Nothing warned Lindsey and Justin Wagner that their second child would be born with a problem.
When little Edith, now 21 months old, arrived, a nurse said, “Not to alarm you, but your daughter has a cleft palate.”
Cleft palates and cleft lips occur when those areas do not form correctly during development. Both situations require surgery.
Cleft lip surgery typically is done at three months of age.
“We feel that particular age balances safety (with general anesthesia) with the ability of very young children to scar better,” said Dr. Joseph DeSantis, board-certified plastic surgeon and the director of cleft lip and palate program at Geisinger. “Children who are very young scar with less of a scar than children who are older.”
The surgery takes about two to two-and-a-half hours, and the child stays in the hospital overnight.
“Keeping them overnight ensures that they are eating well, and everything looks good, and their parents are comfortable with their care,” DeSantis said.
Cleft palate surgery is typically done between 9 and 12 months and takes about two-and-a-half hours.
“At that age, technically the surgery is easier and more successful,” DeSantis said, “because the child has had time to grow a bit, so we have more tissue to work with.”
Lacking a roof
In Edith Wagner’s case, both her hard palate (at the front of her mouth) and soft palate (back by the uvula) were affected.
“She had no roof of her mouth,” Lindsey said. “She had absolutely nothing. Sometimes when she would drink milk, it would go right out her nose. That was rough.”
A cleft specialty bottle, which requires very little sucking, helped her drink.
Edith was also diagnosed with Pierre Robin Sequence (PRS) because her chin was flat — possibly from jamming her hand against it in the uterus, but no one knows for sure.
“I did a lot of ‘mom guilt,’” Lindsey said. “I thought it was my fault.”
She felt better after talking with DeSantis’s colleague, Dr. Christian Kauffman, plastic surgeon and chair of the Department of Plastic and Reconstructive Surgery at Geisinger. As far as they can tell, Edith’s cleft palate was a fluke. Kauffman performed her surgery when she was just shy of 11 months old.
“I kept it really cool. I cried a little bit when I handed her off to the nurse,” Lindsey said. In recovery, she was allowed to hold Edith. “She started crying, but then she saw that I was there, and she snuggled in on my chest.”
Doing surgery before 12 months is important because that’s when children start meaningful speech. The palate closes the nose from the mouth and allows us to make consonant sounds like P, B, S and T. In a cleft palate, air goes out through the nose, making it impossible to form those sounds.
“We don’t like to go too much after 12 months,” Dr. DeSantis said. “We want children to learn those sounds early on.”
Cleft palate patients commonly stay in the hospital two days.
“It does take a little longer to start feeding again,” Dr. DeSantis said. “It can be painful, or it just feels different to them, so they almost have to get used to it.”
All would have been well with Edith, but she developed complications, bleeding more than she should have after the surgery. A second surgery was performed later that day. She had to be intubated and sedated for three days. She then had trouble eating, but once she started that, she was allowed to go home. Lindsey and Justin stayed all six days with her in the Pediatric Intensive Care Unit.
“Our surgery story is not typical,” Lindsey stressed. “It’s not a typical cleft palate repair.”
As she recovered, Edith was allowed to eat only from a bottle. Lindsey sought occupational therapy for her to help her learn to chew and swallow – she had never really developed those muscles. To help prevent reflux, Lindsey learned to feed her with Edith sitting upright against Lindsey’s chest.
DeSantis reassures anxious parents that their babies are in competent hands.
“We do these surgeries pretty much on a monthly basis,” he said. “We also have pediatric anesthetists here who specialize in kids. We have a lot of people involved here. Whatever your child needs is available for them.”
Geisinger has one of four credentialed cleft teams in Pennsylvania. The other three are in Philadelphia, Pittsburgh and Lancaster.
Children with cleft palates, particularly, have a high incidence of ear infections and tend to have more dental problems. They may need help with speech, and their noses can grow asymmetrically.
The cleft team contains an ear, nose and throat doctor who specializes in children, a pediatric dentist, orthodontist, oral surgeon, speech therapist, social worker, pediatric psychologist and a geneticist. The children meet once a year with all members of the team, which then meets as a group to come up with a “game plan.”
“That is coordinated care that is really necessary with a cleft palate child because there’s a lot of components and a lot of specialists,” DeSantis said. “The whole key is to avoid problems or deal with them as early as possible.”
Pressure on parents
Parents of a child with a cleft lip must brace themselves for the reaction and whispers heard when they take their baby out in public.
“It’s very tough on parents,” DeSantis said, adding that parents have cried at seeing the before-and-after photos of his work. “It’s a pretty dramatic change. It’s really life changing for that family. That’s a great thing about doing this work.”
As for Edith, “She’s doing really good now,” Lindsey said. “Chewing and swallowing and able to eat.”
She is small for her age and a little delayed in speech. Her favorite words are “Out,” which she says from her crib in the morning, and “No.”
“It really does get better with time,” Lindsey said. “It seemed like a never-ending tunnel.”
Social media support groups helped her through the rough times, and she credits Kauffman, the nurses and Dana Rubin, physician assistant.
“Dr. Kauffman is a very caring, wonderful doctor,” she said. “It was horrible to go through, but Kauffman and Dana both made it amazing. I can’t say enough good things about them.”