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Donor funds
Breathe new life into CDH research

Eight-year-old Danny Murphy dreams of growing up to be just like
his idol, Arnold Schwarzenegger. But the real hero in Danny's life
is Jay Wilson, MD, a surgeon at Children's Hospital Boston. In turn,
Wilson's heroic work has been aided by a woman Danny has never met.
Dina Zahid's generous gift to Children's gives kids like Danny,
born with congenital diaphragmatic hernia (CDH), a chance for survival
her own child missed.
CDH occurs in one of every 2,500 births. In the developing fetus,
the diaphragm — the muscle that separates the chest and abdominal
cavities — fails to close, allowing the abdominal organs to migrate
into the chest, compressing the heart and lungs. While surgery can
correct the diaphragm shortly after birth, the underdeveloped lungs
cannot deliver enough oxygen and a CDH newborn requires immediate
high-tech respiratory support.
A chance to draw breath
Twenty years ago in the U.S., only about one in five babies with
CDH lived. Now more than half pull through, and at Children's, the
survival rate has reached 90 percent. That's because Jay Wilson,
the leading CDH expert, directs the world's only CDH clinic and
has advanced research and treatment in the disease. "With Russell
Jennings, MD, and the Children's Center for Fetal Diagnosis and
Treatment, we've pioneered one of the most successful procedures
for CDH newborns," says Wilson. "EXIT to ECMO is a heart and lung
bypass performed while a baby is still attached to the umbilical
cord and before he or she draws a breath." ECMO — extracorporeal
membranous oxygenation — gives infant lungs precious time to rest
and heal before and after corrective surgery, and, hopefully, to
grow strong enough to breathe on their own. "But ECMO can be used
for only a few weeks," Wilson says. "Sometimes that isn't adequate
for enough lung growth."
For the past 14 years, Wilson has studied normal lung development,
discovering that lung growth rate is not fixed, and can be dramatically
increased both in the fetus and newborn. Searching for ways to accelerate
growth before and after birth, he found that temporarily filling
animal models' lungs with perfluorocarbon, an inert liquid, stretched
and stimulated them to grow.
Beating the odds
By January 1996, Wilson was ready to launch a small pediatric clinical
trial — just in time to help Danny Murphy. CDH had left Danny's
left lung, which normally would be the size of a lime, only as big
as a quarter, and his first week on ECMO wasn't enough to increase
his lung capacity. "Dr. Wilson told us perfluorocarbon might help,"
says his mother, Helen. "We were willing to try anything to save
his life." Danny spent a critical second week on ECMO, while perfluorocarbon
gently expanded his tiny lungs. It worked, and after four months
in the hospital, Danny was able to go home, where he spent another
year attached to an oxygen tank. Today he's an eager second grader
who loves reading, swimming in the family pool and being a protective
big brother to his 4-year-old sister, Krista. "He's my miracle child,"
says Helen. "Dr. Wilson sent his lung X rays all over the world
to show what perfluorocarbon treatment can do."
Despite promising results, further clinical trials were stalled
when the manufacturer of perfluorocarbon went out of business and
funding was depleted. Then Wilson's research received help from
an unexpected source. In 1997, Dina Zahid learned her unborn child
had CDH, and her doctors in London were discouraging. "When we heard
about Dr. Wilson, we knew we'd have a better chance in Boston,"
she says. Her son Mohammed was born here January 29, 1998, and placed
immediately on ECMO, but his condition was complicated by an unexpected
heart ailment. Tragically, he survived only a few weeks. "We have
a saying in Islam that babies who die become birds in heaven, where
they wait for their parents to join them," Mrs. Zahid says. Before
returning to England, she and her family made a gift of $250,000
in Mohammed's memory, to be used for further CDH research. With
the gift, Wilson and his team resurrected the research, even without
a source of perfluorocarbon. "Our combined clinical and research
efforts increased CDH survival rates from 70 percent in 1997 to
90 percent today," says Wilson. More important, these results attracted
further support from a new pharmaceutical manufacturer. This year,
Children's will participate in a national multicenter perfluorocarbon
trial that promises gentler and more effective therapies for thousands
more CDH children.
Dina Zahid can see the seeds of her generosity flourishing. "Baby
Mohammed was very much loved — it was terrible to
see him suffer and not be able to comfort him in intensive care,"
she says. "I knew then I wanted to help increase the chance of survival
for other babies with this disease." Today, her loving gift means
the breath of life for someone else's child.
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