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 xerodernia 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 night and ride it as though it were a great black
stallion.
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 tenuouslydid 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 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