Family raises questions after rare disease kills Gallatin man - WSMV Channel 4

Family raises questions after rare disease kills Gallatin man

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GALLATIN, TN (WSMV) -

It's hard to image something so deadly it can steal your sight, speech, body and brain in a few months.

It is something so rare, doctors can spend an entire career and never see a case. It can spread, and it's virtually impossible to kill with chemicals, radiation or even incineration.

This disease killed a trucker in Gallatin just seven months ago, and this too is hard to image: no one was allowed to see or touch his body again.

Was that a necessary precaution, or tragic misinformation?

Jim Smith had an ordinary name, but he was no ordinary guy.

"He was crazy. Silly. Just laughed and carried on all the time," wife Joyce Smith said.

Jim was a tough-as-nails trucker and race fan, with a soft spot for his pet cat.

But in February, on a regular road trip to Florida, came the first clue that Jim, a guy with the most common name in the world, had a very uncommon, oddly-named problem.

"I had called him and asked him what he was doing, and he said he was parked alongside the road waiting on his mother to call him, and his mother's been dead for 10 years. And I knew right then," Joyce Smith said.

The experienced trucker was in a panic, had no idea where he was, or how to get home.

"That GPS," Joyce said. "He kept hitting the home button, and it told him how to come home."

His wife took him to a walk-in clinic first, then the emergency room.

The bad headaches might be an inner ear problem, they said. The disorientation, could have been a stroke.

"On March the 5th, I took him back because he got worse," Joyce said. "They told me there was nothing wrong with him, bring him back home. The ambulance came and got him the 13th because he had no idea who he was. No idea who we were."

Doctors tested Jim for encephalitis, rabies and tick fever.

"They asked me if he had ever ate monkey or monkey brains," Joyce said.

Had he been poisoned from truck exhaust he might have breathed in?

No, what was destroying Jim, and boring sponge-like holes into his brain, was a one-in-a-million disease called Creutzfeld-Jacob Disease.

CJD is not mad cow disease but is very similar and closely-related.

"He had seizures. The seizures were so bad he would come out of the bed, and he was tied in the bed. We couldn't hold him there, and they kept telling me they didn't know what it was. They did not," Joyce said.

Yet, hospital records show doctors did have a pretty strong suspicion from the start that Jim had some sort of what's called Prion disease.

The Prion may be the most vexing scientific problem still out there.

"It's not a bacteria, it's not a virus, it's not a fungus infection," Dr. Brian Christman said. "This is very different than anything else we usually see."

With no RNA or DNA helping it reproduce, it essentially breaks all the rules.

Somehow, something folds protein into a harmful form, duplicating to do its destruction.

"It's sort of like stacking spoons in a drawer. You keep putting them one next to each other and they line up perfectly, but suddenly if you turn one spoon the other way and you keep putting in the spoons, pretty soon there's this terrible disarray. And that's what happens in the brain tissue, and the brain tissue starts to lose its function over time," Dr. Christman said.

In practically no time, the disease is untreatable and universally fatal.

Radiation can't kill it completely, nor chemicals, nor super high heat.

And it can strike anyone, for no known reason.

"Did they ever mention CJD? No, no," Joyce Smith said. "I guess after he died, when they called me and told me he wouldn't be able to be seen. That he had CJD."

Joyce says she has concerns about how Jim's body was handled after he died.

"I want to know if he's in the grave, since nobody could identify his body. He was put in two body bags when the guy picked him up from the funeral home, and the guy was told do not open these bags under any circumstances," she said.

And asks how loved ones could be so close to Jim in the hospital, but kept away after he died.

"I couldn't understand, if you've got something, this disease is supposed to be so contagious. But yet still you have doctors, you have nurses, you have the people that draw the blood, myself, friends, step-daughter, everybody goes in and they hug him and kiss him. And then he dies, and then you're not allowed to touch him or nothing," she said.

It turns out that what Joyce was told how Jim's body was handled was unnecessary and wrong.

Funeral director Bob Kassai and his wife Marie have spent 20 years crusading for the CJD cause after Marie's mother died of it.

"If common sense is used and precautions are used, the family can have an open casket and there can be viewing," Bob Kassai said.

Bob says one phone call to the CJD foundation could have provided all the information about funeral preparation and precautions.

And like the early days of AIDS, fear and ignorance are leading to heartache for grieving families.

"My question would be to the pathologist: why did you double-bag this patient and think this patient could not be viewed," Kassai said. "The emotional impact that has on the family is not only unethical, it is almost like a crime. Because what you're doing is destroying these people, because they don't have the chance to say goodbye to their loved one."

Joyce Smith is still so troubled over the ordeal she says she's willing to exhume Jim's body to be certain his remains are really there.

"They evidently knew the day after I took him there what he had, most likely. The care I got was good. The information was not good at all," she said.

Jim Smith was a patient at the Nashville V.A. Hospital and his autopsy was performed at Vanderbilt.

We asked who gave the order not to open or prepare the remains for a funeral, but V.A. officials would not comment. Vanderbilt also declined an interview.

Smith's funeral director says he saw no choice but to follow what the medical professionals advised.

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