THE VANISHING FAMILY: Love, Fate, and the Quest to End Dementia, by Robert Kolker
Robert Kolker’s new book, “The Vanishing Family,” opens on a note of suspense.
Barb, 41, has received multiple envelopes from the Mayo Clinic, beseeching her to learn her genetic fate: Does she or does she not have the mutation that guarantees she will develop the rare disease frontotemporal dementia as early as her 40s, just as her mother and — so far — five of her eight siblings have?
A mother of two young children, Barb throws many of these envelopes away, unopened — even though, months before, she had in fact traveled to Rochester, Minn., alone and in secret. But when confronted with the chance to find out her results, she isn’t ready to know.
Barb and her siblings family had seemed the ideal American brood, residing in a five-bedroom colonial in suburban Pennsylvania. Their father, Ollie, was an engineer; their mother, Jean, devoted herself to domestic perfection, cooking two dinners a night — kid and adult — often in high heels.
But at some point in her 40s, Jean began to undergo a strange metamorphosis, sitting for hours in front of the TV in a haze of indifference; in response, Ollie began to absent himself from the household altogether.
The family attributed Jean’s changes to her increasingly heavy drinking. But when they staged an intervention, and she readily gave up alcohol, it didn’t help; her old personality continued to disappear. Barb, 10 years younger than her next sister, was often the only person to witness her mother’s disintegration.
“The Vanishing Family” is Kolker’s gripping follow-up to the best-selling “Hidden Valley Road,” in which his subject was a family of 12 siblings, six of whom developed schizophrenia. Here, as in that book, Kolker displays his genius for the immersive detail: calm, purposeful prose that builds to an extraordinary sense of knowing an entire family quite well — especially their sibling dynamics under extreme duress.
Just as he did with the field of schizophrenia science, Kolker gives us a sweeping view of evolving brain research. We learn about Phineas Gage, the unfortunate railroad foreman who, in 1848, accidentally launched the field of neuropsychology when an iron rod blew through his skull, piercing his frontal lobe. Gage survived, but with personality changes, becoming a walking illustration of the way our brains underpin our minds, and our selves.
Until the 20th century, the consensus was that dementia was synonymous with aging, rather than a distinct condition with a potential cure. Alzheimer’s, a disease that causes symptoms of dementia, was rarely referenced in medical textbooks through the 1960s. And yet people were living ever longer and more commonly developing the memory and cognition loss that are dementia’s hallmarks.
In 1976, a neurologist named Robert Katzman argued that most cases of dementia — and there were millions of them — should be reclassified as Alzheimer’s. In this way, Alzheimer’s would come to qualify as one of the world’s most common causes of death. The campaign worked: “Alzheimer’s disease was neuroscience’s public enemy number one,” Kolker writes. Yet it also subsumed other dementia disorders, like the one that afflicts Barb’s family.
Alzheimer’s is marked by brain changes visible in autopsy — the distinctive plaques and tangles that Alois Alzheimer, the doctor for whom the disease is named, had observed decades before. The plaques, researchers later discovered, were made from beta-amyloid protein, and the tangles from a protein called tau. A major split occurred in the field: What mattered more, the amyloid or the tau?
By the early 2000s, the debate seemed settled, in favor of the “baptists,” or those who focused on beta-amyloid protein. Tau tangles were now commonly seen as an artifact, “the word scientists use to describe something they think doesn’t matter at all,” Kolker writes. Never mind that frontotemporal dementia is marked by an overabundance of the tau protein.
Even when the field had appeared to form a consensus around amyloid plaques, there were always researchers looking elsewhere. Tom Bird, a neurogeneticist at the University of Washington, had, by the mid-1980s, been studying Alzheimer’s for a decade when he ran across a Seattle family that didn’t fit the hallmark symptoms.
Their dementia, which often developed young, didn’t cause them to lose their memory, as Alzheimer’s patients do, but brought alarming antisocial behaviors: They covered their windows with tin foil, or chased visitors with a pitchfork.
Bird thought this condition must be hereditary. As family members began to die, he discovered in autopsy that their brains were defined by tau tangles, with no visible proliferation of amyloid proteins. It would be more than another decade before Bird and his team, with the help of this family, identified the actual genetic mutation responsible: V337M.
Barb was the family member most proactive about understanding the condition that befell her closest relatives, one by one; she pressed her older siblings into research studies, attended medical conferences, learned the field. By the time Barb submitted her own DNA sample to the Mayo Clinic, she knew that nightmare string of chromosomal digits all too well.
FTD is a catastrophe for those who suffer from it, pulling them away from the people they love and the values they hold. It is a profoundly alienating disease, and researchers have made groundbreaking observations in recent years that correlate to its relational aspects — like a significant deficit of the specialized “von Economo” neuron that likely helps humans understand themselves in a social context, and behave accordingly.
In Barb’s family, some afflicted siblings have affairs; another compulsively eats and roams, sometimes into neighbors’ houses; another becomes estranged from her son. The tragedy of FTD is also pronounced for those who must take care of their relatives, often for decades.
Yet in Kolker’s story the devastation is most acute in relation to the third generation, the young nieces and nephews whom we don’t get to know as well as the original nine but whose genetic fates we will learn.
This is something that subtly distinguishes “The Vanishing Family” from “Hidden Valley Road”: the knowledge that these young adults are finding out in the present tense whether or not they are destined for frontotemporal dementia, and trying to make their lives the faces of this burden.
One of Barb’s nephews, in his early 30s, sits down to compose a letter to his mother years after learning he is positive for the mutation. It’s a message he needs to express, though his mother is no longer able to truly absorb its meaning. “There’s still no cure, but we know so much more,” he writes. “I hope it happens soon enough for me. If not, I hope it happens soon enough for my little brother, my cousins. I wish that it had been soon enough for you. I miss you. I love you.”
THE VANISHING FAMILY: Love, Fate, and the Quest to End Dementia | By Robert Kolker | Doubleday | 368 pp. | $32
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