Adelyn Swain underwent two life-saving surgeries to correct rare birth defects in the first 10 months of her life. Not that one could tell now, as she runs seemingly nonstop, throws crackers across the room, and bears only a slight, pale scar on her torso as evidence of her surgeries.
She is expected to have a normal childhood with no restrictions on her activities.
“Sometimes I have to remind myself of everything that she went through,” said her mother, Tiara Marsh of Saco.
Adelyn, 18 months, was born with gastroschisis, a condition in which part of the intestines are outside of the abdominal wall. Gastroschisis – which occurs in one of every 2,000 births and for unknown reasons has become a more common birth defect compared to decades ago – is usually discovered during ultrasounds and requires immediate surgery upon birth.
When she was an infant, Adelyn Swain had two life-saving surgeries at Maine Medical Center. Gregory Rec/Staff Photographer
Forty years ago, gastroschisis occurred about once in every 10,000 births, and scientists aren’t sure why the defect has become more common since the 1990s, although research is ongoing.
Adelyn also was born with congenital pulmonary airway malformation, or CPAM, a life-threatening defect that required the removal of the lower left lobe of her lung when she was 10 months old. The rate of CPAM as a birth defect is in dispute among scientists, but the range is one of every 8,000 to 30,000 births. Little is known about the causes of CPAM – a lesion on the lungs – but survival rates are up to 95 percent.
Dr. Jeffrey Halter, the pediatric surgeon at Maine Medical Center who performed both surgeries, said the conditions are unrelated, and it was coincidence that Adelyn had two serious birth defects.
“She had two unique congenital malformities,” Halter said. “We had never seen that combination.”
Adelyn also benefited from new, improved procedures that help speed patient recovery, Halter said.
Adelyn was the first baby with gastroschisis for whom Maine Med physicians used a technique called “sutureless closure” to put the intestines back in place and repair the abdominal opening. The procedure uses the baby’s umbilical cord, requires fewer follow-up surgeries and is less invasive than other procedures.
Hers was also the first case in which doctors used VATS – video-assisted thoracoscopic surgery – to remove the lower lobe of the lung. This meant doctors only needed to make an incision a few inches long to do the surgery. For both procedures, Adelyn was back home within weeks.
Over time, Adelyn’s lung will grow to replace the part that was removed, and she should have no respiratory constraints, Halter said.
“She could be an Olympic athlete if she wanted to be,” he said.
If Adelyn had been born before the 1970s with the same defects, she likely would have died.
“We have very good survival rates for these kids now,” Halter said.
Marsh, Adelyn’s mom, said she learned of Adelyn’s condition when she was 14 weeks pregnant – after doctors examined her ultrasound – and was confused about why it was happening.
“It was hard to wrap your head around because there’s no reason why, there’s nothing they can point to,” Marsh said. “It just happens.”
But Marsh said although it was a “dangerous situation,” she still somehow stayed on an even emotional keel.
“I always had confidence things would turn out OK,” she said. “I’m not going to say there weren’t times when I was scared, because there were definitely those times, but I knew I was in good hands.”
Adelyn was born on Nov. 26, 2017, and was home before Christmas.
Tiara Marsh holds her 18-month-old daughter, Adelyn Swain, at their Saco home. Gregory Rec/Staff Photographer
“I remember (after the birth) feeling so very proud and happy, just that she was here with us. She is a fighter,” Marsh said.
Megan Rauen, president and founder of the Avery’s Angels Gastroschisis Foundation nonprofit, said it’s heartwarming to hear stories about children like Adelyn who survive and thrive after surgery as infants. But she cautioned that many babies who survive gastroschisis surgery can suffer from complications later in childhood or as adults, including severe, life-threatening gastro-intestinal conditions.
“It’s impossible to tell when they are really young how well they will do later. There’s a huge disparity in outcomes,” she said. Rauen said about 60 percent of those who survive the initial surgery don’t have lifelong complications, but 40 percent do. “While survival rates have improved, the incidence of gastroschisis has increased, and we don’t know why.”
She said some suspected reasons are environmental, such as pesticides, or the use of anti-depressant medications, but the evidence is thin and much more research is needed. Also, babies born with the condition are more common in younger mothers, and it’s unknown why.
Marsh, 24, was 22 when Adelyn was born.
For now, Adelyn is showing no signs of any complications, said Marsh, who also has a 4-month-old boy, Hazen. Her career is on hold with two young children at home, but she hopes to go into veterinary work.
Marsh has brought Adelyn in for visits to Maine Med since her recovery from the surgeries.
“It’s a good feeling to see how animated and charming she is now,” said Halter, her surgeon. “We are very lucky in pediatrics to do what we do.”
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CORRECTION: This story was updated at 7 p.m. on June 20, 2019, to correct that Maine Med has been performing surgery on babies with such birth defects since the 1970s, but this is the first time they’ve used this particular method that is less invasive.
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