No link found between brain disease, game

But victim's family faults deer meat

By Brent Boyer
Special to The Denver Post

Sunday, September 29, 2002 - Otto Berns' family celebrated his birthday Aug. 17 with a solemn dinner at a local steakhouse, a marked contrast from the family's tradition of home-cooked venison roasts and backyard games.

Berns would have been 64 years old, but he died July 10 of complications from Creutzfeldt-Jakob disease, or CJD, a fatal brain disease that strikes one in a million people worldwide.

Like 90 percent of CJD cases, Berns was diagnosed as having "sporadic" CJD, which randomly inflicts victims with no apparent cause. The majority of people who get it, including Berns, are older than 55. By all accounts, his case fits the typical makeup of CJD victims.

But Nicki Berns, his daughter, thinks otherwise.

She believes his case, and numerous others, point to a more tangible cause of the horrific, rapidly occurring dementia that is CJD's calling card. She believes chronic wasting disease, a neurological disease ravaging deer and elk largely in the West, is the cause of her father's CJD.

Otto Berns was an avid hunter whose annual fall hunts in northern Colorado produced a freezer full of venison, which always was replenished by the following year's hunt.

"I love the taste of venison," she said. Hunting "was our source of meat. It would last us the whole year."

Ironically, it was the venison that killed her father, Nicki Berns said.

"I'm 100 percent certain," she said. "I have no doubts in my mind. There are too many hunters getting this disease."

Nicki Berns is not the first to express this belief. Other hunters who died from what is believed to be CJD have garnered considerable attention from national media, especially as chronic wasting disease spreads through deer and elk populations.

Experts still unsure

Health officials and medical experts have for years been unable to find a direct link between CJD and CWD, and studies have produced nothing to sway them from that position.

Dr. Ermias Belay, an epidemiologist with the U.S. Centers for Disease Control and Prevention, said extensive study of numerous CJD cases and steady rates of infection provide no evidence of a link between the diseases.

"There's limited data available in this area," Belay said. "The data we have indicate there is no relation."

But, he cautioned, the lack of evidence underscores the need to monitor the disease.

"I think we should be very, very cautious," Belay said. "We need to continue surveillance."

The CDC established the National Prion Disease Pathology Surveillance Center in Cleveland in 1997 to monitor and investigate all suspicious or atypical cases of human prion diseases, with the focus on CJD.

Creutzfeldt-Jakob disease is part of a family of diseases known as transmissible spongiform encephalopathies (TSEs). Mad-cow disease, scrapie and chronic wasting disease are TSEs.

Researchers believe TSEs are caused by abnormal prions, or proteins, that mutate and eventually eat holes in the brain.

Colorado, where chronic wasting disease was first discovered in 1967, also has a human TSE surveillance program. The goal of the program, said state epidemiologist John Pape, is to find and document all Colorado cases. To date, surveillance has not revealed a noticeable prevalence of cases involving hunters, he said.

"Most of our cases were not hunters," Pape said. "Everything we've looked at so far has turned out to be sporadic CJD."

Though the number of CJD cases in the state increased slightly over the past six years, from one documented case in 1996 to six in 2001, Pape attributed the rise to Colorado's population boom.

With 4 million residents, "we expect to see four or five cases a year," he said.

Area of interest

In early August, state health officials announced they would analyze death records from the chronic wasting disease endemic area in northern Colorado, and compare the number of people who died of brain diseases there with the death records of people in other areas of the state.

A higher rate of brain disease in the endemic area may indicate a better chance of a link between CJD and CWD, according to the state health department.

Most CJD victims die within a year of the onset of symptoms.

For Otto Berns, the end came much quicker. He died two months and seven days after the first symptom - memory loss - appeared. Nicki Berns said her father came home from his job as a baggage system worker at Denver International Airport on May 3 and complained he couldn't remember numbers that identify particular areas of the airport.

Three days later, a doctor told Berns he had suffered a stroke. Berns' condition continued to worsen. Initially, doctors feared he was continuing to experience strokes, but further tests ruled that out.

"All of a sudden he couldn't remember simple things around home," Nicki Berns said. He couldn't remember how to make a sandwich, put condiments on a hot dog or take a bath, she said.

On a walk around the neighborhood one evening, he stood awestruck in front of a sprinkler as it watered a neighbor's lawn. He acted like he had never seen one before, Nicki recalled, even though the family had sprinklers of their own for years.

"It was like a shell of my father," she said. "It just wasn't him anymore."

Doctors advised the family to admit him to a hospital, where more tests were performed, but no diagnosis came. On June 10, Berns was transferred to St. Anthony Hospital, where doctors would perform a biopsy on his brain.

The family was told to expect results within a couple of days. When those results were not available, the family pushed for answers. Eventually, they were told the brain tissue sample was sent to the National Prion Surveillance Center.

On June 19, the family was told he had CJD, a little-known disease with deadly consequences. With no treatment for the disease and Berns' condition declining at an alarming rate, the family opted for hospice in late June.

"He couldn't do anything," Nicki Berns said. "It was terrible."

Otto Berns' feeding tube did little to curb his rapid weight loss, and by the beginning of July he stopped talking. Nicki estimated that he slept up to 23 hours a day.

Nicki Berns now worries about the health of her 2-year-old twin daughters, who ate venison many times. She said she also ate deer meat while she was pregnant.

She remains unconvinced by health officials who assure hunters and others there is little risk in venison consumption. The state Division of Wildlife has issued guidelines for hunters who handle deer and elk carcasses in the state.

It is mandatory for hunters to submit the heads of all deer and elk killed in the endemic area of Colorado. There is no fee, and hunters are told to expect test results within a couple of weeks.

Last year, limited testing facilities led to a backlog of samples, and many hunters waited many months for results.

Belay said those guidelines are appropriate.

"We have made sure there's an immense amount of information available to the people who hunt in CWD areas," said division spokesperson Todd Malmsbury.

This year, the division has increased the number of testing facilities to produce quicker results for hunters.

The delays slowly unnerved Otto Berns' confidence with the Wildlife Division, Nicki Berns said.

"He didn't trust the state testing," she said.

It got to the point where she had to convince him to go hunting every year. After waiting six months for test results last season, Otto Berns said he had had enough.

There would be no more hunting seasons.

"He finally refused," Nicki Berns said. "It is so weird because (getting sick from eating venison) was what he was afraid of, and that's what he got."