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June 5, 2008

Losing Stephanie

The temper flare-ups and misspelled e-mails were the first clues. Why was this charismatic life force growing less recognizable by the day?

creutzfeldt jakob disease

Jim and his youngest, Demitri.

Photo Credit: Juliana Sohn

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Jim Papoulis pushes hard on the accelerator, racing through the blackness back to Manhattan from the eating-disorder clinic in Princeton, where his frail and delirious wife, Stephanie, is sobbing on the floor.

He runs into their Upper West Side brownstone on this late June night, riffles through Stephanie’s purse, and plucks out a business card. Holding his breath, he punches the digits into the phone. It’s 9:30 at night. Crazy, he knows. But he’s a desperate man.

“Do you remember my wife, Stephanie Martini?” Jim asks Dr. Mehmet Oz, of Oprah fame. Though Dr. Oz had only met Stephanie briefly, at a luncheon the previous May, of course he remembers her, he says. Stephanie Martini is not someone you forget. “I need your help,” Jim says as the tale tumbles out of his mouth.

In just a few short weeks, Stephanie has morphed from a vivacious, energetic, brilliant woman, Jim says, to a person who has trouble speaking, eating, and walking. She’s been in and out of the hospital, and no doctor has been able to tell him what the hell is wrong with her — that’s why she’s in Princeton. Her friends, completely frantic, thought that since some 30 pounds had melted off her 5-foot-7 frame seemingly overnight, maybe she had an eating disorder. Maybe a B-12 deficiency could explain all her bizarre symptoms.

But now that she’s locked down in the clinic with a bunch of 15-year-olds, it’s clear that something much more serious is ravaging her body and her mind. Stephanie, a Phi Beta Kappa graduate of Vassar College — a former soap-opera actress, for Christ’s sake — can’t even tell the difference between shampoo and conditioner. Just the other day, her sister, Andrea, looked Stephanie in the eye and thought, This is not my sister; I know this is crazy, but could she be possessed? Is that possible? As Stephanie crashes between screaming and sobbing, cursing and withdrawing, everything familiar about this 45-year-old woman is gone. Could someone this high-functioning, this capable, just crack up and completely lose her mind in a month? Jim asks the doctors daily. Yes, they say, but more likely she’s severely depressed and should be admitted to the psych ward - though every time they say that to Stephanie, she screams, “I’m not fucking depressed! Something’s wrong with my body, and I don’t know what it is!” Then she tries to escape, and Jim has to physically restrain her until she crumbles, depleted and defeated, to the floor.

Yesterday, the head of the clinic finally told Jim that if this were his wife, he would get her to the best neurology clinic in the country — New York University Medical Center — and have them exhaust every possiblediagnosis. Jim called. There is a six-month wait. Stephanie clearly does not have six months.

“Can you help us?” Jim asks Dr. Oz.
Five minutes later, the phone rings in the brownstone. It’s the neurology department at NYU. Stephanie will be transferred first thing in the morning.

When they wheel her in on Saturday, she weighs less than 100 pounds and looks, says her neurologist, Dr. Harold Weinberg, like she’s been through a Holocaust experience. She’s barely verbal, except when she’s cursing at him. “Are you like all the other bastards?” she screams, and then refuses to let anyone touch her for even the most rudimentary tests for three days. This must be a psychiatric case, Dr. Weinberg thinks.

But with each passing day — with each passing hour — Stephanie gets worse. She’s no longer able to lift her head to throw up, and she can’t control her bowels; she chokes on her drool as her body convulses with seizures. The doctors begin to run neurological tests on her. One by one, they rule out everything that might explain her symptoms — thyroid issues, infections, vitamin deficiencies — until only one explanation remains. They tap her spine and send the fluid out to Cleveland, to the only laboratory in the entire country that can run this particular test to confirm their suspicions.

It will take 10 days to get the results. In the meantime, doctors continue to hope that this is, indeed, some-thing psychological. “My sister is not crazy,” Andrea, exasperated, says to one doctor.

“I just want to caution you, that would be the better scenario,” the doctor replies. “If you’re going to pray for something, pray for it to be something psychiatric.”

The day Stephanie came screaming into the world — on Valentine’s Day, of course — her grandfather stood on his head, right there in the Philadelphia hospital. That was the kind of reaction people had to Stephanie. You just couldn’t help but be drawn in by her crackling charisma. “Stephanie did more things in a day than I did in a month,” says Andrea, who clearly idolizes her. “She was always in motion. She would exhaust all of us.” Stephanie was so busy chasing down life that her father, a college professor, had to sing “Ah, Camminare” nightly — in Italian — to get the whirling dervish to fall asleep.

She danced naked with her brother and sister, singing the off-color lyrics to “Hair” at the top of her lungs; she also petitioned her Catholic church to accept female altar girls (but was denied). “She was an incredibly intellectual person but an actress at the same time,” says her close friend Dawn Trachtenberg. “I think that a lot of people didn’t really know the depth of her intellect and the seriousness that was underneath the drama and theatrics that they saw.”

But that’s the woman — “the very smart drama queen,” he calls her — that Jim Papoulis, himself an interesting cocktail of Ivy League athlete and music conductor/composer, fell in love with and married 18 years ago. They lived as near a fairy-tale life as can be written — walking their three children through Central Park to school every day, collecting interesting and artsy friends, and throwing rocking parties, such as Stephanie’s yearly “Come With a Heart-On” Valentine’s/birthday blowout.

All of which would have made it easy to peg and dismiss Stephanie as just another pampered and entitled uptown wife — except that she was genuinely grateful for, and wanted to share, her good fortune. She worked tirelessly to raise scholarship funds at the Rudolf Steiner School, her children’s very expensive private school, so that less-fortunate kids could also attend. And after Jim’s music company, which Stephanie managed, became successful providing pop tunes for the likes of Aretha and Celine, film scores, and orchestral pieces, Stephanie created a foundation for children’s charities in 1999, called the Foundation for Small Voices; their gift drive has given away tens of thousands of presents over the past eight years

“Stephanie was a person who you stood back in awe of and watched the speed at which she was able to get things done,” says Dawn, with whom Stephanie volunteered at the Steiner School. “Many of us would look at her and say, ‘We can’t keep up with you. You get done in a day what it would take 10 people to do.’”

And so, the day Stephanie stood outside the school in late May, struggling with her BlackBerry and telling Dawn that she felt like her brain could not keep tabs on all of the things she needed to do, Dawn just thought, Of course you can’t. You simply can’t cram that much living into a life. What else would you think?

The spelling mistakes are the first clue. Stephanie simply does not make spelling mistakes. But it is the day in late May when Stephanie tells Jim she can’t read a phone number he had just e-mailed her that he knows something is wrong. There’s a helplessness in her voice. It strikes Jim right in the gut.

He has ignored some weird behavior in the past few months. Stephanie, normally optimistic and loving, was suddenly plagued with irrational fears — keeping him up late at night worrying that if he didn’t work for 16 months they would be homeless — and it was starting to annoy him. Stomping around, her brow constantly furrowed, he says, she just wasn’t fun anymore. One day he called his oldest daughter’s cell phone while he was away on a business trip. All three kids were hiding out in the park close to dinnertime. “We just want to stay out of Mom’s way,” his daughter explained. “She gets so upset.” Jim was stumped. Stephanie was a nurturing mother. So nurturing that their youngest son, Demitri, could only calm himself when upset and drop off to sleep at night by putting his finger in her belly button. She was his security blanket.

But by now, in late May, her symptoms can no longer be ignored. Stephanie is forgetting things; her weight has plummeted from 130 to 105; she is unsteady on her feet; her handwriting looks like that of a young child — she can’t even form the S in Stephanie. She blames it all on stress. Everyone believes her. Between refinancing and renovating the brownstone, as well as juggling the foundation work and the school fundraising, she has simply hit a breaking point, she says, and needs to unplug for a few days. She heads up to her sister’s place an hour outside the city but just gets worse. Andrea begs her to see a neurologist, but Stephanie refuses. “I think she was afraid that she was losing her mind, going crazy,” Andrea says. “And that scared her.”

Stephanie is clearly scared by the time she is taken to Princeton in mid-June. If anything happens to me, she says to Dawn and another friend, please tell my children and my husband I love them, and tell funny stories about me. “I think,” says Dawn, “she knew that she was not going to come out of this.”

Her loved ones are considering that possibility, too. As she lies in bed on the neurology floor at NYU, some days — like the time she hums along with her favorite musical, Wicked — they cling to hope. But most days, she seems catatonic. Seven weeks into this nightmare, they still have no idea why.

“It’s called Creutzfeldt-Jakob Disease,” Dr. Weinberg says.

It’s 4th of July weekend when Stephanie’s neurologist gathers the family together. The test results won’t be back for a week or so, he says, and while we should all hope for the best, it is time to prepare for the worst. CJD is a very serious brain disorder, he says. Please don’t Google it.

But Jim fires up his computer anyway, and for the next six hours, he reads with horror about this mysterious and controversial disease. CJD (aka “classic CJD”) is one of a group of degenerative brain disorders known as TSEs (transmissible spongiform encephalopathies) — like mad-cow disease in cattle — in which it is believed that an infectious and indestructible form of a protein called a prion invades the brain and creates spongelike holes. As the brain disintegrates, the human or animal descends into dementia and loses all control of its body. There is no treatment for CJD; it is 100 percent fatal.

All the symptoms — the weight loss, the personality transformation — fit. So when the test results on Stephanie’s spinal fluid come back from the government-funded surveillance center at Case Western University in Ohio a week or so later indicating that she does, indeed, appear to have CJD, Jim is not surprised. It barely even registers when a woman from the Centers for Disease Control shows up at the hospital and gives him her card. She is probably there to make sure his wife doesn’t have mad-cow disease, he thinks.

The public was first introduced to bovine spongiform encephalopathy (BSE), or mad-cow disease, in 1986, via images on the news of staggering and falling cows throughout the English countryside. Scientists traced the likely cause of the epidemic to the shocking practice of diseased cows being ground up and fed to other cows. Ranchers had in effect taken an animal that was an herbivore and not only forced it to be a carnivore but also a cannibal.

It had been assumed that CJD, the very rare human TSE, could not be contracted from eating diseased meat (beyond isolated cases involving human cannibalism in New Guinea); it almost always occurred spontaneously, for no reason beyond apparent bad luck. But then, in 1996, alarm broke out in British medical circles when 10 citizens came down with a new type of CJD, which would be identified as variant CJD (vCJD); another 156 cases in the U.K. would be diagnosed by 2007. Scientists linked vCJD to the victims’ eating mad-cow-contaminated beef, prompting the government to slaughter and incinerate up to 4.5 million cows, a full-blown catastrophe for the British beef industry.

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