Such an interesting journey that we take with the Baxley family as we delve into the mystery of inherited diseases. Important yet difficult questions are confronted, including the most challenging: Would you want to know if you were going to get it?

Excellent book. We not only reviewed and discussed it on BookBrowse. I have also recommended it to many personally. Thank you

This book relates the parallel paths as researchers and a family confront a rare, genetic, disabling illness. The scientists want to understand the disease to gain notoriety and fame, as well as help those afflicted. At the same time, the family, of which several members are physicians,only want to find out what is wrong and what they can do to stop this from progressing through their family. As a physician I found both threads fascinating; some readers may find the scientific discussion tedious although the jargon was minimal. Ultimately, it was a story of perseverance, commitment, and courage that anyone should appreciate.

For any family facing a life with genetic health issues, I would highly suggest reading this book. It is filled with the powerful story of a deeply emotional journey between what is considered the "right" way and what your heart can handle. The details that fill the lives of the Baxley family are shared in a way that shakes what you think you know to the very core. The details of the disease, the journey to understanding, and the life the Baxley's must lead once they find out the truth are laid out in depth. The story centers around Amanda Baxley and the reader is drawn to her immediately. I finished this book in one sitting. It reminds us that we can never be sure of anything until the moment we come face to face with understanding.

A poignant story about unraveling the mystery of an untreatable illness. The Baxley family is a pillar of their community but a strange illness is taking family members at early ages. They consult with leading doctors but no diagnosis or treatment is in sight. As the youngest generation comes of age they must confront the prospect of the illness affecting them and make choices that could alter their lives forever.

In this book, Gina Kolata traces both the Baxley family and the science behind their family illness in expert fashion. This book reads more like a story than a case report and I think this is largely due to the structure she uses. The chapters alternate back and forth between the scientists working to decipher the cause of the illness and the family as they deal with its consequences. It moves at a really good pace and the alternating storyline keeps the reader engaged and wondering what happens next. This story is both poignant and tragic, and full of hope. I really enjoy a book where the author can take a very scientific subject and make it approachable for the average reader. I highly recommend.

If there was a strong possibility that you carried a defective gene that causes a disease that leads to an early painful and prolonged decline and ultimately an untimely death, would you want to know? If you have the gene, it's not a matter of if the disease strikes you, but when. So, which path do you choose? It's not an easy question, even hypothetically, but that is the very real situation in which Amanda Baxley finds herself at the beginning of Gina Kolata's moving tale of a family's battle to stop a killer gene in its tracks.

Mercies in Disguise takes readers on a journey to New Guinea where tribes were struck down by a mysterious disease known only as kuru. From there, readers follow the path scientists took to discover what is known today as the "prion," the culprit behind Mad Cow Disease, Chronic Wasting Disease, and Scrapie. Kolata also looks at the speculative future of prion disorders, thinking about how that might play into Alzheimer's research going forward.

While the science is an important aspect to this book, the human element is by far the most interesting. The Baxley family has been struck down, one by one, over several generations by a mysterious and debilitating disease. Sometimes diagnosed as Alzheimer's, sometimes diagnosed as Parkinson's, and sometimes diagnosed as Alzheimer's complicated by Parkinson's the Baxley's are in need of answers before the disease claims another victim. In a family of doctors, they begin to experiment on themselves. Finally, a genetic link is found leaving the next generation, that of Amanda and her siblings, to decide what to do with their knowledge while simultaneously watching their father die after losing their uncles to the same terrifying disease.

Gina Kolata balances the science and the human extremely well. There is enough science and technical jargon for someone in the medical field but she also breaks down the medical facts in a way that is approachable for the casual reader. Kolata primarily follows Amanda's struggle which allows the reader to really develop a personal connection with the family. From the very first line, this book drew me in and refused to let me go until the final word. Certainly a must read!

The question threading its way through the narrative nonfiction book, Mercies in Disguise, by Gina Kolata is one I’ve pondered before. If a hereditary degenerative disease showed up often in your family and you could be tested to see if you carried the gene, would you have the test?

This story of the Baxter family exemplifies the odds of a fifty/fifty chance of having a gene for a disease that steals both body and mind slowly and ends in death. The frame is Amanda’s choice. It begins as she waits to hear the results of her test before going on to the backstory, and ends with the phone call and her response to it.

Irony lies in the fact that the Baxter family is filled with doctors. By chance, they begin to notice a similarity in family deaths that are attributed to Parkinson’s and other degenerative diseases. Once they realize the commonality of the rare disease, they are able to establish that it has been passed from one generation to the next, often receiving various labels since doctors themselves are not aware of the disease. In their bafflement, they label the sickness with the closest equivalent in their knowledge base.

Rogue scientists fill in a parallel story along with that of the family. They have the correct label, discover the gene marker, and find a way to test for it. As expected, family members differ in whether to test or not to test, sometimes finding a conflict between the possibilities of science and reliance on their faith.

This true story reads like a mystery novel with page-turning urgency. I found myself sympathizing with those who wanted to know and take every opportunity afforded by science and with those who wanted to live life with a faith that assumed they were in the fifty percent clear of the gene. Following Amanda feels like following the protagonist of an intriguing novel with the added urgency of knowing her story is true.

As for the question of whether I would want to know, I’m still pondering.

This book is about a well established family The Baxley's who fought an extremely rare genetic disease GSS that mimicked Alzheimer's and Parkinson's disease. It was about this family's dedicated efforts in finding out what was killing their family's members. It was also about one family member in particular who made the extremely hard decision to be tested to see if she too would be afflicted with this disease.
Overall I rated this book four stars out of five. It was an excellent read that covered this family's journey and the history behind this disease in great detail, all while capturing your attention throughout the book. It made me think and put myself in this family's shoes in what would I do if it was me? What decision would I make? Do you put it in God's hands or do you leave it to science? The only criticism that I would make is that there was one chapter in the book where it went into the history of In Vitro Fertilization that I felt was unnecessary to add in the book. I think that it would have been better to leave this chapter out because it brought up a whole new set of questions that took the focus away from the real story that was going on. But again it was still interesting learning about the history of it anyways so I don't put a lot of weight on this criticism. I would definitely recommend this book for all to read.
I would like to thank Netgalley, Gina Kolata, and St. Martin's Press for the opportunity to read this book in exchange for an honest review.

I simply can not imagine being in the shoes of Amanda Baxley. I can't imagine that my grandfather, uncle, and father have died from a degenerative brain disease. I can't imagine that I being faced with the decision of whether to find out if I have the same disease. And I can't imagine the myriad of ways in which life would be different if I found out that the genetic test was positive, or negative. "Mercies in Disguise" follows the story of the Amanda Baxley, and the Baxley family as a whole, as they deal with the emotional and physical blow of this terrible disease. The book also delves into the background, research, and discovery of several degenerative brain diseases.

Non fiction with great storytelling
While I really enjoy reading non fiction, I am definitely more particular when choosing a book from this genre. This is likely because I most enjoy a fictional narrative, and I've found that in many non fiction books I've read, the "characters" were very difficult to connect with and the "story" too dense to follow. My favorite non fiction books, however, are written by authors who walk the line between fact and storytelling, using one to enhance the other. Gina Kolata did an excellent job of this when writing "Mercies in Disguise. I felt invested in and connected with the Baxley family as they tried to determine what illness was afflicting their family and what recourse they had for treatment. My heart broke and leapt for joy at Amanda Baxley and her struggles and success in life with the threat of a genetic disease looming overhead. I was equally impressed by the author's ability to make potentially less relateable characters come to life. In addition to the Baxley family, "Mercies in Disguise" also follows the research and discovery of degenerative brain diseases since its early roots in a New Guinea field hospital in the 1950s. These parallel storylines as generations of Baxleys and scientists separately piece together the symptoms of these diseases and make connections, further added to the storytelling aspects of this book, and my enjoyment of it.

Just enough science, but not too much
I was a biology major for one arduous year. When the math of science out paced my reading comprehension skills however, I was out of there - a decision I sometimes, but rarely, regret. I love science, but I love literature more, and overall I am glad I switched to English Literature my sophomore year- and I have a feeling so are the chemistry tutors at my alma mater! "Mercies in Disguise" had just the right amount of science for me to enjoy the story without feeling like I was reading a textbook. I do think a background in science aided my enjoyment of this book, but I don't think it is necessary. If you've watched a couple of episodes of Grey's Anatomy- you'll be up to the task. The science behind the range of degenerative brain diseases discussed in "Mercies in Disguise" is fascinating and I enjoyed seeing the accumulation of knowledge, and the impact, privilege, and burden that can be for families today. "Mercies in Disguise" showed me the heartbreak families experienced for generations when afflicted by these, then mysterious, diseases- but also the burden families now face, knowing the disease and having all of the information, but being faced with decisions previously thought impossible in genetic testing. I really enjoyed reading Amanda, and her siblings, divergent points of view regarding the tests and how the knowledge of the disease affected their lives.

Things I Struggled With
Nothing! I don't really have any bones to pick with "Mercies in Disguise". Overall I really enjoyed this book and would definitely recommend it to others readers who enjoy nonfiction, are curious about degenerative brain disorders, genetic testing, and medical ethics.

Mr. Baxley is having physical problems. He doesn't find any help with the local doctors. They can't figure out what is wrong with him. There are suggestions but they are only guesses. Eventually the science worl finds something but still don't know how to cure it or understand why it happens the way it does. Amanda has watched her grandfather died from this and her father. What would you do if you knew you might have inherited a disease that had no cure? Would you want to know or not? There is a test that can tell her. Will Amanda get marry or have children if she has inherited the disease? I discovered that rare and/or uncommon diseases do not get the support needed to fund the research so desperately needed.

Even though this is nonfiction, it is easy to read as the author has done an excellent job of writing on this subject. In some ways it is like reading a medical thriller. As I read the book, I became involved with the family and the problems they had with their disease. It is not something that I will forget. Read it, its am important book.

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.

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.

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).

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.

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.

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.

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.

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 recommened.

[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.

I knew nothing about this book when I started reading, just that I love Gina Kolata's writing. Her nonfiction is as compelling as good fiction and this book was every bit a page turner as The Immortal Life of Henrietta Lacks. I have a very weak science background, but no matter, Kolata made everything I needed to know easy to understand. The work of researchers independently looking for answers to GSS and CJD was woven together with a plight of a delightful family, many physicians, who were plagued with the killing gene. The only disappointing thing is that,while scientists are uncovering ways to deal with the fallout so that the afflicted can have children free from the gene, there is as yet no cure. I hope she revisits this subject in the future.

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-life, 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.

Mercies in Disguise by Gina Kolata is excellent narrative non-fiction. Kolata explains the history of Prion diseases without being boring, and she transitions seamlessly into a narrative about the . Baxley family. Her writing made me care.

The Baxter family members had varied lifestyles and opinions about how to handle the disease that plagued their family, but Kolata represented each of them with respect.

I particularly liked the fact that this book was an excellent conversation starter. My sons and I discussed what we would want to do if we were faced with an inevitable, incurable disease and what factors would we consider when making decisions about unborn children with disease.

I was provided an advance copy of this book through Netgalley

Admittedly, I do love a good medical story: The Tennis Partner by Abraham Verghese, Better by Atul Gwande, The Remedy by Thomas Goetz. If you are interested in genetics, medical research, impact on families, then this book is your thing. The Baxley family is one of those "All-American," stereotypical, quintessential Southern families, the type whose father was the small-town doctor, church attendees, and tight ties with one another. However, a mysterious genetic disease has ruined the picture for generations. This book is the search for an answer, not a cure. Author Gina Kolata does a masterful job of looking back at the history of this strange disease, one that causes the sufferer to slowly lose control of his or her body, speech, and brain. Kolata goes far back in time and space to New Guinea where a young doctor sees a people devastated by disease and isolated culturally. Kolata slowly builds the puzzle as doctors fight charges of quackery, advances in testing creates more questions, and false roads are taken. As DNA testing evolves, we see all the pieces start to come together, all while the story of the Baxleys is threaded throughout. It is a profound look at how science can impact a family, what one might do if given a chance to see their future, and the often futile attempts for normalcy in the face of great challenges.

One Family’s Genetic Roulette Wheel

Science reporter Gina Kolata follows the Baxley family through their navigation of a discovered highly heritable mutated gene that belies a brutal and fatal neurodegenerative disease with no cure. This highly charged story starts with Amanda Baxley, one of the family members whose grandfather, father, and uncle all succumbed to the terrible illness known as Gerstmann–Sträussler–Scheinker syndrome (GSS). Amanda is one family member who chooses, in her twenties, to be tested for the mutation so that she may plan accordingly. The opening is a cliff hanger, leaving off at the point where the test results are in and she is about to discover her fate.
What are the implications of having this knowledge ahead of time? Would you choose the same path as Amanda?
Next, we are taken to eastern highlands of New Guinea in the 1950’s where a small group of scientists attempt to track the origin of a strange and similar sickness that resembles a combination of both Alzheimer’s and Huntington’s but with decidedly different characteristics.
The shunned scientific hypothesis regarding distorted proteins that could be inherited through gene mutation finally gave way to the groundbreaking realization that this hypothesis proved to be correct.
Meet the Baxley’s, a high achieving, morally pious Baptist family living in a small town South Carolina. A family full of doctors and medical professionals, they pieced together an unlikely puzzle to discover that their family carried within its walls, this rare gene mutation that could be diagnosed with a blood test.
Part narrative non-fiction, part scientific inquiry, and part human interest story, Kolata traces one family’s destiny through the story of a courageous young woman.

BRB Rating: Read It

I received a copy of "Mercies in Disguise: A Story of Hope, a Family's Genetic Destiny, and the Science That Rescued Them" from NetGalley for an honest review. I wish to thank NetGalley, St. Martin's Press, and Gina Kolata for the opportunity to read this book.

This was a fascinating book about a deadly genetic disease. It was heartbreaking to read about the disease affecting the large, extended family in such a devastating way.

Even though the story was sad, there was hope. There was quite a bit of religion sprinkled throughout the book as the family were devout Baptists, primarily. That really didn't impact the story at all for me and shouldn't be an issue for non-religious readers - I am just putting it out there to be fair.

This is definitely a must read for fans of "The Immortal Life of Henrietta Lacks" by Rebecca Skloot. This is a very different premise of a book, but it is a medically-based book heavy on the ethics. It will be on my mind for a while just mulling it over more and more.

Highly recommend!!

This was a well researched and well written book about a rare genetic neurological disease that strikes around middle age that looks like a cross between Parkinson's and Alzheimer's diseases. The book covers the history of the research into this disease and the difficulty with identifying its cause but spends a great deal of time with a family that has the defective gene that causes the disease.

The background of the science behind the disease was fascinating but I thought the author did a great job with telling the story of the Baxley family. The Baxleys' stumble upon the diagnosis as another member of the family slowly deteriorates from the disease. Now that the diagnosis is confirmed in the father, if you are one of his children, would you get tested? The book covers many questions about pros and cons of finding out (knowing there is no cure) and how to move on with life if you get the news that you have the genetic defect. I really enjoyed this book--well researched and the author told the delicate story of the Baxleys' with care.

Very good, but tragic story of a South Carolina family that carries a gene for a deadly and untreatable disease. Book deals well with the issues of gene therapy and selective IVF