New research raises questions about mad cow disease

Julia Harris' tall, strong husband was trimming the hedges at their summer place in New York when the clippers suddenly got heavy and he couldn't remember simple things, like how to write his name or add 9 and 5. That was in May 1995.

Two months and five days of living hell later, Arthur Harris died, blind in one eye, unable to walk, talk or eat, muscles jerking out of control, his brain so full of holes it looked like cheese on the CAT scans.

Doctors at Beth Israel Medical Center in New York City told his wife he had a strange sounding illness the Harrises had never heard of: Creutzfeldt-Jakob disease. Then they told her some things that seemed preposterous.

It probably had been incubating in his brain for five years, they said, ever since he'd had a tumor removed at Bethesda Memorial Hospital in Boynton Beach, the Harrises' winter home. They said the surgeon had patched the hole with material routinely used in brain and spinal cord operations -- freeze-dried membrane from cadavers -- that could have been infected.

There was more: The sickness was related to a disease among cannibals in New Guinea and to an epidemic killing tens of thousands of animals and causing panic in Europe -- "mad cow" disease.

This was too much for Julia Harris. Her beloved Artie's brain destroyed by a mysterious disease from a dead person's tissue that they didn't even know had been implanted in his head, and somehow it was linked to cannibals and mad cows?

"I almost dropped dead, I was so stunned," she said. "I just fell apart and didn't know how to answer. . . . The funeral home didn't even want to handle his body, they were so afraid when the doctor told them what he died from." Until recently, this was how people usually learned about Creutzfeldt-Jakob, a disease so rare it's believed to occur naturally -- and still inexplicably -- in only one person in a million, mostly to people in their 50s and 60s.

However, new research in Florida indicates the toll may be a lot higher because the disease has been misdiagnosed as Alzheimer's and other illnesses that attack the nervous system. Now CJD and BSE -- bovine spongiform encephalopathy, or mad-cow disease -- have leaped out of medical obscurity and into headlines, TV shows and rampant speculation on the Internet. Scientists and governments are scrambling to pin down their cause, stop their spread and keep a suspected link between them from turning into a worldwide disaster. So far:

-- BSE, diagnosed in 1986, has infected more than 168,000 cows in England and nearly wiped out the British beef industry. -- Sixteen people in England and one in France have died of a new form of CJD that appears nearly identical under a microscope to the cow-killing plague. Scientists think they got it from eating meat contaminated by pieces of brain, spinal cord or intestines, or by handling such tissue. -- No BSE has been documented in U.S. cattle. But to keep BSE from getting into the American food supply, the Food and Drug Administration has ordered changes in animal feed and meat processing rules that could cost $200 million a year. A leading consumer organization says the move still leaves the door open to the brain diseases.

-- The World Health Organization is about to recommend surgeons stop using human dura mater membrane -- the stuff doctors believe killed Julia Harris' husband -- for brain and spinal cord surgery. Worldwide, at least 69 people have died of CJD traced to infected membrane, most produced in the 1980s by a German company.

Only two dura-mater deaths have been documented in the U.S., but estimates of the incubation period for CJD are growing. More deaths will be traced, as awareness and knowledge of the diseases grow.

Cannibalism of one kind or another has caused the worst spreading of the brain-eating diseases. In a popular new book titled "Deadly Feasts," author Richard Rhodes gives a clinical and stomach-turning account of a "mortuary feast of love" among the Fore (FOR-ae) people of Papua New Guinea in the 1950s. Fore families honored their dead by cooking and eating them; special parts, including the brains, went to special people, mostly women and children.

Coincidentally, the Fore also suffered horribly from kuru, a disease they called "laughing death" and blamed on sorcery. It caused tremors, loss of balance, bizarre laughter, violent jerking and, finally, when they could no longer move or swallow, slow death by thirst or starvation.

Kuru killed more than 100 Fore a year -- mostly women and children. It declined quickly after the 1950s, when the tribe gave up eating their dead relatives. A few cases still appear, indicating incubation periods of 40 years.

Scientists from the National Institutes of Health examined the brains of kuru victims and found plaque deposits and holes similar to those described by two German doctors who encountered a puzzling brain disorder in the 1920s -- Hans Creutzfeldt and Alfons Jakob. But they still didn't know what caused the disease or how it was spread.

Researchers later saw the same type of spongy damage in the brains of sheep infected with scrapie and of British cattle killed by BSE in the 1980s. It didn't all come together until they focused on food.

Meat processors long ago found a great solution to the problem of what to do with billions of pounds of animal remains: Cook the carcasses, separate the fats and oils for gelatin, glue, pharmaceuticals and cosmetics; then grind the bones, hides, hooves and scrap meat into "bone meal," a mixture rich in calcium and phosphorus favored by nurseries and gardeners for fertilizer.

Then the food industry started feeding it back to cattle, sheep, horses, hogs, poultry and pets -- some of the same creatures from which it was made -- as protein supplement.

The animals, like the Fore, began eating their relatives.

And somewhere, at some point, the brains of a sheep that had scrapie or a cow with BSE, or maybe both, got into some bone meal produced in the United Kingdom. It infected more cows, whose remains were fed back into the animal-cannibal system, and an epidemic was born.

BSE ultimately broke out in 33,000 dairy and cattle herds. Countries banned imports of British beef, and UK officials began wholesale slaughter of infected herds. Meat became a scary thing in Europe.

In March 1996, the British government -- often accused of denying and hiding facts about the epidemic -- made a shocking announcement. Ten people, all under age 42, had died from a new strain of CJD that looked too much like the bovine disease to ignore. The nation's best scientists still couldn't pin down exactly how it had happened, but they feared the infective agent, whatever it was, had crossed the species barrier from animal to human.

At that point, the U.S. government, meat growers and processors knew they had to do more than ban British beef and test the brains of cows that acted strangely. If an animal with a spontaneous case of BSE or scrapie got into the rendering apparatus, it could create a panic that would bring the whole industry down, in addition to a disease epidemic.

"The implications of one case of BSE on this industry (are) enormous," said Gary Weber, an official with the National Cattlemen's Beef Association. "We know that a 1 percent change in the consumer's purchase of our product is $350 million immediately in farm and ranch income. In the (whole industry) it's at least $1 billion."

The FDA hurriedly put together a rule that would partly curtail the use of meat-and-bone meal among food animals. It would classify protein from ruminants like cattle, sheep and goats as a food additive and forbid feeding it to them or other ruminants. The cud-chewing animals have four stomachs and take longer to process food, making them more susceptible to food-borne infection.

Renderers pointed out there was no way to tell whether a bit of cow or sheep carcass got cooked into the 45 billion pounds of animal parts they recycle per year, so it would be better to ban the use of all mammals as food for ruminants. The FDA agreed and published the final rule June 5, saying no mammal protein can be fed to ruminants. But there were exceptions.

"You have to read the fine print to find out that their definition of mammal doesn't include pigs and horses," said Michael Hansen, food safety researcher with Consumers Union, which publishes Consumer Reports magazine. "By failing to include swine, FDA has left the door open for a mad cow-like disease to circulate in the United States."

Scientists can cause the disease in pigs by injecting contaminated material into their brains in the lab, but they haven't found a natural case. An outbreak was suspected among pigs coming into a slaughter plant in Albany, N.Y., in 1979, but slides of the damaged brain tissue weren't good enough to be conclusive when examined last year.

Most pigs are less than a year old when marketed, Hansen argued, so the disease wouldn't have had time to show its symptoms. He said the FDA should follow the British example of banning all mammal tissue in feed for farm animals and in fertilizer used on land where they graze. Renderers say they're glad to comply with the rule, regardless of cost.

"We'll probably take a $200-million-a-year hit, but we believe it's a fair price to pay to protect the American food supply," said David Evans, a Greensboro, N.C., plant owner who chairs the industry's committee on spongiform brain diseases.

Doubt and debate will go on as long as science can't solve the puzzles of CJD and its animal equivalents. After 40 years of tests, most researchers agree that they are like nothing ever seen in biology or medicine.

Though they behave like slow viruses, they appear to be caused by certain proteins on the surface of nerve cells. Protein molecules have three-dimensional shapes that have to fold in precise ways for the cells to function. In spongiform diseases, scientists think a protein molecule "goes bad" and folds in a distorted way; then it causes the molecules around it to fold wrong, and the distortion spreads out like ice crystals forming from a single pattern or template. The cell dies, and when enough cells die, they leave a hole.

Scientists have labeled the proteins "prions," and some say they could be in all species -- meaning all species could be susceptible to the prion diseases.

The crucial question is: What triggers the first molecule's mutation?

About 10 percent to 15 percent of known CJD cases are inherited, and most of the rest occur without any apparent cause. Incubation can take up to 40 years, so cause and effect are long separated. The discovery that CJD was infectious and could be transmitted through injections of human growth hormone -- 80 cases worldwide -- cornea transplants and contaminated surgical supplies and tools was a jolt.

The first case of dura mater infection turned up at Yale University Hospital in 1989, only 18 months after the patient's brain surgery. In cases tracked since then, incubation times of up to 15 years have been found, said Dr. Paul Brown, medical director of the U.S. Public Health Service.

The World Health Organization wants surgeons to stop using the human membrane entirely, but Brown said the dura collected and processed in the U.S. is "almost certainly safe." He said the FDA probably will propose tighter controls on it at a meeting in August.

Only about 10 percent of the 200 brain operations performed annually at Good Samaritan and St. Mary's medical centers require membrane grafts, and surgeons there haven't used processed dura mater for several years, said Dr. Jordan Grabel, chief of neurosurgery.

"Even if the chance is minute, there's simply no reason to take that chance," Grabel said. "It can be very easily substituted by a synthetic substance, and there's also a nice substance called fascia lata, membrane taken from the thigh, that doesn't present a health hazard."

Bethesda Memorial Hospital, where Arthur Harris' brain surgery was performed, hasn't used human membrane since 1992, spokeswoman Ernestine Ziacik said. And as in most other hospitals that do cranial surgery, she said, instruments such as scalpels, needles and suction tubes that come in contact with brain tissues are all disposable now.

As scientists chase prions in labs and government tries to keep a lid on mad-cow hysteria, two Florida researchers are quietly doing tests and recording results that may add to the ever-changing perceptions of such diseases.

Florida's Office of Vital Statistics records an average of a dozen deaths a year from CJD, about the worldwide average of one per million. But in the past 16 months, 11 cases have been diagnosed in the Tampa area alone, said Dr. Michael Gold of the University of South Florida.

Gold and Dr. Amyn Rojiani of the University of Florida send spinal fluid samples to the National Institutes of Health for testing when they suspect CJD. And they are becoming more and more suspicious, Gold said.

"I'm not ready to tell you we have an epidemic," Gold said, "just that we have an excessive number of cases."

It could be the result of more awareness of CJD, more willingness to test for it and a better way to diagnose it, Gold said. Or simply that Florida has so many older people who are ripe for spontaneous CJD and have been misdiagnosed with Alzheimer's disease -- or shut up in psychiatric wards when symptoms appear.

"The other working hypothesis," he said, "is that there's a lot more Creutzfeldt-Jakob out there than we think there is."

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