The Book of Kehls
CHAPTER ONE
SHORTLY AFTER THE END OF THE CIVIL WAR, ALL BY herself, wearing her first pair of shoes, my nine-year-old great-grandmother Bridget Moore waved good-bye to her Mam, and her Da, and her nine brothers and sisters (including the never-named little brother who "walked funny," and fell down) and left Galway Bay for New York City, and a job as a Fifth Avenue "ladies' maid." Bridget brought to America a small steamer trunk, a pair of rosary beads, a clay pipe, and as near as we can figure out, Duchenne muscular dystrophy. In Ireland, she'd had little schooling. She'd never learned to add, or subtract, which was a bit of a hindrance when she'd finally saved enough to buy a Greenwich Village grocery store that she promptly lost when she couldn't figure out how much anybody owed her, and before it spoiled, simply gave her food away. Since Bridget had never learned to read, or write, she never knew what became of her family. She never heard from anyone in Galway Bay again.
But maybe Bridget Moore remembered her little brotherwhen her daughter Mamie's two sons, Harold and Raymond, and her daughter Sadie's two sons, William and Theodore, began "walking funny" and falling down. Maybe Bridget prayed for him, if she prayed for them, when she watched the sickness cutting through their legs like pinking shears through onionskin. Maybe Bridget finally mourned for her brother when she mourned for her grandsons, who were all under twelve years old when they died, of pneumonia, days apart, in February of 1922. In the front rooms of adjoining Hell's Kitchen tenements, at 403 West Fifty-sixth Street, an address that is now a parking garage, the four boys were laid out in two caskets. Perversely benevolent at the end, muscular dystrophy had whittled them small enough to lie in each other's arms.
While Bridget Moore was learning to live in New York, on the other side of the Atlantic, Dr. Guillaume Amand Duchenne, a French physician from a family of Marseille seafarers, was busy photographing the human face, or more specifically, the muscles of the human face, for which he received a prize. It was Dr. Duchenne who observed that genuine smiles not only use the muscles of the mouth, but also the muscles of the eyes--hence, the "Duchenne Smile." After Dr. Duchenne's young wife died in childbirth, he grew despondent, and left both medicine and Marseille. He resumed his career in Paris, where he became intrigued by a mysterious "wasting disorder" striking young boys, and opened a clinic. It was Dr. Duchenne who invented a "harpoon," an early form of muscle biopsy, to penetrate the skin of living subjects, and extract muscle tissue for examination. I can't imagine there were many "Duchenne smiles" after a procedure like that.
Dr. Duchenne's 1868 report, still in use today, lists fully the signs and symptoms of the "wasting disorder" affecting the legs, the arms, the hips of young males--all of the muscles except the muscles of the face. Enlarged calf muscles--ah, those enlarged calf muscles, melting into water and fat, where the dreams are first dashed--were the first symptom Dr. Duchenne noted. His patients died in adolescence, when their heart muscles, or their respiratory muscles, in the days before antibiotics, failed. Duchenne's discovery of the "dystrophy" that bears his name was not greeted with universal applause. "I thought humanity to be afflicted with enough evils already," one of Duchenne's colleagues pronounced, "I do not congratulate you, sir, upon the new gift you have made it."
In our family, it's the gift that keeps on giving.
Or, depending on how you look at it, taking.
After Mamie and Tom Timothy had buried Harold and Raymond, they were left with four other children: three daughters and a healthy son. But after Sadie and Jimmy Doyle had buried William and Theodore (James Jr., their first child, had died, in infancy, of spinal meningitis), they were left with only one child plucked from the wreck--a three-year-old girl, my mother Helen. Mom said that she didn't remember her brothers, or her cousins. She said that until she was about eight, and overheard her mother say something about "the boys," she hadn't known that she'd had any siblings at all, and then, Mom told me, their absence was what filled therailroad-style rooms. She was afraid to ask her mother and father about "the boys," her brothers, and so she asked Sadie's sister, her Aunt Nellie. "Your brothers' names were William and Theodore," Aunt Nellie said. "They had 'muscular apathy.' When they were only little boys, they stopped walking. They couldn't go to school. They were so weak that they had to be tied into kitchen chairs not to fall over. Then they died." And that was it. Mom said she'd wanted to know everything about William and Theodore. What they had looked like, how their voices had sounded, if they had "liked" her--even a little bit, but Aunt Nellie, who warned her not to be upsetting Sadie and Jimmy with her questions, no more questions, wouldn't say any more.
Then Mom said when she was about ten, while she was sitting on the stoop of the building, watching a neighbor's little girl learning to walk, the terrible thought struck her that she might have learned to walk while her brothers, tied to those kitchen chairs, were forced to watch her. She had to know, she said, and at the same time, was afraid to know, if her brothers had resented her.
But when Mom asked her cousin Evelyn, Mamie's daughter, twenty years Mom's senior, to tell her about "the boys," Evelyn had only laughed, and imitated how they'd hollered at her whenever Mom got into their things.
It wasn't until the 1950s that scientists realized that Duchenne muscular dystrophy (or DMD), like hemophilia, is a "sexlinked" disorder, carried by females and inherited by males. It wasn't until the 1960s, around the time that my brother Richiewent into the wheelchair, that scientists discovered that female carriers of Duchenne muscular dystrophy (like afflicted males) had elevated blood levels of creatine phosphate kinase, or CPK, a muscle-burning enzyme. In those infantile days of genetic research, Mom, Pam, and I gave blood to the brand-new "genetics" department at Elmhurst General Hospital in Queens, New York. They found that although Pam and I had elevated CPK levels and were probably carriers, Mom's CPK level was normal--and she was not a carrier. "But Richie had DMD--how can that be?" we asked. They shrugged. They admitted that genetic testing had a long way to go. (Nearly forty years later, and detection of carriers is still only around 70 percent.) It would be years before they'd realize that at menopause, a carrier's CPK level often reverts to normal.
They didn't yet know that Duchenne is the most common and rapidly progressing dystrophy, or that the disease occurs in one out of every 4,000 male births. Even into the 1980s, figuring out which women were carriers, and which sons might be affected, was largely a guessing game. They knew that there was a 50 percent chance that daughters born to carrier mothers would be carriers themselves, and if they were carriers, a 50 percent chance that their sons would have DMD. They knew that despite the faulty X gene a female carrier inherits from her carrier mother, the healthy X gene she inherits from her father compensates. And unless there is a rare mutation, a female carrier will show no sign of DMD herself. If a son inherits his carrier mother's healthy X gene, he will be healthy, but if he inherits his carrier mother's faulty X gene, he will have DMD. A male gets only one Y and one X--there is no other X to compensate.
In the near future, the scientists said, a woman with cause to worry about DMD could find out, in the fifth month of pregnancy, if her fetus was male or female, and base her decision to "therapeutically" abort solely on the baby's gender.
For me, that would have meant aborting Patrick Jr., the deepest, most absolute joy of my life.
Like Mom, Pam and I are carriers. Duchenne muscular dystrophy is our heritage, our background, our pedigree. Harold and Raymond, William and Theodore, all died before their weakness became profound. Every skill my parents, Pam and Charlie, and Patrick and I had taught Richie, Jamie, Chris, and Jason, from brushing their teeth to combing their hair, to tying their shoes to cutting their food, and feeding themselves, DMD forced us to take back, until, in some terrible parody of parenthood, we were all bathing, dressing, carrying, feeding handsome bearded men in their twenties. You might have thought we'd have raised pedigree dogs, or blue-ribbon pumpkins, or even another kind of First Lady rose. The Ireland great-grand uncle, the Hell's Kitchen uncles and cousins, our brother Richie, my son Jamie, Pam's sons Chris and Jason, all the way from Woodrow Wilson through the second George Bush, we've been DMD's nieces, cousins, sisters, mothers, or some amalgam of them all. Always a bridesmaid, never a bride--although it sometimes seems about to get us too. Though there is no Paris clinic, or modern laboratory, where heartbreak and regret can be studied, they're wasting diseases too, eating though spirit and soul. (Even Sister Prejean, the death row activist, says that she can't imagine anything worsethan seeing your child condemned to death through circumstances that are partially your fault.) Though we've had our moments, there weren't a lot of smiles, Duchenne or otherwise, in our hard lives.
If I could have, I would have slipped into one of those wheelchairs. I would have given any of the lost boys my healthy body. I feel as though I owed my brother Richie, my nephews Chris and Jason, my son Jamie, especially Jamie, that much. Richie had twenty-two years and Jamie had twenty-four. In Connecticut, in their increasingly more highly powered power chairs, now equipped with ventilator shelves, Chris and Jason, twenty-seven and twenty-six, are DMD geriatrics, struggling and enduring, day by day, indignity by indignity, breath by breath.
For over fifty years, I've had more than my share of movement. I've been walking on two good legs, moving two good arms, breathing, eating, swallowing without hesitation, without a single gasp, and all the while complaining about toothaches, headaches, head colds, paper cuts, bad hair days, free-floating ennui. Unlike the boys, I can turn over in bed, stalk out of a room, go for an angry walk, throw an innocent, blue-flowered Corningware bowl full of macaroni and cheese at Patrick Sr., who, though I don't remember why, certainly deserved it.
But DMD is a beastly thing, taking not only body and soul, but stepping on the throat of spontaneity, cleansing fits of pique, an awful lot of life's fun. In DMD, there are no young arms thrown carelessly around a shoulder, no pickup games in the school yard, no snowball fights, no diving boards, nohome runs, no touchdowns, no goals, baskets, girls, or weddings, no daddies, no babies smiling for the camera.
It was tempting to agree with the shrinks who thought they were helping when they encouraged me to blame the Catholic church for what was really our decision. "After all," so many of them asked, "doesn't your church forbid birth control? Isn't it the Pope himself who wants you to propagate and fill the pews?" But the truth is that we were lukewarm Catholics and the church was innocent. "Follow your heart," was what the renegade Father O'Connor told us back in 1970, when Patrick and I were about to be married, and asked about birth control, and that was exactly what we did. Patrick Jr., all curls and eyes, was the child of our hearts. When Pam and Charlie looked at him, he was the child of their hearts too. Without any rational reason to believe it, we were sure that DMD would never happen to us.
When I was eighteen, just graduated from high school, I worked for a rusty old bank in New York City's garment district. Patrick was twenty, an electrical engineering major in Manhattan College. We had very little money and so we spent a lot of nights sitting on a bench in a small park on Sutton Place, where he'd gone with his old girlfriend, the senator's daughter. It was as though he'd never been on his way to the seminary, where they told him he was too young and should wait a year--long enough, as it turned out, for him to change his mind--though he never completely abandoned the idea. When we talked about getting married, he said he wanted the Papal Blessing. When we spoke about having children, healways said "if we should be blessed with children ..." the both of us oblivious to the fact of Richie at home, in the wheelchair, watching Combat on TV. "Blessed with children," Patrick said, when everyone we knew was fussing with hair, burning draft cards, going nose to snout with the New York City mounted police. The nurses in the hospital thought Patrick was crazy when he insisted that our eight- and nine-pound, apparently healthy baby boys, be baptized right away--just in case. He said that parenthood was "a sacred trust" and I think those were the three little words that made me want to marry him, years before I'd wonder how "blessed" Jamie was with us.
It doesn't seem possible today that there was ever a time in my life when I hadn't heard the words muscular dystrophy. Though I couldn't yet read, and didn't know it had anything to do with our family, on one dog-eared page of an old medical book I'd found underneath my grandparents' bed was a picture I kept turning to again and again, a dozen boys wearing leg braces and sitting in wicker wheelchairs.
THE BOOK OF KEHLS. Copyright @ 2005 by Christine Kehl O'Hagan. All rights reserved. No part of this book may be reproduced in any manner whatsoever without written permission except in the case of brief quotations embodied in critical articles or reviews. For information, address St. Martin's Press, 175 Fifth Avenue, New York, N.Y. 10010.
