Did Mad Cow Kill Cluster Of
18 People In PA 13 Years Ago?

From Patricia Doyle, PhD

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, or CJD.
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, Del.
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 the 1980s.
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 cattle industry.
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 Agriculture.
''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 1997.
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 my diet.''
Brown and Little spent months reviewing the medical records of local people who died of CJD in the five-year cluster and interviewing relatives.
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 researchers said.
''I believe we did what we could to determine the cause of the outbreak in the Lehigh Valley,'' Little said. ''But, we were unsuccessful.''
610 820-6745
Copyright © 2004 The Morning Call
Patricia A. Doyle, PhD
Please visit my "Emerging Diseases" message board at:
Zhan le Devlesa tai sastimasa
Go with God and in Good Health



This Site Served by TheHostPros