Bovine Spongiform Encephalopathy:

1
Bovine Spongiform Encephalopathy Mad Cow
Disease

• Melody ODonnell
• URBS 515 Race Poverty and the Environment
• Professor Raquel Pinderhughes
• Urban Studies Environmental Studies Programs
• San Francisco State University
• Spring 2004
• Public has permission to use the Material herein,
  but only if author(s), course, university and
  professor are credited.

2
This presentation focuses on the history and
impacts of Bovine Spongiform Encephalopathy (BSE).

• It is designed to inform the public about the
  danger of this disregarded disease in the United
  States.
• It analyzes and describes the origins and
  transmission of this disease, paying particular
  attention to the social, environmental and public
  health impacts associated with BSE.
• We start by analyzing the rendering process that
  is thought to be a major factor in transmission
  of this disease. We then look at a brief history
  of its effect on humans and other animals. This
  is followed by a comparison of the safeguards
  taken by the U.S. compared to other countries.
• We conclude with proposals to protect those who
  consume beef products from the United States.

3
Introduction

• Why is it important for us as Americans to know
  about a disease that is thought to be a problem
  only in Europe, and more recently, in Canada?
• Almost 77 million Americans eat beef every day. 1
• If the research is true that humans are
  susceptible to the human form of Spongiform
  Encephalopathy, and this disease is in fact
  transmissible from consuming infected beef
  products, why isnt more being done to protect
  the American public?

4
Transmission

• BSE can be transmitted from mother to fetus, as
  well as from bull sperm to the female.
• Also contracted when infectious agent (prion) is
  ingested in food.
• Adult cows are fed rendered animal protein to aid
  in bulking up.
• Calves are fed a baby formula made from bovine
  blood because it it much less expensive than
  milk, not to mention the comparative resale
  values of the two liquids.

5
Protein Concentrates rendered animal protein
feed

• This inexpensive, high protein food is sold as
  granules or dry food pellets to animal farmers
  for vigorous animal growth.
• It is sold in greatest quantities to cattle
  farmers because administration of hormones such
  as RGBH to their cows for increased milk
  production requires them to increase the cows
  protein consumption.
• This feed is also given to
• Pigs
• Turkeys, chicken and ducks
• Horses
• Catfish, salmon and shrimp
• Domestic animals (cats and dogs)
• Zoo animals

6
Rendered Protein Ingredients

• Animals unfit for human consumption such as
• Sick cows
• Sick pigs
• Sick turkeys, chicken and ducks
• Horses
• Sick catfish, salmon and shrimp
• Cats and dogs
• Zoo animals
• Road kill

7
More ingredients

• Frying oil from restaurants
• Brains, spinal cords, feathers, hooves, skins,
  hair, fur, whiskers, bones, teeth, etc. remaining
  from slaughterhouses
• Sewage sludge
• Manure
• Sawdust/wood scraps
• Newspaper
• Cement dust
• Maggot infested grains

8
Rendering Process Health and Environmental
Effects

• The animals and other ingredients are processed
  into smaller pieces in a Double Screw Press
  (top left) and then boiled down in huge vats
  (bottom left) at extremely high temperatures with
  dangerous chemicals. Infected tissue from a
  single animal has the potential to mingle with
  tissues from thousands of others, and then be
  distributed widely in feed.1

9
Worker Hazardous Exposures

• Dangerous chemicals such as hydrogen sulfate,
  potassium permanganate, chlorine gas, sodium
  hypochlorite, lime, formaldehyde, lye, acetic
  acid and phosphoric acid.
• Extreme Heat
• When heat of process was lowered in response to
  worker safety issues, process does not
  sufficiently remove contaminants Salmonella, E.
  coli, infectious prions pesticides,
  pharmaceuticals and hormones. These contaminants
  not only pose a problem for the animals ingesting
  the food but also for the workers handling the
  product.
• Aerosolized fat mist that sprays out of the vats
  coats the walls and floor of the plant, making
  all surrounding surfaces very slippery and
  creating an extreme fire danger.

10
Recycling?

• The rendering industry defends their work as a
  form of recycling-
• 1.3 million tons in the U.K. in 19881
• 15 million tons in the U.S. in 19922
• This is waste that would have otherwise gone into
  landfill. But is the risk belong taken worth the
  tradeoff?

11
Not only is this rendered protein used in the
food we feed our pets and food animals, it is in
the products we use everyday. The fat is skimmed
off of the top of the vat during the rendering
process and used to make marshmallows and cereal
bars, lipstick and hand lotion and garden
fertilizers, tires and yogurt and breath mints.
12
Therefore, not only have we turned herbivore cows
into not just carnivores but cannibals, we have
put ourselves and other animals at risk . The old
adage you are what you eat has never been more
true-not only are we the food that we eat, we are
also what our food consumes. Humans, being at the
top of the food chain, are consuming all of the
toxins, chemicals, hormones and diseases that
affected our food before we consumed them.
13
Symptoms of Bovine Spongiform Encephalopathy

• restlessness
• aggressiveness
• loss of motor function
• loss of appetite
• convulsions
• blindness
• self mutilation

14
the initial discoveries of BSE

• In April of 1985, the first identified case of
  BSE was initially believed to be grass
  staggers, a common illness caused by Magnesium
  deficiency. The cow was observed as seeming to
  hallucinate.1 Given an Unknown diagnosis, as a
  possible brain tumor or lead poisoning. The brain
  autopsy revealed spongiform patterns.
• On March 20, 1996 the UK Department of Health
  announced that BSE was in fact transmissible to
  humans.
• The announcement was so devastating to the UK
  cattle economy that many ranchers were forced
  into bankruptcy beacuse of the immediate loss of
  entire herds of potentially contaminated cattle,
  as well as the immediate consumer boycott of beef
  and beef products. This downturn was so terrible
  in fact that there was an epidemic of suicides
  within the ranching community.2
• School districts began banning beef in school
  lunches and vegetarianism rose in popularity for
  the general public in the UK.
• In June, 1987 John Wilesmith, a veterinarian
  epidemiologist for the Ministry of Agriculture,
  Fishers and Food (MAFF) made the link between
  BSE and cattle feed made from scrapie infected
  sheep.3

15

• On July 7, 1988, a settlement was offered by
  British Agriculture of payment for 50 of the
  worth of the cow if reported to the government.
• This in fact gave the ranchers an incentive not
  to report suspicious cases, as they would make
  the full profit from sneaking past inspection and
  selling the meat into the market versus reporting
  the problem and only receiving 50 compensation.
• Not only did ranches lose money from BSE reports,
  they lost credibility as well as their customer
  base, and in effect became blacklisted.

16
The effect of BSE in the E.U.

• Millions of suspect animals in the UK and
  across Europe have been destroyed since 1986, but
  the disease is still very much present.

17
Causative agent of BSE What is a Prion?
18
prions

• Identified by Dr. Stanley Prusiner, who in 1997
  won the Nobel Prize for his research on prions.
• Does not contain RNA or DNA (therefore not
  technically alive like other infectious agents
  like viruses or bacteria)
• Does not evoke any detectable immune response or
  inflammatory reaction in host animals.
• In most animals, 3-7 years dormant incubation
  where the animal is asymptomatic.
• Average age of cows in the U.S. is 4 years old,
  meaning that the animal may be infected but still
  asymptomatic at time of slaughter.

19
Prions in the body
Found in mainly in the brain, spinal cord and
nervous tissue, with increasing research
discovering prions in glands and blood as well.

• Physical Attributes of the affected brain
• Enlarged astrocytes- Star shaped cells attached
  to blood vessels in brain.
• Holes where neurons used to be.
• Amyloid Plaques-flower shaped protein waxy
  buildup.

BSE Brain
Scrapie Brain
20

• Prion protein is indestructible
• by heat up to 1000 F (350 C)
• Hot enough to melt lead.
• In 1986, 4.5 million cows were incinerated in the
  U.K. after the discovery of BSE. The ashes,
  stored in underground concrete containers, were
  retested again in 1998 and found to still be
  infected with active prions.

21
So how do these prions affect humans?
Kuru Creutzfeldt-Jakob Disease (CJD) New Variant
Creutzfeldt-Jakob Disease (vCJD)
22
Kuru

• Cannibal culture of Papua New Guinea.
• Affected mostly women and children, with a small
  amount of men. anywhere from 5 to 10 percent of
  the population died each year from kuru.1
• When loved one died, men ate muscle portions and
  women and children were left with the lesser
  organs and brain, where we now know prions tend
  to cluster.
• The rare male cases occurred because of the
  possible 20 to 30 year dormancy period of prions
  where the infectious agents were ingested as
  children.

23
Kuru (continued)

• Analyzed by New Yorker Carleton Gajdusek and
  Lithuanian Dr. Vincent Zigas (both in photo) in
  1957.
• Initially believed to be a virus causing
  encephalitis (swelling of the brain), with the
  same symptoms as Parkinson's, Alzheimer's, and
  MS. However these were degenerative, not
  infectious diseases, and not epidemic as kuru
  was.
• After autopsy, Gajdusek made the connection of
  brain damage to recently discovered CJD.
• No treatment was ever found, and when cannibalism
  was eventually phased out of the culture, so too
  came the disappearance of kuru.

24
Creutzfeldt-Jakob Disease (CJD)

• Discovered in 1921by Dr. Hans Gerhard Creutzfeldt
  and Dr. Alfons Jakob, colleagues at the
  University of Hamburg Germany.
• Now more common that rabies.
• Physical attributes of the affected brain
• Enlarged astrocytes- Star shaped cells attached
  to blood vessels in brain.
• Holes where neurons used to be.
• Amyloid Plaques-flower shaped protein waxy
  buildup.

Microscope slide of brain affected by CJD
25
CJD Symptoms

• (first 7 same as BSE)
• restlessness.
• aggressiveness (biting and hitting).
• loss of motor function.
• loss of appetite.
• convulsions.
• blindness.
• self mutilation.
• inability to swallow.
• 90 of deaths usually occur within one year of
  diagnosis, difficult to confirm diagnosis until
  post mortem.

26
CJD Transmission

• Humans can acquire the prion by exposure to meat
  that has come in contact with the brain or spinal
  column of the animal.
• Surgical equipment can be unknowingly infected by
  use on a patient with CJD, and because
  sterilization techniques do not kill the prion,
  the are transmitted to the other patients in
  subsequent procedures.

In common slaughtering practices, the animal is
often sliced at least once through the torso,
severing the spinal column and exposing all the
the surrounding flesh to the infectious agent.
27
CJD

• Similarity to Alzheimers disease
• very similar patterns of dementia.
• because of late onset of CJD, both usually occur
  later in life.
• CJD often misdiagnosed as the more common
  Alzheimers, as only way to differentiate is post
  mortem brain autopsy (which most families do not
  agree to.)
• However, a 1989 article in the journal Neurology
  explains that autopsies of 54 dementia patients
  at the Veterans Medical Center in Pittsburgh, PA
  revealed that 3 of the had actually died of CJD.
  Given this figure we can infer than as many as 5
  of Alzheimers patients are actually suffering
  from CJD.
• In Georgia, according to state law, autopsies
  cannot be performed on suspected CJD cases
  because the equipment cannot be sterilized.
• As with all TSEs there is no cure or proven
  treatment.

28
New Variant CJD (vCJD)

• Much earlier onset but same symptoms as classic
  CJD, often with prolonged life expectancy.
• A recent test on surgical equipment used for
  tonsillectomies in the U.K. revealed that 50 of
  tools were infected with vCJD, even after
  sterilization and autoclaving. (The tonsils are
  one of the major glands where the body stores
  prions.)

Jonathan was diagnosed with vCJD at 17 and
treated with the drug Pentosan polysulphate (PPS)
, commonly used as an arthritis treatment for
dogs. This extended his life by several years,
but did not cure him.
29
Other Forms of Transmissible Spongiform
Encephalopathies
30
Scrapie

• Known to have existed for at least 200 years
  without being transmitted to humans, while being
  endemic in sheep populations all over the world.
• There are 23 variations of prion mutation
  possible, each with a different incubation period
  as well as patterns of amyloid plaque in the
  brain.
• Each variation is known by a unique symptom, such
  as drowsy, hyper, or fat building.

31
Domestic and Exotic Felines

• The first case of domestic Feline Spongiform
  Encephalopathy was discovered in the U.K. in May
  of 1990. Max was a 5 year old Siamese cat. The
  FSE was discovered in a rare autopsy (which are
  thought to be too costly and unnecessary by most
  pet guardians).
• Lab tested cats are not susceptible to scrapie
  but extremely susceptible to BSE. These results
  reinforced the findings that it was possible for
  a species (such as humans) to be susceptible to
  BSE without being susceptible to scrapie, despite
  their overwhelming similarities.

A Puma and 3 cheetahs died from FSE in 1989 at
the London Zoo from rendered food.
32
Other Zoo Animals
Gemsbok
Arabian Oryx
From 1986 to 1989. The London Zoo lost several
varieties of hoofed ruminants (cud chewing
animals), including Nyala, Gemsbok, Kudu, Eland
and Arabian Oryx, (all similar to antelope or
deer), to spongiform encephalopathy. These
animals had all been fed rendered protein.
33
Mink

• Wisconsin is the largest U.S. producer of mink.
• Known as sentinel species, mink often show
  disease before other animals because they are fed
  stock animals unfit for human consumption from
  slaughterhouses and farms.
• Contract many bovine diseases- anthrax, botulism,
  tuberculosis.
• Outbreaks of MSE common, devastating to ranchers.
  a single outbreak could wipe out all of the
  animals on an entire mink ranch.1 100 fatal to
  mink exposed to contaminated feed.
• Isolated prion from autopsy indistinguishable
  from scrapie.

Ranched for their fur, these carnivorous animals
are extremely susceptible to Mink Spongiform
Encephalopathy from eating other infected animals.
34
Chronic Wasting Disease (Deer and Elk)

• Chronic Wasting Disease is the spongiform
  encephalopathy most similar to scrapie.
• The infected animals exhibit more progressive
  degeneration (similar to Alzheimer's) than other
  TSEs.
• Most likely not contracted by ingesting infected
  proteins, but through everyday contact with other
  infected animals in their herds.

35
Lab Tests

• In laboratory tests, animals were injected with
  active prions from cows infected with BSE.
• The infectious agent was not ingested in food as
  most animals and humans would be exposed to the
  prions. Even so, all of the test subjects
  contracted some form of prion disease.
• monkeys
• sheep (different variation of scrapie contracted)
• goats
• mice/ rats
• pigs

36
But weve only had one confirmed case of mad cow
disease in the U.S. ! How can we blame BSE for
all of these other spongiform encephalopathies
occurring in the U.S., especially vCJD?
37
The Cow that Stole Christmas.

• On December 23, 2003, the first confirmed case of
  BSE was reported on a family farm in Moses Lake,
  Washington .
• Initially described by federal officials as a
  downer cow the family later came out that the
  cow was in fact ambulatory, and that the test was
  run not because the animal was sick but because
  the family farm has a special contract with the
  government to collect brain samples from up to
  1000 animals for mad cow testing, no matter what
  their physical condition. 1
• Since the animal was healthy, the program that
  was begun after this discovery by the USDA does
  not fully address the problem, as it only allows
  random testing on Downer Cows.

38
The U.S. Beef Market At Home

• US local beef market not significantly hurt by
  discovery of BSE.
• There was a slight decline in U.S. sales in
  January and February of 2004. However, in many
  places sales of beef, both at retail and
  wholesale dealers, are reported to have increased
  from the previous years due to consumer trust in
  government protection.1

39
The U.S. Beef Market International Exports

• Beef is The United States 1 export
• 4.3-billion annually
• Any damage to this international economy would
  have widespread impacts across the country.
• Because more than 30 nations have now banned beef
  from the United States, Many packing plants are
  laying off workers and feedlots left with
  thousands of unsold animals. Those that do sell
  are at such a low price that some feeders
  feel... lucky if the broke even on each animal
  sold.1 Sales of approximately 9.6 percent of
  total U.S. production, or 3.1 billion pounds of
  beef will be lost if confidence is not regained.

40
Government officials have placed concerns for
the food industry over human health and welfare.
1

• Does this result in a lack of regulation?
• In the U.S. more than 1.8 million cattle collapse
  before slaughter yet are still seen as fit for
  consumption. About 100, 000 of these die
  mysteriously of what is known as Downer Cow
  Syndrome but are not tested and are still
  consumed by humans.2
• only one out of every 18, 000 cows slaughtered
  for human consumption are tested for BSE in the
  United States. (.000055)
• The USDA must authorize every test. When a cattle
  farmer proposed to voluntarily test every cow,
  their request was denied and the practice was
  forbidden across the country. 3

41
What information do these countries have that is
being kept from the American public? Do Americans
not deserve the same protection given citizens
all over the industrialized world?

• Its unlikely the single Holstein discovered
  in Washington state is the only sick animal ever
  imported into the country from Canada, and
  possibly Europe... Since none of the other
  animals was (sic) detected, their infected
  tissues were almost certainly processed into
  cattle feed years ago, spreading and amplifying
  the disease so that cattle in the U.S.A. have
  also been indigenously infected.- USDA Report
  submitted to Ann Veneman, Secretary of
  Agriculture.1

42
So why do we continue with these dangerous
practices?

• In an effort to contain the problem, in addition
  to testing, many public interest groups have
  proposed banning feeding animal protein to
  animals.
• 1997 USDA advisory barring ruminant (cud
  chewing animal) protein from cattle feed seen as
  more as a suggestion than enforceable. (It is
  still legal to feed to all other animals,
  however.)
• The Vice President of the American Feed Industry
  Association, Richard Sellers estimates loss of
  100 million annually if a ban was imposed on
  feeding animal protein to cattle.1

43
Who is more important to the U.S. government,
their people or their businesses?

• The American public trusts the Federal
  government to protect us, as a parent would
  protect their children. As our parent, would they
  take the risk of feeding us something that they
  know has a very good chance of making us sick?
• No parent would ever willingly put their child in
  danger.
• So too should the USDA, FDA, CDC and DHHS care
  for us.

44
Recommendations to USDA and FDA to more
adequately protect consumers

• (Compiled from suggestions by Physicians
  Committee For Responsible Medicine, Public
  Citizen and the World Health Organization)

45
Reccomendations

• Restrict marketing of downer cows for any
  purpose, whether for animal or human feed.
• Create strict regulations for cattle feed that
  match those of the E.U. nations and Japan. We
  need to remember that cows are not meant to eat
  cows or other animals. Sue Jarrett, Colorado
  rancher. 1
• Properly train and equip field personnel to test
  and report possible cases in a timely and
  effective manner.
• Properly label potentially contaminated food,
  (including those product with animal byproducts
  such as gelatin or natural flavorings) with
  stickers similar to salmonella warnings on
  chicken and Surgeon General warnings on tobacco
  products.
• Prohibit the use of animal byproducts in
  cosmetics and medications.
• Increase communication within regulating agencies
  to expedite action if necessary. Research is
  performed by National Institutes of Health (NIH),
  Protection primarily the issue of the FDA, Human
  disease surveyed by the Center for Disease
  Control (CDC) and all of these agencies are
  overseen by the Department of Health and Human
  Services (DHHS). Better communication will mean
  faster response times.

46
If you dont look for it, youre not going to
find it - Howard Lyman, former Montana
cattle rancher.
47
Bibliography

• Animal and Plant Health Inspection Service
  (APHIS)-USDA
• Program Aid No. 1705 Bovine Spongiform
  Encephalopathy An Overview. October, 2001
• Veterinary Services February 2002 Bovine
  Spongiform Encephalopathy Fact sheet
• Bovine Spongiform Encephalopathy (BSE) Response
  Plan Summary
• Barone, John. U.S. mad cow discovery lowers
  int'l. beef exports, prices. Nation's Restaurant
  News, February 2,2004, Vol. 38 Issue 5, p36.
• BBC News
• BSE Experts say Old Meat Is Safe February 4
  2004
• vCJD and BSE - the link October 20, 2000
• CJD Drug Hope September 25, 2003
• The Caledonian Cat Clinic http//www.catclinic.co
  .uk/health/fse.htm
• Center For Disease Control (CDC)
  http//www.cdc.gov/ncidod/diseases/cjd/cjd_fact_s
  heet.htm
• Doughton, Sandi and Scott, Alwyn. Experts
  predict more U.S. cases of mad cow. Seattle
  Times Thursday, February 05, 2004
• Department of Agriculture-Office of the Secretary

48
Bibliography(2)

• McNeil, Donald G. Jr. U.S. Won't Let Company
  Test All Its Cattle for Mad Cow. New York Times
  April 10, 2004
• Morris, Chris. Research raises new human-BSE
  fears Canoe News (Canada) http//www.canoe.com/C
  NEWS/Canada/2004/01/18/pf-317283.html
• Mother Earth News (Green Gazette) Mad Cow
  Disease Hits Home. April/May 2004
• Murphy, Richard McGill.. Truth or Scare What
  Food Producers Need to Know About Americans
  Demands for details on what they buy to eat.
  American Demographics, March 2004.
• Muzzi, Doreen, BSE Still influencing Cattle
  Market Southeast Farm Press March 3, 2004
• Nobel Prize e-museum http//www.nobel.se/medicine
  /laureates/1997/illpres/brain.html
• Organic Consumers Association
  http//www.organicconsumers.org/madcow/GregerBSE.c
  fm
• Ortiz, Jon. California Lawmakers Push to End
  Secrecy over Meat Recalls. Sacramento Bee April
  1, 2004
• People for The Ethical Treatment of Animals
  (PeTA) http//www.peta-online.org/feat/madcow/
• Physicians Committee For Responsible Medicine
  (PCRM) press release Mad Cow Disease February
  11 2004

49
Image Bibliography

• arabian oryx http//www.geoimagery.com/publishers
  /UAE/Gjlb65-0021UAEArabianOryxCrp.jpg
• baby http//www.euraupair.com/Images/page11_b.jpg
  
• beef product flow chart www.mindfully.org/
  Food/Mad-Cow-BSE-GAO25jan02.htm
• BioHazard http//www.pharo.com/science/bse/articl
  es/msbs_05a_other_approaches.asp
• Carleton Gajdusek http//home.sandiego.edu/scar
  e/page0.htm
• cjd brain www.infectiousdiseasenews.com/
  199606/cjd.asp
• cowpilehttp//cfapp.rockymountainnews.com/cwd/kil
  ler/2.cfm
• cow tounge http//www.cite-sciences.fr/francais/a
  la_cite/science_actualites/media/1/1437/QACTU_IMG_
  PREVIEW.jpg
• elk http//www.esd.ornl.gov/facilities/nerp/elk.j
  pg
• Farside comics http//www.r-t-o-l.com/jeroen/
  farside/archive/
• feedbags http//cfapp.rockymountainnews.com/cwd/k
  iller/2.cfm
• gemsbok http//www.incentivetouring.com/english/r
  ight20main/topbar_links/wildlife/mammals/gemsbok/
  gemsbok.gif
• Johnathan http//www.bbc.co.uk/bbcthree/news/7ocl
  ocknews/features/cjd_250903.shtml
• kuru http//pathology.mc.duke.edu/neuropath/cnsle
  cture2/cnslecture2.htm
• lab rat http//news.bbc.co.uk/media/images/381550
  00/jpg/_38155555_guide_mouse2300.jpg
• lipstick www.beautywithin.com/
  images/lipstick.jpg
• lotion http//www.oilchem.com/images/lotion.jpg
• Mad Cow http//www.peta-online.org/feat/madcow/co
  wfoambig.jpg

50
On a lighter note