What do you think?
Rate this book
Mercies in Disguise: A Story of Hope, a Family's Genetic Destiny, and the Science That Rescued Them
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.
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.
272 pages, Hardcover
First published March 21, 2017
117 people are currently reading
2476 people want to read
About the author
Gina Kolata
32 books103 followersKolata graduated from the University of Maryland and studied molecular biology at the graduate level at MIT for a year and a half. Then she returned to the University of Maryland and obtained a master’s degree in applied mathematics. Kolata has taught writing as a visiting professor at Princeton University and frequently gives lectures across the country. She lives in Princeton, New Jersey, with her family.
Ratings & Reviews
Friends & Following
Create a free account to discover what your friends think of this book!
Community Reviews
Displaying 1 - 30 of 225 reviews
March 23, 2022
One by one, members of the Baxley family of Hartsville, South Carolina - respected members of the devoutly Christian community - were struck by a devastating neurodegenerative illness. The first victim was family patriarch Bill Baxley. Bill's symptoms began with a shuffling walk, which escalated to stumbling, weaving, and lurching. As Bill's illness progressed his memory faded, he lost control of his hands, speech became difficult, and he was unable to eat without choking. Eventually Bill could no longer walk or talk. The senior Baxley was confined to a wheelchair, his face a frozen mask, only able to communicate by grunting, nodding, and clumsily pointing to an alphabet board. For sustenance, Bill was fed liquified meals prepared by his devoted wife, Merle. Bill's children - Billy, Mike, Buddy, Tim, and Andrea - looked on with sadness and dismay.
Alphabet board
The medical community was mystified by Bill's illness. Starting in 1988, Bill's sons took him to doctor after doctor, all of whom diagnosed the sick man with 'a combination of Alzheimer's and Parkinson's diseases.' In desperation, sons Buddy and Tim - who were physicians - performed their own medical tests and devised their own treatments, but nothing helped and Papa Bill died in 1998.
Dr. Buddy Baxley tried to diagnose his father's illness with his brother Tim
Serendipitously, a woman at Bill's funeral - after some words of condolence to son Tim - mentioned, "I remember [Bill's] daddy walking through the plant. He had to hang on to the machines." This was the first hint that Bill's disease might be hereditary - passed from one generation to the next. Sadly, the inheritable nature of Bill's illness was confirmed when son Mike researched the family tree and found ancestors with the condition.
In time researchers learned that Bill's illness was related to two other neurodegenerative diseases, Kuru and Creutzfeldt-Jakob Disease (CJD). Kuru is a sickness seen in members of the Fore tribe of Papua New Guinea. In the 1950s and 60s, American doctor Daniel Carleton Gajdusek - who worked with the Fore - discovered that brains of Kuru victims are filled with tiny holes and contain amyloid plagues - similar to the brains of people with Creutzfeldt-Jakob Disease (CJD).
Dr. Daniel Carleton Gajdusek
Boy in the Fore Tribe stricken with Kuru
Brain of a Kuru victim
Gajdusek's research also showed that both Kuru and CJD are caused by infectious agents - thought to be 'slow viruses' - that could be transferred from one animal to another. Gajdusek won the 1976 Nobel Prize for his Kuru work, but was never able to isolate the 'slow virus.'
Following Gajdusek's studies, an Amerian scientist named Dr. Stanley Prusiner became determined to isolate the agents that cause Kuru and CJD. He discovered they aren't viruses at all. In fact, the culprits are defective proteins called 'prions' which can be passed from diseased to healthy individuals. Furthermore, mutated genes code for the nasty prions. In 1997, Prusiner also won a Nobel Prize for his work.
Dr. Stanley Prusiner
Some neurogenerative diseases are caused by defective proteins called prions
In 1999, still unaware of the identify of their disease, additional members of the Baxley family began to succumb. Oldest son Billy fell ill first, followed by his sibling Buddy, and then Aunt Faye (Papa Bill's sister). Enlightenment was on the horizon though. Tim Baxley - studying for his medical boards - came across an article about Gerrstmann-Sträussler–Scheinker Syndrome (GSS).....and came to realize this was his family's ailment Patients with GSS - like victims of other neurodegenerative diseases - have brains filled with tiny holes, plaques, and damaged neurons.
Brain of GSS victim
By the early 2000's a blood test could detect the presence of the gene that causes GSS. This created a dilemma for the Baxleys. Would at-risk family members want to know if they were destined to fall ill.....or would they prefer to remain in the dark? Brothers Mike and Tim were tested early and learned they were free of the deadly mutation. Other Baxleys were more ambivalent, especially sick Buddy's children.
Buddy's daughter Amanda was a rebellious teen who grew up to become a nurse practitioner. For years Amanda wrestled with the problem of being tested for GSS - did she want to know if she had the gene or not? In time, hoping to get married, Amanda HAD to know! A good portion of the book tells her story.
Young Amanda Baxley with her father Buddy
Gina Kolata covers a lot of territory in this book. She gives a brief overview of neurodegenerative diseases and prions - and describes the work of Gujdusek and Prusiner in some detail. All of this is very interesting and informative. Kolata also provides a sympathetic picture of the extended Baxley family, all of whom show remarkable strength and faith in the midst of their ongoing ordeal.
Amanda Baxley Kalinsky, her husband Brad, and their three children
On the downside, parts of the book read like fiction. Kolata dramatizes scenes - providing conversations and inner thoughts - that seem made up. To me, this was distracting.
Still, this is a very good book about an engaging topic. Highly recommended.
[FYI: If I was in the Baxley's situation, I'd get tested in a second. Better to know than live in dread (IMO)]
Thanks to Netgalley, the author, and the publisher for a copy of this book.
You can follow my reviews at http://reviewsbybarbsaffer.blogspot.com/
November 17, 2016
Having a neurological disease is challenging, I live with one, but having a neurological disease that no one can diagnose, that has no treatment, no cure is terrifying. Having the symptoms of both Parkinson disease and Alzheimer, the inflicted slowly degenerate until death mercifully claims them. This is the disease confronting the Baxley family, a disease that has existed for generations but only when their father is inflicted do the two physician sons become aware of this fact. Setting out to find answers this is the story of a family unsure of their future, devastated by what has gone before.
Eventually an answer will be found and a test form the gene necessary to activate this fatal disease and then in the younger generation the question becomes, does one take the test and live with the fear of a death sentence or does one live their life never knowing until or if they are afflicted. This is the dilemma the younger generation, including Amanda, now in her late twenties, her father dying, must decide.
The book chronicles the discovery of this illness, the men behind it and the doctors who discover how to test for this very rare gene. These chapters are interspersed between chapters of the family. A wonderful family, whom one comes to know and care about, a family that has shared much happiness and much sadness. Although they all choose different paths, some of the decisions causing breaks in the family, they all come together in support, caring and a great deal of love and hope. The author does a wonderful job showing us the joy and heartbreak, following this family, chronicling their decisions, their doubts, their fears. That further sufferers of this or other neurological diseases may understand that they are so much more than what they are afflicted with, that life can still full, that making everyday count is perhaps bigger than the disease itself. I thank them for sharing their story with me, it was truly inspirational.
ARC from bookbrowse and St. Martin publishers.
Publishes in March, 2017
Eventually an answer will be found and a test form the gene necessary to activate this fatal disease and then in the younger generation the question becomes, does one take the test and live with the fear of a death sentence or does one live their life never knowing until or if they are afflicted. This is the dilemma the younger generation, including Amanda, now in her late twenties, her father dying, must decide.
The book chronicles the discovery of this illness, the men behind it and the doctors who discover how to test for this very rare gene. These chapters are interspersed between chapters of the family. A wonderful family, whom one comes to know and care about, a family that has shared much happiness and much sadness. Although they all choose different paths, some of the decisions causing breaks in the family, they all come together in support, caring and a great deal of love and hope. The author does a wonderful job showing us the joy and heartbreak, following this family, chronicling their decisions, their doubts, their fears. That further sufferers of this or other neurological diseases may understand that they are so much more than what they are afflicted with, that life can still full, that making everyday count is perhaps bigger than the disease itself. I thank them for sharing their story with me, it was truly inspirational.
ARC from bookbrowse and St. Martin publishers.
Publishes in March, 2017
April 3, 2017
The first 2/3rds of the book was a 5 star. The family story is from the inside perspective and in day to day, year to year as it happened in sequence- reactive reality. Utter bravery in telling the entire; feelings and physical suffering and gaps in understanding between family members (some perceiving to face it head on and others not at all persuaded in the aptitude to such knowledge being positive)but in every sense letting it "all" hang out. Very brave, in a world where such information could at one point or another, be detrimental. Not only within emotions, but within monetary or lawful fall outs.
The history of the research on kuru and prion protein mutations, all the prion related afflictions was 6 star. A neighbor of mine during this last year has died from CJD. Seeing this vastly quick transformation at an age that was middle age put this book into some visuals for me. It's easier to read descriptions than to see it. This GSS family's story is one of the most heart wrenching I have ever read. Doubly so because the cognitive ability toward dementia is not as altered. How cruel!
The last quarter of the book is about the grand-daughter of the original Bill who died from this GSS in his 60's. Her name is Amanda. It's very much like her own personality: dynamic, dramatic, emotive, proactive and choosing to be in the "know".
This is one of several other gene studies books in the non-fiction applications to gene mutations, double sequences, voids or other DNA studies which have developed testing that I've read during the last year. This is written clearer than most non-fiction in this field. Style and flow is appropriate and easily understandable to the layperson of no specific scientific interest, IMHO.
I am extremely interested in DNA testing and for VUS categories of differences that may affect metabolic assimilation especially interested.
The history of the research on kuru and prion protein mutations, all the prion related afflictions was 6 star. A neighbor of mine during this last year has died from CJD. Seeing this vastly quick transformation at an age that was middle age put this book into some visuals for me. It's easier to read descriptions than to see it. This GSS family's story is one of the most heart wrenching I have ever read. Doubly so because the cognitive ability toward dementia is not as altered. How cruel!
The last quarter of the book is about the grand-daughter of the original Bill who died from this GSS in his 60's. Her name is Amanda. It's very much like her own personality: dynamic, dramatic, emotive, proactive and choosing to be in the "know".
This is one of several other gene studies books in the non-fiction applications to gene mutations, double sequences, voids or other DNA studies which have developed testing that I've read during the last year. This is written clearer than most non-fiction in this field. Style and flow is appropriate and easily understandable to the layperson of no specific scientific interest, IMHO.
I am extremely interested in DNA testing and for VUS categories of differences that may affect metabolic assimilation especially interested.
February 2, 2018
The first half of this book alternates between scientific research into a certain type of neurological disease and an Eastern U.S. family experiencing such a disease. It takes years for the family to recognize it’s a genetic disease that’s afflicting many of them. The interspersed scientific discoveries take the mystery out of the family’s searching: The reader already knows what’s wrong with them, so watching the family puzzle it out becomes tedious at time. It would have been a tantalizing mystery to learn along with the family instead.
The second half of the book follows Amanda, a member of the third generation, as she makes decisions about education, marriage, and having children. By this time (2009ish), a blood test reveals whether someone actually has the disease, which can strike at any time in life. Then it goes into detail about the history of IVF, and it feels like the rare genetic disease is forgotten for a time. But it’s still interesting to see how the disease affects the family and their choices and their faith.
I think a bit of restructuring of the material would make it a better book. It’s too scientific to be a memoir/biography, but not scientific enough to be a science book. I also find it odd that people would refuse testing to find out whether they have the disease. If you don’t know, you’re just going to stress about it as much as if you did have it. So you might as well find out in case you don’t have to worry about it.
The second half of the book follows Amanda, a member of the third generation, as she makes decisions about education, marriage, and having children. By this time (2009ish), a blood test reveals whether someone actually has the disease, which can strike at any time in life. Then it goes into detail about the history of IVF, and it feels like the rare genetic disease is forgotten for a time. But it’s still interesting to see how the disease affects the family and their choices and their faith.
I think a bit of restructuring of the material would make it a better book. It’s too scientific to be a memoir/biography, but not scientific enough to be a science book. I also find it odd that people would refuse testing to find out whether they have the disease. If you don’t know, you’re just going to stress about it as much as if you did have it. So you might as well find out in case you don’t have to worry about it.
February 26, 2017
Those pesky prions have needed a popular-science storytelling for quite some time. Gina Kolata, arguably the best science and medicine writer at The New York Times, would seem just the person to do it. The problem is, this is not the definitive word on prions. We do get some pre-Prusiner coverage of how scrapie, kuru, and mad cow disease were linked as early as the 1950s. But there is no detailed discussion of errant protein folding here, no analysis of how prions spread disease not through replication via nucleic acid, but by the spreading of bad information.
This was not the kind of book Kolata set out to write. She wanted to provide a compassionate look at families facing unexpected deaths and disabilities, making moral choices on genetic screening, and the personal feel of an individual being marked for death. That's a worthy goal, to be sure, but it makes her book less interesting as a result.
The first half of the book is quite exciting, as one particular South Carolina family learns of its heritable prion crisis, and family members bond together to create family trees and detailed histories, and to care for the disease that strikes in middle age and acts "a little bit Alzheimer's, a little bit Parkinson's" in its manifestations. But in the second half of the book, as Kolata focuses in on Amanda, we follow this particular member of the family as she goes through courtship, a destination wedding, and decisions about in vitro fertilization, all with the sword of Damocles hanging over her head. Even with the book's sense of genetic dread, it's hard to deny the story drags midway through the book.
Kolata is a rationalist and wants to be sure to provide empathy for the deep faith of the Baxley family, a devout Southern Baptist clan. I wish she had been a little more judicious in how far to extend her understanding. Amanda's mother worries that discarding embryos that carry a gene marker in order to implant a disease-free egg in the uterus is somehow almost as "bad" as abortion. I can maybe go far enough to give her that due. But Amanda's older sister believes in the nonsensical phrase of "God's will," interpreting it to mean that science should never seek to uncover any of the mysteries of nature, and that learning one's genetic fate is somehow a sin. I find that deluded people use the phrase "God's will" to excuse all kinds of faults made by humans, and I personally think that no empathetic quarter be given for those crackpots who believe in "God's will."
Kolata bends over backward to write with passion about the power of the family. I cannot help but think she is paying a type of penance for her own dysfunctional past with her sister Judi Bari. As an outsider, I place the fault of sibling alienation largely at the feet of Kolata, since she believed so strongly in the power of science, she was not willing to see how people might be motivated by environmental passion. Is it possible this book is a backhanded admission that other ways of knowing are as powerful as science?
Kolata no doubt will win many new readers among families facing tough genetic decisions, as well as among Christians. The problem is, she has short-shrifted the science of prions in order to write a compassionate family tale. I think that even non-scientists would have appreciated a longer book with more details on prion science itself, to augment this brief tale of family love. Or maybe that's just me.
This was not the kind of book Kolata set out to write. She wanted to provide a compassionate look at families facing unexpected deaths and disabilities, making moral choices on genetic screening, and the personal feel of an individual being marked for death. That's a worthy goal, to be sure, but it makes her book less interesting as a result.
The first half of the book is quite exciting, as one particular South Carolina family learns of its heritable prion crisis, and family members bond together to create family trees and detailed histories, and to care for the disease that strikes in middle age and acts "a little bit Alzheimer's, a little bit Parkinson's" in its manifestations. But in the second half of the book, as Kolata focuses in on Amanda, we follow this particular member of the family as she goes through courtship, a destination wedding, and decisions about in vitro fertilization, all with the sword of Damocles hanging over her head. Even with the book's sense of genetic dread, it's hard to deny the story drags midway through the book.
Kolata is a rationalist and wants to be sure to provide empathy for the deep faith of the Baxley family, a devout Southern Baptist clan. I wish she had been a little more judicious in how far to extend her understanding. Amanda's mother worries that discarding embryos that carry a gene marker in order to implant a disease-free egg in the uterus is somehow almost as "bad" as abortion. I can maybe go far enough to give her that due. But Amanda's older sister believes in the nonsensical phrase of "God's will," interpreting it to mean that science should never seek to uncover any of the mysteries of nature, and that learning one's genetic fate is somehow a sin. I find that deluded people use the phrase "God's will" to excuse all kinds of faults made by humans, and I personally think that no empathetic quarter be given for those crackpots who believe in "God's will."
Kolata bends over backward to write with passion about the power of the family. I cannot help but think she is paying a type of penance for her own dysfunctional past with her sister Judi Bari. As an outsider, I place the fault of sibling alienation largely at the feet of Kolata, since she believed so strongly in the power of science, she was not willing to see how people might be motivated by environmental passion. Is it possible this book is a backhanded admission that other ways of knowing are as powerful as science?
Kolata no doubt will win many new readers among families facing tough genetic decisions, as well as among Christians. The problem is, she has short-shrifted the science of prions in order to write a compassionate family tale. I think that even non-scientists would have appreciated a longer book with more details on prion science itself, to augment this brief tale of family love. Or maybe that's just me.
May 29, 2018
Recommended by my friend Kate. May review in the future. It was excellent.
May 5, 2017
4.5 stars.
This was a tragic yet amazing story of a family who learns eventually that a neurological disease is destroying their family. This disease relatively unheard of, is transmitted genetically and though it is found out many years after the fact, this disease had taken the life of many members of the Baxter family. Finally, one member of the family realizes that the occurrence in family members follows a pattern, does extensive work on ferreting out family members who have succumbed and have shown symptoms, and eventually doctors are able to understand this neurological illness. The symptoms, mimicking Parkinson and Alzheimer's, leave the person so debilitated that death is actually a relief. The person suffering from this eventually loses all his/her faculties, losses the ability to walk, talk, and eat.
Imagine finding this out, knowing there is no cure, and eventually learning that you carry this gene and this is your fate? Through science and technology members of this family and of course other families can now find out if the wish, that they carry this disease. Through the use of IVF they can, after the fertilized egg has been tested, have disease free children since only healthy fertilized eggs are implanted in the mother's womb. Imagine the decision one makes to have children knowing that you might develop the symptoms of this illness in your late thirties and eventually become a burden to your family and children? This is the decision one young couple has to make in this book.
This was truly heart breaking story that makes one ever so grateful for the blessed gift of health. The death that people who carry this prion disease is an awful fate and hopefully advances in brain research will be successful in helping those affected with an eventual therapy and cure.
The author did a wonderful job presenting the family, the doctors, and the scientists who are dealing with this most awful disease. This book is well worth the read if you are at all interested in the dedication and love a family shares as they face illness and death.
This was a tragic yet amazing story of a family who learns eventually that a neurological disease is destroying their family. This disease relatively unheard of, is transmitted genetically and though it is found out many years after the fact, this disease had taken the life of many members of the Baxter family. Finally, one member of the family realizes that the occurrence in family members follows a pattern, does extensive work on ferreting out family members who have succumbed and have shown symptoms, and eventually doctors are able to understand this neurological illness. The symptoms, mimicking Parkinson and Alzheimer's, leave the person so debilitated that death is actually a relief. The person suffering from this eventually loses all his/her faculties, losses the ability to walk, talk, and eat.
Imagine finding this out, knowing there is no cure, and eventually learning that you carry this gene and this is your fate? Through science and technology members of this family and of course other families can now find out if the wish, that they carry this disease. Through the use of IVF they can, after the fertilized egg has been tested, have disease free children since only healthy fertilized eggs are implanted in the mother's womb. Imagine the decision one makes to have children knowing that you might develop the symptoms of this illness in your late thirties and eventually become a burden to your family and children? This is the decision one young couple has to make in this book.
This was truly heart breaking story that makes one ever so grateful for the blessed gift of health. The death that people who carry this prion disease is an awful fate and hopefully advances in brain research will be successful in helping those affected with an eventual therapy and cure.
The author did a wonderful job presenting the family, the doctors, and the scientists who are dealing with this most awful disease. This book is well worth the read if you are at all interested in the dedication and love a family shares as they face illness and death.
October 14, 2018
This is a nonfiction health & science book. I liked the research. The author showed that in detail and also how a family discovered what disease had plagued it's family tree for so long. The disease was debilitating and robbed the afflicted and their family of normal life joys.
I was hooked from the beginning. However, at times it felt like the story was being fictionalized with dialogue and past events. I would have liked more time to be spent on research and the moral dilemmas the family was faced with. The author glossed over the dilemmas, and I wanted it to be explored a little more.
I was hooked from the beginning. However, at times it felt like the story was being fictionalized with dialogue and past events. I would have liked more time to be spent on research and the moral dilemmas the family was faced with. The author glossed over the dilemmas, and I wanted it to be explored a little more.
February 14, 2017
ICY (like me) MI: The title “Mercies in Disguise” refers to the song “Blessings” by popular Christian singer-song writer Laura Story. The song won a Grammy in 2012 and topped the Billboard Christian Songs chart, according to Wikipedia. Listen to the song here.
Invoking this song by the South Carolina-based Story in the epigraph is an appropriate warning about the story to come in this excellent book. Much of it is about the complex relationships within the network of doctors and healers, as well as spouses and children, who make up the intensely Christian Baxley family of Hartsville, South Carolina. If the family had been a gaggle of mixed-heritage secular humanists from a major US metropolitan area, the book about certain modern medical techniques might have ended up about novella-length, as a lot of narrative conflict results from actions about and opinions which flow from religion-inspired moral belief.
I have patience for that sort of thing, but I predict that a lot of future one-star reviews of this book (as well as book-club grousing) will mention the maddening tendency of members of the Baxley family to quarrel and/or give the silent treatment to other family members at moments (usually medical crises) where a little Christian charity and comfort might be appropriate, because apparently making a great public show of disapproval for the decisions of others is much more important than supporting your family members in their time of need.
I found a lot of behavior in this book mystifying (but then again I have a similar feeling about most human behavior). In this book, for example, the Baxley family's life is made very difficult by the expense incurred for the maintenance of members suffering from an incurable wasting disease. The daughter Amanda and her fiancé must (very reasonably) move into a cheaper apartment and take extra jobs so they can save money to afford an important (but non-essential) medical procedure in connection with the family's sad fate. The two then decide (again, reasonably) that they wish to get married. Once this decision is made (here comes the mystifying and unreasonable part), the only two choices seem to be (a) an expensive “destination wedding” in the Caribbean, and (2) a (surprisingly) even more expensive all-stops-out wedding with hundreds of guests in the bride's hometown. Both of these, obviously, put a further strain on the family's budget. I understand that options (a) and (2) were probably happy dreams of the bride and/or members of her family for many years previous, but given the circumstances wouldn't a small church service and dinner at a nice restaurant been a more sane decision? I mean, you can always resolve to take a luxury vacation later.
Similarly, members of the family seem determined, even to the point of mania, to hide the evidence of their family's tragic affliction and present a facade of normalcy to the community, even perhaps to the point of paying for a wedding they can't afford, and certainly to the point where they withdraw from society so they do not have to endure the horrors of … the sympathy of others. While I certainly understand the urge not to parade the intimate details of our lives, Kardashian-like, before the community, I have to say that, if there's a good time to stop giving a damn what neighbors and strangers think, what they say, and how they will react, the time when you are staring the possibility of a slow wasting death in the face is certainly that time.
I guess I'm giving the impression that this book is a soap-opera-like Southern family drama – it isn't. It's largely about the new ethical dilemmas pushed on us by advances in medicine. It's a brainy read with both long side-trips to other continents as well as explanations of complicated medical research. But at the end it was really a traditionally ripping good read, where I put aside other things that I should have done first so I could see how it all would turn out.
One suggestion I have for the publishers: a family tree with names, birth and death dates, and important genetic information, that the reader could refer back to from time to time, might help the average reader.
I received an free unfinished galley of the ebook for review. Thank you to Netgalley and St. Martin's Press for their generosity.
Invoking this song by the South Carolina-based Story in the epigraph is an appropriate warning about the story to come in this excellent book. Much of it is about the complex relationships within the network of doctors and healers, as well as spouses and children, who make up the intensely Christian Baxley family of Hartsville, South Carolina. If the family had been a gaggle of mixed-heritage secular humanists from a major US metropolitan area, the book about certain modern medical techniques might have ended up about novella-length, as a lot of narrative conflict results from actions about and opinions which flow from religion-inspired moral belief.
I have patience for that sort of thing, but I predict that a lot of future one-star reviews of this book (as well as book-club grousing) will mention the maddening tendency of members of the Baxley family to quarrel and/or give the silent treatment to other family members at moments (usually medical crises) where a little Christian charity and comfort might be appropriate, because apparently making a great public show of disapproval for the decisions of others is much more important than supporting your family members in their time of need.
I found a lot of behavior in this book mystifying (but then again I have a similar feeling about most human behavior). In this book, for example, the Baxley family's life is made very difficult by the expense incurred for the maintenance of members suffering from an incurable wasting disease. The daughter Amanda and her fiancé must (very reasonably) move into a cheaper apartment and take extra jobs so they can save money to afford an important (but non-essential) medical procedure in connection with the family's sad fate. The two then decide (again, reasonably) that they wish to get married. Once this decision is made (here comes the mystifying and unreasonable part), the only two choices seem to be (a) an expensive “destination wedding” in the Caribbean, and (2) a (surprisingly) even more expensive all-stops-out wedding with hundreds of guests in the bride's hometown. Both of these, obviously, put a further strain on the family's budget. I understand that options (a) and (2) were probably happy dreams of the bride and/or members of her family for many years previous, but given the circumstances wouldn't a small church service and dinner at a nice restaurant been a more sane decision? I mean, you can always resolve to take a luxury vacation later.
Similarly, members of the family seem determined, even to the point of mania, to hide the evidence of their family's tragic affliction and present a facade of normalcy to the community, even perhaps to the point of paying for a wedding they can't afford, and certainly to the point where they withdraw from society so they do not have to endure the horrors of … the sympathy of others. While I certainly understand the urge not to parade the intimate details of our lives, Kardashian-like, before the community, I have to say that, if there's a good time to stop giving a damn what neighbors and strangers think, what they say, and how they will react, the time when you are staring the possibility of a slow wasting death in the face is certainly that time.
I guess I'm giving the impression that this book is a soap-opera-like Southern family drama – it isn't. It's largely about the new ethical dilemmas pushed on us by advances in medicine. It's a brainy read with both long side-trips to other continents as well as explanations of complicated medical research. But at the end it was really a traditionally ripping good read, where I put aside other things that I should have done first so I could see how it all would turn out.
One suggestion I have for the publishers: a family tree with names, birth and death dates, and important genetic information, that the reader could refer back to from time to time, might help the average reader.
I received an free unfinished galley of the ebook for review. Thank you to Netgalley and St. Martin's Press for their generosity.
December 6, 2017
Onvan : Mercies in Disguise: A Story of Hope, a Family's Genetic Destiny, and the Science That Rescued Them - Nevisande : Gina Kolata - ISBN : 1250064341 - ISBN13 : 9781250064349 - Dar 272 Safhe - Saal e Chap : 2017
February 6, 2022
If you like crying, this book is for you. Fatal genetic diseases are painful and tragic. Kolata nicely balanced the history of the scientific discovery of the prion disease (GSS) and depicting a specific family's way of making decisions about it and dealing with it.
March 18, 2022
required read for my genetics class LOL but it was actually really good! the first part about the discovery of the disease itself was boring but i really enjoyed how it focused on one specific family and their challenges with GSS, especially because i can relate similarly with the ethics / issues of genetic testing
June 5, 2017
I always say that murder mysteries are my favorite genre because they help me relax and take me away from real life. But, I actually think that a good medical story is much more appealing. Gina Kolata is not a doctor but studied molecular biology and is a science writer for the Times. Her ability to explain the biology behind the story as well as to get in the heads of the people involved is what intrigued me.
I think the most interesting and compelling part of the book was reading about the protagonist having the genetic testing and then about the the decisions that she made based on those results. Her emotions and the reactions of her family will stay with me for a long time.
I think the most interesting and compelling part of the book was reading about the protagonist having the genetic testing and then about the the decisions that she made based on those results. Her emotions and the reactions of her family will stay with me for a long time.
March 31, 2017
3.7 stars. This non-fiction about the way one family discovers that they have an incurable fatal genetic disease and the choices that each one makes is a very interesting and quick read. The human stories are what make this book, much more so than than science of the disease, which the author describes but not with much detail on the state of current research.
January 17, 2025
This was a hard read for me. A lot of it hit home. While the genetic mutation I have, doesn't give me a degenerative neurological condition, it does affect my heart and most likely life span. I understood the debate about when/if to get tested. I understand the guilt of possibly passing the mutation to my children. Knowing that there is research and options for the future does give hope. It's just a hard subject. 3.5-4 stars for this one.
June 18, 2022
Fascinating story about the discovery/identification of GSS, a rare and debilitating genetic disorder, and its impact on the lives of those who might be affected by it.
November 22, 2016
I received an ARC from bookbrowse.com in exchange for an honest review. A variation of this review appears on their site. Decisions, decisions.... Medical science has advanced so rapidly over the past 40 years that the burden of making decisions about one's health has shifted from the medical profession back to the patient. Medicine is no longer as paternalistic and this book clearly illustrates why that shift has come about. Several members of the Baxley family suffer from a neurological disorder that seems to be a combination of Parkinson's and Alzheimer's always ending in death. Two sons of one patient, who are both physicians try to discover exactly what the disease is and how it is passed from one generation to the next. Simultaneously, several scientists in parallel and together are discovering a whole new class of diseases as well as their inheritance patterns. Both groups come together through Amanda Baxley who has lost her grandfather to the disease and is watching her father and uncle succumb to it. She is given difficult decisions to make, the first generation in her family and the responsibility weighs on her. The story is compelling and well written. On a personal note, I have cared for patients who have had to make similar decisions and it just increased my empathy and respect for them. Thank you to bookbrowse.com for the opportunity to read and review this book.
June 21, 2017
Mercies in Disguise tells the story of Gerstmann Straussler Scheinker (GSS) syndrome. Written by a science writer, Gina Kolata, she describes the discovery of this rare neurological disease that is passed down through families.
The disease is extremely debilitating physically and shares some of the symptoms of Parkinson's and the memory loss and dementia of Alzheimer's. It usually strikes people between the ages of 20 and 60.
At this time, there is no cure or treatment for this disease. There are some medications that attempt to slow down its progression. This disease is a prion disease-a flaw carried on one of the proteins in the genome.
In her book Kolata focuses on the Baxley family. When their father, who is a doctor dies, from an unknown disease with neurological symptoms and an older relative recalls that the dead man's father presented with the same symptoms, one of the doctor's sons is curious because he is a doctor himself. The family beings tracing its medical history.
When two of the dead doctor's surviving brothers begin showing the same symptoms and medical testing is done, the Baxley family learns that some members of the family will have inherited this terrible disease.
The latter portion of the book deals with the dead doctor's daughter Amanda. Planning to marry and have children, she decides to be tested to see if she carries the flawed prion.
The disease is extremely debilitating physically and shares some of the symptoms of Parkinson's and the memory loss and dementia of Alzheimer's. It usually strikes people between the ages of 20 and 60.
At this time, there is no cure or treatment for this disease. There are some medications that attempt to slow down its progression. This disease is a prion disease-a flaw carried on one of the proteins in the genome.
In her book Kolata focuses on the Baxley family. When their father, who is a doctor dies, from an unknown disease with neurological symptoms and an older relative recalls that the dead man's father presented with the same symptoms, one of the doctor's sons is curious because he is a doctor himself. The family beings tracing its medical history.
When two of the dead doctor's surviving brothers begin showing the same symptoms and medical testing is done, the Baxley family learns that some members of the family will have inherited this terrible disease.
The latter portion of the book deals with the dead doctor's daughter Amanda. Planning to marry and have children, she decides to be tested to see if she carries the flawed prion.
May 15, 2017
Kolata, Gina. Mercies in Disguise: A Story of Hope, A Family's Genetic Destiny, and the Science that Rescued Them. 7 CDs. unabridged. 8.5hrs. Recorded Books. ISBN 9781501945366.
Narrative nonfiction doesn't get much better than this. Gina Kolata, a New York Times science reporter, brilliantly 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 disease while concurrently telling the story of a loving family in a small town that appears to be harangued by bad luck. When the patriarch of the family dies under mysterious circumstances, his sons take matters into their own hands, trying to track down the mysterious disease and piece together their family tree to see what the future may hold in store for them. Compellingly narrated by Andrea Gallo who doesn't shy away from hard medical pronunciations and foreign names. Her soft, yet endearing voice provides a wonderful reassuring and authoritative voice. A wonderful read, even for those not interested in medical or scientific nonfiction. - Erin Cataldi, Johnson Co. Public Library, Franklin, IN
Narrative nonfiction doesn't get much better than this. Gina Kolata, a New York Times science reporter, brilliantly 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 disease while concurrently telling the story of a loving family in a small town that appears to be harangued by bad luck. When the patriarch of the family dies under mysterious circumstances, his sons take matters into their own hands, trying to track down the mysterious disease and piece together their family tree to see what the future may hold in store for them. Compellingly narrated by Andrea Gallo who doesn't shy away from hard medical pronunciations and foreign names. Her soft, yet endearing voice provides a wonderful reassuring and authoritative voice. A wonderful read, even for those not interested in medical or scientific nonfiction. - Erin Cataldi, Johnson Co. Public Library, Franklin, IN
February 25, 2018
This follows a family with a rare neurologic disease that doctors struggle to diagnose...it shares some characteristics of Parkinson's and Alzheimers, but doesn't seem to be either. Eventually, the family discovers that mutated prions are causing the disease, and the reader sees how each family member chooses to react, each wrestling with decisions about genetic testing, childbirth, and so on. I found the science behind prions, and the difficulties faced by those who studied and tried to understand these rare neurologic diseases really fascinating. The story drew me right in, and I had a great deal of compassion for the Baxley family. I thought this held a great combination of science and (real life) storytelling.
October 30, 2017
I have been reading this book aloud to my husband in installments, whenever on a couple of short road trips. It has been, by turns, frightening, heartbreaking, inspiring, informative. I did not expect to be as absorbed as I was by this true tale of the Baxley clan of South Carolina, a family blessed by extraordinary intelligence and ability, yet stalked by a hereditary disease that claimed 50% of its members. Read it with a box of tissues nearby.
August 1, 2020
A very interesting look at both a little-known genetic disease and the ethical dilemmas it raises. I enjoyed the first half of the book most, as the author traced the efforts to identify the cause of the mysterious kuru disease and, in parallel, the suffering of the Baxley family, which was carrying it in their genes. In the second half, the focus seemed to change from the disease itself to the issues involved in the (then) fairly new procedure of preimplantation genetic diagnosis, which Amanda Baxley, afflicted with the mutated prion, decided to undergo--interesting in its way, but there was perhaps too much tedious discussion of her wedding and other peripheral events. The scientific facts were clearly explained, but otherwise the writing was somewhat cliched (people kept "locking eyes," etc.), and much of it read like fiction, with descriptions of thoughts and conversations that are unlikely to have been recorded at the time they occurred. Still, it was a fairly riveting read; the author did well with creating suspense.
December 7, 2017
Well-written, compelling story. I stopped about halfway through as it's just not striking me as something I want to finish. The story is more about the lives and emotions of the family involved than about the science of genetics, so if you're expecting lots of medical information this isn't for you.
November 6, 2024
I found this book very interesting. I worked with Amanda recently and at first, had no idea that this is what she and her family are dealing with. She is a wonderful person, making her children and husband a priority. I wish her and her family strength in their journey yet to come.
October 17, 2017
Didn't D.T. Max already write this book? Ten or so years ago? Well, I always read Gina Kolata's books, so I shall read this, too.
May 23, 2024
An amazing, heart wrenching, yet hopeful read.
January 23, 2018
I thoroughly enjoyed this book. I also know this book is not for everyone. This story basically follows one family as they are diagnosed with a rare and life ending neurological disease that no one really knows about (a mix of Alzheimers and Parkinsons). It goes through the history of this class of diseases and then tells a tiny bit of what is in store (not much as this is definitely more about the back story). The end of the book talks about IVF with selection for embryos not carrying the gene. This would be a good discussion book. Would you want to know if you carried a gene that would make the last couple of years of your live miserable? What about having children when you may give them the disease?
August 9, 2016
Would you want to know if you carried an inherited gene that causes an incurable disease? A *prion disease ~ a class of diseases that are deadly neurodegenerative disorders with symptoms that start innocently, but progresses to an excruciating death for the afflicted, and for their family. This is the story of the Baxley family who never knew the name of what had plagued their family for generations until they started researching it on their own. Even the medical community couldn't give the family a name for their disease, only that it kills. This book is reminiscent of the book "Lorenzo's Oil" in that the family would not take no for an answer, even when it is found that there is no cure. However, they found hope in another way. You'll have to read the book to find out what that hope is.
*Prion diseases include Creutzfeldt-Jakob disease and kuru in humans, scrapie in sheep, mad cow disease in cows, and chronic wasting disease in deer.
Note: I received a free review copy of this book and was not compensated for it.
*Prion diseases include Creutzfeldt-Jakob disease and kuru in humans, scrapie in sheep, mad cow disease in cows, and chronic wasting disease in deer.
Note: I received a free review copy of this book and was not compensated for it.
November 4, 2016
This was a fascinating explanation of a little known disease that is described as being somewhere between Parkinson's and Alzheimer's. The decisions to be genetically tested seemed agonizing and the consequences of living with the outcome of the testing even more so. Adding the Baxley's stories to the book made it so much more personal and human. Each and every person who stands to inherit the mutant gene for the debilitating and devastating GSS must make their own decisions and then live their lives accordingly as was reflected in the Baxley family. It was thrilling to learn that there may be some options for preventing this disease in future generations but I can only hope and pray that developments arise giving new hope to those already carrying and/or manifesting the GSS gene.
I found this book to be one of most easily understood medical/scientific portrayals I've ever read. It sounds cliche but it read like a mystery/thriller.
Thank you to Book Browse for providing me with the ARC. This book was definitely right in my wheelhouse of interests.
I found this book to be one of most easily understood medical/scientific portrayals I've ever read. It sounds cliche but it read like a mystery/thriller.
Thank you to Book Browse for providing me with the ARC. This book was definitely right in my wheelhouse of interests.
Displaying 1 - 30 of 225 reviews