other organs, until he was riddled with it. In the struggle to survive, he had lost much of his thick white hair.
On the cardiac monitor, the green line began to spike and trough erratically. I watched it with dread.
Dad’s hand closed weakly on mine.
When I looked at him again, his sapphire-blue eyes were open and focused on me, as riveting as ever.
“Water?” I asked, because he was always thirsty lately, parched.
“No, I’m all right,” he replied, although he sounded dry. His voice was barely louder than a whisper.
I could think of nothing to say.
All my life, our house was filled with conversation. My dad and mom and I talked about novels, old movies, the follies of politicians, poetry, music, history, science, religion, art, and about owls and deer mice and raccoons and bats and fiddler crabs and other creatures that shared the night with me. Our discourse ranged from serious colloquies about the human condition to frothy gossip about neighbors. In the Snow family, no program of physical exercise, regardless of how strenuous, was considered to be adequate if it didn’t include a daily workout of the tongue.
Yet now, when I most desperately needed to open my heart to my father, I was speechless.
He smiled as if he understood my plight and appreciated the irony of it.
Then his smile faded. His drawn and sallow face grew even more gaunt. He was worn so thin, in fact, that when a draft guttered the candle flames, his face appeared to be hardly more substantial than a reflection floating on the surface of a pond.
As the flickery light stabilized, I thought that Dad seemed to be in agony, but when he spoke, his voice revealed sorrow and regret rather than pain: “I’m sorry, Chris. So damn sorry.”
“You’ve nothing to be sorry about,” I assured him, wondering if he was lucid or speaking through a haze of fever and drugs.
“Sorry about the inheritance, son.”
“I’ll be okay. I can take care of myself.”
“Not money. There’ll be enough of that,” he said, his whispery voice fading further. His words slipped from his pale lips almost as silently as the liquid of an egg from a cracked shell. “The other inheritance…from your mother and me. The XP.”
“Dad, no. You couldn’t have known.”
His eyes closed again. Words as thin and transparent as raw egg white: “I’m so sorry….”
“You gave me life,” I said.
His hand had gone limp in mine.
For an instant I thought that he was dead. My heart fell stone-through-water in my chest.
But the beat traced in green light by the electrocardiograph showed that he had merely lost consciousness again.
“Dad, you gave me life,” I repeated, distraught that he couldn’t hear me.
My dad and mom had each unknowingly carried a recessive gene that appears in only one in two hundred thousand people. The odds against two such people meeting, falling in love, and having children are millions to one. Even then, both must pass the gene to their offspring for calamity to strike, and there is only one chance in four that they will do so.
With me, my folks hit the jackpot. I have xeroderma pigmentosum — XP for short — a rare and frequently fatal genetic disorder.
XP victims are acutely vulnerable to cancers of the skin and eyes. Even brief exposure to sun — indeed, to any ultraviolet rays, including those from incandescent and fluorescent lights — could be disastrous for me.
All human beings incur sunlight damage to the DNA — the genetic material — in their cells, inviting melanoma and other malignancies. Healthy people possess a natural repair system: enzymes that strip out the damaged segments of the nucleotide strands and replace them with undamaged DNA.
In those with XP, however, the enzymes don’t function; the repair is not made. Ultraviolet-induced cancers develop easily, quickly — and metastasize unchecked.
The United States, with a population exceeding two hundred and seventy million, is home to more than eighty thousand dwarfs. Ninety thousand of our countrymen stand over seven feet tall. Our nation boasts four million millionaires, and ten thousand more will achieve that happy status during the current year. In any twelve months, perhaps a thousand of our citizens will be struck by lightning.
Fewer than a thousand Americans have XP, and fewer than a hundred are born with it each year.
The number is small in part because the affliction is so rare. The size of this XP population is also limited by the fact that many of us do not live long.
Most physicians familiar with xeroderma pigmentosum would have expected me to die in childhood. Few would have bet that I could survive adolescence. None would have risked serious money on the proposition that I would still be thriving at twenty-eight.
A handful of XPers (my word for us) are older than I am, a few significantly older, though most if not all of them have suffered progressive neurological problems associated with their disorder. Tremors of the head or the hands. Hearing loss. Slurred speech. Even mental impairment.
Except for my need to guard against the light, I am as normal and whole as anyone. I am not an albino. My eyes have color. My skin is pigmented. Although certainly I am far paler than a California beach boy, I’m not ghost-white. In the candlelit rooms and the night world that I inhabit, I can even appear, curiously, to have a dusky complexion.
Every day that I remain in my current condition is a precious gift, and I believe that I use my time as well and as fully as it can be used. I relish life. I find delight where anyone would expect it — but also where few would think to look.
In 23 B.C., the poet Horace said, “Seize the day, put no trust in the morrow!”
I seize the
Most of my friends say that I am the happiest person they know. Happiness was mine to choose or reject, and I embraced it.
Without my particular parents, however, I might not have been granted this choice. My mother and father radically altered their lives to shield me aggressively from damaging light, and until I was old enough to understand my predicament, they were required to be relentlessly, exhaustingly vigilant. Their selfless diligence contributed incalculably to my survival. Furthermore, they gave me the love — and the love of life — that made it impossible for me to choose depression, despair, and a reclusive existence.
My mother died suddenly. Although I know that she understood the profound depth of my feeling for her, I wish that I had been able to express it to her adequately on that last day of her life.
Sometimes, out in the night, on the dark beach, when the sky is clear and the vault of stars makes me feel simultaneously mortal and invincible, when the wind is still and even the sea is hushed as it breaks upon the shore, I tell my mother what she meant to me. But I don’t know that she hears.
Now my father — still with me, if only tenuously — did not hear me when I said, “You gave me life.” And I was afraid that he would take his leave before I could tell him all the things that I’d been given no last chance to tell my mother.
His hand remained cool and limp. I held it anyway, as if to anchor him to this world until I could say good- bye properly.
At the edges of the venetian blinds, the window frames and casings smoldered from orange to fiery red as the sun met the sea.
There is only one circumstance under which I will ever view a sunset directly. If I should develop cancer of the eyes, then before I succumb to it or go blind, I will one late afternoon go down to the sea and stand facing those distant Asian empires where I will never walk. On the brink of dusk, I’ll remove my sunglasses and watch the dying of the light.
I’ll have to squint. Bright light pains my eyes. Its effect is so total and swift that I can virtually feel the developing burn.
As the blood-red light at the periphery of the blinds deepened to purple, my father’s hand tightened on mine.
