Booklist Reviews
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 symptoms—failing coordination, muscle pain and weakness, difficulty speaking and swallowing—the 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. Copyright 2017 Booklist Reviews.
BookPage Reviews
An infected family tree
Imagine watching your father die slowly from a degenerative neurological disease so rare that there is no cure. Imagine, then, coming to grips when you learn the disease is genetic and there is a good chance that you carry those genes, and will not only die from the disease but pass it along to your children. If you could take a simple blood test to reveal whether or not you have the genes, would you take it?
Part medical mystery, part family history and part medical history, Gina Kolata's gripping Mercies in Disguise: A Story of Hope, a Family's Genetic Destiny, and the Science That Rescued Them tells the story of one small-town Southern family ravaged by a mysterious illness. The Baxley family of Hartsville, South Carolina, always felt they were special. They were even named the South Carolina Family of the Year in 1985 by former governor Richard Riley. Following family patriarch Bill Baxley's death, they discovered their specialness arose not only from hard work and community respect but from carrying a rare disease that started to afflict almost every member of the family.
After watching her father die slowly from Gerstmann-Sträussler-Scheinker (GSS) disease, Amanda Baxley, Bill Baxley's granddaughter, tests positive for this degenerative illness that is plaguing her family. In poignant detail, Kolata tells of Amanda's fear, hope, strength and courage as she chooses to undergo preimplantation genetic diagnosis, a procedure in which embryos with the disease are discarded while a healthy one is implanted. As a result of this procedure, Amanda delivers twins not long after her father breathes his last breath, and she sees her children as a gift of life from her father.
Kolata's book tells a riveting tale of fear and fierce determination in the face of an overwhelming medical situation that at first seems hopeless.
This article was originally published in the April 2017 issue of BookPage. Download the entire issue for the Kindle or Nook.
Copyright 2017 BookPage Reviews.Kirkus Reviews
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 ans wers, 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 p renatal diagnosis of at-risk human embryos. The panic is palpable in Kolata's moving depiction of a mysterious disease and its frightening consequences. Copyright Kirkus 2017 Kirkus/BPI Communications. All rights reserved.
Library Journal Reviews
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 Library Journal.
Library Journal Reviews
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.
Publishers Weekly Reviews
Kolata (