and I put 17 spoonfuls in the mug, till the
spoon stands up in it, and that's all she'll take.'

When Annie finally joins us, I ask, Why won't you eat?

'I don't know. Penance maybe.'

Penance is for when you've done something wrong.

She shrugs. 'It's not rational, is it? But my child predeceased me. When she
was growing up, I wanted her to have all the experiences I'd never had - not
spoiling, but letting her see and learn things. And now she's had the
biggest experience of all before me. I feel incredibly proud of the way she
did it. I really do. She died with such dignity. But I'm meant to show her
how to do it. Not her show me.'

Did Claire McVey die because she ate a sausage roll at a birthday party in
1989, when she was five years old? The people most likely to attempt an
answer are to be found in a single-storey brick building round the back of
the Western General Hospital in Edinburgh. There is something very British -
a sort of Ealing Tragedy - about the CJD Surveillance Unit, a crummy warren
of rooms full of brainy souls who, in another era, would have been cracking
Enigma in a convivial hut.

'Forgive the mess,' says Bob Will, the head of the unit, steering me past a
prehistoric filing cabinet into his office. Bob - the amiable fiftysomething
neurologist Dr Robert Will - has been here since the Unit was set up in May
1990, coincidentally the same month that John Gummer, then Minister of
Agriculture, fed a beefburger to his four-year-old daughter at a fete to
reassure the public that British beef was safe to eat. 'Did he really do
that then?' marvels Dr Will.

There are still some scientists who, while acknowledging the evidence points
strongly to a link between BSE and CJD, maintain this link has not been
proved.

How certain is Bob Will of the link between the disease in cows and humans?

'I am very confident the BSE agent is the cause of variant CJD and most
scientists agree. Variant CJD occurs almost solely in the UK, and what
happened here that didn't elsewhere? BSE. They have done transmission
studies in mice - where they inoculate the brain tissue - and the protein
deposited in the brain in vCJD looks remarkably similar to that deposited in
BSE.'

In layman's language, the unit is no longer pursuing any other suspects in
connection with its inquiries: they know who the killer is, all they need to
discover is the method of breaking and entering.

Dr Will is sure the disease got into humans through infected meat in the
Eighties. There have been other theories - contact-lens fluid (not one of
the victims turned out to have worn lenses), childhood vaccinations (the
dates don't add up and, anyway, our vaccines were widely exported to the EU,
so why aren't there more cases over there?) - but diet is the only common
factor among the vCJD patients and may even account for why the dead are
getting younger and younger. Of the total 83 known cases of vCJD (the
remaining eight are either still alive or are awaiting post-mortem results),
57 have been under 30. Was it easier for the infection to cross into
children?

'That's clearly a critical question,' acknowledges Dr Will. 'It may be that
age influences how the gut absorbs protein. My belief is that the most risky
food was brain or spinal-cord tissue from cattle because they carry huge
amounts of infectivity compared with any other tissue.' The main culprit, he
says, was MRM or mechanically recovered meat, a hellish paste of bones and
other garbage that goes into the optimistically named meat products. 'Is it
possible that the age distribution of variant CJD in some way correlates to
the usage of mechanically recovered meat in certain foodstuffs consumed by
the young?' Bob Will chooses his long and exquisitely unexcitable words with
care. He caused a fuss in the papers recently for mentioning school dinners
and baby food as possible sources of infection
<http://www.telegraph.co.uk/et?ac=000142527164265&amp;rtmo=lnAwzvlt&amp;atmo
=kkkkkkku&amp;pg=/et/00/7/17/ncjd17.html> , although for anyone up to speed
with the subject, that was common sense rather than scaremongering.

What about Claire McVey at that hypothetical birthday party, eating that
hypothetical sausage roll, I ask. Why her and not the other children?

'Let's say that there was one batch of sausages made from MRM. I think it
may be wrong to assume that the infection was distributed evenly. It may be
that one item of food was the critical item that had enough to initiate
infection.'

So, just incredibly bad luck, then?

Bob Will gives an almost Gallic shrug of helplessness: 'The other much more
difficult possibility is that there may be variation in susceptibility in
people for genetic reasons. This is of great importance to the families
because other siblings could be regarded as being at risk. It's certainly
reassuring that at the moment we have not yet had any families with more
than one member infected.'

Have we got off lightly?

'We just don't know. All we can say is there was a risk taken and that we
still can't tell how many people are incubating the disease.'

The unit has mathematical models which show anything from a limited epidemic
of, say, 100 or so cases to 100,000 or more. Experts have recently suggested
that the upper bounds of such an epidemic look less likely to be very high.
'Personally, I think it's actually a very anxious period,' says Dr Will.
'For the first time, we believe the cases are occurring with a higher
incidence. The previous highest total of deaths per annum was 18 in 1998. In
the first six months of this year, we have already had 14 deaths.'

I think of one among that 14: Claire McVey, now enrolled into the mute,
biddable ranks of statistics. Bob Will seems to read my mind. 'I've seen
people in the papers writing only 75 cases and it's incredibly distressing
for the families. Their suffering - well, you've had a glimpse of it - it's
almost unimaginable. This is a terrible disease. You don't want anyone to
get it.'

Dr Will never stopped eating beef. Along with many colleagues, he took the
view that if brain and spinal-cord tissue was removed from the food chain as
per the guidelines laid down by the Southwood Report in February 1989, then
there would be little risk to anyone after that. 'Families were possibly
exposed in '84 or '85, before BSE was isolated; well, maybe there was
nothing to be done about that. But the minute it was isolated and safety
measures introduced, public health should have been guaranteed. Now we know
that those measures were not fully implemented or enforced, which is a
national tragedy, and I hope that tragedy will be addressed by the Phillips
Inquiry [set up in 1997 to look into the previous government's response to
BSE and vCJD].'

Dr Will offers me a tour of the laboratories. As we are getting up to go, I
broach something that has been bothering me. Does he think the victims will
get any younger?

'Well, we now have a 12-year-old.'

A 12-year-old?

'That's what I said.' He looks almost ashamed.

Girl or boy?

'I can't say.'

But if the incubation period is at least 10 years, then the child was barely
eating solid food when it contracted the infection. 'I'm not saying
anything,' the neurologist says wearily. 'You've got small children of your
own, Allison. You do the maths.'

Along the corridor, I am introduced to James Ironside, the erect, bone-dry
Scot who first identified variant CJD when he was looking down a microscope
in 1996. The name is familiar, anyway. Dr Ironside is the pathologist who
examined tonsil and brain samples from Claire McVey and whose written
evidence, submitted to her inquest in Barnstaple, enabled the coroner to
record a verdict of death by misadventure. (Earlier vCJD victims were deemed
to have died of natural causes, to the disgust of relatives.) 'Now, this is
grey matter,' says Dr Ironside, inviting me to join him at a microscope.
'You see those flowery shapes? We call them florid plaques - that is an
aggregate of prion protein which is a hallmark of variant CJD.'

The cross-section of brain looks like a lunar landscape, with the florid
plaques forming the craters. Is that vCJD itself? 'No, I think of those as
the fingerprints it leaves. We don't know why it forms them.'

Suffused by a pink dye, the section of brain looks enchanting, as if the
moon were made of marshmallow. 'That's memory and personality, basically,'
says Dr Ironside. The prion protein got into that person's spleen, probably
incubated there for 10 years, then entered the central nervous system and
travelled to the brain where it started to throw its weight around like a
chimp at an embroidery circle. The pretty eruptions I have been admiring are
the nerve cell endings - they have exploded, taking memory and personality
with them.

Does Dr Ironside ever think about the patients? 'Never.' Why not? 'I have
teenage children of my own. They ate beefburgers. I can't think about it, it
would be too' - he searches for the technical term - 'distressing.'

The Phillips Inquiry is due to hand its report to ministers at the end of
this month, but as yet no date has been set for publication. (The families'
case for compensation will start four months later.) The inquiry has been
remarkably leak-proof, although some of the evidence Lord Phillips has heard
was described to me as, 'like Yes, Minister without the laughs'. The same
Whitehall source says, 'If you were being darkly cynical, you could say the
wait-and-see policy over BSE was a qualified success. The dead still under
three figures - a bit of a narrow squeak, admittedly, but hardly the
calamity it might have been.'

I call Annie McVey in Devon and ask her about her hopes for the report. 'I'm
worried it will just be an opportunity for this lot in government to score
points off the last lot. That's not what we want. What we need is an
acknowledgment of mistakes and a debate about what duty of care - what
honesty - is owed to us all by the state.' If the business of government is
heartlessness, then the business of mothering - as Annie has shown - is its
exact opposite.

She says she has bad days and worse days, 'days when I get up with my horns
and tail on'. And she writes furious letters - to Jack Straw, to the papers
- but she is training herself to tear them up. She doesn't want to be a Mad
Cow Mother, only because she thinks she can best serve her child's memory by
being as cool and cogent as the lawyer daughter she will never know.

These days, Annie rations her visits to Claire's room, keeping the door
tightly closed: 'I'm so afraid of losing her smell.' But she did go in the
other morning and at the bottom of the wardrobe she found the shoes. 'They
were so new, hardly shaped to Claire's feet at all. She didn't wear them
again after that morning when she told me she felt afraid coming down the
stairs. They're ridiculously high and I thought, how on earth are you going
to walk in those, my girl?'