Boy "Does It All" After Complex Defect
2-year-old named Golisano Children's Hospital Miracle Kid for 2014
Friday, May 09, 2014
Almost five months before their son was born, Drew Lagares and Theresa Cappon already knew something was wrong with their baby. Ultrasounds showed a shadow, although at first no one knew what it meant, so Theresa was monitored once a week, then twice a week before getting a clear diagnosis. Their baby had gastroschisis, a birth defect in which the intestines stick out of the body through a hole in the abdominal wall. The defect is found in about one in every 20,000 babies and creates a wide range of potential complications.
“We didn’t know what was going to happen, and I felt responsible because it was going on inside of me,” Theresa said. “It was scary.”
At a regularly scheduled appointment six weeks before her due date, a stress test showed Theresa was having contractions (“I really didn’t feel anything,” she noted,) and was sent to UR Medicine’s Strong Memorial Hospital where they would have access to high-risk obstetrics and they would be just down the hall from the Golisano Children’s Hospital pediatric specialists their son would need. Once there, Drew went to the vending machine for a snack, and when he returned to the room there were doctors everywhere.
“I said, ‘We’re having a baby today,’” Theresa recalled. “He’d just popped an M&M in his mouth, and he completely froze.”
Andrew was born via Caesarean section, his body placed immediately in a bag to keep his intestines moist before being rushed to surgery at Golisano Children’s Hospital. “My son looked like an alien,” Drew said.
For some, the congenital malformation is mild and can be remedied with one procedure, “but he was as far on the other side of the spectrum as you can get,” said Christopher Gitzelmann, M.D., a pediatric surgeon at Golisano Children’s Hospital. “Not only were his intestines outside his abdomen, they were twisted.”
That condition, called volvulus, meant blood flow was significantly diminished, causing two separate atresias, or bowel obstructions, that left much of his intestines unviable. Gitzelmann put the remaining intestines back inside Andrew’s abdomen, hoping that they – and the infant – would survive those first few days.
Meanwhile, Andrew, who was admitted to the Golisano Children’s Hospital’s Neonatal Intensive Care Unit, was also dealing with short bowel syndrome. Instead of his small bowel measuring the typical 150 centimeters, it was only 25 centimeters long, requiring him to get nutrition intravenously through a method called total parenteral nutrition, or TPN. (He also had a gastrostomy tube in his stomach for feedings.)
Unlike older children and adults, newborns have the potential to “grow their bowel,” said Gitzelmann, who hoped this would be the case in Andrew so the boy could avoid an intestinal transplant a few years down the road. Three months later, as verified through surgery, Andrew’s small bowel had grown both in diameter and length – to 80 centimeters. The news was monumental, signaling the possibility that Andrew might be able to avoid a transplant after all. Instead, the treatment strategy was adapted with hopes the short bowel would grow even more and eventually be attached to what was left of his colon and the first section of the small intestine, called the duodenum. Through a complicated series of procedures, Andrew received two ostomies – openings surgically created to connect an internal organ to the body’s surface – to provide nutrition and collect waste in separate bags.
In addition, Andrew received an ostomy in his colon, with Gitzelmann banking on the possibility that he could “collect the output, put it in a syringe, and refeed it into the colon to help it grow as well,” he explained. “You’re improvising as you go. You have to apply all the knowledge you’ve accrued over the years to come up with something, because there’s no solution or plan or scheme you can get from a textbook when you have a case this complex.”
Three months after that, back in the operating room, Gitzelmann found Andrew’s short bowel had grown yet again; he connected it to the colon, so the baby then would need only one ostomy for nutrition.
Andrew went home from the hospital after that. He was four months old.
Marsha Pulhamus, a pediatric nurse practitioner at Golisano Children’s Hospital, said she was impressed with the care Theresa and Drew, who had to quit his job to stay home with his son, provided. “There was an IV catheter, feeding tubes, ostomy care...It was a lot of work,” she said. “This was a young couple who was shell-shocked, and they really stepped up.”
Multiple surgeries later, including several to place and remove central line catheters related to the TPN, as well as one to connect the duodenum to the remaining part of the intestines, Andrew was able to be gradually weaned off TPN. This was a huge step because TPN ultimately causes liver damage.
Andrew kept his gastrostomy tube until he was 18 months old, however. “He did have some difficulty wanting to take food, because it had been such a long period of time that he wasn’t allowed to have anything,” said Pulhamus, who helped manage the oral feedings. “Babies have a critical window in their first six months of life in which they try to coordinate sucking and swallowing, so that was often a struggle for him.” As a result, Andrew didn’t start regularly incorporating oral feedings into his nutritional program until he was nearly 9 months old.
Gitzelmann, who attended Andrew’s first birthday party, said this was “the most difficult and challenging case of gastroschisis I have encountered in my career, but it turned out great. I get goosebumps just talking about it.”
Drew has a difficult time expressing thanks for what the doctors, nurses and staff at Golisano Children’s Hospital did for his baby. “There really are no words you can say about them taking that situation and making it what it is today,” he said. “They were always, always there for us, and they saved my son’s life. I’m very grateful.”
Other than the scars on his belly, there are no signs of Andrew’s rough beginning. Now 2, he has worked with a number of speech and occupational therapists and needs to be monitored only once every year or so. He laughs when he does something inappropriate, adores any kind of sport, and has a weakness for pizza and broccoli.
“He eats, he runs, he plays, he cries, he throws temper tantrums like a normal 2-year-old,” Theresa said. “He does it all.”
This resilient little boy has been chosen to be one of the hospital’s five Miracle Kids for 2014, to represent the 74,000 children Golisano Children’s Hospital treats every year.
Tune in to the Children's Miracle Network Hospital’s News10NBC Telethon on Sunday, June 1, to meet Andrew and his family, along with four other Miracle Kid families, Golisano Children's Hospital supporters and health care providers. The Telethon is from 10 a.m. to 1 p.m. and 6:30 to 11 p.m. on News10NBC. Dial (585) 241-KIDS during the Telethon or donate online to make a gift.
The Telethon is part of a weekend-long celebration called Miracle Weekend, which kicks off Saturday, May 31, with the 18th annual Stroll for Strong Kids fundraising walk and 5K run. The event starts at 8 a.m. at Genesee Valley Park and wraps up at about 1 p.m.