For eight years, every night is the same. The same routine of countless details, connected in precise order, each mundane, each crucial.
The routine makes the eight years seem longer, until afterward, when because of the routine the years seem to have evaporated.
Wake up to a steady, motorized noise. Something wrong with the water heater. Nnngah. Pause. Nnngah. Nnngah.
But it's not the water heater. It's my boy, Walker, grunting as he punches himself in the head, again and again.
He has done this since before he was 2. He's 11 now. He was born with an impossibly rare and random genetic mutation - cardio-facio-cutaneous syndrome, a technical name for a mash of symptoms. He is globally delayed and can't speak, so I never know what's wrong. No one does.
There are possibly 300 people with CFC around the world. Doctors call it an orphan syndrome.
I count the grunts on the way to his room: one a second. To get him to stop hitting himself, I have to get him back to sleep, which means taking him downstairs and making him a bottle and bringing him back into bed with me.
That sounds simple enough, doesn't it? But with Walker, everything is complicated. Because of his syndrome, he can't eat solid food by mouth, or swallow easily. Because he can't eat, he's fed formula through the night via a feeding system. The formula runs along a line from a feedbag and a pump on a metal IV stand, through a hole in Walker's sleeper and into a clever-looking permanent valve, sometimes known as a G-tube or a mickey, in his belly. To take him out of bed and down to the kitchen to make the bottle that will ease him back to sleep, I have to disconnect the line from the mickey.
To do that, I first have to turn off the pump (in the dark, so he doesn't wake up completely) and close the feed line. If I don't clamp the line, the sticky formula pours out onto the bed or the floor (there are patches of carpet that feel like the Gobi Desert under my feet, from all the times I have forgotten). To crimp the tube, I thumb a tiny red plastic roller down a slide. (It's my favourite part of the routine - one thing, at least, is easy.) I unzip his one-piece sleeper (Walker's small, and grows so slowly he wears the same sleepers for a year and a half at a time), reach inside to unlock the line from the mickey, pull the line out through the hole in his sleeper and hang it on the IV rack that holds the pump and feedbag.
Then I reach in and lift all 45 pounds of Walker from the depths of the crib. He still sleeps in a crib. It's the only way we can keep him in bed at night. He can do a lot of damage on his own.
This isn't a list of complaints. There's no point to complaining. As the mother of another CFC child once told me, "You do what you have to do." If anything, that's the easy part.
The hard part is trying to answer the questions Walker raises in my mind every time I pick him up. What is the value of a life like his - a life lived in the twilight, and often in pain? What is the cost of his life to those around him? "We spend a million dollars to save them," a doctor said to me recently. We were sitting in her office, and she was crying. "But then when they're discharged, we ignore them."
Sometimes, watching him, it's like looking at the moon: You see the face of the man in the moon, but you know there's actually no man there. But if Walker is so insubstantial, why does he feel so important? What is he trying to show me?
To answer that question, I decided to look again at the life he had lived, and the way we had helped him live it - first at home, later in a special community for children like him. I climbed into a car and drove across the continent to meet other children with his syndrome.
All I really want to know is what goes on inside his off-shaped head, in his jumped-up heart. But every time I ask, he somehow persuades me to look into my own.
Before I can slip downstairs with Walker for a bottle, the bloom of his diaper pillows up around me. He's not toilet-trained. Without a new diaper, he won't fall back asleep or stop smacking his head and ears. Detour to the routine of the diaper.
I spin 180 degrees to the battered changing table, wondering, as I do every time, how this will work when he's 20 and I'm 60. The trick is to pin his arms to keep him from whacking himself. But how do you change a 45-pound boy's brimming diaper while immobilizing both his hands so he doesn't bang his head or (even worse) reach down to scratch his tiny, plum-like but suddenly liberated backside, thereby smearing excrement everywhere? While at the same time immobilizing his feet, because ditto? You can't let your attention wander for a second. All this is done in the dark as well.
But I have my routine. I hold his left hand with my left hand, and tuck his right hand out of commission under my left armpit. I've done it so many times, it's like walking. I keep his heels out of the danger zone by stopping his knees from bending with my right elbow, and do all the actual nasty business with my right hand. My wife, Johanna, can't manage this alone any longer and sometimes calls me to help her. I am never charming when she does.
And the change itself - a task to be approached with all the delicacy of a munitions expert defusing an atomic device in a Bond movie. The unfolding and positioning of a new nappy; the immense, surging relief of finally fastening it up - we made it! The world is safe again! The reinsertion of his legs into the sleeper.
Now we're ready to head downstairs to make the bottle.
Three flights, taking it in the knees, looking out the windows as we go. He's stirring, so I describe the night to him. Tonight, there's no moon and it's damp for November.
In the kitchen, I perform the now-ancient bottle ritual. The weightless plastic bottle (the third shape we tried before we found one that worked, big enough for his not-so-fine motor skills yet light enough to hold), the economy-sized vat of Enfamil (whose bulk alone is discouraging, it implies so much), the tricky one-handed titrating of tiny tablespoonfuls of Pablum and oatmeal (he aspirates thinner fluids). The nightly pang about the fine film of Pablum dust everywhere: Will we ever again have anything like an ordered life? The ebb for having such thoughts in the first place. The rummage in the ever-present blue and white dish drainer (we're always washing something, a pipette or a syringe or a bottle or a medicine measuring cup) for a nipple (but the right nipple, one whose hole I have enlarged into an X, to let the thickened liquid out) and a cap. Pull the nipple into the cap, the satisfying pop as it slips into place. The gonad-shrinking microwave.
Back up three flights. He's still trying to smash his head. Why does he do it? Because he wants to talk, but can't? Because - this is my latest theory - he can't do what he can see other people doing? I'm sure he's aware of his own difference.
Cart him into his older sister Hayley's room. Hayley, meanwhile, is downstairs with her mother, so they can get some sleep. We take turns like this, reduced by the boy to bedroom Bedouins. Neither one of us has slept two full nights in a row in eight years. We both work during the day. After the first few months, I stopped noticing much difference.
Lay him down on the bed. Oh, fuck me dead - forgot the pump! Build a wall of pillows around him so he doesn't escape while I nip back. Remember four (or is it six?) cc's of chloral hydrate, prescribed for sleep and to calm his self-mutilation. (I tried a dose once: the kick of a double martini. William S. Burroughs was thrown out of school for experimenting with it.) Reprogram the pump, his night pulse.
At last: Sink into bed beside him, pull the wriggling boy close. He begins to hit his head again, so I hold down his small right hand with my large right one. This brings his left hand up to his other ear - "he's a genius for finding ways to hurt himself," his teacher told me the other day. I grab his left in my left, which I have threaded behind his head. He kicks himself in the crotch with his right heel, so hard it makes me wince: I run my big leg over his little leg, and lay my right hand (holding his right hand) on his left thigh, to keep it still. He's stronger than he looks. Under his birdy limbs, he's granite to his core. He'll mash his ears to a pulp if no one stops him.
There is a chance none of this will work. Every once in a while, the chloral hydrate rebounds and transforms him into a giggling drunk. When he has a cold (eight, 10 times a year), he wakes on the hour. Sometimes we perform the entire routine again an hour later. Sometimes he cries for hours. There are nights when nothing works, and nights when he is up and at it, laughing and playing and crawling all over me. I don't mind those nights, tired as I am: His sight is poor, but in the dark we're equal, and I know this makes him happy. In the night, there can be stretches when he is no different from any normal lively boy. It makes me almost cry to tell you that.
Tonight is a lucky night: I can feel him slip off after 10 minutes. He stops grunting, strokes his bottle, turns his back and jams his bony little ass into my hip, a sure sign. He falls asleep.
I hurry after him. For all this nightly nightmare - eight years of desperate worry and illness and chronic sleep deprivation, the havoc he has caused in our lives, threatening our marriage and our finances and our sanity - I long for the moment when he lets his crazy formless body fall asleep against me. For a short while, I feel like a regular little boy's father. Sometimes I think this is his gift to me - but parcelled out, to show me how rare and valuable the gift is. Walker, my teacher, my sweet, sweet, lost and broken boy.
More than 100 cases of CFC have been reported since the syndrome was first described publicly in three people in 1979. It was 1986 before it had a name. Some CFC children hit themselves, though most don't. Some can speak and sign. All but a few are anywhere from mildly to severely retarded. Heart defects range from serious to unimportant. Their skin is sensitive to the point of agony. Like many CFC children, Walker can't chew or swallow easily by mouth; his vision and hearing are compromised (he has narrowed optic nerves, one more than the other, and skinny ear canals subject to incessant infection); he's thin and wobbly, "hypotonic" in the medical jargon. Like virtually all CFC children, he has no eyebrows, sparse curly hair, a prominent brow, wide-set eyes, low-set ears and an often charming cocktail-party personality.
At 11, mentally, developmentally - I'm terrified to even write these words - he's somewhere between one and three years old. Physically, he's better off than many CFC children (he doesn't have seizures that we know of, doesn't have ulcerated intestines); cognitively, less so. He could live to middle age. Would that be good luck, or bad?
Minus a few new genetic details, this is the sum total of what the medical profession knows about CFC. The CFC population isn't large and politically powerful like that of Down syndrome. It isn't a widely studied condition such as autism. Most parents of CFC children know more about it than the doctors do. Raising Walker is like raising a question mark.
What irritated Norman Saunders, Walker's pediatrician, was that the hospital didn't call him when my wife delivered five weeks early. Certainly something seemed off that day. The way the boy slumped in the obstetrician's hand. His yellow skin. The lost look on his face. That strange flock of wild, curly hair, piled in a strip on top of his oblong head. An unexpected pattern.
But he was premature; naturally he was lethargic. (No one spots CFC at this stage.) He refused his mother's breast on alternate feeds and one of his testicles hadn't descended and he could only open one eye. Still, by the time he saw Dr. Saunders for his first checkup two days later, the kid had gained 300 grams.
Yet even on that inaugural visit - I know this now from examining Walker's medical records - Dr. Saunders began privately to note odd details. The child's palate was unnaturally high. Flaccid muscle tone. Small papebral fissures, or eye openings; lowered, rotated ears; a fold in the skin on his nasal bridge. Hayley had been a star baby. Dr. Saunders wasn't so enthusiastic about her brother.
Two days later, Walker had lost most of the weight he had gained. Johanna was beside herself, deep in a hormonal trance in which her only concern was to get her child to eat.
He didn't seem to be able to suck. He needed an hour to ingest half an ounce of milk. When he did get it down, he threw it up. His body didn't want to survive. "We do want this child to live, don't we?" Dr. Saunders snapped one morning on yet another visit to his office. I decided it was a rhetorical question.
Walker saw the doctor three more times that month. He was puking like a pro; he never slept. His mother was a ghost. Dr. Saunders was now noting anatomical details every visit: oval, spade-like thumbs, mild blepharophimosis (smallish, down-slanted eyes), orbital hypertelorism (they were widespread too). He always used the scientific terms on the boy's private chart - it made for more accurate communication with other doctors. They were serious words, embodying the professional hope of exactitude. But Walker Brown was a hard child to be exact about. His testicles, on the other hand, were now palpable, a small victory.
"It's still too early to get worried," Dr. Saunders told Johanna. He had a talent for reassuring worried mothers, one of the reasons he was considered one of the best pediatricians in the city. He was just turning 50, trim, well-dressed (he insisted on wearing a tie), and he knew how to make easy conversation. Most of the mothers I knew had secret crushes on him. They tarted themselves up when their kids needed a booster shot.
What his patients didn't know was that Dr. Saunders had a long-standing interest in rare afflictions and their human consequences. His wife, Lynn, had been a special-education teacher. Pediatrics didn't pay as well as most other specialties, but it was hopeful medicine: Most kids he could fix with swift and certain action. The times when he couldn't reached a long way in: Dr. Saunders saw something heroic in those children and in their lives. (Shortly before he died of colon cancer last spring at the age of 60, he inspired the creation of the Norman Saunders Initiative in Complex Care at Toronto's Hospital for Sick Children.)
Heroism was his secret passion - he was obsessed with 18th-century British naval history. With the broken kids, Dr. Saunders, too, became a navigator in unknown waters, an explorer and a watcher.
His watchfulness drove Johanna crazy. She'd arrive home from an appointment and struggle through the door with a baby bag and a stroller and some new device to try to feed the boy, hand him to Olga deVera, our nanny, and say, "I'm so upset with Norm. Normally he knows what he's doing. But with Walker he just looks at him."
What Dr. Saunders was doing was trying to figure out whether the just-offness of the boy's appearance - to say nothing of his limpness and failure to thrive - were signs of a syndrome.
But which one? There were thousands of medical syndromes and at least 6,000 rare diseases. On its own, the extra fraction of space between Walker's eyes suggested any number of them. It was like trying to find a particular plant in a vast garden of exotic flowers, each one more bizarre than the next.
Slowly but steadily, as the fall greyed into winter, Dr. Saunders began to refine a diagnosis - not yet that something was wrong, but that something wasn't right. The child was becoming more alert - his eyes, at least, were now tracking objects, even if his head was a bit laggy. He had started smiling. Good signs, the doctor thought.
But at night at home, he began leafing through textbooks on rare syndromes. He didn't like what he found - specifically, a research paper with pictures of children who looked almost exactly like Walker Brown. The anomaly was new and shockingly rare, a random genetic misfire variously called Shprintzen syndrome, velo-cardio-facial syndrome, and cardio-facio-cutaneous syndrome.
This was in the fall of 1996. Walker had all the signs. The potential consequences were more sobering: learning difficulties, hearing loss, intellectual impairment, language impairment. "Socialization skills may surpass intellectual skills," one researcher noted, rather gracefully. Ten per cent developed psychiatric disorders in the teen years.
That November, Dr. Saunders referred Walker's case to the genetics department at the Hospital for Sick Children. At home, what had begun as a normal concern for a preemie baby had mutated into a 24-hour state of turgid alarm. There was something wrong with our boy.
In February, geneticist Ron Davidson confirmed Dr. Saunders's inkling: Walker had cardio-facio-cutaneous syndrome. He was eight months old. That would qualify as an early CFC diagnosis even today.
"Now that we know what's wrong, we'll know what to put right," my wife said, touchingly. She believed in medicine.
Dr. Davidson was even upbeat. "His developmental milestones are being achieved at a rate that was well within the normal range," he wrote in a confirming letter after meeting Walker. (There were always confirming letters.) True, "the feature of the CFC syndrome that raises most concern is the chance of learning problems," but even here there was a light at the end of the tunnel. "As the number of cases reported has increased, several affected individuals have been reported as having completely normal learning histories and normal intelligence."
The syndrome was not hereditary: The chances of having a second CFC child were microscopic, although Walker had a 50-50 chance of having a CFCer of his own. "However, by then we will know a great deal more about the condition and the mutation that causes it, and there will undoubtedly be a variety of options available to him and his wife." Walker's wife! I have to say I never believed it.
His infant head was overlarge and shaped like an olive, but the rest of him was as light as a loaf of bread: I could carry him in one hand. I called him Boogle, or Beagle, or Mr. B, or Lagalaga (because he made that noise), or simply Bah! (He liked B sounds.) Later, as he grew older, we developed a private language of tongue clicks that only he and I speak: All we ever seem to say is, "Hello, it's me, I'm clicking to you, and only to you, because only you and I speak Click;" to which he (or I) reply, I think, "Yes, hi, I see you there, and I am clicking back, I like it that we speak our private language, in fact I find it hilarious." This is very enjoyable for both of us.
I could clap my hands and he would clap back; he especially liked it when I clapped his hands faster than he ever could on his own. He hated having his face touched, but loved his bath: The water seemed to ease his movements, float his knobby joints. It was impossible to take a decent photograph of him, except by chance, and then he looked like Frank Sinatra Jr. on a tear. He smelled warm, baked: His head to this day has the tasty whiff of a Zagnut bar. He never crawled, but began to walk at 2 ½.
The house was a well-organized nightmare. You couldn't survive as the parent of a handicapped child if you weren't organized, and my wife was. There were laundry baskets of toys on every floor; plastic contraptions hanging off the backs of chairs in the kitchen and the living room; tubs of syringes and feeding lines upstairs and down; caches of diapers in a chest by the front door; troops of medicine bottles and ointment tubes; vomit stains on everything.
He loved to touch things. The bottom three slats of every window blind in the house were mangled. His most developed consciousness seemed to live in his hands, in what he could manipulate - the genius light switch, the fascinating toilet paper tube, anything that beeped or flickered. What he could touch he knew.
The best part was the way he exploded with laughter and rocked into a ball of glee at some mysterious thing, which passersby loved. (For a while, I suspected he was rubbing his penis between his thighs, a traditional source of merriment for all boys.) As he grew older, he became slyer. He loved to clear tables and flat surfaces, especially closely guarded ones: He would distract you, wipe the deck and throw his head back with pleasure, momentarily cleverer than anyone else.
He became a great wanderer, a lucky one. Once, when he was 5 (on his beefy days, he looked 3), I left him at the foot of some stairs in a friend's elegant house, while we ate dinner. I knew he couldn't climb stairs and I knew he couldn't open a door. Ten minutes later, I heard a tinkling sound. He had climbed the stairs, opened the door and was gleefully and deliberately smashing the last of seven wineglasses on their Noguchi coffee table. Not a scratch on him. We called that Kristallnacht.
He loved women, the prettier the better. He would raise his arms to be picked up - he couldn't sit up on his own until he was nearly a year old - or, later, climb into a woman's lap and immediately peer down her neckline. Then he would feel her up. I thought it was accidental, but Johanna's friends remarked how intentional it seemed. He loved anything shiny, fingering it close to his wonky eyes. Our friends called him "the jeweller."
Our close friends, that is. To others, at least for the first few years, I never mentioned Walker's difficulties. I wasn't ashamed of him. But I didn't want sympathy and I didn't want him to feel he needed it, either.
He stayed in my mind. Not only darkly, or as worry, but as a mental talisman. So did my daughter, of course. But I was always catching up to Hayley, whereas Walker moved slowly and could be tracked from standing. His aura, the fact of his existence, turned up everywhere, unexpectedly: in the lyrics of a Neil Young song at the gym, between the lines of a Norman Mailer essay read during a bout of insomnia.
He loved the outdoors. He spent hours with Olga, touring the neighbourhood in his stroller. He had a vast circle of acquaintances. Strangers approach me to this day and say, "You're Walker's dad." It makes me feel his brilliance. He was well-dressed too. Olga bought him the latest gear at the Gap for his birthdays and I occasionally snuck out on my own to pick up something. I can't describe the pleasure it gave me to buy his first big boy's shirt - he looked so cool and sharp. I plan to take him, with my father and brother, to buy his first tie. I know it's futile: the bib he wears to catch his drool will cover it. But that might be the only male ritual we pass among the four of us.
By the time Walker was 3, his medical chart was 10 pages long.
A pattern of afflictions emerged early: bad chest, pneumonia, constipation, endless ear aches, scaling skin. He didn't sleep. We thought of him as affable, but he cried half the time.
At least at the doctor's office you could ask questions. Returning home again was like entering a long hallway where the lights wouldn't go on. My wife said she felt "as if a soundproof curtain has dropped over us." Certainly any crisis with any child brings that curtain down: Your focus shortens, you aim straight ahead. The difference with Walker was that the curtain was permanent. Before Walker, the future had looked like a series of discrete challenges, each to be overcome with (possibly callow) glory. After Walker was born, the future was unchanging, sad, full of obligation, until we died, which only raised the gloomy prospect of what would happen to him then.
Early on - this too is common in the families of CFC children - we agreed Hayley shouldn't be held responsible for Walker as an adult. But she alone took him in her stride. One day, I asked her why she thought Walker couldn't walk and talk at the age of 2. "I could walk when I was 1, because I was born with two eyes open," she said. "But Walker was born with only one eye open." She was 4.
The diagnosis was something to go on, but the knowledge didn't improve Walker's health. Dr. Saunders's notes became repetitive: congested and coughing and otitis andfailure to thrive began to appear in every entry . At 18 months Walker spoke and understood no solid words, couldn't walk, had no gestures except "up" and the occasional smile. Saunders wrote DEVELOPMENTAL DELAY in capital letters on his chart. He ordered the installation of a G-tube - there wasn't time in the day to wait for Walker to take in the trickle of food he could swallow. Until he grew stronger, he wouldn't be able to eat; because he couldn't eat, he couldn't grow stronger.
The G-tube made it easier to administer the growing list of medicines Walker needed for his reflux and his ear infections and his sleeplessness and his jitters and his rashes: gentian violet, hydrocortisone, amoxicillin, azithromycin, clarithromycin, erythromycin (ever wider on the spectrum of antibiotic strength), cisipride, Keflex, Betnovate, flamazine, lactulose, colace, chloral hydrate. ... They sounded like the names of ambassadors to an intergalactic conference of aliens. His already-chronic constipation (his muscles were too weak to move things along normally), made worse by the equally necessary chloral hydrate, often required not one but three drugs - lactulose as a sugary starter, colace as the dynamite, and suppositories, the blasting cap itself. You had five minutes to take cover.
Nothing was ordinary. Like most kids, he had diaper rash - but because this was Walker, my compromised son, it was the Chernobyl of diaper blights, requiring a day in hospital. There was so much wax in his ears that we could have started a museum. For a period of 10 months, he developed agonizing blisters on his feet that stymied his already stymied walking. They were three inches across, yellow, and showed up whether he was wearing socks or no socks, shoes or no shoes. They disappeared as quickly as they came. The doctors never did figure out why.
The diagnosis of CFC meant more appointments: the ear specialist, the eye doctor, the dermatologist, the gastro-reflux expert, the neurologist, a foot doctor, occupational and behavioural and oral therapists, the geneticist, the cardiologist, the feeding and sleep clinics, even the drooling clinic (their conclusion: "Mrs. Brown, your son drools"). The dentist needed full anesthesia to clean Walker's teeth.
Oral therapy was important if he was going to learn to speak, but two years yielded nothing. We switched to sign, but his fine motor skills were too coarse, the therapists said, and his head banging didn't help. Better to concentrate of his gross motor abilities, the experts told us. The eye doctor couldn't get an accurate reading on what his eyes needed, and Walker couldn't say. Ditto his hearing. All of this made me feel I had failed him. Added to the 11 times he was at Dr. Saunders' office in 1998 alone, along with trips to emergency, Walker logged a medical visit a week. And that was if he was more or less healthy.
To teach him to walk, we undertook a costly and radical Venezuelan therapy three times a week for two years. The Medek method entailed hanging him upside down and pulling his legs into unnatural positions. He started screaming the moment we pulled into the driveway, but he learned to walk. At least he had that. He could be what his name said he was. Maybe that was why we insisted.
The strange thing was that all this darkness could be relieved by a few pinpoints of light. A reaction alone was notable; a smile or one of his glee sprees charmed my afternoon.
I remember how proud I was the first day he went to school. Play and Learn was a daycare program that integrated normal and disabled children. You could spot the parents of the delayed: They were the ones who looked as if a bomb had just gone off in the back seat of the car. They were starved for contact and longing to tell the truth. One afternoon, I ran into a woman whose severely disabled 14-year-old daughter had died two years earlier. "Do you know the first thing I did - on the way back from the funeral?" she said. "I said to my husband, 'Pull over. Let's have sex.' " She later divorced him.
Walker enjoys exploring objects by manipulating them. He turns objects through his fingers as he looks at them, and has also begun to bang objects together.
Play and Learn's theory was that integrating normals and the disabled would sensitize the normals and inspire the delayed. The school boasted a full-time sensory integration therapist (CFC children are often overwhelmed by their senses and have to be conditioned to them) and an occupational therapist, to teach basic concepts such as sociability - sitting down with others at lunch. To my surprise, Walker became bolder, more outgoing.
The staff (all women) were dedicated teachers of the handicapped, geniuses of the heart who saw hope in everything.
Typically, he produces open vowels and consonant-vowel combinations - which may include any of the sounds [b, n, d, n, l, y]with an "ah." Although he will not initiate an interaction, he enjoys having his peers around. When a peer is holding his hand, he seems to be content.
The provincial government in Ontario, keen to show it was serious about education, insisted that all children be graded. Grades meant standards. The first time Walker arrived home with a report card in his backpack, we learned he was improving at math. Math! And improving! We laughed like hell, and then we kissed him and said, "Well done, Walker! Two plus two is four!" We did that for a long time, held on to it like a rare, delicious treat. Not that we believed Walker could do math as we defined it. But it was something funny about our boy that anyone could appreciate, a detail of his life that made it out from under the soundproof curtain.
What I couldn't tell was what the routines meant to him. Did he know he was "painting" when the teacher was guiding his hands? He had a friend, Jeremy, but did he know what a friend was? He sat at the table with the other kids for Snack - a time named Snack, I loved that - but did he feel the communal buzz? What went on inside that thickened skin, behind that swollen heart? I didn't care if he never threw a ball or tormented his sister or skied beside me or told a joke or dated a girl (though I would love it if he did). What I cared about was whether he had a sense of himself, an inner life. Sometimes it seems like the most urgent question of all.
Throughout these years, on half-sleep, my wife and I fight a lot. It's mostly the same argument. It goes like this: In the middle of the night, though it's Johanna's turn with Walker - and it could easily be the other way around - I can't sleep and head downstairs to read. Five minutes in, I hear Johanna: "No, Walker, no!" A minute later, she appears in the doorway of the living room - naked, brown (even in January), exhausted. Walker has been up for three hours and has just head-butted her and erupted with laughter. "Can you take him?"
I sigh - a mistake - and - another mistake - say, "I had him last night for three hours straight in the middle of the night."
"Forget it." She stalks off.
I follow her upstairs, recanting.
"Never mind! Sorry I asked!"
But I get to Walker's room first and lie down with him. By now my poor wife is so tired she refuses to let go. She shouts, I shout, I close the door. She comes in again, so I close it again and bar it with my foot. I'm not exactly rational. When I open the door, I can hear Hayley asking what's happening from the bottom of the stairs. I begin to apologize profusely. It's not entirely sincere, but sometimes in these irrational, terrified battles - will I ever have a normal life again? - it does the trick.
But there are other times too - moments of unstoppable happiness. Reading the paper next to a lake on a deck chair with Walker in my lap, laughing delightedly as I swear at the news. The four of us in bed together on a Saturday morning, Walker on his knees, towering for once over us all, and never happier. This is something, you see: Every time he is happy, he is as happy as he has ever been. Hayley, a delicate and skilled ballet dancer, twisting with Walker to music on the stereo, Walker on the moon with happiness. Twenty minutes from his life. Everyday occurrences for a normal child. But I know their true value.
Shortly before Walker turns 2, we hear about a CFC study being conducted at the famous Children's Hospital of Philadelphia. We drive 10 hours. It isn't until the end of a day of examinations that we meet a doctor who tells us something we don't know. His name is Paul Wang, a developmental pediatrician.
Dr. Wang conducts a series of tests. He shows Walker some line drawings, a light, a puzzle; Walker throws them on the floor. After an hour, the doctor is done. Walker wanders over and climbs into my lap.
"As you know," says Dr. Wang, a slim man with a high forehead and a quiet voice, "there are three general levels of cognitive delay, or retardation - mild, moderate and severe, sometimes called profound."
"Which one is Walker?" Johanna asks.
"If Walker continues at his present rate of development, then he might be diagnosed with moderate mental retardation as an adult."
"Moderate?" Johanna says, and puts her hand to her mouth. She is already crying. (I hope I held her hand.) "I was hoping for mild. Will he ever be able to read? Or drive a car?"
"I doubt it." This is bad news. Moderate retardation is still catastrophic, and there is nothing to say it won't get worse as he gets older. He will need lifelong supervision, support in his living arrangements. "At this point, little definitive information is known about children with the CFC syndrome." Dr. Wang judges Walker's overall development to be at the 10-month level. Ten months. Less than half his age. "As he gets older, of course, the differences will become more noticeable."
Dr. Wang turns to me. "Do you have any questions?"
"Just one. For the past few summers, we've rented a cottage in Georgian Bay, a couple of hours north of Toronto. It's a very remote place, very quiet. An island, no one around but us. Walker seems to love it. It changes him, calms him. It means a lot to me, that place, and how it changes him. Will I ever be able to explain all that to him?"
Dr. Wang shakes his head. "Not rationally, probably not. But" - he stops, thinks - "it sounds like he already understands it." Another pause. "The Buddhists say the way to enlightenment, to pure being, is by getting your mind out of the way. I'm not trying to be trite, but Walker already knows how to do that. He is pure being. He may be developmentally delayed, or moderately retarded, but in that way, he's already miles ahead of most of us."
This is the first time someone suggests Walker may have a gift the rest of us don't.
And right then, as he turned 2, Walker began to grab his ears and bite himself. He didn't stop for a year and a half. We thought he had a toothache, an ear ache. He did not.
Self-mutilating appears for the first time in his medical chart in March of 1999, shortly before his third birthday. He quickly graduated to punching himself in the head. He put his body behind the punches, the way a good boxer does. Hayley called it "bonking," so we did too.
The irony was that he had been making progress, of sorts: finer pincer movements with his fingers, a little eating. He could track objects and wave goodbye and often babbled like a madman.
Then he flipped into blackness. Was it self-hatred? I wondered about that. We enrolled him in the famous rehabilitation clinic at the Bloorview MacMillan Children's Centre in north Toronto, where he was seen by a behavioural therapist. Everywhere else people saw his bruises and wondered what we were doing to our child.
Cannot communicate, Dr. Saunders noted.
Sometimes he was in agony as he smacked himself and screamed with pain. At others he seemed to do it more expressively, a way to let us know he would be saying something if he could talk. Sometimes - this was unbearably sad - he laughed immediately afterward. He couldn't tell us anything, and we imagined everything.
More specialists crowded into our lives. Walker was diagnosed as being functionally autistic as well as having CFC. Dr. Saunders tried Prozac, Celexa, risperidone (an antipsychotic designed originally for schizophrenia, it has been known to allay obsessive-compulsive behaviour in younger children). Nothing worked. Once, in Pennsylvania, he bit his hand to the bone and, after an hour of surgery, spent a night in hospital. The bill was $14,000.
Dr. Saunders's notes began to track longer and longer stretches of horror. "B onking" ears x 2-3 days. I remember that stretch, especially the grief-stricken look on Walker's face one morning as he bashed himself. He looked straight at me. He knew it was bad and wrong, he knew he was hurting himself, wanted to stop it and couldn't - why couldn't I? His normally thin gruel of a wail became frightening and loud. From June of 2001 to the spring of 2003, every entry in his medical records mentions his unhappiness, his irritability. He began to regress, to lose sounds and gestures.
Did he know his window for learning was closing? Was his vision dimming? 72 hours aggressive behaviour. Unhappy crying x 5 days. Even Dr. Saunders's handwriting became loose and scrawled, distracted by the chaos of those shrieking visits. Screaming all day, needs to be held.
I dreaded his waiting room, with its well-dressed mothers and well-behaved children. They were never anything but kind. But walking in with Walker yowling and banging his head, I felt like I'd barged into a church as a naked one-man band, with a Roman candle up my ass and singing Yes, We Have No Bananas for good measure.
Mother tearful, Dr. Saunders noted on the 29th of December of that awful year. Urgent admission for respite.
I remember that day too. We drove Walker home from the doctor, fed Walker, bathed Walker, soothed Walker, put Walker to bed. I heard his cries subside in stages. Normally Johanna was relieved when he dropped off to sleep, but tonight she came downstairs from his bedroom crying, her arms wrapped around herself.
"He's gone away," she said. "My little boy has gone. Where has he gone?" She was inconsolable.
So perhaps you can understand why, the very next morning, I began to look for a way out. I didn't tell Johanna, but I had to find a place for Walker to live, somewhere outside our home. I didn't realize it would take seven years, that it would be the most painful thing I had ever done and that the pain would never go away.
On my desk at work is a picture of Hayley reading to Walker. This was up north, on the quiet island. They are lying side by side on a bed, and Walker is looking up at the book in Hayley's hands, as if riveted by every word. I'll never know if he understood a syllable. But he can hear her voice, is thrilled to be with her and clearly grasps his smart big sister's affection. He has become the moment and it has become him, because he has nothing else to be. Walker is an experiment in human life lived in the rare atmosphere of the continuous present. Very few can survive there.
The photograph reminds me of a poem I once read in a magazine, by Mary Jo Salter:
None of us remembers these, the days
When passing strangers adored us at first sight
Just for living, or for rolling down the street.
Praised all our given names, begged us to smile.
You, too, in a little while, my darling,
Will have lost all this,
asked for a kiss will give one,
And learn how love dooms one to earn love
Once we can speak of it.
My boy Walker has no worries there. He never asks, but is loved by many. But I doubt it feels effortless to him.
"I hear parents of other handicapped kids saying all the time, 'I wouldn't change my child,' " Johanna said one night. We were lying in bed, talking as we fell asleep. "They say, 'I wouldn't trade him for anything.' But I would. I would trade Walker, if I could push a button, for the most ordinary kid who got Cs in school. I would trade him in an instant. I wouldn't trade him for my sake, for our sake. But I would trade for his sake. I think Walker has a very, very hard life."
LONG ODDS - BUT THEY ADD UP
Ian Brown
The good news is that CFC is rarer than many other single-gene disorders. But the risk of an unfavourable outcome in any pregnancy is far higher than most people realize. The risk of a spontaneous abortion, or miscarriage, for instance, is 1 in 8; the chance of a couple being infertile is 1 in 10. Some more odds:
Incidence of Diabetes mellitus (type 2, the most common, a multi-cause genetic disorder) 1 in 10 to 1 in 20 adults
Risk of a live-born person under 25 having a genetic disorder 1 in 20
Risk of some congenital abnormality at birth 1 in 30
Risk of a severe physical or mental handicap 1 in 50
Risk of stillbirth (North America) 1 in 125
Cleft lip 1 in 250 to 1 in 600
Down syndrome 1 in 800 live births in Canada alone; 350,000 people have this condition in the U.S.
Single-cell disorders
The chance of a disorder caused by a single gene is smaller, but still not as small as one might like. Some examples, and their incidence:
Sickle cell anemia First molecular disease to be identified, causing fatigue, delayed develop-
ment, pulmonary hypertension, stroke and heart failure; 1 in 400 to 1 in 625 persons at birth among African Americans
Familial hypercholesterolemia Coronary heart disease by middle age; in men who have inherited the trait from both parents, a 50-per-cent risk of heart attack by the age of 50; 1 in 500 males
Cystic fibrosis Chronic pulmonary disease, death by late 30s, most common autosomal recessive disease in Caucasian children; 1 in 2,000
Neurofibromatosis type 1 The most common single-gene disorder, causing changes in skin colouring, tumours along nerves, loss of vision, leukemia, scoliosis, ADHD; 1 in 3,000 to 1 in 4,000 worldwide (though very rare in Asians)
Noonan Syndrome Facial deformity, short stature, language and speech difficulties, heart defects, eye abnormalities, occasional mild intellectual delay; 1 in 1,000 to 1 in 2,500
Huntington disease Neurodegenerative disease with progressive dementia, with onset typically in the fifth decade; 4 to 8 in 100,000
CFC syndrome, Costello Syndrome Facial deformity, heart defects, mild-to-severe mental retardation; risk of cancer in Costello syndrome, but not in CFC; CFC incidence is 300 people worldwide, or 1 in 1.24 million (fewer than 10 births a year, worldwide)
Proteus syndrome Excess growth of tissue in body, asymmetrical growth, benign tumours; John Merrick, the famous "elephant man," probably had Proteus syndrome; 100 to 200 people worldwide
CIPA (Congenital insensitivity to pain with anhidrosis) Inability to sweat, fever, inability or reduced ability to feel pain; 100 known cases, worldwide
Ian Brown with Marjan Farahbaksh, Globe and Mail Editorial Research Library
Sources: Genetics in Medicine, W.B. Saunders Co., Philadelphia, 1991; National Down Syndrome Society; U.S. National Library of Medicine; Costello Syndrome Support group.
WHAT YOU CAN DO
People with rare "orphan" syndromes and other complex conditions often challenge medicine's organizational limits and procedures. For this reason, supporters of Walker Brown's long-time pediatrician (who died in March of colon cancer) came together in 2006 to create the Dr. Norman Saunders Initiative in Complex Care at the Hospital for Sick Children in Toronto, with the aim of developing comprehensive care for such children.
To see how you can help, contact Arlene Manankil at the Sick Kids Foundation at 416-813-8742 or by e-mail at .
NEXT IN THE SERIES
Saturday, Dec. 8, Part 2:
Finding Walker's Place
Ian Brown crosses the continent to meet other families with CFC kids for the first time, as he and his wife seek out more help with Walker's care.
Saturday, Dec 15, Part 3:
Walker, My Teacher
What do children like Walker have to teach us, both scientifically and as human beings - and what does society owe them in return?
Ian Brown is a Globe and Mail feature writer.