University of Pittsburgh psychiatry professor Richard Schulz has been one of the nation’s foremost researchers on caregiving stress for
Mt. Lebanon freelance writer Niki Kapsambelis vividly remembers the life-changing April 2009 day she met seven members of the DeMoe family from North Dakota at the Omni William Penn Hotel in Downtown.
The DeMoes were making one of their annual visits to participate in research at the University of Pittsburgh’s Alzheimer Disease Research Center. The family members are susceptible to a rare genetic version of the disease that makes it 50-50 they will acquire the disease as adults — and worse yet, before the age of 60.
Ms. Kapsambelis was preparing to write an article about them for the ADRC newsletter. She listened to their story for two hours in a hotel suite. Five of six adult siblings had already been identified carrying the gene that brings the early-onset version of the disease, and one was in the process of dying in his 50s. The DeMoes were unluckier than most such families, in faring far worse than those 50-50 odds.
Ms. Kapsambelis, who is 48 now and has been a working journalist since college, listened quietly to the story of these ill-fated, persevering family members in their northern Great Plains accents before leaving the interview shaken — literally.
“When I went to my car to drive home, I pulled out my keys and my hands were shaking,” the onetime Associated Press reporter recalled last week. “You just don’t have that kind of visceral reaction normally when you’ve been in this business a long time. … I just felt they had a much more important story than a newsletter article.”
She was right.
On Tuesday, Simon & Schuster, one of the nation’s leading book publishers, is releasing “The Inheritance,” a book five years in the making by Ms. Kapsambelis that weaves together the story of the DeMoes; the Pittsburgh researchers they’ve bonded with through more than a decade of work together; and the science and politics of the painstaking, frustrating efforts nationally and internationally to find a cure for Alzheimer’s.
An agent embraced her book proposal in January 2012. Before and since, and through the course of six drafts of “The Inheritance,” she has gotten to know multiple generations and offshoots of the extended DeMoe family intimately. She has visited them in their homes in North Dakota and Wyoming, as well as sitting in with them on their annual Pitt examinations.
Most Alzheimer’s has no known direct family link, but in 1 percent or less of the cases, those afflicted carry a genetic mutation that virtually guarantees they will develop the memory disease. That predictability makes families like the DeMoes incredibly valuable to researchers at Pitt and elsewhere — even long before any symptoms show up. Their behavior and abilities can be monitored for years and correlated with experimental drug treatments and sophisticated brain scans that show any of the visible changes associated with Alzheimer’s.
The family made a collective decision early on to do everything they could to contribute to research. In sometimes heartbreaking detail, “The Inheritance” describes the impact of the disease on individual family members — some who have it, others who don’t have the gene mutation, and still more who may bear the gene but don’t yet want to know.
The DeMoes gave Ms. Kapsambelis uncensored access to their lives. In an interview, she describes them as not just selfless — a main takeaway from the book — but as funny, unpretentious and a little wild, “like ‘Fargo’ on steroids.” Just like the professionals at Pitt getting to know them over the years, she became friends, almost like family — invited to DeMoe weddings and even to deliver the eulogy when one died.
“You can’t meet people like this and not be affected,” Ms. Kapsambelis explained. “At the same time, you’ve got to tell the truth of their story, warts and all. Just like any other people, they’re humans — imperfect.”
The book focuses on the six offspring of Galen and Gail DeMoe of Tioga, N.D. Galen and two of his siblings inherited the disease from their mother, who died at 54. He died in his 50s also after years of institutional care. Of his six children, two have died in their 50s, one is in later stages of the disease now and two who carry the gene have yet to show symptoms.
Karla (DeMoe) Hornstein, 59, the one sibling born without the Alzheimer’s gene, has orchestrated the family’s role in research. She was the one who identified Pitt as the right spot for them in 2005. It was where two researchers, psychiatrist William Klunk and radiologist Chester Mathis, had developed a breakthrough radioactive dye, Pittsburgh Compound B. It could track progression of amyloid protein in the brain, long deemed one hallmark of Alzheimer’s.
Ms. Hornstein said neither she nor others in the family have ever regretted the long, frequent journeys regarding their involvement at Pitt. Their travel expenses are paid, but they have to take at least several days off from work for the trips.
“We’ve just learned so much about the disease from them, and we sometimes feel like royalty there” because of the care shown by the staff, Ms. Hornstein said.
As for the goal of using their participation to help find a cure, particularly to benefit the next generation that may acquire the disease, Ms. Hornstein said, “It’s been slow, but if you look at the big picture, we’ve seen progress. … When my dad first had it, there was absolutely nothing they could do.”
Her five siblings, who began having children before being aware of the family’s genetic risk, have 11 offspring among them. Many also participate in research here, but only one, 34-year-old Robin McIntyre of Laramie, Wyo., has been told she is carrying the Alzheimer’s gene. Others are free of it or chose not to be told yet either way. Ms. McIntyre, whose mother, Lori, died from the disease, wanted to know her own future in 2012.
“In my thoughts, knowledge is power,” she said by phone last week. “I just want to do everything I can to educate people or create awareness of the disease, and having awareness and education myself was very important.”
The hairdresser, unmarried but living with a partner, has chosen not to have any children herself. She figures she might have 12 to 15 years before symptoms show up, based on family history, and hopes progress against the disease will come in the interim.
Dr. Klunk, portrayed in the book as showing unusually close, caring attention to the DeMoes, has high praise for the multiple generations of them he has gotten to know and study. At least 10 of them are now involved in the international DIAN study for which Pitt is one site, testing out several different drug treatments, though the results won’t be known for several years.
Dr. Klunk says the DeMoes are curious about the progress of research, and he tries to keep them updated on the study and other efforts without sugarcoating it. He has been pained himself when watching several of the DeMoes decline or die after getting to know them well.
“None of them are here based on a guarantee of success,” he said last week. “They’re here based on being a family who isn’t gong to sit back and do nothing. This is a group that wants to fight back, and seems so happy to have an opportunity to participate in the research that they thank us. They don’t ask us what they’re going to get out of it.”
Niki Kapsambelis will be the featured speaker at the Women's Press Club of Pittsburgh's 126th Anniversary Dinner and Journalism Awards at 6 p.m. April 8 at the Rivers Club, Downtown.
Gary Rotstein: firstname.lastname@example.org or 412-263-1255.