A Life Turned Inside Out - Boy Defies Medical Odds

At her 13-week ultrasound, Danielle Daniels thought her baby looked a little different than her first baby, but it wasn’t until the 17-week ultrasound that it was apparent that there was something very wrong with her little boy.

The baby, who would be named James Daniels III, had a hole in his abdominal wall, or an omphalocele. Many of the organs that should have been in his belly – stomach, liver, spleen, intestines – were floating outside his little body. About 1 in 5000 babies are born with the defect, but his was so large it has a special classification of “giant,” and that only happens in about 1 in 10,000 babies.

Danielle and her husband James Daniels Jr. were understandably concerned, but the first task, obstetricians told them, was to rule out any syndromes or genetic anomalies that often go along with omphaloceles.

“When we found out he didn’t have them, we thought we would be out of the woods,” Danielle said. But they weren’t.           

Following good instincts, Danielle and James Jr. didn’t search the Internet for photos of omphaloceles. They turned to their medical team to give them reliable information. They took a tour of the Neonatal Intensive Care Unit at Golisano Children’s Hospital and saw other babies with similar defects. Above their bellies, hung silos or specialized, thick plastic bags that held the babies’ organs as they were gently, and over time, pushed into their abdomens.

A few weeks before the planned C-section, they met their surgeon, Walter Pegoli, M.D., chief of Pediatric Surgery, who would be the leader of the team that would put James’ organs inside his body.

“I’m a really emotional person so I really appreciated that he was blunt,” Danielle said. “I knew what I was dealing with.”

When James was born on June 21, 2011 – at an impressive 10 lbs. 14 oz. – a thin, membranous sac covered his stomach, intestines, liver, spleen, which were all outside his body. The sac was almost as big as the rest of James. James cried, like any other newborn, which reassured his worried parents. Because of the defect and because he developed severe pulmonary hypertension, a common issue in babies with this defect, James had to be put on a ventilator and they wouldn’t hear his cry again for nine weeks.

The surgical team couldn’t use the silos other babies had because James had too many organs to fit into any available manufactured silos, so Dr. Pegoli custom-built one. The team worked carefully and slowly to push the organs into James’ body. After 16 days, they succeeded enough to place a specialized dressing called a wound vacuum-assisted closure (VAC), which applied negative pressure over the area and further helped the organs into the abdomen.

“Not many pediatric surgical centers use wound-VACs like this, but we’ve found it very helpful, especially in patients like James, with giant omphaloceles,” said Heather Goetzman, C.P.N.P., N.N.P., a pediatric nurse practitioner who works closely with Pegoli on the surgical team.

Just about three weeks to the day James was born, his parents finally got to hold him for the first time. It took multiple nurses to help because he was on a respirator, but for his parents, it was worth the extra hands to make it happen.

Every week for almost three months, James was sedated and had his wound-VAC changed, which is essentially a surgical procedure. Many of those procedures occurred in the NICU where he had been since he was born, but partway through the process, he “graduated” from the NICU to the pediatric unit 4-3600. There, he became a kind of mascot for the unit, often sitting in a swing by the nurses’ station when his very devoted parents weren’t with him.

Three months and eight days after James was born, he went into surgery with Pegoli and John Girotto, M.D., chief of Pediatric Plastic Surgery. Together, they removed the wound VAC, closed the little boy’s abdomen, and for the first time in his life, James had a whole belly.

Despite starting to look whole, James still had a long road to recovery. He was back on a ventilator again for two weeks, and he still was not able to eat like other babies. He had a broviac central line into a major blood vessel that goes to his heart that carried the IV nutrients into his blood stream until his digestive system was working well enough to tolerate feedings. James also had an NG tube inserted through his nose down into his stomach that carried very small amounts of breastmilk into his digestive tract.

By Thanksgiving of 2011, the Daniels were ready to bring James home to live with his big sister, Dixon, and his parents. His maternal aunt, Joya Wade, moved from Milwaukee, Wis., to help take care of James.

“She hadn’t so much as changed a diaper when she got here,” Danielle said. “She can now change an NG tube and a broviac dressing like the best of them.”

James had a couple of complications, including an infection and initial prolonged feeding intolerance, but that hasn’t slowed him down. In fact, he’s now eating like most other toddlers, eschewing vegetables for graham crackers and apple slices. But he’s eating by mouth. No more lines into his heart or tubes into his stomach.

Danielle credits her family, especially her sister who put her young life on hold to help, and his many physicians, nurses, occupational therapist and SKIP of NY with the quick progress James has made. But Pegoli said James takes after his parents.

“They’re just amazing. They are always very understanding and patient,” Pegoli said. “They stepped up for every challenge laid in front of them, whether it was changing broviac dressings or getting James to eat.”

This little fighter has been chosen to be one of the hospital’s five Miracle Kids for 2013, to represent the 74,000 children Golisano Children’s Hospital treats every year. Tune in to Children's Miracle Network's News10NBC Telethon on Sunday, June 2, to meet James 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 11:30 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, June 1, with the 17th 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. To sign up or learn more, visit www.givetokids.urmc.edu/stroll.