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Contents
Immediate Demoe Family Tree
Extended Demoe Family Tree
Prologue
PART ONE
One The Enemy Within
Two The Salt of the Earth
Three Family N
Four One in a Million
Five You Are My Sunshine
Six The Ghosts of Anoka
Seven Untamed Hearts
PART TWO
Eight A Blameless and Upright Man
Nine The Fruits of Persistence
Ten Déjà Vu
Eleven When the Fog Rolls In
Twelve Maldición
Thirteen Burden of Proof
Fourteen The Rise and Fall of Golden Boy
Fifteen Favorite Son
PART THREE
Sixteen The Baptists and the Tauists
Seventeen Expelled from Eden
Eighteen Safe Havens
Nineteen A Big If
Twenty Everyone Sees the Power
Twenty-One Landslide
Twenty-Two Something to Shoot For
PART FOUR
Twenty-Three The Silver Tsunami
Twenty-Four The Lucky Ones
Twenty-Five Follow the Science
Twenty-Six Like Father, Like Son
Twenty-Seven All the Cards Are on the Table
Twenty-Eight Coming Home
Twenty-Nine Palpable Momentum
Thirty To the Moon and Back
Photographs
Acknowledgments
A Timeline of Discovery in Alzheimer’s Disease
About Niki Kapsambelis
Notes
Index
For HELEN KAPSAMBELIS
and GAIL DEMOE,
two women of remarkable courage,
without whom this book would
not have been possible.
Prologue
The doctor is businesslike, but his young face is kind. He explains that he’s going to say a name and address, and he wants you to repeat it back to him, now, then once again later:
“John Brown, 42 Market Street, Chicago.”
You repeat it back to him, slowly: “John Brown . . . 42 Market Street . . . Chicago.”
He smiles. Good. He asks a series of other questions.
“And where were you born?”
“What was the last job that you had?”
“How is a turnip similar to cauliflower?”
As the questions hang in the air, the brain works to retrieve the information, stored somewhere in the hippocampus. The answers have to travel from neuron to neuron, across the spaces between the cells. They don’t always arrive quickly. They aren’t always complete. But they do arrive, eventually.
“What’s the difference between a lie and a mistake?”
You know this: A lie is on purpose, and a mistake is an accident. The doctor seems pleased.
Then he asks where you are.
You reach for the thought, but it isn’t there. When it tried to travel from neuron to neuron, it was blocked by a wad of proteins that stuck together in the space between. Without a landing place, the message evaporated, and the thought was gone.
You look around, but you don’t think you’ve ever been here before. There are no clues to help you reach for the answer.
He asks you what day it is: You don’t know. You don’t know the year, or even the season. Those thoughts traveled from a healthy neuron to one that had withered up and died, and again, the message was lost.
He asks you how many nickels are in a dollar. You used to balance your checkbook with ease, but now you can’t answer. It’s embarrassing, because you realize it should be an easy question. You hope he doesn’t tell your boss what you don’t know, or your kids.
You wonder exactly who is watching this. There’s someone sitting across the room, and you’re not sure, but you get the impression that they’re mocking you. Every time you glance their way, you catch them staring. They’re even wearing the same shirt as you. You feel the anger start to rise.
He asks you again: What was that name and address?
You were telling the truth when you said your memory was OK. A lie is on purpose; a mistake is an accident.
You begin: “John Brown . . .”
But the rest of the answer hits another dead circuit.
• • •
In April 2009, I walked into a hotel in downtown Pittsburgh to meet a handful of members from a North Dakota family who were involved in Alzheimer’s disease research at the University of Pittsburgh.
I had never been to North Dakota. In fact, I had never met anyone from North Dakota. My limited impression of the state had been formed by watching the movie Fargo, which is mainly about Minnesota anyway. I was equally ignorant of Alzheimer’s disease—a diagnosis that, to the best of my knowledge, is absent from my immediate family tree. I walked into the hotel simply because I am a journalist, and I had been assigned to write a short article about these people.
Two hours later, I walked out, and my life had changed forever. When I reached my car, I realized I was physically shaking from the impact of their story and the magnitude of what they were doing. I immediately thought that they deserved more than the short article I had been asked to write. They deserved a book.
One day, the world will be free of this devastating disease. When future generations want to know how that became possible, may the following pages help them remember.
• • •
Early in the afternoon of a perfect July day, Dean DeMoe watched his big brother Doug fly a kite shaped like a panda.
Doug was standing at the top of a gentle slope, his fingers gently pinched around the cotton string, his arms raised skyward by the kite as it danced high in the wind that never leaves the North Dakota plains. His face beamed with the innocent, uncomplicated joy of his task; he was proud that his kite floated the highest of anyone’s at the top of the hill.
Farther down the slope, close to where Dean was standing, a church group preparing for a picnic had set up a speaker that played hymns as they worked. At age fifty-two, Doug was wearing diapers. The man who had always crafted his own ice cream for family cookouts—with hands strong enough to knock an opponent out with one punch—now had his meals brought on a tray. Though he’d never been a talker, he was now unable to say more than a word or two at a time. When his language skills had almost completely left him, he broke months of silence with a single word: Dean.
The brothers were nineteen months apart, and though Doug was older, he had always been more of a peer than an elder to Dean. They were two sides of the same coin: Doug was taciturn; Dean was outgoing. Doug was never much of a conversationalist; he let Dean do the talking for him. Doug was a homebody, while Dean loved the adventure of travel. Dean’s self-confidence was infectious, whereas Doug often gave off an air of uncertainty, as though he would rather say nothing than say the wrong thing. Dean was born to be a leader, the kind of man others would obligingly follow; Doug was the perfect employee, never missing a day of work and always glad to be given a task to complete. But on a primal level, they understood each other, their connection encoded in their DNA.
When they were children, people around town called them Deanie and Dougie. They’d been high-school wrestlers, amateur boxers, hell-raisers: the untamed DeMoe brothers. It hadn’t been so very long ago. People in town still told stories about them, the hometown anti
heroes. Their youth had been spent in a flurry of good-natured rebellion, punctuating long stints of backbreaking hours in the oil fields, where their father before them had once made his living. When they weren’t on an oil rig, they might be found playing elaborate practical jokes or organizing parties at their family’s sprawling house near the center of town. The girls had flocked to them. And they always had each other’s back: anybody who crossed one brother would have to contend with the other.
Dean still put in fifteen-hour workdays in the oil fields, supervising men on a rig that could explode if somebody screwed up his job. In middle age, he was still a strong man. His crew trusted him with their lives, but he was powerless to help Doug; he could only watch as his brother slipped further away under the surface of a disease that was overtaking his brain.
As one of the church group’s recorded hymns hit its crescendo, Dean turned his back to the nursing home where Doug now lived, a place Dean vowed he himself would never call home.
Dean got back in his car, slipped it into gear, and headed toward town.
Part
ONE
There are only two or three human stories,
and they go on repeating themselves as fiercely
as if they had never happened before.
—Willa Cather, O Pioneers!
One
THE ENEMY WITHIN
WALK INTO A shopping mall. An amusement park. An auditorium of parents gathered for a school play. Within this crowd, there will be someone—in fact, several people—who are directly and irreversibly affected by Alzheimer’s disease.
In the United States, Alzheimer’s is the sixth-leading cause of death. Next to cancer, there is no condition more feared by human beings than Alzheimer’s, for it means more than a slow death; it robs its victims of the key components of their humanity. They lose shared experiences; they fail to recognize their most cherished loved ones; they forget even their proudest accomplishments. The stress of caring for an Alzheimer’s patient has decimated close-knit families, ended happy marriages, snapped the tensile bond between parents and children. And the disease is as baffling as it is unforgiving.
An estimated 24 to 36 million people worldwide—5.3 million in the United States alone—suffer from the disease or similar dementias. But Alzheimer’s is the least understood of all major fatal illnesses, frequently mistaken for other conditions, especially depression if the patient is young. Only one in four people who have the disease are actually diagnosed. None of them can be cured.
Once thought to be relatively rare, Alzheimer’s is now known to be the leading cause of age-related dementia, and science is only beginning to grasp how common—and how lethal—it really is. For it is always fatal: If patients do not die from secondary causes, such as pneumonia, the disease will eventually move from erasing memory and language to shutting down involuntary functions, such as breathing and swallowing.
In the developed world, most major causes of death—including cancer, heart disease, and AIDS—have undergone great strides in treatment across the past quarter century. People do sometimes survive these diseases. But to date, science has been unable to make any kind of dent in Alzheimer’s. In fact, the problem is actually growing, due to the population bubble created by aging baby boomers.
It is a disease that ignores celebrity, income, character, and gender. President Ronald Reagan had it, and so did one of his most controversial allies, British Prime Minister Margaret Thatcher. So have movie stars, literary figures, sports heroes, criminals, humanitarians, geniuses, and dullards. It has claimed victims among the most wealthy, powerful, and famous: Rita Hayworth, Norman Rockwell, E. B. White, Sugar Ray Robinson, Charlton Heston, Glen Campbell.
• • •
For such a formidable enemy, Alzheimer’s managed to keep a low profile for a surprisingly long time. The disease was first identified in 1906 by its namesake, Alois Alzheimer, a German psychiatrist who was also a neuropathologist, meaning he specialized in diseases of the brain and nervous system. But descriptions of similar symptoms have appeared in literature dating back to ancient times.
In the second century, Roman emperor Marcus Aurelius employed a Greek-born physician who used the term “morosis” to describe dementia. He described people afflicted with this condition as “some in whom the knowledge of letters and other arts are totally obliterated; indeed they can’t even remember their own names.”
In recent years, better diagnostic tools have allowed doctors to understand two sobering facts about the way they have approached Alzheimer’s disease: First, that senility is not a normal part of the aging process; people who were once generally described as “senile” often actually had Alzheimer’s, meaning it is a much more widespread disease than anyone realized. And the second fact, which is more frightening, is that no current medical intervention can reverse it, or even slow it down, because for most of the time science has known about Alzheimer’s, there has been no way to see it coming until it has already wreaked havoc within the walls of the brain.
From 1906, when Alois Alzheimer first described the disease, until well into the twenty-first century, diagnosing Alzheimer’s disease in living patients was little more than an educated guess. Doctors relied on clinical tests, asking questions about the patient’s memory and ability to function. Though these tests depended on the patient’s honesty, doctors might separately verify answers with close friends or family members. There really weren’t objective physical tests, although there were some telltale physical signs, such as a shuffling walk. Mood changes could occur, too; aggression, hallucinations, and depression were common.
But all of these symptoms can also point to other afflictions: meningitis, brain trauma, stroke, syphilis, and medication side effects can produce similar results. Even sleep apnea and urinary tract infections can cause confusion. And while Alzheimer’s disease is the leading cause of dementia, accounting for 60 to 80 percent of all cases, other causes exist, too, such as Parkinson’s and Huntington’s diseases. The word “dementia” is a general catch-all term encompassing many abnormalities.
A study of 852 men diagnosed with Alzheimer’s disease from 1991 through 2012 found that the diagnosis was wrong one-third of the time, correct one-third of the time, and partially wrong—in other words, the patient had a mixture of diseases—one-third of the time. And in situations where a patient is young or a doctor has limited experience with memory disorders, the diagnosis becomes even more elusive.
For most of the time science has known about the disease, a true, definitive diagnosis of Alzheimer’s—not probable Alzheimer’s—could only happen after death, when a neuropathologist examined brain samples under a microscope to confirm the presence of amyloid plaques and tau tangles, the abnormal proteins that are the disease’s grim signature. Plaques are sticky, microscopic clumps of stray amyloid proteins that form outside the brain cells and possibly prevent the cells from signaling each other. Tangles occur inside the brain cell. They are twisted fibers of the tau protein, which—in its normal state—helps transport nutrients. When its strands begin to twist, they choke the transport system and the cell dies.
Current consensus within the Alzheimer’s research field holds that early intervention is key; by the time a person shows what we think of as mild symptoms, such as occasional forgetfulness, the brain may have reached a tipping point from which it will not return. But just how far in advance a doctor would need to give a treatment isn’t known. Is ten years before the onset of symptoms soon enough? Should it be sooner? Can it be later? If scientists were working with a patient who knew that he would develop Alzheimer’s at a specific age, they could answer these questions faster.
So even as they search for a viable treatment, researchers also continue to seek out ways to predict who the disease will strike. If they know who will someday get Alzheimer’s, they want to treat that person before he begins to slip away, much the way possible cardiac patients are now given cholesterol-lowering medication to help them avoid hear
t attacks. But to find such a treatment, doctors need a patient who is guaranteed, with 100 percent certainty, to get the disease—only then will they know if an experimental treatment was successful, by testing it out on that person and then measuring its effect.
Those perfect patients do exist, as one tiny sliver of the population who stand distinctly apart from the rest. They are the people living with one of three known genetic mutations that guarantee they will be stricken. Only about 1 percent of all Alzheimer’s patients fall into this category. They are hit young: Their average age of onset is between thirty and fifty years old. Often, they have children, not knowing they stand a 50 percent chance of passing on the mutation; so the disease has raged silently through generations of families. For as little as science has known about Alzheimer’s, it’s known even less about these mutations.
But in nature, curses are often a double-edged sword. As tragic as mutations are, they may well hold the key to preventing—or at least delaying—Alzheimer’s. Doctors can diagnose patients with mutations years before symptoms appear, even in childhood. By testing preventative drugs in this population, researchers hope—and the rest of the world prays—that they will be able to translate a successful treatment to the rest of humanity before another generation is lost.
To get to that point, quiet sacrifices have been made by the most ordinary of people. They could be your neighbor, your coworker, your high-school classmate. Their lives were sometimes colorful, sometimes simple; but in their mutations, they have become exceptional. For it is their courage, often driven by desperation—sometimes tempered by fear or frustration—that has fueled the science that hopes to beget the solution. These are the people future generations will thank when Alzheimer’s itself becomes a distant memory.
• • •
Alois Alzheimer was a bespectacled, cigar-loving, robustly built man. He took a job in 1888 at the Frankfurt Asylum for the Insane and the Epileptic—a facility housed in a fairy-tale Gothic revival building known colloquially as the “Castle of the Insane.”
The Inheritance Page 1
