Rocky Mtn News

Family fears deer caused dad's illness

Hunter is dying of Creutzfeldt-Jakob

By Lou Kilzer, News Staff Writer

June 29, 2002

Otto Berns didn't hunt for deer or elk last season at his favorite spot north of Fort Collins.

He hadn't wanted to hunt there the season before, but his daughter talked him into it.

Berns was afraid of chronic wasting disease. He didn't trust the test results he got back every year from the Colorado Division of Wildlife which stated his kill was CWD free. He had hunted in an area where the disease is widespread, and he didn't want to take another chance.

On May 3 he came home from work more frightened than ever. Berns, a baggage system mechanic at Denver International Airport, told his family that he was suddenly suffering from memory problems.

"When there is a bag jam, they call out certain areas and you have to go clear that bag and he couldn't remember his locations," said Berns' daughter, Nicki Berns, herself a baggage mechanic at the airport. "He was really upset that he couldn't remember locations."

Today, Otto Berns is in hospice, dying of Creutzfeldt-Jakob disease, which, like CWD, is a transmissible spongiform encephalopathy, but one that occurs naturally in humans.

There are no known cases of CWD causing CJD in humans, and Bern's age -- 63 -- and the speed of his disease are similar to the sporadic, naturally occurring version of CJD.

But her father's seeming premonition that something was wrong with the deer he ate is too much of a coincidence for Nicki Berns. She says she's convinced that the meat she loves caused her father's illness.

There is no proof of that, and there might never be.

A biopsy of Berns' brain was sent to the National Prion Disease Pathology Surveillance Center at Case Western Reserve University in Cleveland. In a letter Nicki Berns received this week, the center said her father suffered a disease caused by a mutant protein called a prion. The center said the biopsy was "consistent with the diagnosis of Creutzfeldt-Jakob disease."

Dr. Shu Chen, head of protein analysis at the center, said that wording does not indicate whether a person had classical CJD or some variant form. That can only be determined with an autopsy, if even then.

Berns' case is important because it is only the second one reported in a hunter who took game from the Colorado/Wyoming endemic area for CWD. Gary Hopkins, a 53-year-old Aurora man, died of CJD in October 2000. Other hunters have died of CJD, but none was known to have hunted in areas where the disease is prevalent.

For now, though, Berns' case will remain a question mark. It is a case that has caused concern, rational or not -- just as the cases of the other afflicted hunters cause concern to their families.

Scientists and politicians alike acknowledge it will be a concern until much more is known about CWD and its potential for transmission to humans.

At the very least, Bern's case illustrates the cruel nature of a disease that destroys memory, mind and, quickly, life itself.

After Nicki Berns moved to Colorado in 1992, she kept in touch with her immediate family in Wisconsin. Soon she discovered the big difference between the two states. "In the wintertime, it would be 30 below in Wisconsin and 60 above here," she said with a smile.

Her parents grew envious and moved to Thornton to be close to their daughter.

One thing father and daughter had in common was their love of hunting. In fact, Nicki Berns hardly ever bought meat at grocery stores, preferring venison to beef.

Father and daughter had a favorite spot to hunt -- near Red Feather Lakes, northwest of Fort Collins.

CWD was already a concern among wildlife managers in the mid-1990s -- not so much as a human health risk but as a nasty disease afflicting deer and elk.

That began to change in 1996 when a similar disease in British cattle was found to have crossed the species barrier to infect humans. The advent of "mad cow" disease in people sparked renewed interest in CWD in Colorado wildlife.

Nicki Berns says she now knows that the testing process cannot detect CWD in deer or elk for several months after an animal is infected and that the incubation period before an animal shows obvious symptoms of disease can last over two years.

But she didn't know it at the time.

Otto Berns grew concerned about CWD and this year put his foot down.

"This year when I was applying for my licenses, he said, 'I'm not hunting. I don't trust the deer meat,' " Nicki Berns said.

Then came May 3rd, and the start of Bern's symptoms.

Nicki Berns reminded her mother, Barbara Berns, to mention the deer meat as she prepared to take Berns to the doctors.

Nicki said that after doctors diagnosed that Berns had suffered a stroke, her mother didn't mention her husband's fear of venison.

Berns continued to weaken, and doctors soon realized he hadn't suffered a stroke. They took spinal fluid to check if he had meningitis. Negative.

Nicki Berns began mentioning her father's affinity for deer meat, but doctors assured her that there were no known transmissions of CWD to humans in the United States.

All the while, Berns continued to deteriorate.

"He couldn't even make himself a sandwich," Nicki Berns said. "We would be eating supper and he would sit there and he didn't know how to put together a hot dog. Everything was sitting in front of him. And he had the hot dog. And he didn't know what to do with it."

Finally, he couldn't tell time. "He woke up at 2 in the morning and got dressed for an appointment that wasn't for 3:30 in the afternoon," Nicki Berns recalls.

By June 10, stumped doctors were out of options. They had to consider that Berns might have CJD.

They performed a brain biopsy, sent it to the University of Colorado Health Sciences Center, which, in turn, sent it the prion surveillance center in Cleveland.

Eight days after the biopsy, the center sent a letter to the university giving its diagnosis of CJD. The final paragraph in the letter read: "Thank you for referring to us this interesting case."

Nothing will convince Nicki Berns that her father's case was a random case of sporadic CJD. She says she senses a cover-up.

She also says she wants to invite everybody that disagrees to come over to her house.

"We'll have a giant barbecue,"she said. "I've got two freezers full of deer meat."

John Pape, an epidemiologist for the state health department, said the best that officials can do to determine the type of CJD a person suffers is to examine brains at autopsy.

So far, the brains of all hunters and venison eaters who have died of CJD do not show signs of a new strain of prion disease. That does not rule out CWD as a cause, because it's possible CWD could mimic classical CJD.

But if the cases of mad cow disease in humans are any road map, CWD would leave its own, definite biological fingerprint.

Nicki Berns isn't buying that.

She arrives at the hospice every day after work and sits with her father, who is now largely vegetative.

And she worries.

Not only did she eat the same meat as her father, but she fed it to her twin girls. "I worry about them," she said.

-- Anonymous, June 29, 2002

Answers

I am eating less and less meat..always been a "meat and potatoes" solidly built country girl....but now am trying to do fruits and veggies more.

but I sure do miss a good steak!

-- Anonymous, July 01, 2002

Steak withdrawal--ya know, a properly marianted and cooked whole portobello mushroom is a wonderful thing! Don't ask, the cookbooks are packed.

-- Anonymous, July 01, 2002

I was thinking of it marinading in my dad's stash of home made.....Stuff...it used to be wine and brandy, but now it will surely grow hair and any number of other things.

I love the delmonico, that melts in your mouth!

-- Anonymous, July 01, 2002

Bunch of recipes came up when I asked Google for "Marinated Portobello Mushrooms." This looks good; have also left in the other recipes on the page. Don't just go with the recommended wine-- anything in SAR's stash would be good, you can even use up those odds and ends of weird liqueurs you bought in the seventies--remember alcohol will flare up on the grill so stand back. Whether using charcoal or gas, many barbecuing basics remain the same. SNIP - But it's the recipes that really shine. Even the basic ones like Salsa 101 are a good brush-up for use on burgers, steaks, chops and fish. (Marty Meitus writes for the Rocky Mountain News in Denver) Salsa 101
Grilled, Marinated Portobello Mushrooms
Napa Red Wine Marinade Salsa 101 Ingredients: 1-1/2 lb. ripe plum tomatoes, seeded and chopped
1/3 cup finely chopped red onion
2 Tbs. fresh lime juice
2 Tbs. chopped fresh cilantro
1 to 2 tsp. seeded and minced jalapeno chilies
garlic clove, crushed through a press
salt
Preparation: Cut the tomatoes in half through their equators. Using your finger, poke out the seeds and cut the tomatoes into half-inch cubes. In a medium bowl, mix the tomatoes, onion, lime juice, cilantro, jalapeno and garlic. Season with salt. Cover and let stand at room temperature for an hour to blend the flavors. Yield: about 2-1/2 cups Grilled, Marinated Portobello Mushrooms Ingredients: 6 large portobello mushrooms
1/2 recipe Napa Red Wine Marinade (see below)
salt
Preparation: Build a charcoal fire in an outdoor grill and let it burn until the coals are covered with white ash. If using a gas grill, preheat on high. Meanwhile, remove the mushroom stems and save for another use. Wipe the caps with a moist paper towel to remove any dirt. In a large glass dish, combine the mushroom caps and the marinade. Let stand, turning occasionally, for 10 to 15 minutes. Lightly oil the cooking rack. Place the mushrooms on the grill and cover. Cook, turning once, until tender, about five minutes. Season with the salt and serve hot. Yield: 6 servings Napa Red Wine Marinade Ingredients: 1 cup hearty red wine, such as cabernet sauvignon or zinfandel
1/3 cup balsamic vinegar
1 Tbs. chopped fresh rosemary (or 1-1/2 tsp. dried)
1 Tbs. chopped fresh sage (1-1/2 tsp. dried)
1 Tbs. chopped fresh basil or (1-1/2 tsp. dried)
2 garlic cloves, crushed under a knife
1/2 tsp. salt
1/2 tsp. crushed hot red pepper flakes
1/2 cup extra-virgin olive oil
Preparation: In a large bowl, whisk together the wine, vinegar, rosemary, sage, basil, garlic, salt and red pepper. Gradually whisk in the oil. Use immediately. Yield: about 2 cups OG Note: I would add that you can reduce what's left of the marinade and make a very good sauce--just heat to boiling and simmer lightly for about ten minutes. Again, watch out for the alcohol--have a lid ready so if it flames you can simply pop the lid on it. If it doesn't flame, light it when you pour it over the food!

-- Anonymous, July 02, 2002