They were also both organized, determined, capable by nature. And it was easier for Karla to brace herself for the possibility of bad news knowing that she had Dean to depend on for help.
• • •
As the DeMoes sat in the neurologist’s office in March 2004, the doctor did his best to reassure the family. No matter what, he told them, everyone would feel good about themselves by the time they left. It would be a relief to know where they stood genetically.
But on the last day of testing, the neurologist was out of town, so another member of his team—a neuropsychologist—sat the family down to discuss Doug’s and Brian’s results, with the two men in the room. His comments were stunningly insensitive.
“They have the math acumen of a third or fourth grader,” the psychologist said, speaking to the rest of the group as if Brian and Doug weren’t there, or were perhaps too stupid to understand. He displayed slides of the childish scrawls the two men had made in their cognitive test answers. He pointed out how poorly they’d performed: “Brian has the reading ability of a third grader and the spelling ability of a second grader.” Brian was nearly forty-eight; Doug was forty-three.
It was embarrassing, having the men of the family depicted like slow children. The reality of their situation was harsh enough, but the doctor seemed unaware of how much worse it felt to be told such devastating news as though Brian and Doug were nothing more than lab specimens.
The imaging technique developed by Bill Klunk and Chet Mathis was not yet available, so the diagnosis was based on a clinical assessment: It was Alzheimer’s disease—the same thing that had stricken Moe, his brother Jerry, their mother, and their uncles.
Gail began sobbing; then Karla also broke down. Dean and his wife, Deb, were incensed at the doctor’s detachment. Doug and Brian just sat, listening; Karla thought perhaps they didn’t comprehend what had been said about them.
The psychologist asked if he could draw their blood and test them for a possible genetic mutation, to see if that was the root cause of the disease in their family. Like the Noonans, Karla thought.
The family agreed, then asked to be referred to any Alzheimer’s research studies that might use them as volunteers. When tragic news breaks, people respond in many ways. In the DeMoes’ case, their immediate response was: Fight back.
On their way out to the car, Brian looked at Doug and said, “Boy, are we dumb, Doug.”
They had understood.
• • •
Setting aside their grief over the diagnosis and their anger over the way they were told, the family did take away an important kernel of information: Just as Karla had suspected, their problem might come from the same type of rare genetic mutation that was in the documentary. A month later, the neuropsychologist contacted Karla, confirming that Doug and Brian had tested positive for just such a mutation.
Doug’s daughter, Jennifer, was barely out of high school and living ninety minutes away in Minot when Doug Latch, the father she so adored, had started to falter. When she talked to him on the phone, they’d get stuck in an endless conversational loop; he’d tell her five times in a row that he had washed his car.
She hadn’t known her grandpa Moe very well, since he died when she was just a little girl. Nobody ever really talked about him, except to say he was a bearcat. When Doug was diagnosed, she was shocked.
“I don’t think anybody thought it would turn into this. I thought only old people got this,” she said. She remembered thinking: “Holy crap. This is going to turn our world upside down. Nothing’s ever going to be the same again.”
She moved back to Tioga and into Doug’s tidy little house, with no specific plan for what she would do next.
“I just thought I had to be here,” she said. “There’s just not a lot of years that we were ever together.”
Though the disease took Jennifer by surprise, one of her cousins—Lori and Steve’s oldest daughter, Jessica—had the opposite reaction. Jessica was now in her early twenties and living in Denver. When she first learned that her uncles carried the gene, she went numb, the natural pessimist in her taking over.
“I thought, ‘Oh crap, both of them?’ And I thought in my mind that was going to happen to everybody,” she said.
Based in the northeast, the Noonans had been able to turn for help to Massachusetts General Hospital; in North Dakota, the DeMoes had struggled to simply find a neurologist with the genetic expertise they needed. Tioga didn’t even have a McDonald’s, let alone an Alzheimer’s research facility.
“I knew we needed to go someplace bigger to see if we could get help,” Karla said. Remembering the study to which her father had contributed through the University of Minnesota, she tried to locate June White, the nurse who had drawn their blood, not realizing that White had retired in 1995. Every road turned out to be a dead end. From the DeMoes’ perspective, it was as though that research had never occurred.
Vic, Moe’s younger brother who was closest to him in age, recalled giving blood, too. What he didn’t remember was getting any meaningful information from the study, and it pissed him off. Somebody transferred jobs somewhere, he thought, and nothing ever came of that early hope. So while he still had his memory tested every year at Indiana University, he refused to donate money to the Alzheimer’s Association.
“I’ve been doing this for fifty years, and you’re no farther ahead than you were!” he told them. “These people. When they run out of grant money, they quit.” And the test subjects who had given of themselves, many of them desperate, were left stranded.
Like other Alzheimer’s patients and family members who contribute to research, the DeMoes were finding that they could often be left at loose ends by scientists who ran out of funds or moved on to other jobs. The patients contributed their tissues, their blood, even their brains after death. They revealed some of the most intimate details about their daily struggles, their fears, their family histories. And then, if a study was orphaned, it seemed as though all that information fell into a black hole, the contribution wasted.
Karla DeMoe Hornstein had been many things in her life: a daddy’s girl, a self-centered sister, a homecoming queen. But when her family needed her, she reached underneath the security of her cheerful façade, elbow-deep into her soul, and found at her core the strength that Gail had been instilling gradually in her for her entire life. When she pulled that strength to the surface, she was permanently altered. Gone was the woman who deferred to others’ opinions and accepted no for an answer. She started searching online for Alzheimer’s research and came across the National Institute of Mental Health, which she knew was where her uncle Jerry had gone when he was diagnosed. She started making phone calls.
And finally—finally—someone called her back. It was a woman who said: “I knew your uncle. I remember him. We’d like to meet you.”
• • •
In August 2004, five months after visiting the doctor in Fargo, Doug and Brian went to Maryland with Karla and Dean to undergo a more detailed examination of their brains than they ever previously had thought possible.
They were introduced to Dr. Pearson Sunderland III, chief of geriatric psychiatry and a longtime Alzheimer’s researcher. Known professionally and personally as Trey, he was handsome and charismatic, and he treated them well, making them feel as though they were in competent hands.
Sunderland had arrived at the National Institute of Mental Health, a subset of the National Institutes of Health, in 1982 for a fellowship, then begun a research program in Alzheimer’s disease and geriatric depression. He spent the next twenty years building a sterling reputation, including a decade as the chair of the NIMH’s institutional review board and head of the medical advisory board for the Washington, DC, Alzheimer’s Association. He was a prolific writer, authoring more than 250 scientific papers as well as a book on Alzheimer’s. It seemed the DeMoes had found their expert.
Sunderland’s specialty was finding ways to detect Alzheimer’s before patients develope
d symptoms. As with most formidable diseases, finding a treatment for Alzheimer’s seemed most attainable in its earliest stages. However, no drugs to date had been able to halt its progress. Once it began destroying neurons, its effects were irreversible—doctors called it “the cascade.” The key, then, seemed to be preventing the disease before it reached that fatal tipping point.
But the Alzheimer’s brain changes years before it’s apparent to the outside world. Nobody even knew what presymptomatic Alzheimer’s looked like, or whom to study, until they found people like the DeMoes, who carried a genetic flaw that guaranteed a future disease. Now, in addition to that population, they had Pittsburgh Compound B, a way to see what the disease looked like before it was evident in a person’s behavior. The field planned to combine the two to refine a target for possible drug interventions.
Beginning in the early 1980s, Sunderland began studying Alzheimer’s in patients who came to the NIH Clinical Center, the world’s largest medical experiment facility. He and his staff collected their spinal fluid in search of biomarkers, or biologically distinct indicators of the disease, such as beta-amyloid and tau proteins. By the time he met the DeMoes, he had collected more than six hundred samples.
In the four days they were in Maryland, the DeMoe siblings learned many important but alarming things about the disease that had been stalking their family for generations. The fact that it was autosomal dominant meant that any of them could have inherited the mutation from their father. If they did, Alzheimer’s disease was guaranteed, and its symptoms would begin showing soon; all of them except Jamie were now in their forties, which was close to the expected age of onset.
That genetic guarantee was a double-edged sword. For science, it meant researchers had perfect specimens to study. But for the DeMoes, it was a catastrophe.
Suddenly, the notion that they all had decades left to spend raising their children, making house payments, and earning paychecks vanished. Worse still, if they had it, there was a fifty-fifty chance they were going to pass it on to their own children, or already had. And, except for Jamie, all of them had kids, much-loved children born into a world with the automatic expectation of good health and long lives. Now those children were suddenly in danger: Kids who were still playing Little League and toting around stuffed animals might be unable to have children without the very serious risk of passing on a fatal illness. The grandchildren their parents dreamed about would be equally at risk.
To be able to predict one’s own death is a weighty business; it affects decisions both before and after the discovery, and its ripple effect touches parents, children, siblings, and family members. It is knowledge that can affect the most profound life choices: whether to marry, have children, participate in research, take a once-in-a-lifetime trip that you can’t really afford. It also affects everyday decisions, such as what to eat, whether to exercise, what kind of insurance to purchase. Do you train for that marathon? Keep trying to learn a new language? Quit the dead-end job that pays the bills? When is it appropriate to tell a romantic interest: The third date? Fifth? Tenth?
In families like the Noonans and the DeMoes, genetic testing for their mutations can predict with absolute certainty from any age, including infancy, whether a person has inherited the disease. In the general population, genetic tests offer less certain results but still carry a burden of knowledge that can be life-altering: a Faustian bargain.
Imaging from a PiB derivative such as Amyvid allows doctors to diagnose Alzheimer’s in a living brain, but without meaningful treatment or means of prevention, giving someone such a diagnosis becomes an ethical land mine. In fact, outside of clinical trials, Medicare will not pay for such scans.
Even a clean bill of health carries drastic implications: How do you care for those around you who tested positive? People who get a negative test for the mutation aren’t guaranteed a long life, either, as the Noonans would eventually find out.
It was that terror of losing the next generation that spurred the DeMoes into action. Despite Jerry’s failure to respond to Cognex, the lack of details from their earlier attempts at research, and the knowledge of what kind of end was in store for those with a positive diagnosis, the DeMoes saw only one path toward hope: If their bodies could help science ferret out an answer, they might save their children. Viewed in that context, there was really only one choice to make.
It would be an emotionally expensive choice, even for those who didn’t carry the gene. For Gail, the center of the family and life of the party, any positive diagnosis from the research was bound to carve years off her life in grief and worry. Only the belief that their sacrifices would save her kids’ children—and other people’s children—kept her from total collapse.
Karla and Dean decided they wanted to know their status. If they were going to assume responsibility for Brian and Doug, they reasoned, they should probably do so with a clean bill of health. The study personnel drew their blood, but before they were allowed to learn their results, genetic experts counseled them about the possible ramifications of that knowledge. Both promised to weigh the decision carefully.
Before they left, Trey Sunderland said he hoped to enroll Lori and Jamie in his study, in case they had the gene or could serve as controls for those who did. In Wyoming, Lori was worried about the possibility of inheriting the disease, but decided to apply for long-term care insurance before finding out her status. Jamie was more concerned with working and chasing women than he was with the possibility of Alzheimer’s; his brothers just seemed so much older than him. Testing was something he eventually planned to do, but he didn’t feel any sense of urgency.
Sunderland also put Karla in touch with Julie Noonan Lawson; for the first time, the DeMoes would be able to speak with one of the few people in the world who knew what they faced. For Karla, being able to share her fears, frustrations, and questions with a seasoned veteran was a gift for which she would be extremely grateful.
Eleven
WHEN THE FOG ROLLS IN
JULIE NOONAN LAWSON was five years old when she first became aware that her mother was starting to fade.
She was just a tiny thing, a kindergartener clad in a coat and hat to protect her against the harsh Boston weather, waiting by the school door for the mother who never remembered to show up. The eighth of ten children in an Irish Catholic family, Julie might have learned one day that such lapses happened occasionally in large clans; but the increasing absence of her mother was an omen that could not be explained away by absentmindedness.
There was a time when Julia Tatro Noonan whirled through her day, baking, singing, attending to her children, listening to their prayers. But her youngest daughter did not get to experience much of that mother. Rather, the mother she knew forgot to change her younger siblings’ diapers, lost her husband’s paycheck, struggled with the coffeemaker, overreacted to small slights. She was only in her late thirties; like the Italian woman Jean-François Foncin stumbled across in his Paris hospital, she had babies at home. It was the early 1960s, and Alzheimer’s was still widely misunderstood. Her behavior baffled doctors.
Thinking Julia might be suffering from postpartum depression, they suggested time off. She took trips away from her family, but her symptoms only worsened over time. As was customary for some cases of the day, she was given shock treatments. Finally, she was taken to the psychiatric unit at a hospital, and in 1967, she was diagnosed with Alzheimer’s disease. She was forty-three.
For the remaining eleven years of her life, Julia was institutionalized. Julie saw her mother strapped to a bed, medicated, wasting away. She died in 1978, her weight hovering somewhere around eighty pounds, curled in the fetal position, unable to recognize her children. Just like Wanda DeMoe.
The ten children Julia left behind were, in descending order, Dennis, Maureen, Patty, Kathi, Fran, Butch, John, Julie, Bob, and Eryc. After Julia disappeared from her family, her husband, John, was overwhelmed by the responsibility of caring for all those children. The youngest went
to live with married siblings. For the next several years, the family fought to regain a state of equilibrium, reorienting itself after the loss of its mother. And then, just when they had started to normalize, the bottom dropped out again.
• • •
When Julia fell ill, her oldest daughter, Maureen—then in her twenties—and her husband, Dick Preskenis, adopted one of Mo’s little brothers, four-year-old Bob—the ninth child in the family, born after Julie. He even took their last name, and he was raised as a sibling to their own children. In a cruel irony, Bob lost his second mother when Maureen also developed the same symptoms that had stolen their biological mother.
When Kate Preskenis was in eighth grade, her mother, Maureen, sat her down to watch a scratchy video of a made-for-TV movie called Do You Remember Love. It starred Joanne Woodward as an English professor whose decline from Alzheimer’s disease affects her family and her colleagues.
Maureen cried through it. Afterward, she asked Kate if she had any questions, and explained that the family thought there was a possibility they had a genetic link to the disease because Kate’s great-aunt Agnes, her grandmother Julia’s identical twin sister, also had Alzheimer’s.
Maureen and her siblings, who felt normal, theorized that the disease might skip a generation: She and her siblings thought they would not have to worry about it, but their children might. Fran’s husband, a doctor, had attended a conference and returned with that suggestion.
But if the theory proved wrong, Maureen’s viewpoint was chilling, albeit a common one for the children of Alzheimer’s patients. If she developed the disease, she said bluntly, she did not want to live. She never wanted to end up like her own mother, curled in a permanent fetal position.
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