- By Ann Wlazelek
- Of The Morning Call
- Government officials say there is only one case of the
human form of mad cow disease in the United States - a young woman living
in Florida who probably became infected by eating contaminated beef in
Britain, where she lived for the first 12 years of her life.
- But, 13 years ago, medical specialists in the Lehigh
Valley reported a rare cluster of Creutzfeldt-Jakob disease, or the human
form of mad cow, that killed as many as 18 residents of Lehigh, Northampton,
Carbon, Monroe and Schuylkill counties between 1986 and 1990.
- The statements appear to conflict, but both are true,
according to leading researchers who spent months investigating the local
cluster in 1990 without finding a link to beef or any other source.
- Drs. Paul Brown and Brian Little said that's because
the cases represented two distinct kinds of Creutzfeldt-Jakob disease,
- The Lehigh Valley cluster involved the traditional, sporadic
form of CJD, which occurs in 1 in 1 million people annually without explanation,
the experts said. The Florida case is the only one in U.S. history of the
newer, variant form, linked to the consumption of tainted meat.
- Both are incurable diseases caused by abnormal proteins,
called prions, that produce spongelike holes in the brain and dementia
similar to Alzheimer's disease. However, traditional CJD strikes people
in their 50s to 70s and kills within months, Brown and Little said. Variant
CJD, sometimes written as vCJD, affects a younger population, in their
teens to 30s, and can take a year to cause death. Also, the traditional,
sporadic form has been around for at least 90 years and claimed thousands
of lives; the variant, only 15 years old, has killed about 150 people,
mostly in Britain.
- As a neuroscientist with the National Institute of Neurologic
Diseases and Stroke, Brown has spent 40 years and traveled the world in
search of answers to CJD. Little is a neuropathologist who used to examine
brains for Lehigh Valley Hospital in Salisbury Township and now is vice
president for academic affairs and research at Christiana Hospital in Wilmington,
- Yet, confusion and controversy over CJD continue.
- ''It disturbs me when the federal government says there
have been no cases of CJD in the United States,'' wrote Susan Felegy of
Whitehall Township in a Jan. 6 letter to the editor to The Morning Call.
- Felegy noted that her mother died of the disease in 1990,
adding, ''I know there were 15-20 other cases in the greater Philadelphia
area in a five-year period around the time my mother died.''
- Felegy said she was familiar with the different forms
of CJD but believes her mother, who was part of the Lehigh Valley cluster,
got the variant form by eating beef when visiting Canada for a week in
- Another letter writer, Peggy Baltrus of Milford Township,
also blamed her mother's death from CJD on a visit to Britain, but believes
the risk of disease extends beyond beef and European soil.
- She said that cows and chickens in the United States
could be infected from feed containing pulverized animal carcasses, from
growth hormones or through processing methods. Baltrus accused the United
States of underreporting CJD to prevent hysteria and protect the multibillion-dollar
- Brown has received similar e-mails at his office in Bethesda,
Md., but said research supports his contention that no American has gotten
either form of CJD from eating beef approved by the U.S. Department of
- ''Whether brain and spinal cord are still permitted in
chicken and pig feed, I do not know,'' he said. ''That will be for the
Food and Drug Administration, not the USDA, to decide. We have a backwards
system in this country in which the FDA decides what animals can eat and
the USDA decides what we eat.''
- Brown said the United States doesn't even have a case
of bovine spongiform encephalopathy, more commonly known as BSE, or mad
cow disease, because the single dairy cow in the state of Washington that
tested positive for BSE, on Dec. 23, came from Canada.
- Also, he called it ''arguable'' whether humans can get
infected eating beef from an infected cow.
- ''Most of us would not volunteer to test that,'' Brown
said. But, he added, two studies showed that muscle from sick cows did
not transmit disease when injected directly into the brains of healthy
mice and cows.
- ''That doesn't mean it would never, ever happen,'' Brown
added. ''But if push came to shove, I'd not lose sleep about it.''
- Little said the practice of using ground-up carcasses
to boost calcium levels in milk cows was banned in this country before
- Yet, if Americans are worried about eating beef, he said,
they should know that steaks don't come from dairy cows and beef cows were
not fed the ground-up bones and meat of other cows.
- Meats that might pose a danger, he said, include hamburger,
hot dogs, scrapple, and anything that is made from scraps of meat.
- ''If you are concerned, avoid beef brains,'' a deep-fried
delicacy at some restaurants, Little said. Because sweetbreads and headcheese
could contain some brain meat as well, he added, ''I'd take that out of
- Brown and Little spent months reviewing the medical records
of local people who died of CJD in the five-year cluster and interviewing
- They asked at least 50 questions about eating habits,
including beef, veal, ham, pork scrapple and sweetbread, which can be the
thymus or pancreas of a calf or lamb. Other questions pertained to surgery,
acupuncture, major dental work, family medical history, hobbies and travel.
- At the time, experts knew traditional CJD could be transmitted
by the injection of human growth hormone, the implantation of contaminated
electrodes or the transplantation of corneas.
- But local residents with CJD had no common link, the
- ''I believe we did what we could to determine the cause
of the outbreak in the Lehigh Valley,'' Little said. ''But, we were unsuccessful.''
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- Copyright © 2004 The Morning Call
- Patricia A. Doyle, PhD
- Please visit my "Emerging Diseases" message
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