Mercies in Disguise

January 23, 2017
Kolata (Flu), a science journalist for the New York Times, shares the gripping story of how one South Carolina family has dealt with Gerstmann-Straussler-Scheinker disease, a deadly and untreatable genetic illness. Her wonderful presentation of cutting-edge biomedical research offers insight into some of the scientists who carry it out while exploring the struggles and unhappiness of patients and their families. Kolata also examines a host of intractable ethical issues associated with the disease: Should individuals be tested to determine whether, sometime in the future, they will fall victim to the flaw in their genome, knowing that no palliative treatment is possible? Should embryos be genetically tested and those with the variant gene discarded? The family Kolata follows is devoutly Southern Baptist, which provides the opportunity to consider the myriad ways religion and science interact in such complex situations. Observing the familial interactions and the manner by which different individuals process the same information proves fascinating. The book’s only real flaw derives from the overwrought manner in which every action, whether mundane or medically critical, gets raised to crisis level. Kolata’s book reads like a medical thriller and readers will be caught up in the lives of the protagonists. Agent: Katinka Matson, Brockman Inc.

February 1, 2017
A family's legacy is haunted by a torturous genetic disease.New York Times science reporter Kolata (Rethinking Thin: The New Science of Weight Loss--the Myths and Realities of Dieting, 2007, etc.) adroitly profiles the plight of the close-knit, rural South Carolina-based Baxley family, hounded by the presence of a "rare, anomalous" neurodegenerative disease. "Abrupt in its onset and unswerving in its course," the incurable, inherited, and ultimately fatal disease commingles characteristics from both Alzheimer's and Parkinson's diseases. Inherited via genetic mutations, the malady renders those afflicted with initial dizziness, followed by severe tremors, facial freezing, mute dementia, and death. Tim Baxley and his three brothers lost their father, Bill, to the disease in the late 1990s after a frustrating cycle of neurologist visits and futile personal fact-finding missions. Throughout the chronicle of the Baxleys and how they scoured their family tree searching for answers, Kolata deftly weaves in the history of kuru disease. She explores the funereal endocannibalistic rituals of Papua New Guinea's Fore people as both the source of its origins and its outward transmission. American doctor Daniel Carleton Gajdusek performed dogged research on kuru, and he received the Nobel Prize for his work in 1976. Another distinguished researcher, Stanley Prusiner, also won the Nobel in 1997 for his work with associated "mad cow disease," just as, several years later, Baxley family members began to succumb to kuru one by one. Kolata puts a human face on this incurable, agonizing disease with an affecting combination of neuroscience and anguished anecdotes centered on a loving family at the mercy of an intricate congenital infection. In the concluding section, the story becomes optimistic as the family's next generation agonizes over the decision to test for the gene mutation but finds hope through a risky, radical profiling procedure that allows for a prenatal diagnosis of at-risk human embryos. The panic is palpable in Kolata's moving depiction of a mysterious disease and its frightening consequences.

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February 1, 2017
A genetically afflicted family in South Carolina, the science of prions, and unconditional love come together in a story that pits hope against fate, courage against uncertainty. When three Baxley men are stricken with a strange set of neurodegenerative symptomsfailing coordination, muscle pain and weakness, difficulty speaking and swallowingthe search is on for an answer. The cause is Gerstmann-Straussler-Scheinker syndrome (GSS), an exceedingly rare autosomal dominant genetic mutation. Children of affected parents have a 50-percent chance of inheriting it. A blood test can detect the mutation, but the disease is fatal. New York Times science and medical writer Kolata poses the paramount question, Would you want to know if you carried a fatal gene mutation? Amanda Baxley, whose grandfather, father, and uncle die from GSS, elects to get tested at 26. Her positive result for GSS irrevocably alters her life. But in vitro fertilization (IVF), preimplantation genetic diagnosis (PGD), and plenty of pluck result in her having twins without the lethal mutation. Kolata delivers an inspiring chronicle of a remarkable family, medical advances, and redefining destiny.(Reprinted with permission of Booklist, copyright 2017, American Library Association.)

November 1, 2016

Like the DeMoes (see Kapsambelis's The Inheritance, previewed above), the Baxleys are a close-knit family undermined by Alzheimer's. They face a dilemma: whether to take the test that can tell them who has inherited the deadly mutated gene. From New York Times science reporter Kolata.

Copyright 2016 School Library Journal, LLC Used with permission.

December 1, 2016

Kuru, an incurable genetic disease native to Papua New Guinea, finds its way to the Baxleys, a conservative Christian family of doctors in small-town South Carolina who battle kuru--a degenerative and deadly mix of Parkinson's and Alzheimer's--while trying to uncover its mysterious origin and possibly escape its fate. New York Times science and medicine reporter Kolata (Rethinking Thin: The New Science of Weight Loss--and the Myths and Realities of Dieting) dedicates the absorbing first half of this book to the parallel accounts of doctors investigating the mystifying disease (neurologist Stanley Prusiner and physician Daniel Gajdusek would both win Nobel Prizes for their work) and the Baxley family's struggle to understand and cope with its devastating effects. The narrative weakens in the second half, when it narrows its focus to Amanda Baxley, whose determination to have children but not pass on the deadly gene leads to a plodding medical journey. But the interweaving tales of science, family, and medical ethics make for a compelling read. VERDICT Though at times the book adopts the tone of a Hallmark made-for-TV movie, its hopeful, struggle-against-the-odds story will find a library audience. [See Prepub Alert, 9/19/16.]--Chad Comello, Morton Grove P.L., IL

Copyright 2016 Library Journal, LLC Used with permission.