To be diagnosed with a rare disease is to have wildness pressed upon you. You are not exactly the secretive quarry that birders travel the world to add to their checklist—more like something sighted with surprise, something that no one was looking for and no one is completely sure how to handle, taken into tentative, uncomprehending captivity. Other diseases are receptive to medicine’s domestication. But your disease rattles the bars of the hospital cage, breaks the rules, raises eyebrows.
My illness connects me to the wild places where medicine itself is still a feral thing—untamed as tropical sunlight and suddenly flooding water and the appetites of big cats. In the Amazon rainforest healing is still rooted in the mysteries of what grows and flowers, in the bark of the cinchona tree—the source of quinine used to combat malaria—and in the leaves from which shamans brew the psychedelic ayahuasa.
I too am now possessed of visions. Fruit of the jungle yucca, corticosteroids drive away sleep, fill me with wild energy, exhaust me. Bells sounded by patients in pain too sharp to bear alone, or in trouble that they cannot understand, penetrate the half-dark, half-quiet night surrounding my hospital room. Mystery hangs in the stuffy air. I dream of going home, I dream of being healed, I dream of my clothes rising out of the battered chest of drawers at the side of my bed where I exchanged them for a worn cotton gown—hardly enough fabric to cover my shame in daylight—and draping me, making me whole again. In my dreams my blood is still as good as the blood of the nurses who sample my veins and the doctors who track my platelet counts, the shamans who hold the power to keep me here. I imagine unhooking the tubes that leash me like an animal to machines, of leaving them opened and dripping—of fleeing through the long hospital corridors, down the elevator emptied of patients and visitors and flapping white coats, to the teeming streets that will reabsorb me into the unceasing life of the city.
If I have met my match, I would like for it to be a thing of muscled beauty, reflecting back to me the grace and the resilience possessed by only a small number of creatures. Fewer than seven thousand snow leopards survive in the remote, chill highland forests of central Asia, still as always living to kill. Hunters pick them off one by one for their gorgeous spotted pelts, yet only a few human beings have witnessed a snow leopard in the wild. Nor will many doctors ever encounter a patient with my disease, for it strikes only five to seven people in a million. And no one knows when my disease will return from hiding, for when I am in remission even a microscope cannot find evidence of it in a smear of my blood.
Day approaches from the edge of darkness, one bed at a time floodlit as a soft voice announces, Vitals. What’s “vital” has been distilled in the hospital to the small world of the body: down to temperature, blood pressure, oxygen (“blood ox”), pulse; a cuff placed silently around an arm, first squeezing and then releasing, a thermometer in an ear, a device clipped over a fingertip; a gloved hand feeling for faint pumping at the wrist, counting. Fitfully the signs of ordinary daily life return, the fidgeting wheels on linoleum, the steamy aroma of eggs and sausage uncovered on trays, the voices of the morning news rising from televisions with the sun.
The city is barely visible at dawn through my corner of hospital window. Alone in my bed I imagine mist slowly burning away and leaving only trees and unmowed grass. Out of the shadows a lioness reaches her long legs and stretches, arching her roughly furred back, and looks directly at me, wrapped in a flannel sheet. She knows me, and she knows where to find me.
I am on a mission, flying alone into unfamiliar territory. I am a medical pilgrim, bearing the baggage of my rare condition.
In the airport at my destination, museum-size screens hang over the escalators, welcoming travelers who have come from far away to seek out specialized medical help. Beneath enormous block letters naming the clinic where I’m headed is a picture of a smiling, gowned patient, completely bald: visual shorthand for cancer. But this is not me, this is not my disease. At the curb outside I watch an elderly couple board an airport shuttle to the Sleep-Eze motel. Some sort of device is dangling from her neck. What’s wrong with her? I wonder. She looks tentative as she climbs the steps into the van one by one, taking long breaths as she goes, checking behind her for her husband. Has she come to this place, like me, hoping to stay alive? And is this how I look, at the airport information kiosk, at the front desk of my hotel: like a passenger coming, desperate, to the end of the medical line?
I follow a map of the giant medical school campus to the lecture room where I am to join a support group for others diagnosed with my disease. I find a spot at a table near the front of the room, sit down and unpack my little notebook and my pen. Everyone in this room knows what I know, everyone here knows what it’s like to live with the uncertainty of when, or where, or if, a relapse will occur. They know about the big machine that siphons off your blood, centrifuging it and replacing, from bags of donor plasma, the portion that’s tainted with rogue antibodies. They know what it feels like to pass long days waiting for treatment, and then to wait for the results of blood work to reveal if your platelets are rising from the scary lows that might already have caused organ damage. They have all wondered what their next hospital roommate will be like—friendly? moaning in pain through the night? accompanied by loud relatives who will ignore the sign saying that the bathroom in this room is for patients only?—and what the dinner they ordered will really turn out to be when they lift the silver dome on the tray. I wonder how many of them can, like me, call up in a second the remembered smell of disinfectant and the sound of mops in the hall at the end of the day, the stale sound of television seeping from every doorway along the corridor, almost an aroma clogging the air.
Off to the side of the lecture room is a table on which bottles of soda and bags of potato chips have been laid out alongside shrink-wrapped sugar cookies studded with colored jimmies. Folks all around me are forming a line that twists around the table, taking plastic cups and filling them, picking up napkins and loading their paper plates, while I watch, immobile—weighted down by disappointment. Could such pale, ordinary stuff be what’s on offer today, on this occasion when patients with our rare diagnosis have journeyed from so many distant places? Can this be what we feed on when we gather, finally, together?
Day and night in cyberspace, families comb the universe for others like themselves who are suffering in isolation. They exchange symptoms—an infant son whose muscles are too flaccid to answer a hug; a toddler’s mysterious failure to grasp a spoon; the itching that will not free them for even an hour—like playing cards whose matching suit must, they pray, be out there, somewhere. Hope depends for them on breaking out of the cocoon of solitude. The National Heart, Lung, and Blood Institute provides an on-line alphabet of “rare heart, lung, and blood diseases” on which it has compiled information: Klippel-Trenauny-Weber Syndrome (KTWS); Cupravalvular Aortic Stenosis (SVAS); Lymphangioleiomyomatosis (LAM). On the web-site of my own disease, Thrombotic Thrombocytopenic Purpura (TTP), I read: “We are stronger TOGETHER!” February 28, 2015 will be the next RARE DISEASE DAY USA, sponsored by the National Organization for Rare Disorders (NORD), its motto, “Join together for better care!” The director of the organization dedicated to my disease has driven through the night from Toronto to central Ohio to speak at our meeting, and to hand out red rubber bracelets stamped TTP that each of us can wear to remind ourselves: I am not alone.
I stretch the bracelet hesitantly over my clenched hand, snap the band against the tender flesh on the inside of my wrist, against the blue veins through which blood is returning to my heart from my extremities. In the food line, others are shyly beginning to trade stories: comparing their first episodes, comparing their relapses, comparing treatments; sharing the sense of anxiety that never entirely abates, and the ongoing confusion of reading their own bodies. But I hold back, reluctant to see myself in their faces, unwilling to abandon the mystery that has claimed me, unready to be dis-enchanted.
If my disease were an animal, what would it be?
Would it be a bird of prey, an eagle dropping without warning from the sky, its talons—the surprising source of the great bird’s power—gripping what’s alive and taking it away? The word “raptor” derives from the Latin rapere: to seize by force. The Harpy Eagle is strong enough to carry off a monkey. In the night, the Great Horned Owl slowly pivots its head two hundred seventy degrees, planning, seeing what cannot see back. The owl’s hearing is so acute that it can register sounds of prey moving beneath the earth’s surface, the scrabbling of fleeing field mice. The vulture’s survival depends on the knowledge he logs from the air: the migration of quarry that other predators will kill and leave in the high grass, opened and bloodied, when their appetite is sated; the places where the light will sharpen as autumn shifts into winter and the stripped trees will reveal the struggling, vulnerable life on the ground that’s most likely to succumb.
My disease finds me without warning, throwing me on the defensive. No one knows its cause. When relapse comes, sticky molecules accumulate in my blood, blocking the fine arteries to the brain—setting a rainbow bar adrift without warning over my field of vision; suddenly jumbling my speech. Each time I feel freshly assailed. My clothes are taken from me, my blood sampled, the pumping of my heart measured from a flimsy emergency room cot, barely set off from others’ raw, animal cries and aggressive threats fueled by pain and by fear. I will be wheeled through long, dull corridors into the blinding lights of surgery, where a masked stranger will carve my living flesh, open a path into my warm throat, find access to the blood circulating deep below the surface.
We imagine the hospital as a vast cityscape of sophistication where human beings wield science against illness—corralling it, mounting it, riding it to its demise. But rare diseases remain wild things, animals on the loose, never not dangerous, the essence of disease itself. Nurses move me in the night to a different floor, where the sight of a machine drawing off my blood supply and reinfusing the plasma from freshly thawing donor bags is not fearsome. Doctors wield instruments, dispense pills, miraculously push illness back from the ledge where it has cornered mortally ill people. But my disease holds its ground, continues to flirt with danger.
In the past two years I have been re-wilded, forced to accept the animal truths of my human body. I have witnessed my own blood drained, exposed to day’s light, mixed with the plasma of strangers—turning me back to the elemental rawness that few must face, much less see, in themselves. I have been crazed by the careening high of prednisone—adrenaline untasked, the body’s own steroid set loose so that it can muscle every moment to emergency-level power. Before my eyes my body has been disassembled, revealed as possessed by an alien force. My doctor presses the cold disk of his stethoscope over the thin fabric of my gown, listening for the brute, animal heart—just a beating thing, really, driven by surging blood, and by the untamable urge to survive.
Yet if the diagnosis of a rare disease means consignment to wildness, it is also a call to the imagination. In my hospital bed I devour books about ravens, birds of perfect blackness, of mysterious, glossy sheen, their dark eyes, unique in the bird world, seeming to signal recognition. The condor—a carrion-eater like the raven—sails the updrafts of the Andes, alert for carcasses of large animals like deer and cattle. Yet even with its soaring, ten-foot wingspan, the condor remains an ugly thing, with a vulture’s featherless head. I envision my illness, instead, as a luminous raven: called like the vulture and the condor by fresh blood, but much more than its own appetite for the wrecked flesh left behind by killers. Like their relatives the magpies and crows, ravens are smart and engaging, thieving employers of strategy. The Haida of the Pacific Northwest have elevated the Raven to the mythic status of a trickster who teases human beings and challenges them, reflecting back to them their own nerve and creativity.
From the deck of a nineteenth-century whaling ship, humans could see the big beasts riding the water, their flesh scarred by encounters with predators, beating maimed fins, dangling embedded harpoons. On their bodies the relentless pitch of survival was made visible to the naked eye, and the dark threat of the unknown. Though he survived his fall overboard into the roiling sea, Melville’s deckboy Pip was forever altered in Moby-Dick by the sight of “God’s foot upon the treadle of the loom.”
Nor can I return to the certain, safe place I came from. Two decades ago I peered over the gritty rim of the universe at the Grand Canyon, into the void where what time and water can do even to solid rock is revealed. But I am no longer a tourist of boundaries. Illness is a wild dream, remote as the far edge of night from what we think we know. And when it envelops us, when it fogs and transforms ordinary life, we can only hope—I see now—to emerge a little more fully awake.
On summer days at our neighborhood Lake Michigan beach my cousins and I used to tiptoe to the sand’s edge, daring one another toward the lapping water. We’d enter in a wobbly line, shivering, our still incompletely formed bodies warmed from the waist up by the sun. And we’d pause in unison, aroused by what we knew would happen next. The sudden immersing cold—an electric, hurting chill—would take us in an instant past the string of floating buoys, to the far-away shores of the three-hundred-mile-long lake and the Canadian frontier that still glowed with Indian and French names. Petoskey (from the Ottawa Pet-O-Sega, or “Rays of the Rising Sun”), where beautiful bits of fossil coral continue to surface after millions of years. Mackinac, Seault Ste. Marie: places where long-ago conflicts left their traces, where ships loaded with beaver pelts docked, shaggy with ice after long travel from the north woods. Lucky, lucky us, to be able to walk from our own backyards to the greatest of inland seas, chattering with one another as we floated toward the vast interior of the continent scooped out by massive, slow-driving glaciers, the raw, unpredictable material of the imagination left in their wake.
To be diagnosed with a rare disease was never my intention—who among us would hope for such a destiny? I am marked for life as a medical curiosity. The treatments my doctor orders constitute the most sober of guesswork. Residents crowd my hospital bed for a first-hand look at a patient with my rare diagnosis. I stare at the blood that fills the tubing in the technician’s gloved hand, subtly tinted in a secret code that even I don’t know. Unlikely to profitably repay investment in research, rare diseases are easy, I have come to know first-hand, to abandon: medicine’s “orphan diseases.” Still I do not mourn my fate as one of the stars that slipped out of alignment in God’s firmament. “Rare,” precious, unique, valuable, unlikely, unprecedented: who would I be now, without this drama and this unknown?
My double-scarred neck reveals the fang-marks of surgeons’ scalpels seeking the jugular, seeking—to save me—the same route to the heart that any killing animal hopes to open. Southeast Asia’s Hmong tribes honor the epileptic, shaken free of the ordinary, as a seer. I too have been seized by a great force, brought into the presence of pounding uncertainties. Twice already my disease has taken me to the brink where the creaky workings of life are laid bare and forced into the light—at once sweet and bitter, unwelcome and amazing. Like a raven my disease takes flight, taking me—taking me with him. He scares me, he enthralls me, he grips tight my attention, he takes me on a wild ride to a place where I cannot count on surviving. I cry out, I growl, I claw my way back to life, strangely renewed. Wings dark as night, eyes bright as stars, my most intimate enemy and my most inscrutable companion, he comes back for me again and again.