Rare disease steals a local life

A rare, incurable brain disease, Creutzfeldt-Jakob disease, recently took local caterer Gayla Bishop from her family.

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Wilton "Bish" Bishop talks about his late wife, Gayla, who died unexpectedly this summer from a rare and incurable brain disease.

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Wilton "Bish" Bishop looks through a file of photos of himself and his wife, Gayla, at the family's dining room table. A card sits in front on him with a picture of Gayla taken on a recent trip to the mountains with fellow horsewomen.

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Wilton "Bish" Bishop and his son Gregg stand near the garden tended by Bish Bishop's late wife, Gayla.

He had 53 years, Wilton Bishop said. "And I'd give anything to have 53 more."

It was nearly seven weeks ago that Wilton - known as "Bish" his whole life - lost half of his heart when his wife succumbed to brain disease not long after symptoms began.

Gayla Bishop was 71 when she died at home last month of Creutzfeldt-Jakob disease, a rare, incurable illness. Recognized since the early 1920s, the degenerative brain disorder strikes fewer than 300 victims annually in the U.S., according to the Centers for Disease Control and Prevention.

Globally, Creutzfeldt-Jakob strikes about one in a million people. In Washington state, there may be six to eight cases a year, noted Harvey Crowder, administrator for Walla Walla County Public Health.

Because the disease, known as CJD, destroys brain material in the same way as bovine spongiform encephalopathy - "mad cow" disease - public perception is rife with misinformation, health-care officials say.

However, the disease in its classic ?form, such as Gayla Bishop suffered from, is distinct from BSE. People in the United Kingdom, however, developed a human variant of BSE after eating meat from diseased cattle in the 1990s.

All the variants affect the human brain similarly, Crowder explained. A protein-like substance appears to spontaneously begin shaping itself differently in the brain, he said.

"And then when they do that, they start making more copies of themselves. Those start taking up space and, along with that for some reason, this process is also accompanied by degeneration of nervous tissue," he said.

Eventually the brain is damaged to the point where it no longer functions, Crowder added.

And, as in most other cases of the disease, no one knows how Gayla Bishop contracted CJD.

The time frame of her illness was frighteningly short for the Bishop family.

It was about six weeks before his mother's Aug. 9 death that the disease first announced itself, said Gregg Bishop, the couple's middle son..

He was preparing for a vacation at the end of June when his parents dropped by "to yak a little bit."

His mom, who sang publicly for 50 years or more, was struggling to finish her sentences, Gregg recalled. "I thought it was a little stroke."

Which echoed Bish's concerns. "She tried to get words out and she couldn't get them out," he said of Gayla while sitting in a living room that his wife had designed and decorated. "I would finish her sentences for her, but then it got worse. She didn't know what she was talking about."

It was excruciating to witness, the Bishop men said. The woman who had a finger in any number of community pies, from Blue Mountain Riders to founding A Gayla Affair catering, found her world shrinking by the day.

Bish and Gayla met when the boy from Mississippi was in the U.S. Air Force and stationed near the tiny burg of Rhododendron, Ore.

The prettiest girl in town was 18 and working at her folks' general store. And she was still 18 when they married on Aug. 16, 1957, Bish said with an easy laugh. "I knew her less than a year."

The family arrived in Walla Walla in 1965, where Bish worked first at Safeway and then at a beer distribution plant. They eventually bought nearly three acres off Baldwin Road, building a house piece by piece and indulging a passion for horses. After a 17-year career in food service at Whitman College, Gayla decided to go out on her own.

A Gayla Affair allowed his wife to marry her twin passions of socializing and cooking, Bish said. "If everybody was eating, everybody was happy."

Gayla operated the business for two decades, hiring her husband after his formal retirement. She rode the family horses in Blue Mountain Riders while Bish rode in Wagon Wheelers, he recalled.

Although Gayla worked the flower beds largely alone, both volunteered at their church and traveled extensively.

"We went on a lot of cruises, from Alaska to the Caribbean. And where we really had a good time was the Las Vegas rodeo," Bish said.

"Those are just the big trips. They were gone every weekend." Gregg noted with a grin.

"We went everywhere we could afford to go," Bish agreed. "We kept the wheels rolling."

His parents were very close, Gregg later said. "They were kind of like lovebirds. Wherever they went it was always together."

That shared history made watching Gayla's vivaciousness drain away terribly painful, Bish said.

Creutzfeldt-Jakob changes the very essence of its victims, Crowder said. Because brain tissue is dying, symptoms typically include personality changes, anxiety, memory loss, uncontrolled muscular movements and difficulty swallowing.

"It changes people's behavior and they become demented," he added, "And they die very quickly. Classically, start to finish is about six months."

The effect becomes profound at the end, Crowder said. "You are watching your loved one die right before your eyes."

That was the hardest piece of news to bear, Bish said. Despite every conceivable test, prayer and diagnostic trips to Oregon Health & Science University in Portland and the Mayo Clinic in Minnesota, there was just one answer, the family found: There is no cure for CJD.

"I thought by getting her in, they could get her some kind of treatment. But it was a waste of my time. There is no treatment," Bish said. "You have to accept."

In the last two weeks of Gayla's life, the woman who knew everyone recognized no one, he recounted.

"She would wake up and it would be hysteria. It scared her to death."

People across the country were praying for his wife's recovery, but that came only with death, Bish believes, pulling his large blue Bible into the crook of his elbow. "God took her home anyway. He had a plan. I don't know what it is, but he had a plan. She's in heaven and I want to go there. I'm ready."

In the meantime, Bish Bishop has some earthly chores to attend. Gayla's beloved horses, Red and Chance, are ready for treats. By the backyard gardens created by his wife, the Tennessee Walkers wait by the fence for the apples Bish will pluck from a nearby tree.

It's here he can freely laugh without tears, holding his palm straight for the rubbery kiss of horse lips seeking every speck of fruit.

"They seem to know she's gone," he said. "We both loved horses."

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