Robert Kolker
Doubleday (US), 29 September; Quercus (UK), 8 October
By the time Barb got the letter with her test results, she had already seen her mother and five of her nine siblings gradually “disappear”. From their forties, they had started to act in unusual ways: they had ignored their children, become unrecognisable to their spouses and lost all sense of social norms.
It made her very nervous about her own fate. She knew she had a 50 per cent chance of inheriting the same gene for dementia that had upended the lives of her loved ones. If she was also destined to have the condition, what would happen to her husband, friends and two children? Who might she become when she was no longer herself?
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Barb’s heart-wrenching story, and that of her family, is compassionately told in by journalist Robert Kolker. This latest work follows his bestselling Hidden Valley Road, a book chronicling the true story of a Colorado family where six of the 12 siblings developed schizophrenia by their early twenties.
His new book tells the story of a Pennsylvanian family’s extraordinary struggle as they find themselves redefined by a rare, genetic form of dementia. It is a tale of enduring love and devastating grief, one that empathetically examines the tug-of-war many members face between living in the present and planning for the future – while trying to accept the past. It also offers a window into the genetics and neuroscience that could one day deliver a cure.
The book, which reads like a medical detective story, spends much of its time introducing Barb’s siblings. We learn, one by one, which sisters and brothers have frontotemporal dementia (FTD) and how it changes them. But those not heading for dementia face a different kind of challenge. Some choose to restructure their lives around caring for their siblings. They must navigate difficult, but necessary, conversations with their siblings’ children. This makes for a read that is tough yet engaging.
In one of the most moving sections, Kolker explores each family member’s decision over testing
Interspersed throughout the book, Kolker skilfully weaves in the history of how researchers, often via extreme case studies (such as a man whose brain was punctured by a pole), unravelled the function of the frontotemporal lobes of the brain, which are most affected by FTD.
FTD makes up less than 5 per cent of dementia cases and tends to occur much earlier in life than Alzheimer’s disease, the most common kind of dementia, which usually emerges after age 65. During the early stages of FTD, people are often impulsive and apathetic, without the memory problems that typify the first stages of Alzheimer’s. The later stages of FTD, however, more closely resemble those seen with Alzheimer’s – people become unable to speak, dress themselves or use the bathroom on their own.
As Kolker deftly explains, Barb’s family have a version of FTD caused by a mutation in the MAPT gene, which encodes for tau, a protein that usually helps maintain the structure and function of neurons. The genetics mean each family member has a 50-50 chance of inheriting the mutation, which causes toxic levels of tau to accumulate in the brain – and can be detected with a genetic test.
In one of the most moving sections of the book, Kolker explores each family member’s decision about whether to get tested. He describes Barb’s dilemma over the repercussions for her children: “Was not learning the truth about herself all this time the same thing as misleading them? Or was Barb protecting them – giving them a grace period, a few blessed years to be free, to feel normal, to keep the future at bay.”
Yet the story ends on a hopeful note. Kolker describes Barb’s efforts to organise and campaign for a cure. Dementia research, and the funding that drives it, has long focused on Alzheimer’s disease, and a protein that accumulates during it, called beta-amyloid. But scientists are increasingly finding ways to deplete toxic forms of tau that also play a role in Alzheimer’s – and contribute to FTD.
As a health reporter who regularly covers advances in dementia research, I have seen this for myself. At the world’s largest Alzheimer’s conference in July, some of the most exciting results came from a of a drug called diranersen that reduced tau levels and slowed cognitive decline. The drug works by disrupting the genetic code, in the form of RNA, encoding for tau. A larger trial is underway.
Some of those affected by FTD formed a group called Cure MAPT FTD that pushes for trials of gene therapies and other tau-depleting drugs that could benefit families such as Barb’s. This makes The Vanishing Family a profoundly human account of what it is like to face dementia – as well as how we might one day end it.
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