The Inheritance

by Niki Kapsambelis

  Eventually, in 1974, Moe was let go from the job. It was a devastating blow, one that cut to the core of his self-image as a hardworking man who supported his family, who could be trusted in the fields. And while he drew some disability pay, the financial impact was significant, too. The family went on food stamps. Karla, ever conscious of appearances, was so ashamed that she refused to go to the store for her mother, unwilling to admit to government assistance in front of friends who were bagging groceries.

  To help support the family, Gail took a job as a nurses’ aide at the Tioga hospital, and Moe stayed home to take care of three-year-old Jamie. The older children weren’t interested in watching their youngest brother—there was too much fun to be had out on the town. For most of his childhood, Jamie would be an afterthought to his older siblings. When he was a baby, nobody would share a room with him, so his crib was parked in the hallway outside Lori’s room.

  On nights when Gail had to work a late shift, Moe sometimes forgot where she’d been and worried she was cheating on him. Alcohol made things worse. In Gail’s memory, he didn’t take out his anger on the kids—just on her.

  “I was the one he looked to for answers,” she said. “I guess you pick on the ones you love the most.”

  But most of the children told a different story. Except for Karla, they all remembered being on the receiving end of his abuse. As much as they loved their mother, they could not tolerate their dad. And the worse he got, the more it tore the family apart. Brian escaped when he graduated high school and moved to Wahpeton, four hundred miles away, to attend the North Dakota State College of Science. Unbeknownst to his siblings, he stayed in touch with Moe—possibly to ask him for spending money without Gail finding out. Feeling increasingly trapped, the other children also began looking for a way out.

  • • •

  In the early Sunday morning hours of a summer heat wave, Lori woke to the sound of her father, “drunker than hell,” yelling at her mother. When Lori told him to quiet down so she could sleep, he came after her, and she locked herself in the bathroom. She would recount it later in a letter:

  He started after Mom again + she sprayed him in the face with a can of tear gas that the boys had bought. We all got the gas but Dad got it the worst. He ran outside to get away from it. He didn’t have any clothes on. When he came back into the house he was really, really pissed. He started to hit + chase Mom and I grabbed him to hold him back cuz I was scared and didn’t know what he would do if he would have caught Mom. He threw me up against the stove but I grabbed him again until Mom could get out of the house.

  With none of her brothers home to help her handle Moe, Lori called the police. When the officers arrived, Gail retreated to the neighbors’ house while they calmed him down and got him back to bed.

  Mom really doesn’t know what to do now. She might put him in some kind of a hospital. I think it should have been done awhile ago. It’s tough on everybody.

  Eventually, Lori left. She found her ticket out while cheering at a high-school wrestling match, where she fell in love with one of the spectators, Steve McIntyre, an out-of-towner from the Pacific Northwest who was visiting family. From the moment they went on their first date, Steve knew he wanted to spend the rest of his life with her.

  After she met Steve, Lori had one foot out the door. While he crisscrossed the western United States working as a track maintenance man for Union Pacific Railroad, she finished high school, started trade school, and then set out to join him.

  She and Steve were both nomads at heart, and she had never been the kind of person to worry about what others thought of her. In trade school, she chose an auto parts program, unusual for a girl—particularly in 1977. In one letter to Steve, she wrote about the new outfit she was sewing for herself—cursing her way through her mistakes—before she abruptly switched topics to the automobile power train system she was studying. Tioga would gradually recede in Lori’s rearview mirror as she and Steve embarked on what she was sure would be a life of adventure. She left her siblings back home to figure out how to coexist with Moe as best they could.

  • • •

  The shouts behind the closed bedroom door continued; Moe seemed to be pushing Gail around. But in her room down the hall, Karla stayed in bed. She didn’t see what her father was doing to her mother, and she didn’t intervene; neither did the boys, holed up in their basement bedrooms. All she knew was if she was headed home at the end of the day and her father’s car was in front of a bar uptown, it was going to be a bad night.

  Karla isolated herself from her siblings, spending as much time as she could with her friends. As an adult, she would regret the lack of support they gave one another during the worst period of their young lives, but she believed they were simply shutting down to survival mode, because they had once been so close.

  “You’re just trying to make it through it all yourself. That’s all you think about: This is awful for me,” she said.

  But the family bond that Gail had created was stronger than any of her children understood at the time. Although they were beginning to scatter, trying to forget the rough parts of their upbringing, they never fully broke free of their roots. And when life soured for them at times, they sought comfort in one another. It was a quality each of them would strive to instill in their own children.

  • • •

  Meanwhile, after Lori’s departure, Dean and Doug were still raising hell around town, and Brian was still in Wahpeton. Karla attended college for a year, then had returned to Tioga to work an administrative job for the town when the escalating situation with her father finally detonated.

  Gail asked Brian to move back home, thinking he could help handle Moe; it didn’t work. In fact, with one more person to confront him, Moe became, if anything, more irrational. Only the police could settle him.

  In August 1978, Karla was at work when the police department’s phone rang over to her desk, as it sometimes did when nobody else was there. It was someone from the sheriff’s office calling to say they were sending a car to pick up Galen DeMoe.

  Karla’s heart sank, but she finished the conversation with the dispatcher and quietly hung up, never revealing that it was her father they were discussing. The shame bubbled in her chest. When would it ever stop?

  Gail had run to Hank’s house to hide, and from there had called the police, hoping they’d be able to tame her husband once again. But instead of calming down, this time Moe’s rage increased. He fought with the officers until the police finally managed to shove him into the patrol car and drive off down the street. Dean, then sixteen, huddled in front of the window, convinced his father would break free and come after him. He didn’t exhale until the cruiser drove away.

  The next day, Gail called Karla, who could hear the fear and pain in her mother’s voice.

  “They’re taking Dad away for good, for my safety,” Gail said.

  Moe’s sons packed his suitcase for him. A judge committed him to the North Dakota State Hospital in Jamestown, 250 miles away, a facility for the mentally ill and criminally insane. Galen DeMoe never came home again.

  Three

  FAMILY N

  IN THE YEARS since Auguste Deter’s death, thanks to Emil Kraepelin’s decision to define Alzheimer’s as a malady striking people under the age of sixty-five, science continued to mistakenly treat it as a rare affliction, separate from the common senility of older people. That error cloaked how widespread Alzheimer’s really was.

  Things began to change in 1948, when a London scientist named R. D. Newton reviewed the previous forty years’ worth of literature written about the disease and concluded that there were no grounds for distinguishing between Alzheimer’s and senility. Newton also found previous reports that Alzheimer’s appeared in families in German, British, and American papers. When he grouped those isolated reports together, he became convinced that a hereditary factor contributed to the disease, whether the patient was old or young. Two more studies in the next dozen years would
work to solidify Newton’s idea that a genetic link was at play, including the one written by Harvard’s Robert Feldman in 1963, the year before Moe’s mother, Wanda, died.

  But the turning point came in 1968, when a group of British scientists—Gary Blessed, Bernard Tomlinson, and Martin Roth—showed that most people over sixty-five who had dementia also had the same plaques and tangles that had overrun Auguste Deter’s brain, firmly establishing that senile dementia and Alzheimer’s were the same condition. Their research debunked the prevailing theory that most senility was caused by atherosclerosis, or what is sometimes called “hardening of the arteries,” when fat builds up inside artery walls, causing them to narrow and stiffen. The only thing rare about Auguste’s condition had been that it struck twenty years early.

  Once Alzheimer’s researchers understood that they were dealing with a much larger patient population than they had realized, they began making tentative steps toward discovering the disease’s causes, including possible genetic factors. But it was hard-won knowledge, collected from small pockets around the world when doctors stumbled across cases so strange, and sometimes so poignant, they demanded further investigation.

  • • •

  On the southern tip of Italy—basically the toe of that famous boot—is the region known as Calabria. Its unstable, mountainous terrain is prone to violent expressions of nature, notably volcanoes, earthquakes, and tidal waves, which have killed tens of thousands of its inhabitants over the centuries.

  It was into this beautiful, sometimes brutal land that French neuropathologist Jean-François Foncin landed in May 1973, hoping to unravel the strange family history of his patient, a forty-four-year-old woman with Alzheimer’s disease. What he did not know was that his journey was going to dramatically expand science’s understanding of the genetic components of the disease, and the way it is inherited.

  A year earlier, the woman—an Italian immigrant who was then living in France with her husband, a tile layer—had gone to a doctor when she had difficulty caring for her newborn, who was her ninth child. Her symptoms pointed to some kind of brain disorder, and she was referred to a neurologist who committed her to a psychiatric institution. She was increasingly disoriented, and doctors struggled to evaluate her memory and speech since she was illiterate and spoke only in her native Calabrian dialect. Suspecting a possible brain tumor, they transferred her to a Paris hospital; in those days before CT scans allowed for a less invasive diagnosis, the surgeons instead drilled a burr hole in the right front section of her skull—an ancient procedure known as trephination—and drained a sample of cerebrospinal fluid from her brain’s ventricle, searching for possible tumor cells. They found none.

  Doctors then performed a cortical biopsy, which means they removed a small sample of brain tissue. Brain biopsies are not undertaken lightly; complications can include seizures, brain infections, even death. But when symptoms are baffling, particularly in young patients, they are sometimes used to diagnose (or rule out) potentially treatable illnesses, such as brain inflammation.

  Unfortunately for the woman from Calabria, the sample revealed something much more insidious: plaques and tangles.

  “There’s no tumor here,” Foncin told his colleagues. “It’s Alzheimer’s disease.”

  Foncin recalled reading the early theories—from Newton and others—that there might be a form of early-onset Alzheimer’s that ran in families in an autosomal dominant pattern. So he asked the woman’s husband about her family history. The husband promptly told him her sister and father had died of the same disease.

  Suspecting he was onto something, Foncin sent a request to Italy for her father’s medical history. He received three sets of records in response: those of the father and two other people with the same surname, all of whom had died with dementia in their fifties.

  The discovery also piqued the interest of Foncin’s mentor, Dr. J. E. Gruner, a prominent French neuropathologist and amateur genealogist. Gruner arranged for private funding to further investigate this possible genetic link to Alzheimer’s disease—which held the potential to explain both how it happens and predict when it might strike next. Together with Gruner, Foncin trekked to Calabria to see what he could find.

  The patient’s relatives, who would come to be known as “Family N” in medical lore to preserve their privacy, were cooperative. By now, the patient had returned to Italy, where she was “profoundly demented and cared for like a baby by her old mother,” wrote Foncin. The mother was also caring for other siblings and cousins with the same symptoms. Municipal records and archives at the local psychiatric hospital yielded a wealth of information on the family dating back to 1880.

  In village churches, provincial Italian priests traditionally kept a journal of the preceding year’s events, annual write-ups summarizing their parishioners’ triumphs and trials: “Giuseppe has started wandering, quite confused; couldn’t find his way back home, just like his father.”

  The woman Foncin saw in Paris had lost her father, grandfather, and great-grandmother, all of them dying in similar ways: in their fifties, with dementia. The diagnosis would read “syphilitic general paresis of the insane,” but the family simply called it “the disease.”

  To further pursue the autosomal dominant inheritance of Alzheimer’s in Family N, Foncin applied in 1977 for much-needed funding through a grant from the French Institute of Health and Medical Research, which was running a program on brain aging. Suspecting that his case might not be exactly what the institute had in mind for its program, Foncin didn’t offer many details about his project. He won the five-year grant on the stipulation that he present his findings halfway through to a committee set up by the institute.

  Using punch cards and a mainframe computer, Foncin and a colleague began to quantify the genetics of Family N. When the time came for his presentation, he was so confident that the family trees he was constructing were illustrating something valuable that he decided to tell the committee exactly what he was up to. He presented extended genealogies traced through the computer and added his opinion that this was the way to solve the Alzheimer’s riddle.

  Disappointed, the committee members did not agree. They had been hoping he was searching for a way to delay the Hayflick phenomenon, which is the number of times a human cell population will divide before it finally dies. The committee cut off Foncin’s grant.

  Demoralized, Foncin put aside Family N for months. But then Gruner learned that one of his former students had established a neurology unit in the hospital that had served as the birthplace of Foncin’s original patient. Foncin’s passion for the topic was rekindled; he flew to Calabria and reached out to one of the doctors in the unit, who agreed to help him with his research.

  They discovered more relatives afflicted with the disease, with branches extending into other countries, including the United States and France. And they also realized that they weren’t the first doctors to take an interest in the family. It turned out that when Robert Feldman wrote his 1963 paper suggesting that genetic inheritance played a role in Alzheimer’s disease, he was basing his theory on the research he had done with members from a branch of Family N.

  Sometimes, doctors who work with extended families become very close to their subjects; such was reportedly the case with Feldman, who died in 2003. He watched helplessly as members of Family N, even the brightest and most high-achieving of the bunch, succumbed to Alzheimer’s at unusually young ages—in at least two cases, they were thirty-one and thirty-two years old when their symptoms began.

  Feldman did the best he could to analyze the family’s heredity with mid-1960s technology, but it wasn’t enough. When Foncin’s group contacted him, however, the answers began unfolding. In 1988, a quarter of a century after Feldman first reported on the family, he, Foncin, and several collaborators were able to trace their subjects’ roots all the way back to a single common ancestor: a woman named Vittoria, born in Calabria in 1715, who had died at the age of forty-three. They had sixty cases
of Alzheimer’s disease spanning ten generations of Family N, and they had identified four thousand descendants across several countries. Their 1985 paper appeared to offer conclusive evidence of a form of Alzheimer’s disease that was repeating itself genetically across the generations, and the disease was the same regardless of the country where the victim had grown up. It was a treasure trove of genetic information.

  • • •

  In the United States—in fact, just a few miles from where Feldman worked at Boston University—a wry young British neurologist and molecular geneticist named Peter St. George-Hyslop was just getting started in his career. As he was finishing his medical training in 1985, he took a position at Massachusetts General Hospital in a lab specializing in neurodegenerative disorders. Since Hyslop was interested in the DNA analysis of Alzheimer’s, it seemed a good place to start.

  In the Mass. General lab, Hyslop found write-ups of a Canadian family with Alzheimer’s whose DNA samples had already been submitted to the National Institutes of Health (NIH). While flipping through a book of the samples, Hyslop found a reference to Alzheimer’s disease on slides labeled with an Italian surname. But when he asked about them, nobody knew where they had originated; they had become the scientist’s equivalent of the mystery contents in the back of Gail DeMoe’s refrigerator. Hyslop left them there.

  During the next few months, Hyslop struggled to find additional families with autosomal dominant Alzheimer’s so he could create a large enough sample size for meaningful research.

  “I was aware that there was this other Italian family from Foncin, but I had never met [Foncin], had no idea how to get hold of him,” Hyslop said.

  Then, his luck turned. Rick Myers, a researcher who gave genetic counseling to potential victims of Huntington’s disease, wandered into the lab and asked whether Hyslop would accompany him to offer a neurological opinion on a case.