After Making Heart Surgery History at Lucile Packard Children’s Hospital Stanford, 5-Month-Old Carmel Smith is Home for Good

Carmel is believed to be the smallest baby in Northern California —
and perhaps the world — to successfully undergo the high-risk Norwood
procedure for hypoplastic left heart syndrome


Initial surgery over the Thanksgiving holiday on her marble-sized
heart was so high-risk that the statistical chance of survival could not
even be predicted

STANFORD, Calif.–(BUSINESS WIRE)–Five-month-old Carmel Smith of Hayward, California, headed home last
month from Lucile
Packard Children’s Hospital Stanford
with parents Uyen and Chauncey
and big sister Chloé. But first, she made heart surgery history.

Born eight weeks premature on Nov. 23 at a weight of only 1.3 kg (less
than 3 pounds), Carmel is the smallest baby ever in Northern
California — and probably anywhere — to successfully undergo a
risky open-heart surgery called the Norwood procedure.
Cardiothoracic
surgeon Frank
Hanley, MD,
performed the operation the day after Thanksgiving, four
days after she was born. The Norwood is the first step in correcting
circulation issues caused by Carmel’s serious heart defect, called hypoplastic
left heart syndrome
.

“To our knowledge, Carmel is one of the smallest babies — if not the
smallest baby ever — to survive the Norwood,” said Hanley, executive
director of the Children’s
Heart Center
at Stanford Children’s Health.

Hanley said Carmel has “defied all expectations” by also doing extremely
well during the high-risk post-operative period that followed, and by
undergoing a successful follow-up surgery on March 11 called the
bi-directional Glenn. Her recovery from the Glenn went well, and she was
released from the hospital less than a month later, on April 8.

“Carmel is our miracle baby,” Chauncey said. “It’s been a long road with
a difficult pregnancy and delivery, the HLHS diagnosis and many ups and
downs. But now we know there’s light at the end of the tunnel, and we
couldn’t be more grateful to Dr. Hanley for saving Carmel’s life.”

Shocking diagnosis followed by reassurance

Babies born with hypoplastic left heart syndrome are missing the left
ventricle, which is the major pumping chamber of the heart and normally
pumps oxygen-rich blood to the body. Surgical treatment includes three
open-heart surgeries
(Carmel will undergo the third one, the Fontan
procedure, when she is 3 to 4 years old). These staged operations
redirect the blood flow so that the right ventricle, which normally
pumps deoxygenated blood to the lungs, can be used as the body’s main
pumping chamber.

Chauncey and Uyen were shocked by the unexpected HLHS diagnosis during a
20-week prenatal checkup at the hospital’s Perinatal
Diagnostic Center
. But the Smiths quickly learned that the Heart
Center’s extraordinary outcomes
meant that they couldn’t be in a
better place for what lay ahead — the surgeries to reconfigure the
circulatory system, as well as lifelong care from congenital heart
specialists to optimize the function of the heart’s single pumping
chamber.

No benchmark for comparison

The Norwood procedure, which Carmel underwent in November, was far
riskier than the two subsequent operations. The Norwood has only a 50
percent chance of success when performed on any infant in the
“low-birth-weight” (under 2.5 kg) category. “The smaller a baby is under
this threshold, the riskier the surgery becomes,” explained Hanley, who
is the Lawrence Crowley, MD, Endowed Professor in Child Health at the Stanford
University School of Medicine
.

With Carmel’s weight at only 1.3 kg and her heart the size of a marble
at the time of the surgery, Hanley could not even provide her parents
with her statistical chances of survival. He only knew that, like
Carmel, they were very small.

While Hanley and his team are known for taking on surgically complex
cases that others can’t or won’t, the confluence of risk factors in
Carmel’s case was extraordinary. “That’s why there have been very few
successful Norwood operations for any low-birth-weight babies,
let alone for one as small as Carmel,” he explained.

But once again, Hanley beat the odds and performed a successful Norwood,
balancing the blood flow between Carmel’s lungs and the rest of her body.

Innovative home monitoring after surgery

Before Carmel could go home for the first time Feb. 5, Chauncey and Uyen
underwent a home care “boot camp” of sorts, learning how to monitor her
during the risky interim period between her discharge after undergoing
the Norwood procedure and her admission for the Glenn surgery several
weeks later.

Historically, the nationwide survival rate during this interstage period
was 85 percent. Over the past decade, an innovative Home Monitoring
Program developed and led by pediatric cardiologist Gail
Wright, MD
, has brought the hospital’s interstage survival rate up
to 98 percent.

The HMP tool kit includes two low-tech but lifesaving pieces of
equipment: a scale and a pulse oximeter, a small device that clips on
the baby’s finger to measure oxygen levels and heart rate. The Smiths
recorded Carmel’s numbers once per day and reported them to a nurse
practitioner at Packard Children’s. The program also supported close
communication between the Smiths, the hospital’s medical team and
Carmel’s community pediatrician.

The HMP did its job by detecting issues with Carmel’s oxygen levels that
required her to be admitted to the hospital for treatment in late
February. “Without this close surveillance, this red flag could have
gone undetected and potentially could have led to death during this
risky interim period,” said Wright, who is a clinical associate
professor of pediatric cardiology at the School of Medicine. “Instead,
Carmel was promptly admitted and successfully treated.”

Once Carmel’s health stabilized, she underwent the Glenn procedure — in
early March, sooner than expected — and it went smoothly. The Glenn
surgery brought the blue (deoxygenated) blood from half of her body
directly into the lungs. The Fontan surgery that Carmel will undergo in
a few years will bring the blue blood from the other half of her body
into her lungs so that her blood can be fully oxygenated.

“We are definitely blessed that both the Norwood and Glenn surgeries
went so well,” Uyen said.

Looking ahead

This experience has brought the Smiths even closer together as a family.
“Even after everything Carmel has been through, her tiny body is still
so strong and resilient,” Uyen said. “It puts things into perspective.”

With Carmel home, the Smiths say their family is whole again. They can
enjoy the small moments of everyday life together that others may take
for granted. Moments like reading Peter Rabbit. “When we start to
read a book, she pauses for a moment, and then she gets this look that
shows how much she’s enjoying it,” Chauncey said. And when Carmel wants
to go for a wagon ride, 2.5-year-old Chloé insists on pulling it.

Now, the Smiths want Carmel’s story to serve as a source of inspiration
and support for other parents whose children are born with serious and
seemingly impossible congenital heart conditions.*

“We have hope now,” Uyen said. “We want other parents to have hope, too.”

Additional information

* Parents who have children born with single ventricle defects can
obtain support through the
National
Pediatric Cardiology Quality Improvement Collaborative
,
including its Parent & Family Resources page.

* Discover more about the Heart
Center
at Lucile Packard Children’s Hospital and Stanford
Children’s Health.

About Stanford Children’s Health and Lucile
Packard Children’s Hospital Stanford

Stanford Children’s Health, with Lucile Packard Children’s Hospital at
its core, is the largest Bay Area health care enterprise exclusively
dedicated to children and expectant mothers. Long recognized by U.S.
News & World Report
as one of America’s best, we are a leader in
world-class, nurturing care and extraordinary outcomes in every
pediatric and obstetric specialty, with care ranging from the routine to
rare, regardless of a family’s ability to pay. Together with our Stanford
Medicine
physicians, nurses, and staff, we can be accessed through
partnerships, collaborations, outreach, specialty clinics and primary
care practices at more than 60 locations in Northern California and 100
locations in the U.S. western region. As a non-profit, we are committed
to supporting our community – from caring for uninsured or underinsured
kids, homeless teens and pregnant moms, to helping re-establish school
nurse positions in local schools. Learn more at stanfordchildrens.org
and on our Healthier,
Happy Lives blog
. You can also discover how we are Building
the Hospital of the Future
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Contacts

Lucile Packard Children’s Hospital Stanford
Erin Digitale,
650-724-9175
digitale@stanford.edu