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Overview
When Bridget Moore left Ireland in 1865, she never suspected that along with her trunk and rosary beads, she was bringing Duchenne Muscular Dystrophy to New York City. It wasn't until Bridget was a grandmother, one who had buried four of her grandsons, that she realized she'd brought MD to the States, a disease that would haunt her family for generations. Years later, her great-grandchildren grew up under the elevated trains of Jackson Heights, Queens—and one of them was Christine Kehl O'Hagan, the author of this moving and insightful memoir.
Christine, her sister Pam, and their brother Richie played in the streets and attended mass every Sunday. But Richie had trouble walking. By the time he was diagnosed with muscular dystrophy, Christine learned that two of her mother's brothers—uncles she'd never known about—had died of MD. Christine eventually married and had a healthy son. But one day she saw her second boy, Jamie, struggle to climb onto the school bus—and she knew knew then and there that this disease would be with her the rest of her life.
Extraordinarily written, with much honesty and humor, The Book of Kehls is the engaging story of a family that has known love, courage, and heartbreak in equal measure—and survived.
Product Details
| ISBN-13: | 9781429932134 |
|---|---|
| Publisher: | St. Martin's Publishing Group |
| Publication date: | 02/07/2006 |
| Sold by: | Barnes & Noble |
| Format: | eBook |
| Pages: | 228 |
| File size: | 430 KB |
About the Author
Christine Kehl O'Hagan is also the author of the novel Benediction at the Savoia. She was born and grew up in Jackson Heights, Queens, and now lives with her husband on Long Island.
Read an Excerpt
The Book of Kehls
By Christine Kehl O'Hagan
St. Martin's Press
Copyright © 2005 Christine Kehl O'HaganAll rights reserved.
ISBN: 978-1-4299-3213-4
CHAPTER 1
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 goodbye 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 brother when 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 the railroad-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 Richie went 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 worse than 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, no home 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, he always 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.
CHAPTER 2I WAS BORN IN 1950, IN QUEENS, NEW YORK. IN 1951, on the G.I. Bill, my parents bought a new house in Levittown, Long Island. By 1952, we were back in Queens. To say that my mother disliked suburbia would be like saying Hitler wasn't very nice. Until the end of her days, Mom referred to Levittown as that "gulag of flora and fauna." On the parkways, all of the moving vans were heading east. Ours was the only one heading west.
So rather than easing into the world wrapped in a warm blanket of sunshine and listening to sweet birdsong, in my first memory, I'm standing under the el, where it's dark and noisy and smells like bus exhaust. Nobody knows how nearsighted I am, that to me, the red and green traffic lights on Roosevelt Avenue look like fuzzy basketballs suspended in midair, and the Grey Nuns of the Sacred Heart whom Mom and I sometimes pass on their way back to the St. Joan of Arc convent, don't seem to have starched white cuffs at their wrists at all, but rather, thick white slices of mozzarella cheese. And when I look at the good sisters' feet, I don't see shoes but casters, like the ones on our hassock. On our way to the A&P, or Teddy's Meats, there are lots of people Mom and I meet, but unless they bend down, and look me full in the face, I don't know who they are. I can't see them.
Although I lived in a solitary, myopic world of hapless possibilities, asking my bewildered, high-strung, anxiety-prone mother if the good sisters chewed on their wrists when they were hungry, and did they take their wheels off when they went to bed, it wasn't what I couldn't see that bothered her. It was my pawing through the roof-dried laundry for the sunshine I told her was trapped inside, and my telling her that I could see Daddy's Aqua Velva crawling underneath my closed bedroom door that finally did it. Then she called Nana on the phone, and sobbed that I'd gone mental.
And then I learned to keep my fantastic world to myself.
Even when the statues in church winked at me, even when I heard the opera singer across the courtyard practicing her scales, and I watched the beautiful notes floating past the fire escape, more thrilling, to me, than any country birdsong — I kept it quiet. It was my first-grade teacher who told Mom that I needed glasses, and ruined everything. I might have stumbled over a lot of curbs, and had some nasty encounters with parking meters but I was happy in the soft, gauzy world I had, and not so happy with the sharp world my pink plastic harlequin glasses gave me. When I put on my glasses, and met the world for the second time, I was sorely disappointed. The statues at church weren't flesh but plaster, and the opera singer's "notes" were only incinerator ash. Everything was so much harder, homelier, grimier than I'd thought, and not nearly as much fun. Until, long before I started school, my mother sat me down, and taught me to read. Then, slowly, the world became a wonder once again.
(Continues...)
Excerpted from The Book of Kehls by Christine Kehl O'Hagan. Copyright © 2005 Christine Kehl O'Hagan. Excerpted by permission of St. Martin's Press.
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Reading Group Guide
1. This book is a true story of one family's struggle with a fatal disease——and a carefully crafted narrative. What are some of the techniques that O'Hagan used to create characters that involve us deeply in her story?
2. Why did O'Hagan choose to present her son Jamie's death scene in the prologue? Do you agree with the decision to begin the memoir on such an intense note?
3. Though this memoir is sad, it is also funny. What impact does humor have on your experience of the book?
4. During much of the book, O'Hagan grapples with her desire for "a life that matters." Discuss whether her desire was fulfilled.
5. In several emotionally charged passages, the author describes the grief that she and her husband experienced right after their son's death. Will this memoir be helpful for parents who suffer the ultimate loss?
6. O'Hagan doesn't credit her Catholic faith or any one source of strength as the "magic bullet" that carried her through. Instead, she writes about following "sparks." What does this say about her religious upbringing? What do you find helpful when confronting life's hardships?
7. The author writes about muscular dystrophy's "gifts." Discuss the idea that difficult circumstances can offer gifts.
8. Though begun on a sad note, the book ends optimistically with the author at the playground with her three-year-old granddaughter. O'Hagan writes that "joy is attached to our hearts with the most tenacious of threads." Discuss whether this statement resonates with your own life experiences.