ITS A MAD, MAD WORLD:
BOVINE SPONGIFORM ENCEPHALOPATHY
Prepared for
Dr. Bruce R. Magee
Acting Assistant Professor
English Deptartment
Louisiana Tech University
Ruston, Louisiana
By
April E. Storey
English 303 Student
May 1, 1996
7095 Sentell Rd
Shreveport, LA 71107
April 29, 1996
Kendra Pratt
Animal and Plant Health Inspection Services
U.S. Department of Agriculture
Dear Ms. Pratt:
Here is my report on Bovine Spongiform Encephalopathy and its possible
link to the human brain disorder Creutzfeldt-Jakob Disease. In preparing
my report, I have learned a great deal about this disease and its possible
epidemic effects on the U.K. and the U.S. I believe my report will be of
help to anyone of interest including the U.S. Department of Agriculture.
Since this disease has become such a hot media topic, I had no trouble
finding sources. Everyone had a great deal of information to give, but
definite answers were hard to find. I found that a majority of the information
on this disease is speculation, which justifies my conclusion that further
research must be done. My research was done by the Internet, libraries,
and medical specialists in this field.
In doing this research, I found that the government plays an important
role in the eradication of this disease. In the United Kingdom, the government
overlooked this disease in years past, and now is having to make amends
for its mistakes. One of my recommendations for the U.S. government is
to keep the public informed about the disease and to not overlook this
disease. Do not make the same mistake the U.K government did.
I would be happy to answer any questions you might have about this report.
Sincerely,
April E. Storey
ABSTRACT
Bovine Spongiform Encephalopathy is a fatal brain disease in cattle. Recent
findings show that there is a definite possibility of BSE contaminated
meat being a link to Creutzfeldt- Jakob disease, a fatal human brain disease.
Since there are no definite anzwers, only speculation, there is a must
for extensive research on this disease. This research needs government
funding from not only the United Kingdom but also the United States.
Bovine Spongiform Encephalopathy, also known as mad cow disease, has
become an epidemic in recent y.ars in the U.K. The cattle get thise disease
from eating ground up sheep parts (known as offal) that are infected with
the sheep form of BSE called Scrapie. If cattle can get this disease from
eating sheep, it is most likely that humans can get this disease from eating
beef. Until the early 1990's, the parts that could cause a CJD infection
in humans, such as the brain, nervous tissue, lymphoid tissue and the spinal
cord, were allowed into the human food chain. This means that a substantial
portion of the British population could be infected with CJD and not know
it, especially since the incubation period of the disease in humans is
up to thirty years. This news has been well publicized in the media, causing
a panic among British citizens. Although BSE is not known in the United
States, the possibility of an outbreak is always there. This possibility
is the reason that the U. S. government must take action so that BSE and
CJD do not became the problem it became in the United Kingdom.
TABLE OF CONTENTS
FIGURES AND GRAPHS
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Mad Cow cartoon
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Sheep brain with Scrapie. Magnification x250
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Sheep brain with Scrapie. Magnification x500
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Human brain with CJD graph
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Meat Consumption Graph
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Minister of Agriculture with daughter
INTRODUCTION
Purpose
The purpose of this paper is to clarify the necessity for the U.S government
to take immediate action to eradicate the possibility of BSE not only entering
the country but also making sure it is not all ready present. The United
States government needs to do the following to keep BSE out of the United
States: keep the ban on British cattle importation, pass a law forcing
farmers to comply with the ban on sheep and cow offal, and research possible
vaccines.
History
Bovine Spongiform Encephalopathy, or BSE, is a fatal brain disease affecting
cattle in the United Kingdom. The disease was first discovered in 1986
in Britain. BSE causes actual holes in the brain and throughout the entire
nervous system. BSE is not a bacteria or a virus; it is thought to be an
infectious protein known as a prion.
Review of Recent Literature
BSE, dubbed "Mad Cow Disease" by British newspapers, has become one of
the hottest media topics in not only Britain but in the U.S. as well. From
local papers to national and international magazines to television programs,
BSE has been well publicized (see Fig. 1). Texas A&M University posted
a special "Fact sheet on Mad Cow Disease" on their home page, which included
a question and answer setup for concerned and interested students. Even
Oprah Winfrey had a special show which included views from opposing sides
on BSE.
Figure 1. Mad cow cartoon. (Minister).
Research Procedure
Since the topic of "Mad Cow Disease" is so recent, my research was done
mostly by way of the Internet. I also consulted several experts in their
fields on their opinions of the recent scare of BSE. Newspapers, magazines,
and television shows were also part of my learning experience on BSE.
IT'S A MAD, MAD WORLD: BOVINE SPONGIFORM ENCEPHALOPATHY
History of Disease
"BSE (see glossary) is a chronic, degenerative
disease affecting the nervous system of cattle" (Pratt).
The disease causes loss of muscular coordination, nervousness, and severe
muscular twitching (Fact Sheet). Infected cattle tend
to stand away from the herd and lose weight, although no loss of appetite
occurs. The disease gets its name "spongiform" because it literally bores
microscopic holes in the brain, making the brain appear spongy (see Fig.
2&3). Once clinical signs appear, the disease is always fatal. Although
a fairly recent disease, over 150,000 cows have been diagnosed.
Figure 2. Sheep brain with scrapie. Magnification 250x (Typical).
Figure 3. Sheep brain with scrapie. Magnification 500x (
Typical).
BSE was first discovered in 1986 in Great Britain. By 1990, BSE had
claimed the lives of just under 20,000 cows (Greger).
"The epidemic peaked in January 1993 at almost 1,000 new cases per week"
(Pratt). Today the number of new cases of mad cow
disease is around 300 cows per week.
History of Prions
"Scientists think that cattle may get the disease from eating protein in
feed that was probably contaminated with a spongiform disease agent. Scientific
evidence suggests that BSE is not a viral or bacterial infection. Rather,
it seems to point to a protein material or 'prion' as the cause of the
disease" (Fact Sheet). Normal protein material is converted
into dangerous ones (prions) by simply changing shape. "Prions are indeed
responsible for transmissible and inherited disorders of protein conformation"
(Prusiner).
Prions are extremely resistant to heat meaning the average cooking heat
will have no effect on them. They are also resistant to normal sterilization
processes. "Without detectable DNA nor RNA, not only does no one know how
they replicate, but the whole concept challenges the basic tenets of biology.
Prions can survive for years in the soil. Even domestic bleach and formaldehyde
have little or no effect" (Greger).
Transmission from Sheep to Cow
In sheep there is a naturally occurring prion disease called Scrapie. When
a sheep shows clinical signs of Scrapie the sheep is slaughtered to prevent
further infection of the herd. This sheep is then usually made into a "protein
concentrate (euphemism for mashed-up bits of other animals) left over from
the slaughterhouse" (Greger). This protein concentrate,
sometimes referred to as offal, is then fed to cows, who are naturally
vegetarians, to increase cow size and milk production. The Scrapie infected
sheep parts then infect the cow, making the cattle from of Scrapie - BSE.
In July 1988, Great Britain banned the feeding of Scrapie infected
sheep parts to ruminants. Britain also made BSE a notifiable disease and
all animals showing signs of BSE were to be destroyed. Farmers that reported
the disease were compensated fifty percent of the value of the cow by the
greedy government. This low compensation would obviously cause a low report
rate because farmers could just overlook a staggering cow and get full
market value for its meat. "Cases of BSE are becoming severely under-reported.
For instance only forty percent of clinical cases of BSE reaches U.K. government
statistics in 1993 (Dealler).
Transmission from Cow to Human
Before the ban in 1988, the nervous lymphoid, and gut tissues of an estimated
two million cows reached human food. BSE is more prevalent is dairy cattle,
and although milk is thought to be safe, most dairy cows are quickly retired
into hamburger (Greger). "Most parts of the cow are
used to make burgers, sausages, pies, stocks and pet food. Until 1989,
this also included the brain" (Lacey).
Recently, a new variant of Creutzfeldt-Jakob disease claimed the lives
of ten people in Britain. "Experts in Great Britain are concerned there
may be a link between BSE and this group of people. If there is a risk,
it would be linked to consumption of brain or spinal cord from infected
cattle" (Fact Sheet). On March 20 the SEAC (see glossary)
met and concluded that "the most likely explanation at present is that
these cases are linked to exposure to BSE before the introduction of the
ban in 1989" (BSE). Sir Kenneth Calman, Chief Medical
Officer also stated on March 20, "these new findings are important and
further research is urgently required and this will be funded" (Calman).
Risk Factors to Humans
Although so far only ten people have shown clinical signs of this new variant
of CJD, the possibility of a BSE caused CJD epidemic is very possible.
Since the incubation period of CJD in humans is up to thirty years, this
disease can become "much more serious than AIDS" in years to come. Dr Richard
Lacey asserts that a "substantial danger for man exists" (Greger).
In an essay written on mad cow disease in 1993 by Michael Greger, he stated
"The earliest we could even expect to see people dying from BSE is probably
around 1995." Well, surprise, surprise, it all began in late 1995.
Yes, the government says its safe to eat British beef, but do
they really mean what they say? "Neuropathologist Sir Bernard Tomlinson
announced to the world that he had personally decided to forgo the humble
hamburger for fear that he might succumb to the ravages of CJD" (Fitzpatrick).
"It is now clear that probably a large percentage of the population will
have been exposed to BSE in the UK (Dealler). "Or
as Dr. Lacey put it, virtually a whole generation of people may die" (Greger)
History of CJD
Creutzfeldt-Jakob disease occurs worldwide. This disease, like Bovine Spongiform
Encephalopathy, bores actual holes into the brain (see Fig. 4). It appears
sporadically, striking one person in a million (Prusiner).
The disease is so contagious that a majority of neurologists refuse to
do autopsies on these patients, making the estimated "1 in a million" possibly
severly underestimated. The incubation period is very long in humans. An
innocent child could be slowly dying of this disease and not know it until
their late thirties. Clinical symptoms start with changes in sleeping and
eating and deteriorate over a few weeks. People with CJD will "wake up
one morning twitching and deteriorate weekly into blindness and epilepsy
while their brain perforates into a sponge" (Greger).
Figure 4. Human Brain with CJD (Creutzfeldt).
CJD claimed the lives of two dairy farmers who had tended herds with
BSE infected cattle. A fifteen year old Welsh girl developed CJD, despite
no family history of the disease, meaning the most probably source of infection
is food (Lacey). Since the new cases of CJD, the U.K
community, as well as other communities, have become not only angry but
scared.
Angry Community
Since BSE and its possible link to CJD has been so well publicized by the
media, many people of the community are scared. When people get scared
they stop doing whatever the cause of the scare is; in this case that means
to stop buy beef. "By May 1990, a quarter of the British population refused
to eat beef (Greger). Also in 1990 a ban was implemented
on British beef in school meals (Lacey). Since the
recent scare with the new variant of CJD the community has again stopped
buying beef. (see Figure 5).
Figure 5. Meat Consumption Graph. (Annual).
U.K Government Actions
In the late 1980's when BSE was first discovered the U.K government seemed
unconcerned with the disease as a whole. The government said the disease
would be completely eradicated by 1995 because of the ban on sheep and
cow offal. They denied the fact that BSE was a disease of vertical transmission
(see
glossary) and overlooked scientists claims
that BSE was more of a problem than first thought. In 1990, a then confident
Minister of Agriculture, John Gummer munched on a burger, along with four
year old daughter, in from of the press to restore community confidence.
(See Fig 6).
May 1990:
Agriculture minister John Gummer attempts to allay public fears about
the safety of beef by feeding his daughter, Cordelia, a hamburger.
Figure 6. Minister of Agriculture with Daughter. (Minister)
Today the British government has an angry and confused public to deal
with about BSE and its link to CJD. On March 29, 1996, European Unions
leaders promised substantial financial help towards destruction of hundreds
of thousands of older cattle. (Webster). Recently,
seventeen incidents where traces of spinal cord - a material banned from
the human food chain - were found on beef carcasses in slaughterhouses
in the United Kingdom. Minister of Agriculture said " that he was considering
tighter safeguards on abattoirs" (Fletcher). On
April 3, Britain agreed to destroy millions of head of older cattle during
the next five years in return for partial European Union compensation for
farmers whose cattle will be incinerated in an effort to eliminate human
exposure to the fatal ailment.
U.S. Government Actions
In the past, the United States government banned the importation of British
cattle to the U.S. Since 1991, there has been a strictly voluntary ban
in place on the use of rendered products from adult sheep in animal feeds
(Fact Sheet). APHIS established a program for BSE surveillance
in the U.S., that now has become known as clumsy, unorganized and ineffective.
Today, as well as in the past, the United States government seems confident,
perhaps over confident, of themselves and their ability to keep BSE out
of the U.S. This overconfidence has an eery ring to it -- sounds all to
familiar to the U.K. government about a decade ago when BSE was first discovered.
The overconfidence of the United Kingdom government got them no where but
into a deeper hole that they are now attempting to dig out of.
CONCLUSION
Summary of Findings
During my research of Bovine Spongiform Encephalopathy, I learned a great
deal. BSE is in epidemic form in cattle in the U.K. Although a ban was
implemented years ago that was supposed to completely eradicate the possibility
of a disease called BSE in 1996, it is still around. A newly found possible
link of BSE contamination to the human brain disease Creutzfeldt-Jakob
disease, causes a definite risk factor to humans. In years past the U.K
government overlooked the fact that BSE could be a problem and is now facing
a very angry community that wants answers. Presently the government is
facing a debt of over five- hundred million pounds to help eradicate the
disease.
Interpretation of Findings
Although the governments of the U.K. and the U.S. tend to overlook the
fact that BSE is a risk to humans, I feel that the community formed their
own opinions which differ completely from that of the government. The U.K.
government finally realized their mistakes and are now taking actions to
eradicate the disease in Britain. The U.S. government remains overconfident
and nonchalant about the situation of BSE. In my opinion, BSE is a definite
possible link to CJD and an immediate risk to any beef consumer.
RECOMMENDATIONS
The following are recommendations for the United States
Department of Agriculture from my research on BSE:
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Keep the ban on British importation of British cattle AND British beef.
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Research for possible vaccines.
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Funding for research.
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Make the voluntary ban on sheep and cattle offal a law.
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Do not overlook this disease
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Keep the public informed; secrets only make them scared and angry.
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Research possible testing in live cattle.
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Even though BSE has never been seen in the U.S., make BSE a reportable
disease, making farmers required by law to report a cow that has possible
signs of BSE.
SOURCES
Annual
percentage change in weekly spending on selected meat products." Http://www.nielsen.com/home/press/uk/beef_2.gif
(April 11, 1996).
"BSE."Statement
by Spongiform Encephlopathy Committee. Http://www.open.gov.uk/maff/bse/seac2.htm
(April 3, 1996).
Calman,
Kenneth. "CJD and Public Health." Department of Health. Http://www.coi.gov.uk/coi/depts/6dh/coi6738b.ok
(April 8, 1996).
"Creutzfeldt-Jakob
Disease." Http://indy.radiology.uiowa.edu/ Providers/TeachingFiles/CNSInfDisR2
(March 29, 1996).
Dealler,
S. "Bovine Spongiform Encephalopathy and Public Health." Http://www.cyber-dyne.com/~tom/sci_abstracts.html
(April 13, 1996).
"Fact
Sheet: Mad Cow Disease." National Cattlemen's Beef Association (NCBA).
Http://www.cvm.tamu.edu/fs_bse.html (March 29, 1996).
Fitzpatrick,
Machael. "A Mad, Mad, Mad, Mad World." Living Marxism. Http://www.junius.co.uk/LM/LM87/LM87_mad.html
(April 8, 1996).
Fletcher,
David. "Tighter Curbs on Mad Cow Disease (BSE)." Mad Cow Disease
in the News. Http://www.envirolink.org/arrs/news/bse_in_the_news.html
(March 29, 1996).
Greger,
Michael. "Much More Serious than AIDS." Mad Cow Disease. Http://www.envirolink.org/arrs/AnimaLife/spring94/madcow.html
(April 14, 1996).
Lacey,
Richard. "How Now Mad Cow?" Viva! Guides: Guide 3. Http://www.cyber-dyne.com/~tom/mad_cow_disease.html
(March 28, 1996).
"Minister
and Mad Cow Cartoon." Http://www.tecc.co.uk/bmj/bse.htm (April 11,
1996).
The Official Mad Cow Disease Home
Page. http://www.mad-cow.org/ (May 14, 2001).
Painter,
Mike. "Spongiform Encephalopathy: Human Cases Linked to BSE." ProMED
Maillist. Http://inet.uni-c.dk/~iaotb/painter.txt (March 30, 1996).
Pratt, Kendra.
"Bovine Spongiform Encephalopathy." Factsheet: Veterinary Services.
Gopher://ahd/ahi.d/ch.d/bse.f (March 20, 1996).
Pusiner,
Stanley B. "The Prion Diseases." Scientific American. Http://www.nmia.com/~mdibble/prion.html
(March 28, 1996).
"Terms
Used by the Scientists and Explained as Simply as Possible." Term
Definitions. Http://www.airtime.co.uk/bse/tse.htm (April 8, 1996).
"Typical
Microscopic Appearance of the Brains of Animals with Spongiform Encephalophthy."
Http://www.vetmed.ucdavis.edu/ bse/bse&cjd4.htm (April 23, 1996).
Webster,
Philip. "Major Raises Hopes to End Beef Ban." The London Times.
Http://www.the-times.co.uk/news/pages/times (March 30, 1996).
GLOSSARY
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BSE:
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Bovine Spongiform Encephalopathy.
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Creutzfeld-Jakob disease:
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A TSE that gives rise to a pre-senile dementia in humans.
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Downer Cows:
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Cattle that show neurological problems (an American term).
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Epidemiology:
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The study of changes in the numbers of cases of disease in relation to
time and space.
-
EU:
-
European Union.
-
Kuru:
-
A disease found in the Fore tribe in New Guinea, and due to the eating
of infected tissue by members of the tribe. In general the women ate brain
tissue rather than the mean and it was the women and children that died
relatively rapidly of the disease.
-
MAFF:
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Ministry of Agriculture Fisheries and Food in the UK.
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Prion:
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This is the protein that makes up the infectious agent claimed by a large
number of groups now to be the infectious particle that transmits the disease
from one cell to another and from animal to another. It is made from the
normal protein PrPc (the c stands for chromosomal) that is produced in
small quantities on many cells and especially in the lymphoid and the nervous.
-
PrP:
-
The prion protein. It can exist in various forms. One is PrPc and is the
normal type of protein that is found in a cell. One is called PrPsc (or
PrPscrapie) that is found in the infectious cells.
-
RNA:
-
Ribonucleic acid. Often found in viruses as the nucleic acid that carried
the genome of the agent.
-
Scrapie:
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The TSE of sheep or goats.
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SEAC:
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Spongiform Encephalopathy Advisory Committee. Tyrrell Committee.
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TSE:
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Transmissible Spongiform Encephalopathy. A disease that can be transmitted
from one animal to another and will produce changes in the brain that appear
similar to a sponge.
-
Vertical transmission:
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The transmission of an illness from the parents to the offspring.