Clos

What Is
Canavans?

Canavans, first recognized about
60 years ago, belongs in the cate-
gory of leukodystrophy diseases,
which result from the destruction
of the normal coating, or myelin,
of the body's nerve cells.
This rare ailment is a genetic
disease (it is not contagious) that
primarily affects, but is not limit-
ed to, Ashkenazi Jews.
There are three types of
Canavans.
The first is the neonatal form,
which comes at birth and res t
in death in the first several wee
of life. It is characterized by irri
tability, lethargy and difficulty in
swallowing and feeding**,..
With the infantile form the
disease appears in the first few
months of life, generally resulting
in death by the time the child is - 3 -
years old. Symptoms are poor psy-
chomotor development, blindness
and seizures.
The third form of Canavans is
the juvenile form, which appears
after the child is 5 and usually
results in death by adolescence.
While there are medicines
control certain aspects of the
ease,there is no cure.
There is, however, a simple
blood test to see whether you
carry the Canavans gene. For the
child to contract the disease, both
parents must have the gene, and
then there remains a 507,-
percent chance that the
child will not be affected.
The most common
genetic disorder among
Jews is Gaucher disease,
a blood and bone disor-
der which affects 1 in 10
Ashkenazi Jews.
The gene for Tay-
Sachs, a fatal neourde-
generative disease, affects
1 in 27 Ashkenazi Jews.
About one in 40
Ashkenazi Jews carry the
gene for Canavans.
For more information,
contact the United
Leukodystrophy
Foundation, 2304
Highland Drive,
Sycamore, Ill. 60178,
(815) 895-3211.

Nurse Simmons says Chaya Leah's
knowledge of Hershy's condition, her
understanding of what he needs, and
her care of her son make her "excep-
tional."
Chaya Leah, for her part, couldn't
make it without the nurses. They help
dress and bathe Hershy, work on his
visual and auditory skills, and accom-
pany him to Wing Lake School where,
Nurse Simmons says, he loves wheel-
chair square dancing, the hydrotub,
and arts and crafts.
Hershy can't go out much these
days because it's too cold. But in the
spring and on mild summer days he
and his nurse often take a walk.
Securing money for nurses' care
and Hershy's many medical needs has
been a challenge of its own. Insurance
pays for part of it, but there are a
number of items, like Hershy's formu-
la, that the family must pay out of
pocket. The problem is that Hershy
has allergies and can only take soy for-
mula, Chaya Leah explains. Insurance
will pay for some formulas, but this
one isn't on the list.
. Soon after she arrives, Nurse
Simmons puts on a tape of happy
children's songs, like "Good Day,
Sunshine" and "Feelin' Groovy." She
finishes dressing Hershy in a white
shirt and soft-blue pants, hoists him
into his wheelchair, and empties his
diaper pail and strips the bed. She
packs a bag with a change of clothes,
medicines, a stethoscope, "everything
he'll need for the day," and places
Hershy's liquid_food in a blue back-
pack attached to the wheelchair.
Chaya Leah then bundles up her

boy, complete with grey-wool socks
over his hands. "We couldn't find
wool gloves," she explains. Hershy's
immune system is especially vulnera-
ble. He can start with a slight sniffle
in the morning, and by 2 p.m. it will
be a 103-degree fever. Untreated, it
can turn into deadly pneumonia.
Chaya Leah cradles Hershy's face in
her hands and smiles at him. "Okey
dokes, we're ready to go!"
They are on their way for a haircut
and to the doctor, but first comes a
minor crisis.
The trouble occurs when Chaya
Leah tries to raise the hydraulic lift,
which brings Hershy's wheelchair into
the van. It jumps up and down, down
and up, but won't go back in place. As
the lift shakes and jolts so does the
van, and Hershy laughs.
It's the noise, Chaya Leah says.
Hershy loves loud noises, and especial-
ly the sound of Velcro on the straps
that hug his legs and head so he won't
fall , out of the wheelchair.
In-between bites on a peanut-butter
sandwich and sips from a Poland
Spring water bottle, Chaya Leah is on
the phone trying to get help. Finally, a
g ood 20 minutes later, a mechanic
arrives. With a few twists of his screw-
driver he manages to fix the lift —
temporarily — then tells Chaya Leah
what to do if it breaks again. So now,
in addition to mother and nurse, she
is a mechanic.
At last, jumping into the van,
Chaya Leah looks at Hershy and his
gaze seems to stay, if just foi an
instant, on his mother. It must take a
great deal of effort on his part to focus

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Like soldiers on duty, medicines are lined up in Hershy's bathroom.

11/28
1997

80

in that glance — a loving, helpless
glance.
"Okay, Hershy," she says, sighing.
Chaya Leah pulls out of the driveway.
"Now we're really going."

C

•

haya Leah Tinman was born
and raised in Detroit. She
completed high school, went
to Israel for a year, started
college, then married Michael
Tinman.
There's a test for it now, but 15
years ago little was known about
Canavans.
Both parents must be carriers for a
child to contract the disease, and even
then the chance is 50 percent that he
will be affected.
The Tinmans, who are now sepa-
rated, had two healthy girls before
fiershy was born, Alarm, now 13, and
Rachel, 11.
Initially, he seemed fine. But then
his eyes began to cross, he was excep-
tionally quiet, and he couldn't hold his
head up like other babies.
It took a long time for Hershy's ail-
ment to be identified, though every-
one knew it was not good. At 17
months, he became terribly ill and
"they told us that he wouldn't live to
see the end of the week, then it was
that he wouldn't live to the end of the
month, and then that he wouldn't live
to see his second birthday," Chaya
Leah says.
"Doctors know a lot, but they're
not God."
Once Canavans was diagnosed, the
W
Tinmans knew their son's time was
limited, and so they do what they can
to make Hershy's life full, joyous.
Until several months ago, he slept
with them in their bedroom. He goes
on all the family vacation's, and to
simpler outings, like movies and the
mall.
"Sometimes he's so sick I don't
know whether he'll make it to the end
of the day," Chaya Leah says.
"Sometimes I have such a fun time
with him that I just can't imagine giv-
ing him back."
The Tinmans' daughters have yet to
be tested to see whether they are carri-
ers, though Chaya Leah says they will
be tested once they turn 18. No ances-
tors on either side of the family were
known to have been affected.
A great deal has changed in the way
the disabled are regarded now and the
way they were treated when Chaya
Leah was young.
"When I grew up, we didn't even
look at a person in a wheelchair," she

•