Physical challenges that are hard to imagine

Devon Connelly could not wait to go outside and practice his jump shot.

Earlier that morning, the incoming sixth-grader at Laguna Beach's Thurston Middle School had been honing his skills at a camp dedicated to basketball, his favorite sport.

Devon was making the most of the waning days of summer vacation. The 11-year-old's zeal for sports, whether basketball, baseball or bodysurfing, belies serious medical struggles he has had to overcome.

Colleen Connelly received the news nine weeks into her pregnancy. Her child would be born with an omphalocele, a birth defect in which an infant's intestine or other abdominal organs, covered in a membrane, protrude through a hole in the belly button area.

"I was more scared than I had ever been because I really had never heard of it," Connelly said.

Connelly sought opinions from various doctors — one suggested she terminate the pregnancy.

Then she started doing her own research and joined a support group of mothers who had children with omphaloceles. Through investigation, Connelly learned that many children have had successful surgery and gone on to lead productive lives.

"Mother's intuition" led her to believe "it was going to be OK."

"But if it wasn't I still had to give him a fighting chance," Connelly said. "When I talked to other mothers, there was so much hope. It was not terminal by any means."

 

Problems right from the start

On Jan. 10, 2005, Connelly gave birth to Devon via C-section at St. Joseph's Hospital in Orange.

Devon had what doctors consider a giant omphalocele, one that is more than 10 inches in diameter. His liver and intestines were contained in a sac outside of his body.

The sac was too big to fit inside his abdominal wall cavity at birth, and so the first of two surgeries to repair the omphalocele was postponed until Devon had grown more.

Devon spent at week at Children's Hospital of Orange County and an additional seven days at Mission Hospital in Mission Viejo. Connelly and Devon rode home in an ambulance from Mission Hospital since the omphalocele made it impossible to fit Devon into a car seat.

Once home, Devon required constant attention. Connelly, who was not working full-time, was his primary guardian.

Eating was difficult. Devon had trouble keeping food down. He vomited every two hours for the first four years of his life, his mother said.

Connelly started Devon on breast milk and progressed to a high-calorie baby formula.

"He gained 3 pounds in the first year and he should have gained 16," Connelly said.

Children with omphaloceles encounter feeding problems because the intestines do not rotate properly as they should inside the abdominal wall cavity, said pediatric surgeon Vinh Lam, who operated on Devon. Lam is a member of Pediatric Surgical Associates Inc., a group of pediatric surgeons based in Orange and Mission Viejo.

"[Acid] reflux can grow out of that," said Lam, who on average encounters one omphalocele a year.

Devon slept sitting upright in an indoor baby swing for three months to prevent choking in case he vomited, Connelly said. She relied on restaurant delivery and neighbors who helped bring her groceries and run her errands.

Connelly waited 3 1/2 months before taking Devon on his first car ride. Connelly used a special brace, made of foam found in surfboards, that would absorb sudden jolts and protect the omphalocele.

A layer of skin grew over the omphalocele in the first year, providing greater protection in case the sac rubbed against another object, Connelly said.

When Devon was 10 months old, Lam performed the boy's first surgery, which involved pulling Devon's skin completely over the omphalocele. At 20 months, Devon had a second surgery, in which Lam closed the abdominal hole by repairing muscle tissue beneath the skin.

There were "no big complications," said Lam, who also performed multiple surgeries to repair an inguinal hernia, which occurs when tissue, such as part of the intestine, protrudes through a weak spot in the abdominal muscles, according to the Mayo Clinic website. Hernias can cause groin pain.

Despite the surgeries, Devon learned to crawl and walk at the standard ages.

 

The cause is hard to pinpoint

One of every 5,386 babies born in the United States each year is born with an omphalocele, according to the United States Centers for Disease Control and Prevention.

Many babies born with an omphalocele also have other birth defects including heart and neural tube problems, or chromosomal abnormalities, the CDC website said, though Devon was born without any of these conditions.

Debbi Kulick of Mission Viejo, on the other hand, gave birth to a son who had a "small" omphalocele but several additional medical problems, including a ventricular septal defect — a hole in the wall separating the two lower chambers of the heart. In addition, his throat was the size of a straw and his vocal cords were fused together, meaning the mother could barely hear him when he cried, she said. And he was in and out of the hospital with respiratory problems.

But in terms of the omphalocele, only the bowels were outside the body, and they were successfully relocated when he was 5 days old, though this necessitated his wearing an ileostomy bag for a few months afterward.

The cause of omphaloceles isn't well known, though CDC researchers said certain behaviors could boost the chances of a baby born with the condition.

Women who consumed alcohol or smoked more than one pack of cigarettes a day were more likely to have a baby with an omphalocele, as were women who used selective serotonin-reuptake inhibitors (SSRIs) during pregnancy, the CDC reported on its website.

"Random defect" is what they told Kulick, she said.

But since she was 36 when she had her son nearly 27 years ago, she questioned her age. She wondered what living near a toxic landfill might have done to her.

As online support grew for those who once felt alone, Kulick discovered that a lot of parents likewise second-guessed themselves.

"People would come on the news group and say, 'How many people were on antidepressants?' " she recalled. "Some were, some weren't. How many were on infertility pills? Some were, some weren't."

Even without knowing what causes omphaloceles, the medical community is far better at handling the cases today. Babies born with the defect stand a much better chance at survival, even if they must wait a year or two for surgery, than children born decades ago, said Holly Hedrick, pediatric and fetal surgeon at Children's Hospital of Philadelphia.

"One hundred years ago you would not do surgery," Hedrick said. "You lived with it. If it got infected, or had complications, you would not survive."

Of greater concern than an omphalocele are the respiratory, cardiac and digestive problems that can accompany the condition, Hedrick said.

 

'On good days, I can touch my toes'

Devon, who stands 4 feet 7 and weighs 70 pounds, said he can shake off an object hitting his stomach.

"It hurts a little bit, but it's not like I need to take a break," Devon said.

In the last year, Connelly said, Devon has worked on his flexibility during physical therapy.

"On good days, I can touch my toes," Devon said while reaching toward his feet.

At night he drinks a thick liquid that keeps inflammation in his esophagus at bay, Connelly said. She is not sure if this is directly related to the omphalocele.

Last year Devon did not miss a day of school because of illness and earns straight A's, Connelly said.

Devon is aware of what he has been through and says he feels deep respect and appreciation for every person who has helped and encouraged him along the way.

"I can't believe I had that [omphalocele] ... knowing what could have happened to me," Devon said.

Connelly is still part of an omphalocele support group — Mothers of Omphaloceles, or "Moos," the same one Kulick belongs to — offering an encouraging word or information for pregnant women and new mothers who have children with the condition.

"I was under the impression that only one child would be born with this," said Kulick, noting her days of isolation before the Internet. "No way was I to know there was another child like this."

She said the group is over 1,000 strong with members from around the world.

"These young ones who come on — we get new members almost daily — with these new ones we are able to say whatever issues you face one of us has dealt with the same thing."

Connelly couldn't agree more.

"The best reason Devon is here today is the knowledge from the support group," Connelly said.

"Sometimes when he does something, not because I'm big with sports, I know what he has been through and how many people said he would never do it. He did it and he's good at it. I feel very proud, not for the sport, but how far he has come."

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Mothers of Omphaloceles, or "Moos," has a Facebook page and can also be found at omphalocele.net.

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Staff writer Debbie Zucco contributed to this report.

bryce.alderton@latimes.com

Twitter: @AldertonBryce

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