New human type of mad cow worries Red Cross

April 14, 2001 The Columbian (Vancouver, WA.) by Ken Olsen

The American Red Cross soon is expected to refuse blood from anyone who has spent time in Europe since 1980 as a precaution against the spread of the human version of mad cow disease.

This significant expansion of mad cow restrictions, which now only applies to people who spent six months or more in the United Kingdom, could chop 360,000 people from the ranks of the Red Cross donors alone. That's tough as blood groups struggle to meet increasing demand from aging baby boomers who routinely get heart transplants and hip replacements. But the uncertainty is too risky.

And that's the trademark of mad cow disease and its human version, new variant Creutzfeldt-Jakob disease: uncertainty.

No one knows whether infected sheep or cattle started this potential epidemic.

No one knows exactly how it spreads.

No one knows how many people the disease will kill or precisely how the infectious agent works. There is no test for the disease short of a brain biopsy -- normally done only after someone dies.

And, because even the incubation period is uncertain, no one knows when the number of cases will peak.

Confusion, speculation and misinformation reign supreme.

No human cases have been detected in the United States. Yet there are hints this country won't entirely escape some version of the outbreak. Four sheep in Vermont, imported from Europe, have a form of the disease never before seen in this country. And the Vermont Public Health Department is cautioning people not to eat cheese made from the milk of the imported flocks, the first time milk products have hit the prohibited list in the United States because of the mad cow scare.

Family of dementia

Deep in the brains of hundreds of people -- or perhaps hundreds of thousands of people -- an errant little protein is twisting, folding, swelling and destroying neurons. At some unknown point, this destruction reaches critical mass, suddenly robbing the victims first of their minds and then, over the next six to 18 months, their lives.

The mysterious culprit is an abnormally shaped microscopic protein called a prion. The errant prions worm their way into a victim's brain, appear to sit dormant for 10 to 30 years, and then turn brain tissue into a functionless sponge.

Prions are responsible for a family of diseases called transmissible spongiform encephalopathies, said Dr. Randal Nixon, a neuropathologist at Oregon Health Sciences University. The diseases include scrapie in sheep; mad cow or bovine spongiform encephalopathy in cattle; chronic wasting disease in elk and deer; plus kuru, Creutzfeldt-Jakob and new variant Creutzfeldt-Jakob disease in humans.

The two human variations with nearly identical names are easily confused. They are distinctly different diseases.

Kuru, which means "the laughing death," comes from New Guinea. Scientists discovered it in the 1950s among people who ate the brains of the dead as a funeral rite.

"You wanted to gain the attributes of the people who died," said Dr. Marcia Goldoft, an epidemiologist with the Washington Department of Health.

When it's not mad cow

Creutzfeldt-Jakob has been recognized in humans for more than 80 years. It is one-in-a-million rare, most often occurs in people older than 65, and initially is misdiagnosed as Alzheimer's. Patients usually die in less than six months.

Scientists do not know what starts most human Creutzfeldt-Jakob infections [Although there is reported evidence it may have come from eating meat from infected sheep--BSE coordinator]. They call the cause spontaneous. Creutzfeldt-Jakob disease has killed about 80 people in Oregon and Washington since 1991. It may have killed Vancouver resident Josephine E. Capps in March.

The cause is frighteningly clear in other cases. Cornea transplants and brain tissue grafts accidentally taken from infected cadavers gave the disease to some people.

More than 50 people afflicted with dwarfism were infected by injections of pituitary growth hormone. The hormone was extracted from hundreds of pituitary glands, also taken from cadavers.

Doctors also did not realize traditional disinfecting techniques, designed to kill viruses and bacteria, do not affect the prions that cause Creutzfeldt-Jakob disease. Dry heat, at temperatures of even 680 degrees Fahrenheit, has failed, Goldoft said. Soaking surgical instruments in disinfectant for weeks has failed.

A combination of pressure, high heat and moisture appear to work.

From cows to humans

New variant Creutzfeldt-Jakob disease is traced back to 1980, when England started making cattle feed out of slaughterhouse sheep and cattle leftovers. An infected cow or sheep became part of this feed supply and infected hundreds of thousands of other cattle. People ate the beef products and started contracting new variant Creutzfeldt-Jakob disease. Cases started appearing in England in 1994 and were striking people as young as those in their 20s.

Psychiatrists often are the first to see new variant Creutzfeldt-Jakob victims, because dementia in such young people is so rare. The victims are assumed to be losing their minds.

Scientists are not sure exactly what cut of meat the victims consumed. Dietary histories are difficult to reconstruct, because it's usually at least a decade between when a person consumes the infected food and the disease appears.

Because mad cow concentrates in the brain and spinal cord, lymph nodes and part of the intestine, scientists assume the victims consumed parts of these tissues. Beef sausage and hot dogs also may be culprits, because they contain all kinds of body parts.

But mad cow disease is distributed throughout an animal's body.

"It's not so neatly placed so you can say there's a pocket of it here and a pocket of it there," Nixon said. "You can find the areas where there are higher amounts."

More than 90 people, most of them in England, have died from new variant Creutzfeldt-Jakob disease, and the number of cases is increasing.

"If I were in the United Kingdom in the late '80s, eating sweetbreads, I would be concerned," Nixon said. "If I ate a lot of fast-food hamburgers or beef sausages in the late '80s, I'd be concerned."

The risk is extremely low in the United States, Nixon added.

Since 1999, the FDA has told the Red Cross and other groups not to accept donations from people who lived in the United Kingdom -- with the exception of the Republic of Ireland -- from 1980 to 1996. People who received bovine insulin or other bovine-based injection from certain countries also are prohibited.

Early this year, the Red Cross asked the FDA to expand those restrictions to include anyone who has spent time in Western Europe since 1980, because new variant Creutzfeldt-Jakob cases are appearing in other countries, said Jim Tinker, spokesman for the Red Cross' national office.

The Red Cross also asked that the threshold be dropped below six months of total time in the countries in question.

The FDA probably will add only people who have spent 10 years or more in France and Portugal to the list of prohibited donors.

So the Red Cross is moving forward on its own, even though no one has shown a direct link between blood transfusions and people contracting new variant Creutzfeldt-Jakob.

"There is a great deal of scientific uncertainty," Tinker said. "You can present the same information to different scientists and get different conclusions."

The Red Cross acknowledges the move could eliminate between 3 and 9 percent of the Red Cross' 6 million blood donors. There is currently no way to screen blood for new variant Creutzfeldt-Jakob disease, and the incubation period is so long.

"The worst case is it could be the tip of an epidemic," Tinker said. "Our commitment is to assure a safe, adequate blood supply.

"It would be wonderful to be able to look back and say this was unnecessary."

Sunday: Clark County farmers feel threatened, not just by diseases but by the possibility of the public losing its appetite for steaks and burgers.

Advice to Travelers

The U.S. Food and Drug Administration now cautions European travelers against eating meat, especially ground beef and sausage.

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