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From: TSS (
Date: November 16, 2004 at 10:19 am PST

-------- Original Message --------
Subject: BSE in America by John Collinge (clinical director of the specialist Prion Clinic at St Mary's Hospital)
Date: Tue, 16 Nov 2004 12:13:06 -0600
From: "Terry S. Singeltary Sr."
Reply-To: Bovine Spongiform Encephalopathy

##################### Bovine Spongiform Encephalopathy #####################

BSE in America

This article, written by Prof John
Collinge QBE, appeared in The Times
newspaper 3 January 2004.

Watching Ann Veneman, US
Agriculture Secretary, announce the
first case or BSE in the United
States at Christmas was profoundly
disappointing, not simply because
another country was blighted by
this degenerative brain disease, but
the presentation was all too
familiar. That agriculture rather
than health secretary sought to
reassure about risk to human
health, and that she said she would
be feeding her own children beef,
was as reminiscent of the early
stages of the outbreak in Britain as
it is scientifically inane. There are
many lessons the Americans could
learn from the British experience,
and the following months will
prove crucial.

The first, and most important, is
that infection with the human form
of so-called 'mad cow disease' may
be more widespread than
commonly assumed. To date
around 140 people have died of
vCJD (the human form of BSE).
While each case is a tragedy, this
relatively small number in public
health terms has been interpreted
to mean the human cost will be
small. The largest number of
reported cases was in 2000, leading
to assumptions that vCJD has
peaked and will now decline with a
death toll of a couple of hundred.
While everyone hopes that this
proves correct, I must point out
some uncomfortable facts that
challenge such optimism.

We are used to infectious disease
epidemics, such as influenza or foot
and mouth disease, that arise and
decline over weeks and months.
However, we know from the
disease kuru, transmitted amongst
the Fore in Papua New Guinea
during cannibalistic feasts with
devastating effects, that human
prion disease epidemics can span
decades with incubation periods
that can exceed forty years, with an
average of around 12 years.

We also need to think about the
effect of the so-called 'species
barrier'. When prions from one
species infect another species, the
incubation periods seen are
typically very much longer. Average
incubation periods of BSE prions
in humans of 30 years or more
would not be surprising based on
these sorts of comparisons.

By definition, those who have
already got vCJD are those with the
shortest incubation periods. These
people do not seem to have been
exposed to more BSE in their lives,
suggesting they may be particularly
vulnerable as a result of their genes,
or the presence of unknown
environmental factors. We know
that our genes have a major bearing
on incubation periods, but until we
understand more about this, we
cannot know if the current vCJD
patients represent a first 'wave',
with other peaks, perhaps much
larger, to follow.

In short, while it would be
marvellous if it were true, the
science suggests much caution in
concluding the human BSE
problem is receding at what is, in
the timescale of human prion
diseases, this early stage.

So has America taken this
problem seriously enough?
Secretary Veneman's later statement
announced some sound measures
to protect US consumers - notably
bans on high risk cattle tissues in
human food, on air-injection
stunning of cattle and the use of
mechanically recovered meat.

However, some of her comments
resonated in the British ear: I will
stress again that our food supply
and the public health remain safe'
and 'Our goal is to see trade
resume as quickly as possible.' But
one piece of scientific advice given
repeatedly to the British
Government with respect to the
risks of BSE is that 'absence of
evidence is not evidence of
absence' and no doubt our US
scientific colleagues will advise

What would reassure is proper
surveillance of animals, using
diagnostic tests at abattoirs and of
fallen stock. It will be interesting to
see if Veneman's scientific advisors
agree that testing 20,000 cattle per
year in the US (out of a total of
more than 35 million slaughtered
annually) is the 'very aggressive
surveillance programme' that she
described. If the US BSE case is
due to contaminated feed, it is hard
to imagine that there will not be
others in other herds.

While agriculture ministers the
world over will quite properly
defend their important beef
industries, it is to be hoped they
learn the UK lesson. Openness
about the uncertainties from the
beginning is in the end less
damaging to commerce than denial
and misleading statements on risk
that cannot be scientifically
substantiated and that, if proved
wrong, severely damage public
confidence in Government (and
science). In these post-9/11 times,
restoring and maintaining public
confidence in Government's
management of risk on both sides
of the Atlantic should be

Editor's note Professor John
Collinge is the clinical director of the
specialist Prion Clinic at St Mary's
Hospital which was set up to provide
a clinical service for patients with or
suspected to have any form of prion
disease. We were very pleased, to
learn that John had been awarded
the OBE in the New Year's
Honour's list. We would like to send
John our warmest congratulations
for this. well deserved recognition of
his personal efforts and importance
working for families affected by all
strains of CJD. (AMEN...TSS)


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