\u201cThe Vanishing Family: Love, Fate and the Quest to End Dementia,\u201d<\/a> Robert Kolker chronicles three generations of a family grappling with just such a cruel reality. The author tells their dramatic story while also interweaving a broader one \u2014 how the medical understanding of dementia has progressed in fits and starts over the past 150 years and how, in the last decade, scientists have come to recognize that families like the one Kolker profiles may be key to finding a cure for all types of age-related brain impairment.<\/p>\n Atthapon \u2013 stock.adobe.com<\/p>\n
The resulting book is an unlikely page-turner, a dual-track narrative by turns absorbing and wrenching, informative and inspiring. It\u2019s a worthy follow-up to Kolker\u2019s 2020 best-selling \u201cHidden Valley Road: Inside the Mind of an American Family,\u201d about the descent into schizophrenia of six members of the Galvin family, and the genetic origins of that terrifying disease.<\/p>\n
In both books studying the science \u201cwas in the service of understanding the people, of walking in their footsteps,\u201d Kolker told The Post. Contacted four years ago by members of this family (their last name is not provided for privacy reasons) the author agreed to do a deep dive into their history just as he did with the Galvins\u2019.\u00a0<\/p>\n
Barb, 50, is the anchoring figure in \u201cThe Vanishing Family.\u201d She was born the youngest of nine children of a prosperous couple, Ollie and Jean, who lived in the suburbs of Pittsburgh. Still at home when her much-older siblings had left, Barb witnessed first-hand the breakdown of her parents\u2019 marriage and the drastic changes in her mother.<\/p>\n
Robert Kolker<\/p>\n
Once energetic and attentive, Jean, while still in her 50s, withdrew from the world and spent her days sitting in front of the television, smoking and drinking. She hid from everyone the symptoms of breast cancer, and after she passed away from that disease at age 62, Barb and her siblings concluded that their mother\u2019s unhealthy lifestyle brought about her death.\u00a0<\/p>\n
Six years later, when Barb was attending college, Christy, the second oldest in the family, and a successful executive with two children, underwent an eerily familiar transformation. Her marriage ended, and Christy, then age 44, stopped taking care of herself. She refused to make meals, acted strangely in social situations, could no longer handle her job. The situation reminded Barb of what had happened with her mother: Both women\u2019s marriages had failed and they lost interest in life. Was there some strain of melancholia that ran in the family?\u00a0<\/p>\n
Barb\u2019s next oldest sister, Jenny, pushed for a more definitive diagnosis. Eventually, Christy was told she suffered from Pick\u2019s disease, a form of frontotemporal dementia that occurs in people under age 65. Most cases of Pick\u2019s are \u201csporadic,\u201d meaning not inherited.\u00a0<\/p>\n
Nonetheless, in 2001, Jenny contacted an expert at the University of San Francisco where scientists were beginning to link certain genes to dementia. She, Barb, Christy and another sister, Sue, who gave up her career to take care of Christy, submitted DNA samples. After hearing nothing back from the lab, they assumed they were in the clear.\u00a0<\/p>\n
As the author makes clear throughout the book, denial is a frequent response to mysterious medical events. \u201cOf course, there is a difference between failing to find a hereditary link to a rare disease and proving without doubt that the disease isn\u2019t hereditary,\u201d Kolker writes.\u00a0<\/p>\n
Four years later, Barb and her husband were living near Denver when they received an unexpected visit from her one of her older sisters, Mary, then 47. Mary couldn\u2019t explain why she had come, did not extend everyday courtesies to Barb\u2019s friends and family and spoke about her complicated profession in juvenile language.<\/p>\n
A conceptual 3D illustration shows an elderly person with progressive impairments of brain functions and destruction of neurons and their networks. Dr_Microbe \u2013 stock.adobe.com<\/p>\n
\u201cThat weekend Barb saw it all \u2014 the same movie, playing again,\u201d Kolker writes. \u201cThe old Mary was disappearing, with nothing to replace her.\u201d<\/p>\n
For Barb that was the moment when she became convinced that Jean, Christy and Mary suffered from a genetic condition, and \u201cwe might all have it.\u201d\u00a0<\/p>\n
In the suspenseful chapters that follow \u2014 which Kolker based on his lengthy interviews with numerous family members \u2014 Barb and her sisters piece together clues, including those offered by distant relatives, and consult experts. They eventually discover that a mutation on chromosome 17, V337M, which causes a rare form of early-onset FTD, runs in their family. Offspring of those carrying the mutation have a 50\/50 chance of inheriting it themselves. Everyone in the family thus has a choice: Should they take the test and learn if they will succumb to dementia while still in midlife?\u00a0<\/p>\n
Thanks to Kolker\u2019s intimate, empathetic writing, the fate of individual family members takes on great urgency. The family pulls together to support and care for each other as the bad news unspools.\u00a0<\/p>\n
\u201cI think every reader will naturally ask themselves if they had a crystal ball would they really want to know?\u201d the author told the Post.<\/p>\n
Atthapon \u2013 stock.adobe.com<\/p>\n
The scientific chapters of \u201cThe Vanishing Family\u201d contain their own drama and poignancy. For most of history, dementia was considered a natural, generalized condition, evidence of a brain inevitably slowing down. Then, in the early 20th century, Dr. Alois Alzheimer identified unique brain abnormalities \u2014 amyloid plaques and neurofibrillary tangles of a protein called tau \u2014 present in the brain tissue of those who died with dementia.<\/p>\n
By the 1970s Alzheimer\u2019s was widely accepted as the leading cause of cognitive decline in the elderly, and many researchers fixated on the \u201camyloid cascade hypothesis\u201d \u2014 the theory that the accumulation of plaque brought on the disease. \u201cIt\u2019s a story of group think, the kind that often happens in science,\u201d Kolker explained to The Post. Even as drug after drug designed to clear plaque ended up having no positive benefit for patients, the way abnormal changes in tau might contribute to Alzheimer\u2019s progression remained relatively unexplored.\u00a0<\/p>\n
Only recently have scientists grasped that rarer, simpler forms of dementia, including FTD, can, in Kolker\u2019s words, \u201cactually teach you something about how to treat Alzheimer\u2019s.\u201d Barb\u2019s family\u2019s mutation \u2014which causes normally helpful tau proteins to malfunction, and negatively impact behavior, personality, and cognitive function \u2014 falls in this category.<\/p>\n
Barb and other family members have, through the Association for Frontotemporal Degeneration, lobbied scientists to pay more attention to the disease. Kolker reports that \u201ctau-targeting drugs are currently in the testing phase, and also gene therapy and gene editing may address specific mutations in future.\u201d\u00a0<\/p>\n
But it\u2019s vital that patients get tested and come forward. \u201cDoctors don\u2019t want to bother testing for incurable rare diseases because what\u2019s the point,\u201d Kolker told The Post. \u201cBut if there is to be a hope of a cure, they need patients on the rolls to convince researchers and drug companies that it\u2019s worth trying to find a treatment.\u201d The estimated number of FTD cases in the US \u2014 60,000 \u2014 is likely a massive undercount.\u00a0<\/p>\n
\u201cThe Vanishing Family\u201d provides a fascinating window on innovations the future could hold, but also what might happen to any of us as we \u2014 and our loved ones \u2014 change over time.\u00a0<\/p>\n","protected":false},"excerpt":{"rendered":"Do the crossword every day. Exercise 150 minutes per week. Follow the Mediterranean diet.\u00a0 While for most people…\n","protected":false},"author":2,"featured_media":862762,"comment_status":"","ping_status":"","sticky":false,"template":"","format":"standard","meta":{"footnotes":""},"categories":[34],"tags":[18368,223,1103,97,149,1337,42337,79,673],"class_list":["post-862761","post","type-post","status-publish","format-standard","has-post-thumbnail","category-health","tag-alzheimers-disease","tag-books","tag-dementia","tag-health","tag-lifestyle","tag-neuroscience","tag-postscript","tag-science","tag-wellness"],"_links":{"self":[{"href":"https:\/\/www.newsbeep.com\/us\/wp-json\/wp\/v2\/posts\/862761","targetHints":{"allow":["GET"]}}],"collection":[{"href":"https:\/\/www.newsbeep.com\/us\/wp-json\/wp\/v2\/posts"}],"about":[{"href":"https:\/\/www.newsbeep.com\/us\/wp-json\/wp\/v2\/types\/post"}],"author":[{"embeddable":true,"href":"https:\/\/www.newsbeep.com\/us\/wp-json\/wp\/v2\/users\/2"}],"replies":[{"embeddable":true,"href":"https:\/\/www.newsbeep.com\/us\/wp-json\/wp\/v2\/comments?post=862761"}],"version-history":[{"count":0,"href":"https:\/\/www.newsbeep.com\/us\/wp-json\/wp\/v2\/posts\/862761\/revisions"}],"wp:featuredmedia":[{"embeddable":true,"href":"https:\/\/www.newsbeep.com\/us\/wp-json\/wp\/v2\/media\/862762"}],"wp:attachment":[{"href":"https:\/\/www.newsbeep.com\/us\/wp-json\/wp\/v2\/media?parent=862761"}],"wp:term":[{"taxonomy":"category","embeddable":true,"href":"https:\/\/www.newsbeep.com\/us\/wp-json\/wp\/v2\/categories?post=862761"},{"taxonomy":"post_tag","embeddable":true,"href":"https:\/\/www.newsbeep.com\/us\/wp-json\/wp\/v2\/tags?post=862761"}],"curies":[{"name":"wp","href":"https:\/\/api.w.org\/{rel}","templated":true}]}}