New law helps ease burdens of those with cleft lips, palates

When Lianne Hasegawa-Evans was an adolescent, summer often meant another oral surgery.

She was born with a cleft lip and palate, and needed multiple operations as her face, bones and teeth grew.

“Growing up, I didn’t see a lot of other kids with a cleft lip and palate,” said Hasegawa-Evans, who now counsels families of children with the condition. “Especially in your preteen and teen years, you just want to be like everybody else. You don’t want to feel different. For me it was a very isolating experience.”

Her family had to scramble to pay for the orthodontic work, which, unlike the actual cleft repair, wasn’t covered by private insurance. And her mother had to “figure everything out on her own,” traipsing from one specialist to another.

Children with cleft and other facial anomalies have a lot more support these days, with multidisciplinary medical care, peer group connections and awareness efforts such as Craniofacial Acceptance Month in September.

A new Hawaii law requires private insurers to cover medically necessary orthodontic work of up to $5,500 for oral-facial anomalies. That brings the Aloha State in line with 16 other states and Medicaid insurance.

Hawaii has a relatively high rate of oral cleft, caused when a baby’s lips and mouth do not form properly during pregnancy, leaving an opening. Nationally, about 1 out of every 600 babies is born with cleft lip, palate or both, according to the Centers for Disease Control and Prevention. In the islands the rate is 1 out of 500 live births, according to the state Health Department.

The Kapiolani Cleft and Craniofacial Center gives parents a one-stop shop for coordinated care, bringing together specialists in oral and plastic surgery, orthodontia, audiology, speech pathology, genetics and more. Founded in 2007, it started with a handful of children and now has 520 active patients.

The center accepted 96 new patients last year, 40 with cleft and the rest with various craniofacial, or head-face, problems. They come from across the Hawaiian Islands, American Samoa, Guam and Micronesia.

“I love being able to work with families, with kids in the clinic,” said Hasegawa- Evans, a genetic counselor for the state Health Department and a mother of two. “It’s nice for them to see me and know it’s really OK, you can grow up to have a happy life.”

Oral cleft is one of the most common birth defects in Hawaii, more prevalent than Down syndrome. It is known for its dramatic effect on appearance, but the effects are far more than cosmetic. Cleft hampers basic functions such as eating, speaking, hearing and even breathing.

In most cases cleft occurs without an obvious cause, although it has been associated with factors such as smoking, diabetes, malnutrition and use of anti-seizure medication. Genes also play a role. Rates are higher for Asians and Pacific Islanders, and lower for African-Americans.

“Most people don’t realize how common it is,” said Dr. Michael Pharaon, a craniofacial and plastic surgeon at the Kapiolani center. “Another thing that folks don’t generally realize is that it’s not just one surgery in infancy. Most of the time it involves multiple procedures over the first two decades of life.”

“It’s not just repair of the lip and repair of the roof of the mouth, it’s repair of the dental structure. A lot of that is time-dependent — kids don’t have their adult teeth in infancy. In terms of reconstruction, the dental anatomy is just as important as the external appearance of the nose or the lip, from a psychological and functional standpoint. That’s why this legislation is important.”

The new Hawaii law was named for Anya Maga, a 6-year-old with waist-length red hair who regularly visited the Capitol with her mother to support the bill that became Act 213, introduced by state Rep. Gregg Taka- yama. She was at the final hearing on her birthday and was surprised to be serenaded with the “Happy Birthday” song.

ANYA has already had four surgeries, the most recent to improve her airways.

“This bill will really help a lot of our families, being in that working class, who have private insurance,” said her mother, Michele Pestel-Maga. “It was a tough financial burden for parents to bear. They would delay orthodontics. It is a medically necessary procedure. It is not cosmetic.”

Pestel-Maga is president of the Lifetime of Smiles Hawaii, a family support group, which had its annual picnic Aug. 23 and holds quarterly educational events and other get-togethers.

“It’s for all families and individuals, whether they’re receiving care at Kapiolani or at the clinic at Kaiser or Tripler, or if they are people who are older and may have traveled outside Hawaii for surgery,” Pestel-Maga said. “It’s for all ages. We know that preteens and teens need the support to get through those hard times.”

One of the newest features of the Kapiolani center is “bibliotherapy,” a collection of books that address bullying and facial differences and offer inspiration. The mobile lending library, geared to various ages, is so popular that kids often ask to keep the books. They are gifts of the Sascha Julia Franzel Fund, an endowment created for the center.

“The books help with the recovery of the children,” said Sonia Franzel, Sascha’s mother. “They help with the psychological problems people have because they speak differently, they look differently, they are being bullied. It helps the child and parents understand what’s going on and gives them tools to handle themselves and help others understand what they are going through.”

SASCHA, a voracious reader, volunteered at Kapiolani Medical Center as a high school student, her sights set on becoming a craniofacial surgeon. She died of a severe asthma attack at age 19 in 2011.

Her mother said Sascha’s career goal traced back to her own childhood when she met a little boy at the Nuuanu YMCA who started following her around because they both liked to sing. His appearance scared her at first.

“He had already had a lot of surgeries, but he still had a lot of craniofacial issues,” Franzel recalled. “People were afraid of him because of how he looked. It was a shock; you had to get used to it. The kid was a completely normal, happy child, but he looked very different. They became good friends.”

For her master’s thesis, Hasegawa-Evans interviewed women with oral clefts. One of her questions was whether they would have altered the circumstances of their birth if they could.

“Every single one of them said they would still choose to be born with their cleft,” she said. “They believed it made them more understanding of other people with differences. It made them more appreciative of everything they have.”