Moultrie infant recovering from life-threatening disfigurement

Lori Glenn

MOULTRIE — Chin up, Doodlebug!

Around Christmastime, locals might have seen T-shirts bearing those words of encouragement around town. Their meaning is at once sad and hopeful.

First-time parents Tramese and Jeremy Merritt, owners of Georgia Boy T-shirt graphics and design company on Overlook Drive, were overjoyed at the sight of their new baby girl, Raley. But soon afterwards, Karen Newberry, a nurse at Colquitt Regional Medical Center, broke the news to the Merritts that she had to give their baby a feeding tube. Local pediatrician Dr. Andy Wills examined Raley and advised that she had a rare disorder, Pierre Robin Sequence (PRS). PRS, which occurs in one in every 9,000 births, is potentially fatal if left unchecked.

Because of her position in her mother’s womb, Raley’s jaw failed to develop properly. Her tongue was in the way so that the palate didn’t close. As a result, she couldn’t breathe or eat very well. The danger was that her tongue would fall back, cutting off her air supply.

Fortunately for the Merritt’s, Wills found craniofacial surgeon Dr. Fernando Burstein, medical director at the not-for-profit Children’s Healthcare of Atlanta at Scottish Rite. The Children’s Healthcare of Atlanta Center for Craniofacial Disorders is one of only a few centers in the country to perform the unique surgery that can correct PRS.

Burstein, who gained national fame when he operated on Baby Noor, and his counterpart Dr. Joseph Williams are PRS experts and have pioneered a cutting edge surgery, mandibular distraction, that pulls the jaw forward to alleviate the danger. Burstein has performed hundreds of these procedures with a 98 percent success rate, he said, over the past decade. He and Williams have successfully performed the procedure on older children and even teen-agers, he said, and have since perfected it.

Burstein’s resume is as impressive as it is long. Among his accomplishments, he was named chair of the American Academy of Pediatrics Plastic Surgeon Section in 2006, as one of Atlanta’s Top Physicians by Atlanta Magazine 2000, 2001 and 2005 and as one of the “Best Plastic Surgeons” by Atlanta Magazine in 1999.

Raley was 10 days old by the time she had the surgery, said Tramese. During the first 10 days of her life, Raley gasped for air and at times turned blue from lack of oxygen, she said. Feeding difficulties resulted in obvious dehydration. It was a harrowing time for the Merritts.

“I was so scared that I wouldn’t even be able to even feed my baby,” Tramese said, choking back tears.

But hope was just four hours up the road. The Merritts expected a consultation and instruction on how to feed Raley. Instead, said Tramese, the Merritts were stunned that Raley was immediately placed in intensive care and scheduled for surgery the next day.

Raley wasn’t breathing well at all when she arrived at the Atlanta center and went straight into intensive care, Burstein said.

“It would have been very difficult at home, frankly, for her to have gotten a breathing tube in, so she could have been in real trouble, so I was really glad they sent her up,” the surgeon said.

In the old days, a child would have to undergo a tracheostomy to breathe, he said. Some would grow out of their dependency on a trach in toddlerhood. Some less fortunate had to use a trach their whole lives, which requires round-the-clock nursing, he said.

Children’s Healthcare of Atlanta Center for Craniofacial Disorders was one of the first centers to perform mandibular distractions. First, a small incision is made on either side of the jawbone to access the jaw. The jaw is then cut with a tiny saw and affixed with the distracter that acts like a screw jack. Tramese described the distracter as looking like a door hinge.

The resorbable mandibular distractor was adopted from a technique that has been around for decades to lengthen fingers, arms, even legs. Soviet doctors in the 1960s used the technology to make dwarfs and midgets taller.

Raley had what looked like antennae protruding from each of her cheeks — hence her nickname, Doodlebug — which were adjusted using a small screwdriver for two weeks post-surgery. The distracter slightly stretches the cut segments of the jaw apart only a millimeter each day of treatment, and as it does, bone grows in the gap. Raley’s jaw was pulled forward a centimeter, a big difference for a newborn, Burstein said.

What makes Raley’s surgery so cutting edge is an advancement developed by Burstein and Williams. They’ve designed a distractor that the body can absorb like a dissolvable stitch.

“So the cool thing about this is that you can do the procedure, get everything stretched out and the little gizmo gets taken out instead of [a surgeon] having to take it out,” Burstein said.

The risks are real.

“If you don’t do it, you can die,” he said, adding there are risks associated with the surgery just as other serious surgeries.

Raley was placed on a breathing tube after surgery and could only be touched on her fingers. The pain of seven days of not being able to hold her first baby was evident by tears that rolled down Tramese’s cheeks as she relayed the ordeal. Raley spent her first Christmas in the hospital.

She isn’t out of the woods quite, Burstein said. She still needs surgery to correct her cleft palate, but she is lucky. Sometimes, a doctor will diagnose the cleft palate but miss the PRS.

“It can be common to misdiagnose in the pediatric office because they probably don’t watch the baby feed and probably aren’t as familiar with a recessed chin or jaw as we would be,” said registered nurse Alison Repass at Children’s Center for Craniofacial Disorders.

“We’ve seen a lot of babies like Raley come in here where they have been discharged from the hospital and they’ve seen their pediatrician, and parents are really kind of surprised when we show them that ‘look, your baby is having difficulty feeding,’” she said.

The center, of course, treat patients with cleft palates as their main problem. A cleft palate could be the result of a number of factors, including genetics or environmental exposure, such as to the acne medicine Acutane. One in every 2,000 babies are born with a cleft palate as the sole abnormality, Repass said. A genetic syndrome is found in about 50 percent of cleft palate cases where cleft palate is the sole disorder, she said.

Since babies with cleft palates don’t have the proper musculature to suck properly, the center’s feeding team teaches parents how to properly feed their infants with specially-designed bottles.

Although she still relies on a feeding tube, Raley uses two kinds of special bottles, Tramese said, so she won’t forget how to eat. When she turns 1, she will be ready for speech therapy and will be evaluated for dental care and any further surgery. She will travel to the Atlanta center on a yearly basis after that for follow-up.

While at Scottish Rite, the Merritts were struck by how they were scooped up into the care of the varied staff who attended to any need before they could even think of it.

“They’re extremely thorough. It’s amazing,” Tramese said.

“Children need a multidisciplinary approach when they have a facial difference or a craniofacial disorder,” Repass said. “ The Children’s Craniofacial Center has multispecialists who are all are involved on one team. ... We also collaborate the child’s care, so when a child comes to the craniofacial center, they can see a surgeon, a dentist, a speech/language pathologist and an orthodontist all in one visit.”

Many on staff that they encountered prayed for their child’s recovery — from housekeeping all the way to the doctors. It was the Christmas season, but the Merritts have a sense that the children’s hospital is just as caring year round.

“We met a Christian every step of the way,” Jeremy said.

There were teddy bears everywhere, nurses crocheting beanies, gifts of blankets and an outpouring of sincere support. They felt as if they were the ones being swaddled in the tight folds of their expertise and confidence that everything was going to be all right.

Two months later, the incision marks are barely noticeable, and Raley is alert and intent on learning about the good in her world now that she has a break from the pain. Her mother can’t hold her enough. During the interview, she smiles at Tramese and issues gravelly coos and hiccups. Tramese marvels at her daughter.

“She’s a strong little girl,” she said, kissing Raley on the head as she did every time she looked at her bright-eyed girl throughout the interview.

Ironically, Tramese knows a thing or two about resilience. As a 4-month-old infant, she underwent surgery for a club foot. She remained in leg braces for two years. During her teen-age years, she had a devastating car crash and extensive reconstructive surgery. Just like looking at her daughter now, you couldn’t guess anything had been wrong. They are blessed, she said.

“She’s got some tired guardian angels that’s for sure,” Tramese said.

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