British 14-Year-Old Girl Dies From CJD / Meat link doubted

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British 14-Year-Old Girl Dies From CJD

RTR

Oct 28 2000 12:57PM

 

 

LONDON (Reuters) -

A fourteen-year-old girl, who appeared on British television earlier this

week to highlight the plight of people suffering from the human form of

" mad cow " disease, died Saturday, police said.

 

" Zoe Jeffries passed away today, " a spokesman for Wigan police, who were

handling media inquiries, told Reuters.

 

Zoe''s mother Helen allowed television cameras and reporters into her home

this week to tell the terrible tale of her daughter''s fight against new

variant Creutzfeldt-Jakob disease (vCJD).

 

" One morning she got up and just didn't do anything. It was as though she

went to bed one person and got up a completely different person, " she said

on Channel Four television.

 

Zoe was diagnosed with vCJD, which destroys the brain, when she was 12,

shortly after her father died of a heart attack. She was the 86th confirmed

victim of the disease in Britain.

 

Earlier Saturday scientists from the National CJD Surveillance Unit in

Edinburgh said that a 74-year-old man had died from vCJD last year. Until

now, most of the known victims of vCJD have been young people from 12 years

old upwards with only a handful of deaths among more mature people aged up

to 55.

 

DEATH TOLL ESTIMATES MAY NEED TO BE REASSESSED

 

Scientists may now have to reassess the likely death toll due to vCJD and

factor in the possibility of older people dying from the disease.

 

" We are trying to redo the analysis at the moment because we'd been

somewhat misguided by the considerable clustering of the cases in the

younger age groups, " a government adviser,

Professor Roy Anderson, told the Independent newspaper.

 

" This one case somewhat changes that view so we are in the process of

taking into account the rise of the numbers in the light of a considerably

broader age range, " he added.

 

His computer predictions had originally forecast that up to 6,000 people

had been infected between 1980 and 1996. Now that figure could rise as high

as 130,000.

 

Zoe''s death and the announcement of the 74-year-old''s infection came just

after an official report into mad cow disease, bovine spongiform

encephalopathy (BSE), was published Thursday.

 

The report, led by senior judge Lord Phillips, concluded the government had

misled the public for years about the dangers of British beef and the

chances of BSE being spread to humans. It also said bureaucracy had

hampered the response to the crisis.

 

Leading British scientists who first sounded the alarm about BSE said ''we

told you so'' Thursday after the report of the 2 1/2-year inquiry

vindicated the concerns they had voiced more than a decade ago.

 

The British government has said it is keen to set up a compensation scheme

for vCJD victims which could run into millions of pounds.

 

 

ISSUE 1983 Sunday 29 October

2000

 

After years of inquiry no-one knows how

many lives vCJD will claim

By Robert Matthews and Lorraine Fraser

 

Millions watched Zoe's final hours

 

AS epidemics go, it could have been worse: a sudden outbreak of media

coverage, a rash of unpleasant headlines, some feverish editorials.

 

Now, just three days after the publication of the Phillips report into

government's role in the BSE crisis, those who seemed likely to succumb to

its effects appear well on the road to recovery. Some will return to work

tomorrow after following spin doctor's orders and staying at home, and

avoiding stress - and all media contact.

 

Yet while the former ministers and civil servants named - and almost blamed -

last week have moved on, up or out, one group involved in the BSE debacle

has no such obvious escape route. It is that of the scientists, still

struggling to

understand what went wrong with Britain's cattle, and what might now be in

store for humans.

 

Despite the blanket coverage that it received last week, the Phillips report

was really just an exercise in bureaucratic archaeology. It was a study of the

BSE epidemic and the response of ministers up to March 1996, when the

Conservative government of the day finally conceded a possible link between

eating meat and contracting new-variant CJD, the human version of BSE.

 

Anyone scouring the report will look in vain for definitive answers to such

questions as how cattle came to acquire BSE, the origins of vCJD and the

likely human toll: no one knows. Indeed, anyone who reads the scientific parts

of the Phillips report is likely to be stunned by how little is known even now,

four years after the government announcement what so many had feared.

 

At the centre of the mystery of BSE and vCJD is a microscopic bundle of

chemicals known as prion protein. Found in the brains and nerve tissue of

animals ranging from mice to men, this protein plays an important role in the

nervous system - but what that role is has yet to be ascertained.

 

In cattle and humans affected by these diseases, healthy prion protein is

somehow altered into another, rogue form, with the deadly ability to corrupt

other healthy protein around it. The result is damage to the nervous system,

first subtle, then progressively more severe. Within as little as a few months,

the rogue protein has turned the brain into a spongy mass of dense-looking

" plaques " , surrounded by holes.

 

It is this characteristic appearance that first led scientists to link vCJD to

eating infected meat. Neurologists had identified several different forms of

CJD over the years - ranging from genetic cases passed down families to

cases that seem to strike out of the blue. But, under the microscope, none

looked exactly like this new variant of CJD.

 

After finding 10 patients with brain tissue showing the vCJD pattern,

scientists

at the National CJD Surveillance Unit in Edinburgh tried to find a common

link. Unable to find any obvious risk factors such as connections with

BSE-blighted farms, the team was forced to conclude that the most likely

explanation for the vCJD of these patients was exposure to infected meat

products.

 

The link was strengthened by a search of medical archives for brain tissue

showing the vCJD pattern. Not a single case has been found dating before

1994 - as would have been expected if human vCJD had followed the

emergence of BSE in cattle. Detailed comparisons of the prion protein in BSE

and that in vCJD have since confirmed that the two are, indeed, very closely

related.

 

Yet even now - four years after the government's announcement - there is still

no definitive proof that eating infected meat causes the human disease. If

anything, as more vCJD cases emerge, the picture has become cloudier.

Scientists remain baffled by the fact that most of the 85 cases of vCJD

identified so far have occurred in people under 45 years old, with about a

third of them under 25. The youngest victim was struck down at the age of

just 12.

 

Classic CJD, in contrast, is chiefly a disease of the old. Yesterday's

identification of a 74-year-old man with vCJD has led to suggestions that

many cases in old people had been misdiagnosed as other dementia.

However, scientists believe that the surveillance systems in place since 1990

are unlikely to have missed large numbers of cases among the elderly.

 

Equally perplexing is the emergence of a cluster of five vCJD cases around

the small Leicestershire village of Queniborough. While chance cannot be

ruled out, scientists are trying to find a common factor linking the disease to

the village. The growing suspicion is that there is more to the BSE-vCJD link

than just eating meat: that some other factor may be involved.

 

Genetic susceptibility is one possibility. Every case of vCJD so far has

occurred among people carrying two copies of part of a gene linked to an

amino acid called methionine. The suspicion is that such people are more

susceptible to vCJD. As 37 per cent of the population falls into the same

genetic class, that means that vast numbers will eventually die of vCJD, unless

some other risk factor is involved.

 

What that factor might be has prompted the most disturbing line of research

now under way: that the apparent link between vCJD and meat eating is a

coincidence, caused by an environmental factor that struck humans and cattle

at the same time.

 

One potential culprit is organophosphates (OPs), chemicals with potent

biochemical action that are widely used in Britain to treat warble fly

infection.

Since the 1980s, the role of OPs in the BSE-vCJD link has been doggedly

pursued by Mark Purdey, an organic farmer in Taunton. Long derided or

ignored by establishment scientists, Mr Purdey's research was finally taken

seriously by the Phillips inquiry, which conceded that exposure to OPs could

boost susceptibility to vCJD.

 

Mr Purdey has recently uncovered a link between CJD-like diseases and

levels of trace metals in the environment. This follows his discovery that

CJD-like diseases in animals are more prevalent in areas with low levels of

copper but high levels of manganese.

 

Again, the Phillips inquiry conceded that Mr Purdey's findings might be

significant, and pointed to new research by scientists at Cambridge University

into the mystery of what healthy prion protein actually does.

 

Dr David Brown and his colleagues at the university's department of

biochemistry have found evidence that healthy prion protein affects the use of

copper by the body. The protein also seems to play a role in protecting nerve

cells from so-called free radicals, extremely reactive fragments of molecules

that can damage DNA.

 

Dr Brown and his team have discovered that, to perform this protective role,

healthy prion protein needs to be bound to copper atoms. If, instead, it is

exposed to manganese, the prion protein changes - giving the protein some of

the characteristics of the rogue protein that causes BSE and vCJD.

 

These new findings all support the possibility that the link between vCJD and

eating infected meat is an illusion. What might have happened instead is that

changes in farming practice subtly altered trace element levels that - perhaps

when combined with OP use - led to both BSE and vCJD emerging at about

the same time.

 

While mainstream opinion insists that eating infected meat is the most likely

cause of vCJD, even the scientific establishment is now suggesting that it

might

not be enough by itself.

 

Professor John Collinge, of Imperial College, and one of the Government's

independent scientific advisers, has conceded that the bizarre pattern of vCJD

cases so far points to the existence of other risk factors. He has suggested

that mouth ulcers and infections of the tonsils or gastro-intestinal tract may

increase vulnerability.

 

As scientists struggle to fill the vast holes in their understanding of

vCJD, the

truth is unfolding month by month, as new cases are added to the toll. In

August, researchers at Oxford University produced the best estimate yet of

the figure that everyone now wants to know: how many people will die of

vCJD?

 

With so many unknowns - from the numbers of infected cattle that entered the

food chain to the incubation period of vCJD - the task would seem hopeless.

Yet the team believes that it has arrived at a fairly reliable overall

picture, after

checking five million permutations of the various possibilities. According to

their computer model, the worst-case scenario is that 136,000 people will

eventually succumb - a figure based on the assumption that vCJD has an

incubation period of at least 60 years.

 

While so long an incubation period cannot be ruled out, evidence from other

forms of CJD suggests that it could be too pessimistic. Kuru, a form of CJD

that appeared in New Guinea in the 1920s among tribesmen who ate the

brains of dead relatives, had an incubation period of about 15 years. A similar

figure emerged from studies of patients who died from a CJD-type disease

after being given human growth hormone.

 

Assuming that the incubation period of vCJD is between 20 and 30 years, the

Oxford University computer model estimates that the number of deaths from

vCJD will be a few thousand.

 

Even that figure could prove too pessimistic, if the growing suspicions about

the involvement of other risk factors for vCJD prove correct. What scientists

now need to establish is how important those other risks are. The disturbing

fact is that some of those scientists are starting to wonder if the link

between

eating meat and vCJD is an illusion, and that the whole BSE debacle was

based on a misconception.

 

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The Report

 

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