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A Helpless Vigil

Peter Putnam has Creutzfeldt-Jakob disease, but how did he get it?

FRIDAY, Sept. 26, 2003 (HealthDayNews) -- Peter Putnam turns 35 years old today.

Some of his five siblings, along with his grandparents, will gather at his parents' home in Twin Lakes, Idaho, to celebrate what in all likelihood will be his last birthday.

It's unlikely that Putnam will recognize any of the relatives and guests. It's also unlikely that he'll make any sense when he talks. Almost certainly, he'll need help eating pureed foods and walking. About the only thing his family can still recognize in Peter Putnam is an occasional smile.

In February, Putnam was diagnosed with Creutzfeldt-Jakob disease (CJD), a degenerative brain disorder that causes a rapid onset of dementia and death. A form of the disease recently appeared in humans who had eaten contaminated meat in England.

The disease affects only one in 1 million people around the world each year.

There are three basic forms of CJD. The most common, which has no known cause, is called sporadic and accounts for about 90 percent of cases. Roughly another 10 percent are genetic and the remaining 1 percent are acquired through a transmission, such as that which can occur from unsterile medical tools.

Putnam's case is a mystery. The average age for sporadic CJD is the late 60s and the mean time to death is 5.5 months. Putnam is much younger and has already lived close to a year since symptoms began to appear. Genetic cases tend to be younger, but Putnam has no family history.

That raises the question of whether this could be the kind of CJD that comes from contaminated meat, such as happened in England recently. Although these patients tend to be younger, there has never been a case of this form of CJD in the United States, nor has Putnam ever been to Europe.

There's one other theoretical possibility, that Putnam contracted the disease from eating sheep, deer or elk meat from an animal that had chronic wasting disease. Again, this is unlikely. "There's no evidence as yet that that's transmissible from sheep or elk to man," says Dr. Richard Johnson, a professor of neurology, microbiology, and neuroscience at the Bloomberg School of Public Health at Johns Hopkins University School of Medicine in Baltimore. According to his family, Putnam wasn't a hunter, and it's unknown whether he ate game meat.

Instead of any answers, Putnam's family is left a set of looming questions -- and a son and brother who is rapidly dying.

Last October, Putnam confessed to his mother that he was having trouble remembering things. One night, he couldn't find his way home. Putnam's relatives noticed that when he came home for his grandfather's 80th birthday, he kept asking the same question over and over again. Soon he was showing up at his office (he sold commercial insurance in Anchorage, Alaska) at 2 or 3 in the morning to catch up. He was supposed to fly home for Christmas but never got on the flight. A policeman found him wandering around the airport.

In January, Putnam's boss put him on a plane home back to Spokane, Wash., where his parents were living, in the hopes that he would get the medical attention he obviously needed. An MRI, CAT scan, and spinal tap all came out negative.

Finally, his family admitted him to a hospital where he underwent 10 days of tests culminating in a brain biopsy. "That's the one we had to do because they could not find anything," says his mother, Jemie Turnley.

The doctors quickly saw that something was wrong with the brain fluid. One doctor approached the family. "There's no more question that you have a real sick boy on your hands," he said, according to Putnam's mother.

The samples were sent to the University of Washington in Seattle for confirmation.

Eight days later, Jemie got the worse news imaginable. Her husband, John, answered the phone and the doctor told him, "Put your wife on the phone and make her sit down. This is the hardest phone call I've ever had to make."

"What do we do now?" Jemie asked.

"Just take him home and love him," the doctor responded.

Soon after, Jemie and her husband moved Putnam to their lake home in northern Idaho.

He was declining rapidly. In December, he had delivered a keynote address to a business group. A month later, he could not form a complete sentence. "It's a terrible disease," Johnson says. "Cognitive function just dissolves from day to day. You can say something today and you can't say it tomorrow."

Already, Putnam's legs buckle up when he goes for walks in mountains he loves so much. He is incontinent and his mother expects that he will soon be bedridden. The one bright ray is that her will still occasionally turn his attention to a Mariners game on television.

For his parents and his doctors, there are still no answers. In all probability, Putnam has sporadic disease that just happened to occur in someone much younger that the norm. "There is a spectrum [of ages]," Johnson notes. Cases have been seen in people as old as their 90s and as young as Putnam, though it's rare.

Still, Putnam's brother, Chris, has arranged for his brain to be shipped to Case Western Reserve University in Ohio after he dies to see if experts there can come up with anything more definitive.

Putnam's mother believes he still knows everything that is going on. "The other night," Jemie recalls, "he just looked at me and held my hand."

More information

The National Institutes of Health has more on CJD, as does the CJD Foundation. For monitoring of CJD, visit Case Western's National Prion Disease Pathology Surveillance Center.

SOURCES: Jemie Turnley, Twin Lakes, Idaho; Richard Johnson, M.D., professor, neurology, microbiology and neuroscience, Bloomberg School of Public Health, Johns Hopkins University School of Medicine, Baltimore; Chris Turnley, Seattle
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