Page 334 - A Little Life: A Novel

plank, sliding off the chair and beneath the table, his eyeballs rolling
upward, his throat making a strange, hollow clicking noise. It lasted only
ten seconds or so, but it was awful, so awful I can still hear that horrible

clicking noise, still see the horrible stillness of his head, his legs marching
back and forth in the air.
My father ran and called a friend of his at New York Presbyterian and we
rushed there, and Jacob was admitted, and the four of us stayed in his room
overnight—my father and Adele lying on their coats on the floor, Liesl and
I sitting on either side of the bed, unable to look at each other.
Once he had stabilized, we went home, where Liesl had called Jacob’s

pediatrician, another med-school classmate of hers, to make appointments
with the best neurologist, the best geneticist, the best immunologist—we
didn’t know what it was, but whatever it was, she wanted to make sure
Jacob had the best. And then began the months of going from one doctor to
the next, of having Jacob’s blood drawn and brain scanned and reflexes
tested and eyes peered into and hearing examined. The whole process was

so invasive, so frustrating—I had never known there were so many ways to
say “I don’t know” until I met these doctors—and at times I would think of
how difficult, how impossible it must be for parents who didn’t have the
connections we did, who didn’t have Liesl’s scientific literacy and
knowledge. But that literacy didn’t make it easier to see Jacob cry when he
was pricked with needles, so many times that one vein, the one in his left
arm, began to collapse, and all those connections didn’t prevent him from

getting sicker and sicker, from seizing more and more, and he would shake
and froth, and emit a growl, something primal and frightening and far too
low-pitched for a four-year-old, as his head knocked from side to side and
his hands gnarled themselves.
By the time we had our diagnosis—an extremely rare neurodegenerative
disease called Nishihara syndrome, one so rare that it wasn’t even included

on batteries of genetic tests—he was almost blind. That was February. By
June, when he turned five, he rarely spoke. By August, we didn’t think he
could hear any longer.
He seized more and more. We tried one drug after the next; we tried them
in combinations. Liesl had a friend who was a neurologist who told us
about a new drug that hadn’t been approved in the States yet but was
available in Canada; that Friday, Liesl and Sally drove up to Montreal and

back, all in twelve hours. For a while the drug worked, although it gave him