Almost
every American baby at birth
receives a needle stick to
collect a few drops of blood
to test for phenylketonuria,
or PKU, a rare, inherited
birth defect. A Mayo Clinic
Web site reports that a person
born with PKU is “missing
or deficient in a specific
enzyme needed to process an
essential amino acid called
phenylalanine.”
The
gene defect occurs mostly
in people from Northern European
backgrounds. If including
too much protein in their
diets, these people can develop
mental retardation and a host
of physical problems. They
have to monitor everything
they eat.
Jenny
Barnes, from the Midwest,
and her younger brother, were
both born with PKU. “I remember
in kindergarten,” said 35‑year‑old
Barnes in a telephone interview,
“when we had snack time. I
couldn’t eat what the other
children had. Instead of milk,
I drank Tang. For lunch, my
mom would circle on the school
menu the items I could eat.”
Her parents always stressed
that she could never depart
from her diet.
But
when she was in second grade,
doctors experimented by allowing
her to eat protein for a while,
in order to monitor whether
her system could handle it.
She said, “I remember my
parents feeding me a burger.
At first, I took a bite and
spit it out. I didn’t like
the taste.” The experiment
didn't last long.
In
high school, she said only
her best friends knew about
her having PKU. Later in life,
she said that on occasion,
while eating out with new
friends, she had ordered items
containing protein and not
eaten much of it because she
didn’t want to go through
all the hassle of having to
explain PKU.
In
2006, doctors learned Barnes
also had diabetes and she
began insulin shots four times
a day. Because of PKU, she
must drink a special formula
with necessary nutrients
each day.
Having
PKU and not being able to
freely eat protein, seems
odd given her occupation.
She and her brother work at
their family’s restaurant,
one well‑known in part
for quarter‑pound hamburgers.
“It’s
never bothered me,” Barnes
said of being around meat.
“Working in a restaurant is
my life, my livelihood, and
my brother and I want to take
over from our parents some
day.”
One
thing: She and her brother
will never be accused of eating
the profits.
