Mercies in disguise A story of hope, a family's genetic destiny, and the science that rescued them

Gina Bari Kolata, 1948-

Book - 2017

"The phone rings. The doctor from California is on the line. "Are you ready Amanda?" The two people Amanda Baxley loves the most had begged her not to be tested-at least, not now. But she had to find out. If your family carried a mutated gene that foretold a brutal illness and you were offered the chance to find out if you'd inherited it, would you do it? Would you walk toward the problem, bravely accepting whatever answer came your way? Or would you avoid the potential bad news as long as possible? In Mercies in Disguise, acclaimed New York Times science reporter and bestselling author Gina Kolata tells the story of the Baxleys, an almost archetypal family in a small town in South Carolina. A proud and determined clan, ...many of them doctors, they are struck one by one with an inscrutable illness. They finally discover the cause of the disease after a remarkable sequence of events that many saw as providential. Meanwhile, science, progressing for a half a century along a parallel track, had handed the Baxleys a resolution-not a cure, but a blood test that would reveal who had the gene for the disease and who did not. And science would offer another dilemma-fertility specialists had created a way to spare the children through an expensive process. A work of narrative nonfiction in the tradition of the The Immortal Life of Henrietta Lacks, Mercies in Disguise is the story of a family that took matters into its own hands when the medical world abandoned them. It's a story of a family that had to deal with unspeakable tragedy and yet did not allow it to tear them apart. And it is the story of a young woman-Amanda Baxley-who faced the future head on, determined to find a way to disrupt her family's destiny."--

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Subjects
Published
New York : St. Martin's Press 2017.
Language
English
Main Author
Gina Bari Kolata, 1948- (author)
Edition
First edition
Item Description
Includes index.
Physical Description
viii, 262 pages ; 22 cm
ISBN
9781250064349
  • Introduction
  • Part I. A disease
  • Prologue
  • 1. The Prophecy
  • 2. "No Goddamn Bastard on Earth Has Ever Seen a Disease like This Thing"
  • 3. Revelation
  • 4. An Uncertain Inheritance
  • 5. Truth like Science Fiction
  • 6. Birthright
  • 7. Insomnia
  • 8. Naming the Problem
  • 9. No Return
  • 10. Tested
  • Part II. Amanda Baxley: The Next Generation
  • 11. A Life Transformed
  • 12. Options
  • 13. A Foreign Land
  • 14. The Envelope
  • 15. Of Two Minds
  • 16. A Unique Way
  • 17. "We Have No Other Options"
  • 18. Prescription Fertility
  • 19. New Lives
  • 20. Carrying Forth
  • Epilogue
  • Notes
  • Acknowledgments
  • Index
Review by New York Times Review

HILLBILLY ELEGY: A Memoir of a Family and Culture in Crisis, by J.D. Vance. (Harper, $16.99.) Vance uses the lens of his childhood to analyze the despair and stagnation of his white working-class America. He doesn't hesitate to blame Appalachian culture, which he says "increasingly encourages social decay instead of counteracting it," but offers a compassionate primer on the struggles of the white underclass. COMPASS, by Mathias Énard. Translated by Charlotte Mandeli. (New Directions, $18.95.) The narrator, a Viennese musicologist dying of an unknown illness, spends a sleepless night dreaming of his travels to the Levant; the careers and work of other scholars of the East he has known; and his great love. The novel won the Goncourt, France's most prestigious literary prize, in 2015. MERCIES IN DISGUISE: A Story of Hope, a Family's Genetic Destiny, and the Science That Rescued Them, by Gina Kolata. (St. Martin's, $16.99.) Kolata, a science reporter for The Times, follows members of the Baxley family, who were blindsided after learning their father had a rare neurodegenerative disorder. As they grapple with their own risk, the story poses a wrenching question: Would you want to know if you carried a fatal genetic mutation? SWIMMER AMONG THE STARS: Stories, by Kanishk Tharoor. (Picador, $16.) In tales that leap across time and space, Tharoor considers the tensions of cultural preservation and loss, and the burdens of power. A princess's gesture has profound consequences for an elephant; diplomats orbiting Earth must choose a new headquarters for the United Nations. And in the title story, ethnographers pay a visit to the last speaker of a language, who tries to invent words for modern terms: astronaut, tractor, prime minister. JANE AUSTEN: The Secret Radical, by Helena Kelly. (Vintage, $17.) In the 200 years since her death, Austen has remained categorically misunderstood, and deserves to be read with an eye to Britain's politics of the time, Kelly argues. She is particularly convincing on the Austen family's designs to neutralize Jane's image over the decades; as our reviewer, John Sutherland, put it, "Colin Firth's wet shirt is hung out to dry." GIRL IN SNOW, by Danya Kukafka. (Simon & Schuster, $16.) After the murder of a beautiful teenager, Lucinda Hayes, three misfit characters offer clues to the crime: the outcast who loved - and stalked - Lucinda; a classmate who couldn't stand her; and even the police officer investigating the case, with a personal connection to a leading suspect. This thrilling debut novel has strains of "Twin Peaks."

Copyright (c) The New York Times Company [August 30, 2019]
Review by Booklist Review

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.--Miksanek, Tony Copyright 2017 Booklist

From Booklist, Copyright (c) American Library Association. Used with permission.
Review by Publisher's Weekly Review

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. (Apr.) © Copyright PWxyz, LLC. All rights reserved.

(c) Copyright PWxyz, LLC. All rights reserved
Review by Library Journal Review

Narrative nonfiction doesn't get much better than this. New York Times science reporter Kolata brings a family's harrowing medical crisis to life and tells the story of how a brave young daughter decides to reshape her future and put an end to the genetic disease that had been killing off her family for generations. Kolata researches the history of the mysterious illness while concurrently telling the story of a loving, small-town family that appears to be plagued by bad luck. When the patriarch dies, his sons take matters into their own hands, trying to track down the condition and piece together their family tree to see what the future may hold in store. Compellingly narrated by Andrea Gallo, who provides a wonderfully reassuring and authoritative voice. VERDICT Will appeal to general listeners as well as those interested in medical or scientific nonfiction. ["The interweaving tales of science, family, and medical ethics make for a compelling read": LJ 12/16 review of the St. Martin's hc.]-Erin Cataldi, Johnson Cty. P.L., Franklin, IN © Copyright 2017. Library Journals LLC, a wholly owned subsidiary of Media Source, Inc. No redistribution permitted.

(c) Copyright Library Journals LLC, a wholly owned subsidiary of Media Source, Inc. No redistribution permitted.
Review by Kirkus Book Review

A family's legacy is haunted by a torturous genetic disease.New York Times science reporter Kolata (Rethinking Thin: The New Science of Weight Lossthe 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. Copyright Kirkus Reviews, used with permission.

Copyright (c) Kirkus Reviews, used with permission.