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Home / Articles / News / Features /  Home in the World
03.23.11-Emily-Rapp-cover

Home in the World

A fatal diagnosis leads to an unexpected transformation

March 23, 2011, 1:00 am

I don’t know if it’s still there, but when I was growing up, there was a giant dollhouse in the lobby of Denver Children’s Hospital. My small dollhouse at home had white walls and rickety furniture, but these rooms were elaborately decorated. The red wallpaper in the living room was striped with delicate gold. Tiny chandeliers glimmered from the ceilings. A pink canopy bed in the corner of one bedroom looked sweet as wrapped candy, and a white bassinet sat near the window in the baby’s room. I remember thinking that the doorways were tall enough that I might actually fit inside—but the house was surrounded by glass. It was tantalizingly perfect, but impossible.


I was a frequent patient there. Because of a congenital birth defect, my left foot had been amputated at age four; this was followed by endless X-rays, prosthetic leg fittings and consultations with my orthopedic surgeon. After these humiliating and occasionally painful appointments, I insisted that my mother leave me alone for a bit with the dollhouse. I circled it, watching my reflection in the smudged glass move over those beautifully appointed rooms. I longed to sprawl on the shiny patterned chaise lounges in the living room with its oval mirror and polished lamps, or to sit down for dinner at the table with its silver forks and gleaming white plates. During the holidays (when I was often back in the hospital for more surgery), a fake Christmas tree glittered in the corner, garlanded with popcorn strings and sequined ornaments and with miniature wrapped presents stacked underneath. Life inside the dollhouse was the antidote to life at the hospital: infused with soft light, perfect and contained, procedure-free and comfortable. Someday, I thought, I’ll live in a house like this, and everything will finally be set right. 


Though their time with their son is limited, Emily and Rick try to enjoy each moment with Ronan.
Credits: Tony O’Brien

This notion, that happiness and fulfillment hinge upon radical transformation, has followed me throughout my life. From an early age, I had fantasies of being “healed” of my disability, a miracle I envisioned as rather more Disney than biblical. As my body levitated from the bed in a haze of glitter, my hair, spun in a French twist, would sparkle. My skin would shine. Free of my wooden leg and the need for it, ball gown spinning out around me, my perfect body would land in an adult-sized version of that dollhouse along with my adult-sized husband (attractive and successful) and slightly smaller children (brilliant and Ivy League-bound). I would effortlessly serve elaborate dinner parties, be the perfect mother, and write a bestselling novel every month. I would finally be at home. 


My real-life search for home was exhaustive and nomadic, spanning numerous cities and countries, houses and apartments, jobs and relationships. It took years, but by the time I arrived in Santa Fe last summer, I thought I had finally figured it out. I was married to a good man and close to my family. I had a slew of terrific and loyal friends, one book published and another almost finished, a steady teaching job—and Ronan, a beautiful, healthy boy born one year ago. That first night in Santa Fe, after Ronan was asleep, I lay in bed and watched monsoon rain waterfall over my window and listened to thunder pummeling the seemingly endless New Mexico sky. I remembered that silly dollhouse dream as I have so often over the years and thought: This is even better. 


Shortly after the new year, everything changed. I had been worried for some time. Ronan was experiencing developmental delays, missing important milestones. I would rush home from work each day hoping he’d started to crawl or had said his first word. He was the same sweet, happy, gurgling baby—but that was the problem. He was the same at 10 months old as he had been at 6 months. During what was supposed to be a routine eye exam, an ophthalmologist found “cherry red spots” on the backs of Ronan’s retinas and he was subsequently diagnosed with Tay-Sachs disease, a rare genetic metabolic disorder that will claim his life sometime in the next few years. Exactly when may depend in part upon the choices my husband Rick and I make about medical interventions like feeding and breathing tubes. There is no cure. The damage begins in the womb. As soon as Ronan was born, as the mother of one Tay-Sachs child bluntly put it, “He didn’t have a chance.”

Ronan’s body lacks hexosaminidase A, an enzyme critical for brain development, and his brain is, as they say in the neurology world, “devastated.” Nerve damage progresses quickly, leading to dementia, decreased interaction with the environment, seizures, spasticity and eventually death. Before he dies, Ronan will become paralyzed, lose his sight, his hearing and his sense of touch. As the doctor delivered these details of Ronan’s prognosis, I remembered being pregnant and watching my stomach muscles move apart one cold winter morning as I sat writing in my room at an artists’ colony, the darkness still waiting at the windows. I remembered meeting a boy called Ronan at Trinity College on a warm, rainy day in Dublin in 1994 and deciding on that name for a future child. For years I wrote “Ronan” in longhanded scrolls across notebooks like a lovesick teenager copying over and over again the name of her secret crush, tracing the curved letters like fingering a magic stone. 


There is no cure for Ronan’s condition, but that doesn’t prevent Emily and Rick from loving him and appreciating the moments of happiness he brings to their lives.
Credits: J Weber

January felt straight out of a Victorian novel: I was hysterical, inconsolable, stricken. I had the urge to run down the street in pajamas (for lack of a period nightgown) tearing at my hair and wailing. Eventually, we started to think, strategize, adjust. Instead of investigating future day care options, Rick and I found ourselves driving to Albuquerque for appointments with specialists. Floating down hospital hallways, I felt both a great numbness and an almost brutal heightened awareness. The lights felt bright enough to burn. The smiling clowns on the circus posters lining the walls of the pediatric wing seemed to leer and mock. In the waiting room, kids drew with crayons, looking impossibly normal and cheerful, all with that one irreplaceable enzyme sprinting easily through their blood.


We drove to Bernalillo to meet the parents of another child born with Tay-Sachs, the only other recent case of which we are aware in New Mexico. (In the entire United States, only a dozen or so babies are diagnosed with Tay-Sachs each year.) Years before, this couple’s son had received the same diagnosis as Ronan in the same office, from the same ophthalmologist. We saw pictures of their little boy at Ronan’s age, and then older, and then in the weeks before he died. We learned about books he liked, toys he preferred and activities he enjoyed. We learned about massage techniques that might aid with digestion and the wonders of water therapy. We were warned about shirts with tight collars, since Tay-Sachs babies sometimes develop enlarged heads.


Driving home on the dark snake of I-25, we felt empty and alone, the stars mean and sharp and close.


That couple also put us in touch with their pediatrician, who later met with us and counseled us to prepare for a “marathon.” Meanwhile, I started to network with other moms of Tay-Sachs children. This is a mighty, indefatigable group of people who give me all the grim details, compassionately but matter-of-factly, and without hesitation or pity. Rick and I signed up for a training at Upaya Zen Center designed for caregivers with terminally ill patients, and began discussing end-of-life options. We leaned heavily on our family and friends who visited in droves, trying to help us bear a load of grief we could not lift alone. Now, months into the diagnosis, we wait and watch for signs of decline. “Things have a habit of changing around their birthdays,” we’ve been warned by other parents. The traditional milestones are turned on their heads. We no longer wonder, “What if he starts talking today?” but, “What if he stops smiling, cooing?” A daily list of tyrannical what-ifs.

An elaborate taxonomy of transformation exists in our culture. Change your body, change your life, take charge of your financial future, stop wrinkles. We are driven by future-directed resolutions. We thrive on the idea of change, the business of ambition. My own childhood fantasy of transformation was just a more extreme variation on this theme. But Ronan will never speak, or write, or do much with his hands besides spin the little lizard inside the plastic egg on his bouncer, turn the pages of a soft book, and bat the chimes of his dragon toy. He will never understand a story, or love in the way we mean the word. He has, literally, no future. How do we understand the meaning and purpose of his life in a society dedicated to progress and achievement? 


The damage to Ronan’s brain, caused by a deficiency in hexosaminidase A, a necessary developmental enzyme, began prenatally. He will never speak and, as the condition advances, his interaction with the environment will decrease.
Credits: J Weber

The medical establishment is likewise necessarily geared toward protecting the future. To doctors, saving lives means extending lives. That’s their job. By contrast, when we met with a children’s hospice team, the conversation was about quality of life, not quantity. Rick and I discuss this constantly. When Ronan can no longer swallow, do we place a feeding tube no matter how his other faculties have been affected, or let nature take its course? At each stage of potential intervention, hospice care asks: “What does it lead to? What is it for?” Will we know how to listen to what Ronan’s body is telling us? Or will we just desperately cling to our son?


What is it possible to learn from a dying baby? Rick and I spend each day with Ronan, trying to enjoy him, loving him, taking him for walks, to the zoo and the aquarium. We’re not worried about what college he’ll attend, or what he’ll do with his life. We are not living for him, or through him; we are living with him, and there is a terrible, euphoric freedom in that. Alongside the terrible knowledge of his not-so-distant death are distilled, almost perfect moments of happiness. He jabbers at the sunset; he wrinkles his nose before he laughs; he eats the food Rick blends for him; he, as our physical therapist told us, “melts into your arms.” His life is a collection of singular, unrepeatable moments that are not analyzed, remembered, or regretted. 


When Ronan was a few months old, a friend of ours asked, “Isn’t it interesting that, of all of us, Ronan has the most life ahead of him and yet he’s the least worried about it?” The first part turned out not to be true, of course. Ronan’s life will be short—but he will not be worried about its length or quality. He will never feel shame, fear or regret. He will never hate himself or us. He will never sit and stare at a house inside a glass box and wish for his life to be different; he will just live it. He will always be at home in his body (the only one he knows, a body he doesn’t question). He will always be at home in the world. 


Ronan’s story is not a Movie of the Week. It is, to put it bluntly, the truth about life: that it exists side by side with death. Other cultures and traditions are acutely aware of this intimate pairing. I once celebrated the autumn festival of Chu’sok with my host family in Seoul, South Korea: a raucous, boozy party at the family grave plot, complete with music and the favorite foods and drinks of the departed. In one Día de los Muertos image, a robust, rosy-cheeked man walks with his skeleton rattling in his arms.
One afternoon, I walked with Ronan on the arroyo path near our house, his smiling face peeking up at me from the front pack, the last of the day’s sun warm on my shoulders, the mountains darkening to purple in the distance, and thought, This is a peaceful place to die. Since that day, I have been slowly learning a lesson that I had been avoiding for years, an avoidance that had fueled my frantic search for a home while simultaneously making it impossible to find. 


Ronan’s life will be short. He will never grow up enough to wish for his life to change, but he changes the lives of those around him.
Credits: J Weber

In Frances Sherwood’s novel Vindication, a fictionalized account of the life of Mary Wollstonecraft, the tortured and occasionally suicidal protagonist is desperate to discover the right way to live. She tells her longtime friend and publisher that she is overwhelmed by “all that might be, not be, so be it, your mind going a hundred thoughts between this moment and the next.” Her friend replies: “But my dear, you have arrived. You are here, at your life. Put yourself down, settle in. It is yours. You have been living it all along.”


And so I finally got the transformation I longed for, but it didn’t happen in the way I expected. I’m weirdly at home in my world, my new home under this big sky with my beautiful, dying child. Walking on the arroyo, listening to him laugh, watching him eat and sleep and play, sitting with him on the couch, sometimes for hours, teaching my classes and writing my books. It is not the life I imagined, not the dollhouse, but it is home.  SFR

 

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