Duchenne MD: A Family Legacy

A family explores impact on five generations

by Christine Kehl O'Hagan on January 1, 2005 - 4:16pm

In the late 1860s, 9-year-old Bridget Moore left Ireland for New York, bringing with her “a small steamer trunk, a pair of rosary beads, a clay pipe, and as near as we can figure out, Duchenne muscular dystrophy.” Now her great-granddaughter, Christine Kehl O’Hagan, has written a memoir about her family’s multigenerational history with DMD. The Book of Kehls, published by St. Martin’s this month, traces this bittersweet history with candor and humor.

The book, endorsed by MDA National Chairman Jerry Lewis, beautifully tells an often heartbreaking story, but one shot through with love and hope. It’s a powerful and much-needed acknowledgment of the complex emotional impact of a genetic neuromuscular disease on every family member, regardless of medical and scientific advances. Above all, the story demonstrates the perseverance that sustains a family in the face of a devastating heritage. With this book, O’Hagan has turned the Kehl legacy into a bequest of compassion and courage.

Christine Kehl O'Hagan
Christine Kehl O'Hagan

In this chapter, O’Hagan recounts how she and her sister, Pamela, as children in the 1950s, delighted in and protected their little brother, Richie, even as their mother and grandmother watched with dread the boy’s growing difficulties in walking and climbing. Both sisters would inherit the family legacy and have sons with DMD.

O’Hagan invites readers to write to her at chrisohagan@optonline.net.

 

Maureen McGovern & Alexis Villa

Time passed, Richie looked better, less red, more pink. His grayish eyes turned an amber brown, and white blond hair sprouted on top of his head. He looked like a pink cupcake with coconut frosting and smelled like the vanilla in Nana’s rice pudding. At every baby sound Richie made, Pam and I ran to the bassinet, but we could’ve walked. The apartment was so tiny we were never more than a few feet away. My father used to say that our apartment was so small that he could shave with his right hand in the bathroom, and make coffee, in the kitchen, with his left.

Everything about our cupcake baby brother was delicious from the soft folds in his neck, to his wrinkly fingers and toes. Pam and I could hardly keep our hands off of him. When Mom wasn’t looking, we crept into our parents’ bedroom, reached into the bassinet by Mom’s side of the bed, and held his hands, or pretended to bite his toes. Or we stood behind his head, and made silly noises just so he’d turn and look for us. When he tried to turn over, in his diaper and T-shirt, the back of his neck looked so tender and soft I could barely stand it. I had to turn away. He was just so small. Though sometimes he hated it, it was more fun to press our noses to his, and stare into his dark eyes — until he cried, and shook his head as if he were trying to wake from a nightmare. Then Pam and I were in trouble, and Mom sent the both of us down the twelve-inch hallway, and into our bedroom. Pam’s was an old iron bed that some ancient Irish relative had expired in, and mine was a maple bed with an expensive “horsehair” mattress that my parents had splurged on when they thought I’d be their only child. We sat on our beds, across the room from each other, and our knees touched.

When he grew too big for the bassinet, Richie was moved into our room…. Every morning, it was like a present to sit up in our beds, and see him sitting quietly in his peach-colored crib just waiting for us. It amazed me that the ugly, Fred Mertz baby he had been was now so pretty with big bright cheeks, and eyebrows and eyelashes so thick they seemed to be made of felt, like Mr. Potato Head’s. Coming down the four flights on our way to school, Pam and I laughed at how silly he’d looked, sitting in his high chair in his yellow footie pajamas with pablum cereal smashed all over his face. He was just waking from his morning nap on Mom’s bed, when we came home for lunch, and then we laughed hysterically at the chenille tracks in his pink cheeks. At dinnertime, he sat in his high chair, his sticking-out ears bright red from the cold, when he was supposed to be feeding himself, but fumbled with his silly white spoon instead, and dropped most of his dinner on the floor. Once, when she was clearing the table, Mom showed me the spinach cross that had formed on Richie’s plate. “A blessing in disguise,” she said, but I didn’t know what she meant. I didn’t notice anything “in disguise.” We were up to our eyeballs in blessings then, and Richie was just one more.

Maureen McGovern & Alexis Villa

But Richie didn’t walk until he was fourteen months old, and when he “ran,” it didn’t look like running at all, but hopping. Richie hopped. First on one foot, and then on the other. He had trouble climbing the stairs. Clinging to the banister, Richie pulled himself from one step to the next.

Mom said that Richie was just “immature,” that some kids are just a little slower than others. “A lot of kids have trouble with the stairs,” Mom said — although I couldn’t think of any. All of the kids on the street, even little ones like Richie, were climbing the stairs two at a time.

“A lot of kids take a while to learn to run,” Mom said, “you never ran fast either,” she said to me, and it was true. Pam was much faster. And we could see for ourselves that Richie was very short, and for him, the stairs were very high. Mom said that Richie would learn to climb the stairs in time, and he would learn to run, but as time passed, he didn’t get any better at these things. If anything, he got worse.

Maureen McGovern & Alexis Villa

When Mom thought we were asleep, she tiptoed into our bedroom and leaned over Richie in his youth bed, and rubbed his calves. Though she never realized I was watching her, she rubbed his calves when he was lying on his belly on the brown rug watching cartoons and she rubbed his calves while he was sitting on the sofa next to her, playing with his toy soldiers. Whenever Richie sat next to Mom, she rubbed his calves and whenever Pam or I sat next to her, she rubbed our calves too, holding our legs this way and that, pressing her index finger into our flesh. All she knew of the muscular dystrophy that had killed her brothers and her cousins was that it started with big calves.

But Mom didn’t remember the boys and she didn’t know what else to look for.

Later on, Mom would talk about one particular “moment of knowing,” when Richie, then only a few months old, was sitting on her lap in the front seat of our aqua-and-white Ford, and his legs felt, as she described it “peculiar.” By that time, Pam and I’d had our own “moments of knowing” — Pam, in a Missouri Air Force Base, when the pediatrician spent a long time looking at Christopher’s eight-month-old legs, and me, at a birthday party in Queens, when a father elbowed me, pointed at Jamie’s legs, and said, “He’s gonna be some bruiser!” and it was as if an icy metal cleaver neatly sliced my life in two. Then my sister and I knew exactly what Mom had meant.

Maureen McGovern & Alexis Villa

My grandmother, who knew exactly what muscular dystrophy looked like, who couldn’t even bear to look at the only picture of two of her three lost sons, never said a word about Richie’s troubles. And despite her fears, Mom never mentioned his “immaturity” to the pediatrician. The doctor never said a word about his legs, never asked him to climb stairs, run, or even get up from the floor, something that was getting harder and harder for him to do. Richie was never sick, Mom told us, and his checkups were fine. Why look for trouble? Even we could see that Richie was a happy little boy with a gentle temperament who laughed a lot and ate everything in sight. He was funny, and he was smart. Even Dad, who didn’t have a lot to say and was rarely ever home, said that Richie was so bright, we should call him “Sunny.”

Kehls
Helen Kehl with (from left) Pam, Richie and Christine, in 1958. Richie died in 1979.

And “Sunny’s” two-step, running/hopping didn’t seem to bother him much. Richie’s own attempts to run seemed to strike him as ridiculous anyway. When he saw us watching him try to run, he rolled his eyes, and made us laugh. But then he began to fall. He fell on the sidewalk. He fell in the bathroom. He fell in the kitchen, and in the living room, right on top of his plastic soldiers spread all over the rug. Then he stopped rolling his eyes, and we stopped laughing. We told him it wasn’t his fault. We blamed the uneven pavement, the wet spots near the tub, the glossy linoleum by the door, the toys under his feet, the lumpy spots on the rug. In the winter, we told ourselves and anyone who would listen, that Richie fell down so much because of his bulky gray snowsuit and in the summertime, we blamed the heat, or maybe he needed glasses, or his sinuses made him dizzy, desperate for reasons, hurtling through space, losing the idea of the “normal family” we’d been what seemed like only moments before. What had happened to us? What was wrong with Richie? What would happen next? Mom, Pam, and I were forever picking Richie up from the sidewalk, steadying him on his feet, dragging him up the four flights of stairs. We had to protect him from eager dogs, unsteady toddlers, from the crew-cut boys whizzing through the streets on skinny English racer bicycles with baseball cards clothespinned to the spokes, from the curious eyes of the neighbors, who, like us, couldn’t understand, in the Thunderbird days of the late 1950s, a little boy who had such trouble moving about in the world.

“He has a little trouble with the stairs,” Mom, Pam, and I said to the mailman, and the Con Ed man, and the super trying to pass by. My mother watched her son struggle with the stairs, and my grandmother watched him struggle with the stairs, and Aunt Nellie too, and they looked at it and wouldn’t see it, and hoped, I imagine, that it would all just go away. My father was the only one who couldn’t stand to watch, and when Dad was around, he carried Richie up and down, and anywhere he wanted to go. Like Richie, Pam and I couldn’t understand what was happening. We were as bewildered as he was. At dinnertime, when it was time to go home, and she had to start dinner, my mother went upstairs ahead of us, leaving Pam and me to get Richie home. Pushing him up the stairs from underneath his small backside was slow and tedious work. It was quicker to pick him up by the belt loops of his pants, one stair, steady him, and then the next, and if that didn’t work, then I just carried him, and Pam carried his coat, though Mom didn’t want me to do that. She was afraid that we’d all fall. I could barely stand hearing his groans, his colossal effort, and then how exhausted he seemed, eyes shut, pink face pressed against the banister.

Kehls
O’Hagan’s son Jamie is flanked by her nephews Christopher (left) and Jason Willmer in the late 1980s. Jamie died in 1998 at 24. O’Hagan’s son Patrick Jr. doesn’t have Duchenne MD.

“He has a little trouble with the stairs,” we whispered to cranky old Mrs. O’Brien, who seemed anxious to pass, but instead, stood glaring at us from the bottom of the staircase. “What’re ye mumblin’?” she demanded, but we were too ashamed to answer her, too ashamed to look up and see our trouble-making selves reflected in her black, raisin-like eyes. Everest climb for us, and we couldn’t look any of the neighbors, who thought we were playing and were angry at the inconvenience, full in the face — and so we stared at their shoes, feet passing by us so effortlessly on Mercury-like wings. Mrs. O’Brien, in her black orthopedic shoes, Mrs. O’Neill, the old bitch who lived beneath us, in her cut-up carpet slippers, with the bunion that looked like a parsnip — even the slender Meenahan bride, impatiently tapping her delicate pink Capezio, her signal for us to move aside and let her pass, the Geir’s Meats bag dripping blood behind her. Softly, quietly, we cheered for Richie when he managed one step, and then the next one, huffing and puffing worse than Nana with her arthritis, and worse than Aunt Nellie with her “bum hip,” and they were in their seventies, and he wasn’t yet four. Pam and I had homework to do, cartoons to watch. We were hungry and sometimes we got angry at him. “You’re so slow!” we hissed, “come on, Rich!”— but both of us had to look away, for his dark eyes, the eyes I still see in my dreams, were trying so hard. Every step pulled Richie further and further away from the beautiful baby he had been, and the family we thought we were, and the blessings we thought were ours.

In the days and weeks and months that followed, Richie fell so often that scabs were mixed in with the freckles on his nose, and he often looked as if he had the measles. His chin was black and blue. He had purple lumps on his forehead, new scabs or the old ones ripped open on his knees. Those poor knees, shredded too many times to count. Mom followed him with icebags, face cloths, iodine, Band-Aids. Boys will be boys, people said, and despite her early moment of knowing, it was her moment of denial, for Mom agreed. One injury after another. That’s what happens when you have a son. Meanwhile, Richie hit his face so often that he lost his baby teeth before they were wobbly. Even his knuckles were rubbed raw from the sidewalk. We couldn’t understand why he didn’t put his arms out to break his fall, not knowing that he had already lost that response. It was amazing that he never broke any bones, or needed stitches. Someone was always around to stand him up, dust him off, pretend that things were fine, for as weak as he was, he was somewhat flexible, and many of his falls were not abrupt but more like a gradual sinking as his legs gave way. He was frightened, staring at us from the pavement. When we got him to his feet, he banged his fists against his thighs, calling for the kids on the street to wait up. Sometimes, when they were waiting for Richie, not looking where they were going, the kids smashed into things — trees, the streetlights, each other — and then they fell down. At this, someone else on the sidewalk for a change, Richie roared with laughter — but then he fell. He fell if he laughed too hard, if he had too much fun, if other little boys pulled off his hat, if the little girls tried to grab his hand, and pull him along. They were just kids, like Richie, like us, and there was nothing Pam or I could do but leave the jump rope and hopscotch and stand near Mom, in case one of the kids frightened Richie, made a funny face, patted him too hard on the back, and down he’d go. “Go and play with the kids,” Mom said, “I’ll take care of Richie,” but we couldn’t do it. It was too hard. When Richie was on the sidewalk, games didn’t matter. Nothing but Richie not falling down mattered. I couldn’t wait for Richie to grow up, get older, be better, and until then, I wanted to be there in case he fell.

Note: The Book of Kells is an Irish manuscript created by monks in the late eighth or early ninth century and contains the four gospels. It’s considered the richest, most beautiful example of medieval Celtic art, featuring extensive use of illuminated lettering. The manuscript survives at Trinity College in Dublin.
Reprinted by permission of Christine O'Hagan.
Copyright © 2005 by Christine O'Hagan

 

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