Mercies in Disguise: A Story of Hope, a Family's Genetic Destiny, and the Science That Rescued Them
272
Mercies in Disguise: A Story of Hope, a Family's Genetic Destiny, and the Science That Rescued Them
272eBook
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Overview
"[Kolata] is a gifted storyteller. Her account of the Baxleys... is both engrossing and distressing... Kolata's book raises crucial questions about knowledge that can be both vital and fatal, both pallative and dangerous." —Andrew Solomon, The New York Review of Books
New York Times science reporter Gina Kolata follows a family through genetic illness and one courageous daughter who decides her fate shall no longer be decided by a genetic flaw.
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, 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.
Product Details
| ISBN-13: | 9781250123992 |
|---|---|
| Publisher: | St. Martin's Publishing Group |
| Publication date: | 03/21/2017 |
| Sold by: | Macmillan |
| Format: | eBook |
| Pages: | 272 |
| Sales rank: | 302,189 |
| File size: | 1 MB |
About the Author
Gina Kolata is a science reporter for The New York Times and the author of Flu: The Story of the Great Influenza Pandemic of 1918 and the Search for the Virus That Caused It, Clone: The Road to Dolly, and Sex in America. She lives in Princeton, New Jersey.
Read an Excerpt
CHAPTER 1
The Prophecy
Tim Baxley never even got the name of the woman who told him the story that set the horror in motion. It was a June evening in 1998 and Tim was at the end of the receiving line at a viewing the night before a funeral — his father's funeral.
Tim's father, Bill Baxley, had been a chemical engineer at a local packaging plant. Tall and imposing, with a stern and serious face, he had been a deacon in his church and head of the Sunday school program. He was known in his community as a problem solver, someone whom people would go to for advice or solace.
The Baxley family had lived in or near Hartsville, South Carolina, for generations. A town that, with its suburbs, comprises twenty-one thousand people and sixteen churches — eight of which are Baptist — but not a single mosque or synagogue, it is a deeply Christian population in the heart of the Bible Belt.
The lure of the town, its magnetic appeal for the Baxley family and many others, is its sense of community. Residents frequent family-owned stores because they like to buy from people they know. They support the town's free medical clinic, funded by churches, foundations, businesses, and individuals and staffed by volunteer doctors and nurses who treat those who cannot afford to pay. They cherish family life — restaurants close on Sunday nights so employees can eat dinner at home with their families. High school sports make front-page news in the town's paper. The church basketball teams take time-outs during the games for devotions. It is a place of conservative tradition, of exacting conformity, stifling to some but a comfort to those who have come to feel that Hartsville is their true home.
Bill Baxley proudly fit in. So staunch a patriarch and so perfect a picture of traditional southern living was he that, in 1985, he was awarded a plaque from Governor Richard Riley naming his clan South Carolina Family of the Year "in recognition of the exemplary qualities of family life exhibited by the William H. Baxley, Jr. family."
It was a confirmation of Bill's belief, shared by his family, that the Baxley clan was special, maybe even indestructible — certainly a testament to the grace conferred by faith, discipline, and hard work. Even Bill's viewing at the funeral home seemed to confirm this belief.
Tim knew the respect his father commanded, yet he was still astonished by the size of the crowd that night. The viewing was supposed to start at 6:00 p.m. and end at 8:00, but people lined up outside the door at 5:45. The last did not leave until 10:00. Hundreds arrived — more than the funeral director had ever seen — a steady stream of people coming despite the thick heat of that June evening. The line snaked around the wall on the right side of the front room of the funeral home, then extended out the door and through the parking lot and down the sidewalk. The family formed its own small line in that front room, greeting people as they came by. Bill Baxley lay in an open casket in a small room just behind them. Many could not bear to see him — they left after paying their respects to the family. Others, as is the Baptist tradition, filed past the casket, with no kneeling, no signs of the cross, just a solemn acknowledgment of the revered man.
The Baxley men were readily identified by strong, dark brows shading large, dark eyes, a head of thick chestnut hair, a narrow oval face and a straight, angular nose. They all had a sensuously full upper lip with a pronounced Cupid's bow. From generation to generation, the Baxley boys looked so similar that it was difficult at times to tell them apart.
But this man in the casket — this man looked nothing like Bill Baxley. He was a stranger. A rippled sateen pillow tipped a shriveled face up to the line of passing mourners. Bill's gray hair had been pulled back, the skin drawn tight against his forehead. Heavy makeup was smeared over the scar where his brain had been removed in a vain attempt to figure out the illness that had robbed him of his life. His favorite charcoal gray suit hung loose on his shrunken body; his red tie with a South Carolina emblem did nothing to draw the eye from the desolation of his body.
Tim was at the front of the receiving line, standing ahead of his older brothers, Billy, Mike, and Buddy. He glanced back at his mother — tiny, frail-looking Merle — who stood near the entrance to the room where her husband's casket lay. It would be a long night for her; she'd borne a heavy burden. And yet, after years of watching her commanding husband degrade into an invalid, aging twice as quickly as he should have, after years of helplessly watching as aides transferred his weight from wheelchair to toilet and back again, after spoon-feeding him his meals, after waiting while he communicated by grunting or nodding or slowly pointing to letters on an alphabet board, she felt in spite of herself a kernel of relief that his suffering was now over.
Between greeting visitors, Tim observed his mother shake well-wishers' hands and acknowledge their sympathy. She was bearing up better than he had dared to hope. He developed a kind of rhythm. He'd accept a "sorry for your loss" with a grateful smile and then turn his head to see his mother doing the same thing.
Soon, the motions became automatic. So he paid little notice to the plump gray-haired woman from the nearby town of Darlington when she made her way up to him, one of so many in that sea of mourners.
"I am so sorry for your loss."
Now her eyes turned down, her smile faded slightly. She looked up at him again, holding his glance meaningfully.
"I remember his daddy walking through the plant. He had to hang on to the machines."
This seemingly innocuous comment was delivered so quietly that she had already moved on by the time Tim recognized its meaning.
* * *
Throughout his father's illness — abrupt in its onset and unswerving in its course — Tim Baxley had sought answers from one doctor after another — general practitioners, pathologists, neurologists. And he'd never received one that satisfied him. His father, doctors said, suffered from a rare, anomalous disease, kind of a blend of Alzheimer's and Parkinson's, but his was a case like none any of them had ever seen or heard of before. And yet, suddenly, in the briefest of exchanges, this woman had conveyed that she had seen it before in his grandfather — a crucial detail Tim had never known. That loss of balance, it was one of the first signs, wasn't it?
Standing there in the funeral home, Tim was barraged by memories and images, one after the other. His dad shuffling as he walked; soon the shuffling turned into stumbling and then a sort of weaving, like he was drunk. The way his father careened through the living room, clutching at the wall for support, hanging on to chairs and sofas. And that was in the beginning, when things just seemed off, but nothing so strange that it could suggest a fatal problem.
Bill's memory seemed to slip around this time as well. Tim had first noticed it ten years earlier, in 1988, at Sunday dinner at his parents' house. He had been looking for something in the basement when he came upon an object from his childhood. Bill had fought in the Pacific — but one of his friends, who had fought in Europe, had given him a compass that he had snagged from a German general. It had a steel case that opened to reveal the compass; on the other side, there was a little mirror with the initials of its owner etched on it.
Tim would often bring this to his father and ask him to talk about the war, to tell the story of his friend and how he came to get his hands on that compass. That night, Tim cradled the little compass in his hand, carrying it upstairs to the kitchen. He proudly showed it to his father. Like a small child, he wanted to hear his dad tell the story one more time. It had been years since he had asked him about it.
But when Bill looked down at what his son was holding, he seemed baffled and muttered that he had no idea what Tim was talking about. Tim reminded Bill that they'd looked at that compass many times over the years. No, Bill shot back, he didn't remember any of that. "I've been through several cases of burnout at work since that time," he offered as feeble defense.
Tim sadly trudged back downstairs clutching the compass and replaced it on the shelf — it may still be there to this day. He was devastated and tried to find a logical reason for his father's puzzling memory loss. Maybe it was depression, he thought. But what did his father mean when he offered that odd excuse? What was he telling Tim when he said "since that time"?
* * *
There would soon be more worrying signs of trouble.
The time Bill had been in a meeting at work and suddenly realized he had no idea what was going on. It was as if he had blacked out, yet he was fully conscious. This was the first signal that something was wrong, which he predictably kept to himself for a while. He didn't want to worry anyone. He didn't want to admit weakness. Eventually he told Merle, who kept it quiet for many years. Maybe it was nothing, she reasoned. It could happen to anyone with too much on his mind.
* * *
The day when Bill and Merle were out walking, a doctor who lived next door noticed that Bill was lagging behind, dragging his feet and walking unsteadily; he finally asked Merle if Bill was okay. He suggested that he do a physical exam. But when Merle brought Bill in, the doctor was confounded. Maybe a neurologist could figure it out?
* * *
The time Tim's older brother, Mike, drove Bill and Merle to a neighboring town. They were picking up windows for a sunroom Bill was building onto the house. They'd emptied out the station wagon, leaving the trunk clear to transport the unwieldy sheets of glass. Mike carried them from the store to the car and then waited for Bill, who had always taken charge in these kinds of situations. But instead Bill just frowned at the stack of glass, unable to figure out what to do. He had the frustrated look of someone asked to solve a puzzle beyond his abilities. Mike cringed, trying to seem unconcerned as he loaded the windows into the trunk. "Something is wrong with Dad." Mike was the first to say it out loud, to Merle, finally speaking the words everyone had been thinking.
* * *
Bill grappling in vain with a little package of crackers at a Sunday family dinner, fighting his mounting frustration. That crinkling sound seemed never to end as Bill tried fruitlessly to tear open the plastic with his trembling hands, his weak fingers, never saying a word to his family as he furtively worked away at the wrapper. The rest of the family — the four sons, the three grandchildren — silent, staring at their plates, not wanting to embarrass him, knowing he would not accept help. Finally Mike reached over and, without asking, quietly opened the package.
The family watched with sorrow as Bill lost coordination in his hands. His speech blurred. As one scary symptom followed another, it was impossible to avoid the unthinkable. Something terrible was happening. Bill became desperate for a diagnosis, started begging for help. Could no one figure out what was causing this? His son Buddy was a doctor. Maybe he could assist. Tim remembered those Sunday dinners when Buddy arrived with his family. Bill would usher him into the back bedroom, saying he wanted Buddy's advice on what to do, how to get a diagnosis at least. But Buddy was at a loss.
* * *
And so, that same year — 1988 — when Bill's symptoms had become so distressing, Tim and his brothers began ferrying their father from doctor to doctor. This went on for years as his disease worsened and they sought help in an ever-widening area: Hartsville, then nearby towns, then the Medical University of South Carolina a couple of hours away. But the answer was always the same — some glorified version of "I don't know" — a strange illness with a relentless progression that made Bill Baxley stagger, grab chairs, tables, anything he could reach in the early stages when he could still walk. Eventually, it took away his ability to talk and to eat without choking. It humbled the proud man who never countenanced weakness. It was a disease like Alzheimer's — but not. He may not have remembered the compass, but he still remembered who his sons were, what his wife meant to him, how it felt when he could walk and talk and eat. It was a disease unique to Bill Baxley, the doctors said.
There must be an answer out there somewhere, Tim thought. Though the local doctors had been empathetic, it was obvious they were not going to be able to help. But what about a place where doctors specialize in rare diseases, a major medical center where researchers study mysterious illnesses? The closest place like that was Duke University, 150 miles away. The brothers conferred: they were aware of just how grave a situation their father was in. The symptoms had remained subtle for quite some time, but now — nearly eleven years after that episode when Bill did not recognize the compass from World War II — he was no longer able to stand without assistance. Buddy was a family doctor; Tim was starting a residency in neurology. But so far, their combined medical training was not helping.
When Tim and Buddy explained to their brother Mike that they were hitting a wall, he offered to take their father to Duke. Mike knew it was not going to be easy to get his father there, given his condition, but he felt he had to try.
When Mike picked Bill up at his house on Tuesday afternoon, January 21, 1997, they set off in a cold, driving rain. Bill slumped down in his seat, his face ashen, and dozed off. As it turned out, Bill had sleep apnea so every few minutes or so, he would stop breathing in the midst of rattling snores. The seconds-long silence, following a loud snore, was terrifying. Mike kept swerving as he sped down the slick highway, accidentally pulling the steering wheel every time he'd reach over to give his father a shove, checking if he was still alive.
Every little mundane thing became an ordeal. Bill had to use a bathroom. Getting him in and out of the car and to a restroom along the highway was agonizing. When they finally arrived at their hotel, Mike exhausted himself just getting his father from the car to the lobby to the room. Still, as he later told his brother Tim, he harbored a hope that it would all be worthwhile.
The next morning, Mike dressed his father in an oxblood flannel shirt, khaki pants, and a fedora, put bright white socks and black slip-on shoes on his feet. He and Bill arrived on time for their appointment at the neurological disorders clinic. When they were called to go in for Bill's exam, Mike helped his father rise from a chair and gently held him up as he walked into the exam room with tiny steps. He sat his father down in a chair, facing the neurologist. Bill sat rigid, his hands on his knees, his face stiff and expressionless except for his bulging, searching eyes.
The neurologist began to question Bill, who replied with effort, sometimes with just a single word in an almost inaudible voice as he laboriously struggled to speak. Bill tried to explain that in the last week he had become pretty much unable to walk unassisted, falling after a few unsteady steps. Then the neurologist asked Bill to try to cross his arms over his chest and stand.
He cannot possibly do that, Mike thought. He will tip over. He watched as his father attempted to comply, arms crossed over his chest, bending over and struggling to rise. Bill moved only a few inches from his seat before he collapsed back onto the chair, listing to the side, his masklike face giving no sign of the panic he must have felt.
The neurologist sat impassive in his chair, doing nothing to help. Mike stepped in, hoisting his father up just before he fell to the floor.
When the neurologist asked Bill to walk back and forth in front of the wall, Mike jumped up, helping his father stand, supporting him as he tried to walk with mincing steps. The doctor continued to sit calmly in his chair — no look of sympathy to Mike, or effort to comfort Bill.
"He seemed to be so distant from the human tragedy in front of him," Mike later explained to Tim. "He didn't have that personal empathy or sympathy. He would have let him fall."
(Continues…)
Excerpted from "Mercies in Disguise"
by .
Copyright © 2017 Gina Kolata.
Excerpted by permission of St. Martin's Press.
All rights reserved. No part of this excerpt may be reproduced or reprinted without permission in writing from the publisher.
Excerpts are provided by Dial-A-Book Inc. solely for the personal use of visitors to this web site.
Table of Contents
Acknowledgments
Introduction
Part I: A Disease Revealed
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 Revelation
15 Of Two Minds
16 A Unique Way
17 “We Have No Other Options”
18 Prescription Fertility
19 New Lives
20 Carrying Forth
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