Thirteen
BURDEN OF PROOF
IN NORTH DAKOTA, threads were beginning to pull loose from the carefully mended fabric of Gail’s family. When the lab sent Brian’s and Doug’s genetic testing results, the accompanying letter also recommended genetic counseling for the rest of their relatives. Karla swung into action, contacting John Martsolf, head of the Division of Medical Genetics at the University of North Dakota’s School of Medicine and Health Sciences. For the sake of efficiency, they set up a single meeting with him for the extended family in August 2004, inviting all the siblings, siblings’ children, cousins, aunts, and uncles they could find. Karla was thorough, arranging to videotape the meeting for anybody who couldn’t attend. Jerry’s widow, Sharon, and their two daughters in Oklahoma were among those who missed it.
Among those who did make the trip were Moe’s sister, Pat, now sixty years old, and her three grown daughters, Dawn, Robin, and Colleen—Gail’s nieces, and cousins to the DeMoe kids—all of whom still lived in northern Wisconsin and now had families of their own. For three years, Pat had been exhibiting signs of Alzheimer’s disease—much later than her brothers Moe and Jerry. At the meeting, Pat saw herself in a monitor that was connected to the video camera Karla had brought. She squealed in childlike delight: “I’m on TV!”
Dawn, the oldest of Pat’s daughters, enjoyed a close connection with her cousin Lori McIntyre. Both were creative, lively women who had the knack for making friends wherever they went.
At the meeting with Martsolf, Dawn asked insightful questions. Since her midthirties, she had developed a habit of repeating herself. Her moods were starting to shift dramatically, too, straining her relationship with her daughters. Doctors had prescribed antidepressants, but her symptoms weren’t improving. Now she was forty-two, and her work as an architectural draftsman was beginning to suffer. She was looking for answers.
• • •
Steve McIntyre made the trip to gather information, but Lori did not. After learning of her brothers’ diagnoses, a nugget of fear had settled in the back of Lori’s consciousness. Though she had taken the practical step of applying for insurance to cover her long-term care if necessary, neither Steve nor her daughters noticed any changes. Still, she continued to quietly worry, and her fears were starting to erode her usual upbeat confidence. Of course, her self-perceived “symptoms” could simply be aftershocks from the stress of learning about Brian and Doug.
But in her heart, she didn’t think so.
Eight months before the family meeting, she had called her three best girlfriends, all still living in Nebraska, and suggested they meet in Cheyenne for a slumber party on New Year’s Eve. The occasion was vintage Lori, replete with party hats, noisemakers, and belly laughs. Her best friend, Robin Tjosvold, took photos of them cutting up with the other patrons at the hotel bar: for one night, they were neither mothers nor wives, just four women living in the moment, acting like the teenagers they’d left back at home.
The next morning, when everyone was awake, Lori went into the bathroom, pulled out the hotel-supplied box of Kleenex, and dropped it in the middle of the bed. She gathered her friends in a circle and announced the real reason she had called them together: “I have to talk to you about something.”
And then she confessed what she feared: She had Alzheimer’s disease. The memory of her father’s descent into madness weighed heavily on her mind, and she was worried about the three daughters back home who would soon be adults—fearless, she hoped, but still fragile, still in need of their mother. She asked her friends to make a vow that they would do for her what she, had the circumstances been reversed, would have done for any of them.
“If I do have it, and it’s going to run the course it had with my father, then I need you guys to promise me that you will be mothers for my girls and grandmothers for the grandchildren that I don’t have yet,” she said. “You have to promise me those things, because they will need you.”
Robin was skeptical. A nurse with experience working in hospice care, she hadn’t seen any changes in her friend; she thought perhaps Lori was simply emotionally overwhelmed. But the women, sitting in a hotel room on New Year’s Day 2004, vowed that they would do as she had asked.
Just as they had throughout their relationship, Steve and Lori moved again—this time to Alpine, Texas, for the start of their youngest daughter’s sophomore year of high school—to follow Steve’s Union Pacific railroad job. Their daughters Jessica and Robin, now old enough to live on their own, stayed behind and began their adult lives as workingwomen. They decided to move to Denver, where they talked about one day opening a beauty salon together.
At first, Texas was like every other state the McIntyres had lived in: an adventure. Lori served as a companion for a fussy elderly woman who needed someone to drive her around on errands. The woman drove her own daughters crazy, but she and Lori got along. Like Gail, Lori had a warm, empathetic personality that drew people to her.
“We clicked,” Lori said, though she had nothing bad to say about the woman’s daughters. “It’s different when it’s not your mom.”
Later, she went to work at the high school as an aide for the gym teacher, setting up equipment—the same routine, every day. Steve remembered this detail distinctly whenever anyone asked him when he first noticed that Lori was laboring with her memory.
He didn’t notice anything different, but the gym teacher did. Lori could not remember the same instructions from one day to the next. It was a strange disconnect, because at home, she was as self-sufficient as always. Steve wasn’t sure what it meant.
• • •
The year after the meeting with Martsolf, Dawn went to the Mayo Clinic and learned that she was positive for the mutation. She had been visiting a friend in downstate Wisconsin, and she got lost on the way home. What should have been a four-hour trip took ten. Worse, a state trooper had found her driving the wrong way on Interstate 84, against ongoing traffic.
The Mayo Clinic reported that the extent of her brain damage was much worse than she had anticipated; her forty-three-year-old brain was equivalent in size to an eighty-year-old woman’s.
Dawn’s college-age daughter, Leah, was furious at how long the health care system had taken to diagnose her mother.
“She had been going for years to her behavioral health doctor. She’d been going on for years saying, ‘Something’s wrong. I have this in my family.’ And no one would put it out there as ‘You’re sick,’ ” Leah said. “It was for years. She was trying to tell somebody.”
Dawn’s mother, Pat, also had the disease, though she was almost twenty years older when her symptoms began to show. But Dawn was so young that she was misdiagnosed as depressed. It was a common mistake in early-onset patients, because the diseases shared many of the same symptoms: memory loss, impaired concentration, interruptions in sleep patterns.
Dawn wasn’t the only person affected by the misdiagnosis. Her marriage crumbled under the strain of her mood swings. Her daughters were robbed of their mother for much of their childhood, often left to fend for themselves when Dawn was incapacitated. Her extended family offered little support to help any of them weather each crisis.
Still, Dawn was hopeful. After she finally learned it was Alzheimer’s, the doctor reported that science seemed close to finding a cure. The idea bolstered her confidence. When she started taking Aricept (the trade name for donepezil, a widely prescribed drug meant to alleviate Alzheimer’s symptoms), she announced that she felt smarter. That matched the findings of a study that would be published in 2012 suggesting that patients taking Aricept would perform cognitively better for about a year longer than patients who took a placebo.
Her younger daughter, Alayna, was skeptical that her mother could be cured: “How do you grow a brain back?”
• • •
After Brian and Doug were diagnosed, Dean and Karla agreed that as part of their pact to oversee their brothers’ care, they would share power of attorney so they coul
d jointly make difficult decisions. Remembering the toll the disease had taken on Gail’s emotional health when their father deteriorated, they wanted to alleviate her burden. Since they were both still in North Dakota, where they could physically assist with Brian and Doug, their offer seemed to make sense. Gail was grateful that her children were willing to shoulder the burden.
At Karla’s urging, Dean agreed to fly to Bethesda to participate in Trey Sunderland’s study at the National Institute of Mental Health, which compared biological samples such as blood and spinal fluid to people’s symptoms and needed controls in addition to active subjects.
The biggest takeaway Karla brought back from her first visit with a genetic counselor was what a positive diagnosis of the mutation could mean for her children, Amber and Cole. They were her greatest joy, such bright, happy kids, so focused on their family. They were both in college now, and starting to plan futures; Cole was training to become a golf pro, and Amber was training to become a physician assistant. Karla simply could not stand not knowing if she’d somehow saddled them with faulty genes. Finally, she called Sunderland and told him she was ready to learn her status.
In October 2004, two months after her initial blood draw, Karla returned to Bethesda, this time just with Matt. They tried to distract themselves with sightseeing but were too restless and worried. They had decided not to tell either of their kids where they were going or why. If it was bad news, they’d wait until the college year was over, then break it to them over the summer.
The next day, Trey Sunderland sat down with Matt and Karla, who were sitting together like the high-school sweethearts they had been. They listened quietly as Sunderland began unspooling his usual speech about what they should expect if they learned Karla had the mutation. Finally, he interrupted himself and asked if Karla would just like to know.
“Yes,” she said.
She was free.
For the rest of her life, she would never forget that moment, never forget the relief and joy that washed through her, knowing that her children, and their children, would forever be safe from this unbearable inheritance. Cole and Amber had just been given the greatest gift of their lives.
Sunderland and the staff were elated to give her the news. Karla and Matt were euphoric. Finally, after so much fear and desperation, they had something to celebrate.
They left messages for everyone they could think of. When Cole answered the phone in his dorm in Michigan, he was surprised to hear his mother sobbing about the good news—he hadn’t even known she was away, let alone where she was.
The next day, Karla and Matt flew back to Fargo, arriving early enough that they both went back to their jobs to finish out the workday. Karla’s coworkers surprised her with balloons and a cake frosted with the inscription: Now your only excuse is you’re blonde.
• • •
Inspired by his sister’s celebration, Dean told Sunderland he also wanted to know his genetic status and flew to Bethesda in October 2005 to learn the results. Since he and Karla were already making plans to assume legal control of their siblings’ care, he figured he might as well make it official.
Deb and Dean sat impatiently in Sunderland’s office, Dean in a distracted fog, waiting for the doctor to deliver their moment of truth.
“You just want to know, don’t you?” he asked, as he had with Karla. And then the bomb dropped: Dean had tested positive.
Deb remembers crumbling from the shock. How could he have it? There wasn’t a thing wrong with him; he was clever; he was different. He worked with his brain as well as his hands. He didn’t process information the same way as his brothers.
Her mind screamed at the improbability of what she’d just heard.
At first, Dean did not speak. His thoughts remained purely his own. He was a son of Galen DeMoe, a veteran of the hell that was this disease. He refused to allow the news to touch him.
He would not pity himself. There were people counting on him. His children; his wife.
He was a man who provided. He was a man, period.
Finally, he turned to his wife and said something she would repeat many times: “Deb, it’s not what you’re dealt in life. It’s how you deal with it.”
But wasn’t this situation different? she wondered.
“To me, you can apply that to anything,” Dean said.
Years later, she would still cry as she recalled how stoically her husband reacted to the worst news of his life. His first instinct was to reassure her. He took her to the building’s fourth floor, where pediatric cancer patients were fighting desperately for survival.
“You want to feel sorry for me? Look at those kids who aren’t even three or four and know they’re terminally ill, and don’t even have a chance,” he insisted.
Later, she would write down what he’d said, and draw strength from it.
Looking back, she said, “I knew, right then, he was going to be just fine.”
They returned to their motel room, knowing that the whole family was waiting for the celebratory phone call. They’d all been so confident that Dean had escaped the mutation. The disease had fooled them all. How could the two of them even begin to explain that mistake?
They told Karla first. It was morning; it felt like the last day of the world. Someone drove her home from work. Her mother. She didn’t want Gail to be alone when she heard the news. She called one of Gail’s friends to go to the house to be with her when the call came from Deb and Dean.
Like Dean, Doug had said nothing immediately after his own diagnosis. But when Doug learned about his brother, his closest sibling, he wept inconsolably.
Then Karla went to her bed, where she stayed for days. Lori was so far away; Jamie was so young. There was no one to help her. Her beloved younger brother was going to leave her. She was alone.
After a few difficult phone calls, Deb and Dean stopped calling people. Sunderland had been right to make them come to Bethesda to learn the diagnosis; such news should not be delivered over the phone. They decided to head home and tell the kids in person—their children, so loved, the center of their lives. Now they, too, were in danger.
Sixteen-year-old Tyler was playing in a basketball tournament that week, while nine-year-old McKenna stayed with her aunt’s family in Thompson. When the kids were finally home, Deb and Dean sat them down. The room went silent and tears welled in everyone’s eyes as Dean revealed the news.
Lindsey Sillerud, Dean’s older daughter, was attending college in Bismarck. Though she and Dean had stayed close, she had not yet heard from him when she opened her email and noticed a message from her cousin, Karla’s daughter.
I’m so sorry to hear about your dad’s test results, it read. I really didn’t think he had it.
Lindsey sat in shock, looking at the electronic shimmer of the letters on her screen. Karla had broken the bad news to her own kids before her father had ever had a chance to tell her.
“One thing with the DeMoe family: Once you tell somebody one thing, it goes,” she said later. “It spreads like wildfire.”
She called Dean, who apologized.
“That’s not how we wanted you to find out,” he said. He had wanted to tell her himself, in person, and not by phone; it hadn’t occurred to him that she might find out some other way before he had the chance to do that. As with Tyler and McKenna, he tried to reassure her that everything would be fine.
She was breathless. It was the first time she had ever imagined something bad might happen to him.
• • •
Two nights later, while Dean was fast asleep, Deb heard a noise that woke her. Downstairs her nearly grown son was sobbing. She went to him.
“Baby,” she said, and gathered him into her arms, his long legs draped over hers, and let him cry. They talked for hours, all through the night. That was the only time Tyler DeMoe really broke down, thinking about his father.
Fourteen
THE RISE AND FALL OF GOLDEN BOY
FOR BOTH THE DeMoe and Noonan famil
ies, Trey Sunderland had felt like a godsend. Not only was he empathetic to their disease, but his role at NIH gave him real power to help them; he was heavily involved in research that seemed to be truly meaningful. At last, they felt as though they had found someone they could trust, who offered them hope. He was beloved at the NIH as well—his office nickname was Golden Boy—yet Sunderland’s days as the families’ savior would prove to be numbered.
Depending on whom you ask, Sunderland either got what he deserved or was crucified for doing exactly what many NIH scientists, before and since, have always done: bridge a gap between public and private research to marshal all available resources in the fight to solve a public health crisis.
At first glance, few people seemed less likely to fall from grace. He won NIMH’s Exemplary Psychiatrist Award in 2000, the NIH Director’s Award in 1998, and a 1990 Public Health Service Commendation Medal. He headed NIMH’s institutional review board, which governs the agency’s ethics, for ten years.
But there was another side to the Golden Boy, one that yearned for recognition from the upper echelons of the Alzheimer’s research community. In the end, his ambition would be his undoing, and bring down with him years of painstaking contributions from families like the Noonans and the DeMoes.
• • •
As a government agency, the National Institutes of Health aimed to objectively assess treatments without concerning itself about profits or losses. To NIMH director Thomas Insel, the NIH was “Camelot,” and its internal research program—the largest biomedical research institution in the world—was the crown jewel. “It is a place for highly innovative, exciting science,” he said.
As such, it was critical to him that its scientists were above reproach: “It is not good enough to just be clean,” he said. “It has to be the place where no one will have any question about conflicts of interest. There has to be some place in the United States where the public knows that there is no taint, that there is no question, that there is no outside investment that is involved, that this is being done for the public good. This is the place.”
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