Hebron, Louisiana
Saturday, August 28, 8:55
A.M.
Time Remaining on
Extinction Clock: 99 hours, 5 minutes
Rabbi Scheiner was an old man, but he had
bright green eyes and a face well used to smiling. However, as he
walked beside his nephew, Dr. David Meyer, the rabbi’s mouth was
pulled into a tight line and his eyes were dark with
concern.
“How sure are you about this, David?” the
rabbi asked, pitching his voice low enough so that the nurses and
patients in the ward could not overhear.
David Meyer shook the sheaf of papers in his
hand. “We ran every test we could, and the lab in Baton Rouge
confirmed our findings.”
“It’s unfortunate, David. but it does
happen. You know more than I do that there’s no cure for this, and
that the best we can do is screen young people and counsel them
before marriage. Warn them of the risks.”
“That’s the point, Rabbi,” insisted Meyer.
“We did those screens. We have a very high concentration of
Ashkenazi Jews here, most of them from families that fled the Rhine
as things were going bad in the late nineteen thirties. Virtually
everyone in Hebron, Tefka, and Muellersville has been screened-we
still get grants from Israel to run the polymerase chain reaction
techniques, and they’re very accurate. We know the carriers, and we
have counseled them. If these occurrences were within the group of
known carriers, then I wouldn’t have called you.”
“Then I don’t understand. Haaretz reported
that the disease was virtually eradicated. You yourself told me
that there had been no babies born to Jewish families here in
America with the disease since 2003.”
Meyer took the rabbi by the arm and led him
into a small alcove.
“I know; I know,” said Meyer. His face was
bright with stress, and sweat beaded his forehead. “However, in the
last month clinics throughout the area have been reporting many
cases of patients presenting with classic symptoms: slurred speech,
difficulty swallowing, unsteadiness of gait, spasticity, sharp and
sudden cognitive declines, and a variety of psychiatric illnesses
that include psychosis typical of schizophrenia. Individually any
one or two of those symptoms in an adult would not suggest LOTS,
but when five or six symptoms present in virtually every patient.
then what else could I think? I sent nurses out to take samples for
genetic testing and we ran our own enzyme assay tests, but they’re
not as precise at genetic testing as PCR tests, so I had the
samples shipped to a lab in Baton Rouge.” He shook the sheaf of
papers. “These are the results.”
Rabbi Scheiner reluctantly took the papers
from Meyer and quickly read through them. In the comment notes he
read: “Late Onset Tay-Sachs (LOTS) disease is a rare form of the
disorder, typically occurring in patients in their twenties and
early thirties. This disease is frequently misdiagnosed and usually
nonfatal.”
He looked up.
“So you have several patients who have
become sick?”
Meyer shook his head slowly. “Rabbi. I’ve
had eleven patients here in Hebron, and there were nine in Tefka
and six in Muellersville.”
The rabbi caught the phrasing. “You say you
‘had’ eleven patients.. ”
Meyer gave him a bleak stare. “Three have
already died. Two more are. well, they have lapsed into comas. The
others are getting sicker almost as I watch. The muscles needed to
swallow become atrophic and paralyzed. We’ve intubated them, and
I’ve even trached a few, but the paralysis spreads so fast. I don’t
know how to treat any of them.”
“There’s no cure.. ” The rabbi said it as a
statement. “God help us.”
“Researchers have been looking into gene
therapy and other treatments, but even if we had a genetic option
in hand, these people don’t have the time for it.”
“These are all children?”
Meyer shook his head. “No, and that’s what
scares me the most. Infantile and Juvenile TSD are both fatal, but
not LOTS. And yet every one of these patients is over twenty. Some
are in their forties and fifties. It doesn’t make
sense.”
“Could. could the disease have
mutated?”
“It apparently has,” said Meyer, “but how?
It was nearly eradicated. We’d beaten it. We’ve never had a single
case here in Hebron, or in the other towns, and most of the people
here are second- and even third-generation American born. We
haven’t married strictly within the communities of Ashkenazi Jews,
which means statistics should be on our side.”
Rabbi Scheiner put his hand on the young
doctor’s arm. “Be strong, David. Tell me. what will you
do?”
“I’ll have to report this. Now that I have
the results from the genetic tests I can reach out to the major
university hospitals.”
“What about the disease people?” asked the
rabbi. “What about the Centers for Disease Control up in Atlanta?
You went to them with the botulism problem a few years
ago-”
“No,” said Meyer, “this is a genetic
mutation, not a pathogen. It’s not contagious in any way that could
cause an epidemic.”
Rabbi Scheiner’s eyes were intense, probing.
“Are you sure?”
“Of course,” said Meyer. “It’s an inherited
disorder. You can’t just catch it.”
The rabbi nodded and turned to look out of
the alcove at the patients in the ward. “Are you sure?” he asked
again.