Shortly after Mary Gundrum found out that her unborn son was
developing with part of his brain outside his skull, she found herself
searching the internet to find out more about the baby's diagnosis and
potential treatment.
Her next search would be for infant-sized coffins.
"He was only supposed to live a couple of hours,"
she said.
Instead, baby Dominic was born "screaming and kicking
and full of life," but he had a Tessier midline facial cleft, which means
the sides of his face didn't fuse together properly during early pregnancy,
causing it to split down the middle. Dominic also had an encephalocele, a rare
cranial abnormality that means a sac of brain tissue and fluid formed outside
the skull. Dominic's encephalocele filled the space between the cleft in his
face, splitting his nose in two with a fluid-filled bubble and causing some of
his brain matter to sag into his mouth.
Dominic was not in immediate danger, but potential
life-threatening complications down the road for a child with his conditions
included a puncturing of the sac or infection.
Today, Dominic is seven months old, and developing
normally for his age. Thanks to a six-hour craniofacial surgery, the only sign
of Dominic's birth defect is the thin scar on his forehead that fades with each
passing day.
"It's superexciting to think about his future,"
said Gundrum, who homeschools her seven other children in Milwaukee. "I
never would have imagined such a small, tiny line that's already continuing to
fade. It's beautiful."
Dr. John Meara, the chief plastic surgeon at Boston Children's
Hospital, led the surgery that saved Dominic's face this December, prompting
the family of 10 to move from Milwaukee to Boston for five weeks in December.
Meara specializes in craniofacial surgeries like the one
Dominic needed, having done three encephalocele surgeries in six years during
his time in Boston. Before that, he worked at the Royal Children's Hospital in
Melbourne, Australia, where he researched craniosynostosis, or birth defects
associated with skull development. Because of Melbourne's proximity to
Southeast Asia, where many children are born with clefts and other birth
defects, Meara got a lot of practice treating them.
"In the 20 years I've been in the specialty, I've not
seen a case exactly like this," Meara said.
He said that the occurrence of a Tessier midline cleft and
an encephalocele together in one person is a rarity.
"I'd be lying if I didn't tell you that I had a number
of sleepless nights," he said. "But I felt comfortable in the sense
that we had an excellent team."
Surgeons would have to move the brain into its proper
position, close the two halves of Dominic's face, and create a floor for
Dominic's brain (and a roof for his mouth). Blood loss during the surgery was a
concern because Dominic only had about 650 ccs of blood in his entire body –
about as much as "two cans of Coke," Meara said.
But Meara and his team, including neurosurgeon Dr. Mark
Proctor, rehearsed the procedure's 37 steps on an acrylic model of Dominic's
head. They went into the operating room at 7:30 a.m. on Dec. 18, began the
surgery at 9:30 a.m. and finished up at 3:30 p.m. It took the Boston Children's
Hospital team only six hours, compared with the 18-to-20 hours it took doctors
when they first started doing these procedures in the 1970s, Meara said.
In that sense, Dominic was lucky, said Dr. William Magee,
the plastic surgeon who co-founded Operation Smile with his wife in 1982.
Operation Smile has provided more than 200,000 free surgeries for cleft
palates, lips and other facial deformities in more than 60 countries to date.
"What you see is a lot of this in developing
countries," Magee said of facial deformities, adding that the children who
survive childhood are often branded as outcasts. "They certainly don't go
to school. They certainly don't get employed. It's horrible, and it's a very
correctible problem in the right hands."
He added that about 80 percent of the world's population
doesn't have access to surgeons like Meara. A child is born with a cleft every
three minutes worldwide, according to Operation Smile, and the Centers for
Disease Control and Prevention estimates that one in 10,000 babies in the
United States are born with an encephalocele.
Still, Dominic risked infection and other complications,
Meara said.
Despite a bout of possible fungal meningitis, which Meara
said he thinks was perhaps not a true case of the illness, Dominic is doing
well.
Dominic still has some issues with his pituitary gland --
which was moved to its proper place during surgery – causing problems with
sodium levels, hemoglobin and water balance, Meara said. In the three weeks
Dominic has been home in Milwaukee, Meara has checked on him with a
camera-equipped robot in the Gundrums' home. Dominic has not had to return to
the hospital.
"I have to say, handing my son off to them [the
surgeons] was the most difficult thing I think I've ever done in my entire
life," Gundrum said, adding that she knew her son would never look the
same again. "As much as his face was different, we fell in love with that
face. I don't know that anyone can really understand that."
Gundrum remembers one of her 11-year-old sons pulling a
blanket over Dominic's face in a store shortly after Dominic was born so that
shoppers wouldn't say anything mean about his deformities. But Gundrum took the
blanket off and taught her son to show others he was proud of Dominic the way
he was. Her 13-year-old daughter would later comment that she thought normal
babies' noses were "weird" because she was so used to seeing
Dominic's split button nose.
"You mourn the loss of the look and face that you fell
in love with because that's the baby you knew," she said. "Now,
there's a new Dominic, and we're falling in love with him the way he is."
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