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Jews in Arkansas worried about Form of Creutzfeldt-Jakob disease.

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2004-01-12 18:44 | User Profile

[url]http://epaper.ardemgaz.com/Daily/Skins/Arkansas/?AW=1073925182724[/url] January 11, 2004 Arkansas Democrat Gazette Arkansas Section page 19/146

Jews in Arkansas worried about Form of Creutzfeldt-Jakob disease.

Brain ailment leaves families with questions

Form of Creutzfeldt-Jakob affects memory, coordination BY NELL SMITH ARKANSAS DEMOCRAT-GAZETTE

When Harvey Herrin began acting oddly, his wife thought he might be depressed. But his behavior only got stranger.

[img]http://www.arkpower-light.com/sitebuildercontent/sitebuilderpictures/jewessclutchingmemories.jpg[/img] [SIZE=2]Arkansas Democrat-Gazette/ KAREN E. SEGRAVE Carol Herrin holds a photograph of her husband, Harvey, taken in 1958. Harvey died in October 2002 of Creutzfeldt-Jakob disease, a close cousin of an affliction linked to mad-cow disease.[/SIZE]

[img]http://www.arkpower-light.com/sitebuildercontent/sitebuilderpictures/jew.jpg[/img] [SIZE=2]Harvey Herrin[/SIZE]

Carol Herrin first noticed that her 68-year-old husband was losing interest in hobbies, like puttering around his shop. Then he began forgetting things. While eating dinner, Harvey would ignore a side dish on the table. He forgot it, he told her. Other times she would see him staring into an open drawer. "He would just stand there and stand there and stand there forever," she said. He began complaining that his legs hurt and told Carol he thought he shouldn’t drive. She called the doctors. A battery of tests in July 2002 turned up nothing. But his neurologist suspected Harvey had a rare illness known as Creutzfeldt-Jakob disease. A spinal fluid test indicated the doctor was right. Harvey had contracted Creutzfeldt-Jakob disease, which rapidly eats away the brain. Soon, Carol said, Harvey lost the ability to walk or talk or even turn over in bed at their North Little Rock home. Eventually he could no longer swallow. Within a matter of weeks, he was dead. "You just can’t imagine seeing someone really healthy and in six, seven, eight weeks, they’re gone," Carol said. No one knows how her husband contracted the infectious disease. Doctors told her it is not the form of Creutzfeldt-Jakob linked to mad-cow dis- ease. But Carol said there is enough mystery about these diseases to make her uneasy — especially considering a recent U.S. report of mad-cow disease in one cow detected in Washington state. "I’ve quit eating beef," she said. "I can’t even put that in my mouth." Americans have so far avoided "variant Creutzfeldt-Jakob disease," the kind linked with eating contaminated beef. Federal authorities have tried to shut down the spread of the cattle disease by keeping it out of the food supply. But tainted beef isn’t the only cause for concern about more common forms of Creutzfeldt-Jakob. The disease has been studied since its discovery in the 1920s, but it is still not well understood. There is no definitive test to diagnose it and no treatment to cure the always fatal disease. A few researchers even disagree about what infectious agent actually causes it. Like AIDS in the 1980s, many doctors and pathologists are reluctant to handle tissue from Creutzfeldt-Jakob patients. And many familiar with the disease doubt it is really as rare as the one-in-a-million estimates. So many unanswered questions leave uncertainty and fear in their wake. "I felt like I had the rug pulled right out from under me," Carol said about her husband’s disease. "It’s just the biggest mystery I’ve ever had."

‘STRANGE ILLNESS’

Creutzfeldt-Jakob is one of a handful of neurological diseases that eats holes in brain tissue. Other similar diseases include bovine spongiform encephalopathy — commonly known as mad-cow disease in cattle, chronic wasting disease in deer and elk, and scrapie in sheep. The human form of mad-cow disease is known as "variant Creutzfeldt-Jakob disease," while other forms are referred to as "classic Creutzfeldt-Jakob disease." Harvey Herrin had the classic form. While classic and variant Creutzfeldt-Jakob diseases are similar, the two cause distinct symptoms. The classic form typically affects people 55 to 75 years old, while the median age at death of people with the variant form is 28. Psychiatric problems, such as depression, are early symptoms for variant Creutzfeldt-Jakob disease. Patients with classic Creutzfeldt-Jakob have neurological problems such as memory loss or coordination difficulties. And classic Creutzfeldt-Jakob typically kills its victims within a few months of the first symptoms, not the year or more that the variant form takes. Most scientists believe an abnormally shaped protein known as a prion is the culprit of both forms of the disease. No one knows why the naturally occurring proteins misfold, only that those prions can be transmissible from one person to another. "It’s a strange illness because we think of most infections as being from viruses and bacteria," said Dr. John Schwankhaus, a North Little Rock neurologist, "and these entities are really just protein membranes." At least one scientist, Dr. Frank Bastian, a research professor at Tulane University in New Orleans, thinks the problem is actually a bacterium. The prion, he believes, acts as a receptor attracting the bacteria to healthy cells. The disease is infectious, but not like the flu or HIV, the virus that causes AIDS. Creutzfeldt-Jakob is not spread through casual contact like a sneeze or even exposure to blood. But some cases of the disease have been acquired through exposure to infected human tissue or contaminated surgical equipment. Creutzfeldt-Jakob disease has been linked to cornea transplants and human-growth hormone made from infected cadavers, but so far not other donated organs. Fear of the disease is so acute among health-care professionals, few will agree to autopsy the bodies of patients or do brain biopsies, the only way to definitively diagnose a living patient. "We tried to find someone in Little Rock who would do [an autopsy] and no one would touch it," said Dan Hild, whose brother Robert, 62, died of the disease in February. "I called the medical examiner in Little Rock, and he said no way." Scientists believe that other cases of the classic form of Creutzfeldt-Jakob have been passed genetically. But most cases — about 80 to 85 percent — are considered sporadic. No one has identified the source of infection.

ONE IN A MILLION?

More than 150 cases of variant Creutzfeldt-Jakob disease have been identified worldwide, but so far there have been no documented infections in the United States. Symptoms typically follow an incubation period, so someone could be infected without showing symptoms. "By the government saying we haven’t had any cases in this country," said Florence Kranitz, executive director of the Creutzfeldt-Jakob Disease Foundation, "what they’re really saying is any cases of mad cow-induced CJD seen under a microscope at the [Centers for Disease Control and Prevention] level." Many people even believe the prevalence of classic Creutzfeldt-Jakob disease has been underestimated. Researchers believe one in a million people — or about 300 in the United States — will contract Creutzfeldt-Jakob disease annually. According to the National Prion Disease Pathology Surveillance Center in Cleveland, 72 cases of classic Creutzfeldt-Jakob disease were reported in the United States from January through November 2003. Still, some say even the estimates seem low considering anecdotal evidence. "It couldn’t possibly be that rare," said Kranitz, noting six cases in northeastern Ohio. "It just doesn’t make sense." Arkansas had four cases in 2002 and at least four in 2003, according to state Department of Health statistics. That’s one in about 680,000 people. Carol Herrin said she doesn’t believe it is as rare as one in a million. Her husband died in October 2002, and Robert Hild, who also lived in North Little Rock, died of Creutzfeldt-Jakob disease four months later. "Here’s a disease they say one in a million gets and two show up in North Little Rock with it. Go figure," she said. Despite those two cases occurring near each other, there hasn’t been an unusual clustering of Creutzfeldt-Jakob disease in Arkansas, said Dennis Berry, a state Health Department epidemiologist. Kranitz said Creutzfeldt-Jakob disease is frequently misdiagnosed as Alzheimer’s disease, which causes similar neurological problems, but at a much slower pace. But physicians say the differences are too obvious for widespread misdiagnosis. "It’s just such a severe overwhelming problem, it would be hard to confuse it with something else like Alzheimer’s," said Dr. Lee Archer, associate professor of neurology at the University of Arkansas for Medical Sciences in Little Rock. Other people complain the disease isn’t tracked consistently or with the best information. Only 24 states, including Arkansas, require any kind of tracking of Creutzfeldt-Jakob disease. "This one to two per million I think is ridiculous," said Frank Bastian, the Tulane professor. "There’s no good epidemiological way they’re handling this. The CDC is using death certificates, and that’s not very reliable." But Dr. Shu Chen, with the National Prion Disease Pathology Surveillance Center, says that despite spotty reporting in the United States, the estimates for classic Creutzfeldt-Jakob disease are time-tested. "It’s not a reportable disease in this country," he said, "but it’s reportable in other countries. People have studied it, and this prevalence is very stable worldwide." Carol Herrin doesn’t believe that. She can’t stop seeing the disease everywhere she looks. She scans the obituaries wondering if any of them had Creutzfeldt-Jakob disease. She still has trouble sleeping. The image of her husband awakened by fright but unable to explain it still haunts her at night. But what bothers her most are the unanswered questions. When Harvey died, she said, "My whole life just went. And they have no idea where he got it or why."