IT=S A MAD,
MAD WORLD:
BOVINE
SPONGIFORM
ENCEPHALOPATHY
Prepared for
Dr.
Bruce R. Magee
Associate
Professor
English
Deptartment
Louisiana
Tech University
Ruston,
Louisiana
April
E. Storey
English
303 Student
May 1, 1996
7095
Sentell Rd
Shreveport,
LA 71107
April
29, 1996
Ms.
Kendra Pratt
Animal
and Plant Health Inspection
Services
U.S.
Department of Agriculture
Washington,
D.C. 10021
Dear
Ms. Pratt:
Here
is my report on Bovine
Spongiform Encephalopathy and its possible link to the human brain
disorder
Creutzfeldt-Jakob Disease. I believe my
report will be of help to the U.S. Department of Agriculture in
preparing its
response to to BSE.
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
further questions you might have on this subject.
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 years 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
page
LETTER OF TRANSMITTAL ........................................................................................
ii
ABSTRACT...............................................................................................................................
iii
FIGURES AND TABLES.........................................................................................................
v
INTRODUCTION.......................................................................................................................
vi
Purpose..........................................................................................................................
vi
History............................................................................................................................
vi
Review of Recent Literature.........................................................................................
vi
Research Proceure.......................................................................................................
vii
IT=S A
MAD, MAD WORLD: BOVINE SPONGIFORM ENCEPHALOPATHY..................
1
History of Disease........................................................................................................
1
History of Prions...........................................................................................................
2
Transmission from Sheep to
Cow..............................................................................
2
Transmission from Cow to
Humans...........................................................................
3
Risk Factors to Humans..............................................................................................
4
History of CJD...............................................................................................................
5
Angry Community.........................................................................................................
6
U.K. Government Actions............................................................................................
7
U.S. Government Actions............................................................................................
8
CONCLUSION..........................................................................................................................
9
Summary of findings.....................................................................................................
9
Interpretation of Findings.............................................................................................
9
RECOMMENDATIONS..........................................................................................................
10
REFERENCES........................................................................................................................
11
GLOSSARY..............................................................................................................................
13
FIGURES
AND GRAPHS
page
1. Mad
cow cartoon
. ...............................................................................................................
vii
2. Cow
brain with
BSE. Magnification x250.........................................................................
1
3. Cow
brain with
BSE. Magnification x500.........................................................................
1
4. Prions
....................................................................................................................................
2
5. Prions ...................................................................................................................................
2
6. The
prion
disease cycle ......................................................................................................
3
7. Human
brain with
CJD.........................................................................................................
6
8. Meat
consumption
graph.....................................................................................................
7
9. Minister
of
Agriculture with daughter..................................................................................
8
INTRODUCTION
Purpose
The purpose of this paper
is to attempt 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. (AMinister@).
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.
Cow
brains with BSE. (ATypical@). Figure
3.
Magnification 250x
Magnification
500x
|
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
The prion is an unusual
cause for
disease, being neither a virus nor a bacterium.
What is it then?
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 (AFact 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). One
deformed prion can change another normal
prion into its abnormal shape,
Figure
4. Prions. (AAbout
Prions@)
Figure
5. Prions. (AWhat Is a Prion?@)
as shown in Figure 6.
These deformed proteins build up in the system and are almost
impossible
to destroy. 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" (Gregor).
Figure
6. The prion disease cycle.
(APrion@).
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" (Gregor). 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 in 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" (Gregor). In
an essay written on mad cow disease in
1993 by Michael Gregor, 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 severely
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" (Gregor).
Figure 7.
Human
Brain with CJD. (Jayachandran and Faust).
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 (Gregor). 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 8).
Figure 8.
Meat
Consumption Graph. (AAnnual@).
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
figure 9).
Figure 9.
Minister
of Agriculture with his daughter. (Levy)
|
May 1990: Agriculture minister John
Gummer attempts to allay public fears about the safety of beef by
feeding his daughter, Cordelia, a hamburger. |
Today the 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. (Major).
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" (Cowell).
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
BSE is an epidemic among
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:
1. Keep
the ban on British importation of British cattle AND British beef.
2. Fund
research into possible vaccines.
3. Fund
research into other preventions and treatments.
4. Make
the voluntary ban on sheep and cattle offal a law.
5. Keep
sick cows out of the food chain.
6. Keep
the public informed; secrets only make them scared and angry.
7.
Fund research for BSE tests in live
cattle.
8.
Make BSE a reportable disease, making farmers required by
law to report a cow that has possible signs of BSE.
SOURCES
AAbout
Prions.@ The Prion Institute.
http://www.prioninstitute.ca/index.php?page=webpages&
menucat=35&id=9&action=displaypage&side=1
(April 5, 1996).
AAnnual
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.html (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/
cnsinfdiskz
(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. (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://envirolink.
org/arrs/animallife/spring94/ madcow.html (April 14, 1996).
\
Jayachandran, Priya, and
Hilary
Faust. Prions.
http://www.stanford.edu/group/virus/
prion/2005priya/clinical.html (March 29,
1996).
Lacey, Richard. "How Now Mad Cow?" Viva!
Guides: Guide 3.
Http://www.cyber-dyne.com/~tom/mad_cow_disease.html (March 28,
1996).
Levy, Andrew.
AFamily
friend of John Gummer is killed by CJD aged 23." Mail
Online. http://
www.dailymail.co.uk/health/article‑487074/Family‑friend‑John‑Gummer‑killed‑CJD-aged‑23.html
(October 11, 2007).
"Minister and Mad Cow
Cartoon."
Http://www.bmj.com/mbj/mse.html (April 11, 1996).
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).
APrion.@ The New World
Encyclopedia. http://www.newworldencyclopedia.org/entry/Prion
(March 27, 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).
AWhat Is
a Prion?@ Prion
Space:
The Space of Prions.
http://www.prionspace.net/prion.htm (March 23, 1996).
GLOSSARY
BSE: Bovine
Spongiform Encephalopathy
Creutzfeld-Jakob disease: A
TSE that gives
rise to a pre-senile dementia in humans.
Downer Cows: Cattle
that show
neurological problems (an American
term).
Epidemiology: The
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 men, and it was the women and
children that died relatively rapidly of the disease.
MAFF:
Ministry of Agriculture Fisheries and Food in the UK.
Prion: 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: The
TSE of sheep or goats.
SEAC: Spongiform Encephalopathy
Advisory Committee. Tyrrell Committee.
TSE: 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: The
transmission
of an illness from the parents to the offspring.