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'Bubble Boy'
Fighting the Odds
by Jim Ritter, from the
Chicago Sun Times, November 6, 2002
For more articles visit
www.bridges4kids.org.
From the day he came home from the hospital, Jacob "Ray Ray"
Davis couldn't stop coughing.
When he was 5 months old, Ray Ray suffered viral diarrhea for
17 days. A few weeks later, he caught pneumonia after going to
church.
Last May, doctors discovered what's wrong with Ray Ray. He has
a gene defect that causes "severe combined immune deficiency,"
better known as "bubble boy" disease. Ray Ray's immune system
doesn't work, making him dangerously susceptible to any and
all germs.
The disease has been reported in fewer than 1 in 500,000
babies, although it could be more common because some infants
die before they are diagnosed.
In the past, immune-deficiency babies almost always died in
infancy from runaway infections. In the 1960s, doctors began
isolating them in sterile environments. The most famous case
was a Houston boy who spent his entire life inside a plastic
bubble with filtered air. He died at age 12.
But patients don't have to live inside bubbles any more. A
remarkable drug approved in 1990, Peg-ADA, supplies the
critical ADA enzyme that's missing in immune-deficient babies.
Without the ADA enzyme, the infection-fighting white blood
cells don't work.
Last May, Ray Ray began receiving twice-weekly shots of
Peg-ADA. His cough stopped immediately, and he hasn't been
seriously ill since. Now 1 year old, Ray Ray is learning to
walk, and his teeth are coming in. He's a happy, good-natured
baby who loves being read to.
"He's a fighter. He wants to live," said his mother, Zina
Berryhill. "I call him my chosen one. My survivor. My gift."
But Ray Ray's immune system still doesn't work normally. Once
every four weeks, he must get an IV infusion of antibodies.
Scratches take months to heal. And Berryhill takes great care
to avoid germs. Only family members are allowed to visit their
south suburban home. They take off their shoes and wash their
hands, and if they have so much as a sniffle, they stay away
from Ray Ray.
For now at least, Ray Ray is relatively healthy. But about 10
percent of patients develop resistance to Peg-ADA. Others
develop chronic lung disease, said Dr. Barbara Hendrickson of
the University of Chicago, who is treating Ray Ray.
So some day, Ray Ray may need to get a bone marrow transplant
from a donor who has a healthy immune system. Bone marrow, the
soft fatty tissue inside bones, is where disease-fighting
white blood cells come from.
But no one in Ray Ray's family matches Ray Ray. Nor are there
any matches on the National Marrow Donor Program. A patient is
most likely to find a match from someone of the same ethnic
group. Ray Ray is African American.
Nationwide, about 380,000 blacks have registered as potential
marrow donors. Nearly seven times as many whites--2.6
million--have registered. "My people need to be more
educated," Berryhill said.
LifeSource Blood Services has held about 10 marrow donor
drives for Ray Ray in African-American neighborhoods. About
250 people have registered, and LifeSource hopes to increase
the number to 1,000 by January. The next drive will be held
from 10 a.m. to 2 p.m. Saturday at Liberty Baptist Church,
4849 S. King.
It's a long shot any of them will match Ray Ray. But 3,000
other patients with leukemia, blood disorders and other
diseases are waiting for marrow transplants, so it's possible
that someone who registers to help Ray Ray will end up saving
the life of someone else.
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