gloves, minimize contact with brain and spinal cord tissues,
discard the brain, spinal cord, eyes, spleen and lymph nodes and
definitely not eat them. There is no evidence that CWD can cross
over from deer and elk to humans, but there was no firm evidence
that mad cow disease could afflict humans until 1999, so wildlife
officials in the Rocky Mountain states say caution is warranted.

Writing in the BOSTON GLOBE, Terry J. Allen reported in late 1999
that, since 1996, Creutzfeld-Jakob disease has been identified in
3 Americans younger than age 30.[3] All three are known to have
hunted extensively or eaten venison. There is no evidence that
CWD disease has jumped from deer or elk to humans, but the
appearance of this extremely-rare disease in young people was the
first evidence of a problem in England, so health authorities in
the U.S. say they are aggressively investigating all the
possibilities.

A statistician at the federal Centers for Disease Control (CDC)
in Atlanta told Terry Allen that, if one more case of CJD had
surfaced in a person younger than 30 in the U.S., it "might tip
the balance," meaning it might convince authorities that
something truly unusual was occurring. Dr. Michael Hansen of
Consumer's Union says, "Given how rare the disease is in young
people and how difficult it is to make a diagnosis, the
possibility that some cases go undetected cannot be ruled
out."[3]

Indeed, of the 3 cases detected in the U.S. since 1996, one
nearly went undetected. Last year in Utah, Doug McEwan, 28, began
to show an array of mysterious symptoms: loss of memory, loss of
motor control, mood swings, and disorientation. His wife, Tracey,
says his doctors conducted hundreds of tests but could not
diagnose his disease. She happened to see a TV program on mad cow
disease and she insisted that Doug's doctors must test for CJD. A
brain biopsy confirmed the diagnosis.

One of the three young CJD victims had eaten deer shot near
Rangely, Maine, so last November federal officials took samples
of brains from 299 deer shot in western Maine. Authorities said
at the time they were quite sure Maine deer are not harboring
CWD. So far, test results have not been released.

Federal authorities have quarantined two herds of sheep in
Vermont because they say the sheep may have been given feed that
contained parts of animals afflicted by mad cow disease. The
sheep had been imported into Vermont from Belgium and the
Netherlands, where they may have been fed improperly. A similar
herd of sheep in New York state was recently purchased by the
federal government and slaughtered.[4]

Meanwhile, a 68-year-old Indiana man with a fondness for
beef-brain sandwiches died of CJD last summer. Beef-brain
sandwiches are a local delicacy in Indiana, introduced years ago
by German immigrants. The EVANSVILLE (INDIANA) COURIER reported
that John Hiedingsfelder, a forensic pathologist in Evansville,
said he had seen three cases of CJD in the past year. No
connection to mad cow disease has been established in the Indiana
cases. Roberta Heiman, a staff writer for the EVANSVILLE
(INDIANA) COURIER reportedly received a warning from a
cattleman's association not to publish any further articles about
this subject.
============

[1] Unless a specific source is cited, information in this issue
of Rachel's was taken from www.mad-cow.org, a web site maintained
by Thomas Pringle of Eugene, Oregon. Sources of information are
cited at www.mad-cow.org.

[2] Michael R. Scott and others, "Compelling transgenic evidence
for transmission of bovine spongiform encephalopathy prions in
humans," PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES
Vol. 96, No. 26 (December 21, 1999), pgs. 15137-15142.

[3] Terry J. Allen, "Rare, Animal-Borne Disease a Medical
Mystery; Officials Examine Maine Deer in Hunt for Clues," BOSTON
GLOBE December 12, 1999, pg. C26.

[4] Matthew Taylor, "Mad Cow Fears, Anger on Farms; Two Imported
Sheep Herds Quarantined in Vermont," BOSTON GLOBE October 31,
1999, pg. F24.

Descriptor terms: mad cow disease; england; france; montana;
wyoming; vermont; maine; deer; elk; bse; tse; central nervous
system disorders;

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