Not every 11-year-old can say he’s danced in front of a crowd of 500, but Will Sexton of Bauxite can. At an age when most boys are avoiding the dance floor, Will stole the show with the help of Arkansas personality Steve “Wild Man” Wilson last year when the duo knocked out a boot-scootin’ boogie at the Dancing With Our Stars gala benefiting the Children’s Tumor Foundation of Arkansas.
Will’s mother Ashley remembers it as a show-stopper in more ways than one. A few years ago, Will was diagnosed with neurofibromatosis (NF), a neurological disorder that’s the target of CTF’s efforts, and the family had been working to come to grips with the diagnosis ever since.
“Will is still trying to wrap his head around it. He can feel real anxious about it and so we haven’t given him more information than he’s needed,” she says. “Being an 11-year-old boy, he’s getting to that age where he’s a little bit self-conscious about everything.
“For him to see he’s not the only one [with NF] and to see that there are many kids out there with many different levels of it, dealing with different aspects, and to see that he’s not alone has been an encouragement to him.”
NF is the most widespread disorder you’ve never heard of. A genetic condition that causes tumors to grow on nerves throughout the body, NF has the potential for serious complications including brain tumors, cataracts and other vision problems, scoliosis, learning difficulties and chronic pain.
NF is broken into three types. Neurofibromatosis Type 1 (NF1) is by far the most common and usually diagnosed in childhood while NF2 and schwannomatosis are more rare and generally diagnosed in older teens and adults.
Like a lot of people, Sarah Wengel didn’t know anything about NF prior to performing at Dancing With Our Stars three years ago, but learning more about the disorder and meeting affected families has made her a passionate advocate. She now sits on the CTF advisory board.
“There’s such a low awareness of the disease; it’s actually more common than most people realize,” she says. “NF1 affects one in 3,000 births. That’s more common than cystic fibrosis and Duchenne muscular dystrophy.”
As the daughter of Gov. Asa Hutchinson and First Lady Susan Hutchinson, Wengel has had a ringside seat to the challenges of addressing health issues in a poor, rural state like Arkansas. On the CTF board, she’s seen how much more difficult the matter becomes when the problem isn’t a household name.
“I think a lot of parents don’t even know that’s something to be concerned about,” she says. “And the more spread out a state is, like Arkansas, the harder it is for families to have the resources to get to a city like Little Rock for a proper diagnosis.”
This is why much of CTF’s work goes toward educating Arkansas’ medical community on recognizing the disorder across all specialties statewide. It’s easier said than done; NF’s subtle early symptoms — dark skin patches called café au lait — are all too often dismissed as mere birthmarks, as in Will Sexton’s case.
“We noticed that Will had these skin discolorations from the time that he was born, but it never caught the eye of any doctors,” Ashley says. “When he was seven, we took him in for an ear infection and that doctor happened to notice a cluster of the café au lait spots. She got the ball rolling from there.”
Lesley Oslica, CTF chapter president, relates to what the Sextons and every other NF-affected family are going through and then some. It’s wasn’t that long ago she was in their shoes; a concerned mother who’d read about NF and, noticing her young daughter displayed the telltale dark spots on her skin, brought the matter up with her pediatrician.
“He dismissed my concerns and actually chastised me, saying, ‘You shouldn’t be concerned about that; don’t believe everything you read,’” she recalls, her voice still simmering. “I left feeling very stupid for overreacting.
“Four and half years later I take our daughter to the dermatologist for dry skin and he noticed the café au lait spots, the same ones I had asked about. He said have you had these checked on and I said, yeah, our pediatrician said it was nothing to worry about. And he said, ‘Well actually she has neurofibromatosis, you need to get her to Children’s Hospital and get her diagnosed.’”
Once the diagnosis was confirmed, Oslica remembers the additional difficulty of sorting through all the required medical specialists and moreover, getting them to work in partnership on her daughter’s treatment.
“When we were going through NF, I had to make all the appointments separately,” she says. “We went to cardiology, we went to neurosurgery, we went to neurology and endocrinology and nephrology and all these specialists. But none of those doctors knew we were going to all those places and nobody was talking to each other.”
Going through that, Oslica envisioned a better way for NF-affected families. She started working with the national CTF organization in 2001 and officially launched the Arkansas chapter in 2004. She also set her sights on a dedicated NF clinic, an idea for which she found a willing ally in Dr. Greg Sharp, pediatric neurologist at Arkansas Children’s Hospital.
“At that time, people thought they had to go to St. Louis or Dallas for the closest NF clinic,” she says. “I said listen, the Children’s Tumor Foundation has a national NF clinic network where we could get networked nationwide so that when people click on the map in Arkansas looking for a doctor, they find our clinic.”
Today the clinic, part of the neurology department at ACH, dedicates time every month exclusively to NF patients and manages a waiting list to get others in as needed.
“It’s working great now and we’re getting everybody through the system and there’s not a big waiting list to get through the NF clinic,” Oslica says. “But at the same time, the clinic is full every single time they hold it.”
The clinic also fulfills another of Oslica’s goals, to bring all needed specialties under one roof to improve communication among departments and reduce the amount of legwork for families.
“I think one of the things we’re doing right is that we’re very dedicated to having a multi-disciplinary clinic where we have neuropsychology, neuro-oncology, neurology and genetics in addition to having our CTF representative present every single time,” says Dr. Kevin Bielamowicz, pediatric oncologist and a member of the NF team.
“That doesn’t mean the family has to see everybody every time, but just that everybody is available and everybody meets new patients. We have a clinic coordinator that reaches out to families and kind of funnels all the contacts through that one person. We’re very dedicated to doing that and doing that well.”
Bielamowicz, who came to Little Rock from Houston two years ago, says Arkansas faces challenges in providing services, but that progress is being made.
“Medicine is just so specialized these days and [physicians] have to be aware of so many things,” he says. “With regard to patients with neurofibromatosis, I do feel like primary care physicians are good at recognizing some of the early features of the disease.
“I think, in general, we are getting better at working to educate pediatricians in the community and establishing our program and our multidisciplinary clinic as the go-to place for patients with this condition.”
Along the way, CTF Arkansas formed an advisory board of community movers and shakers like Wengel and organized several events to help fund research and pay for programs, including one that sends NF-affected teens to summer camp. Wengel says working alongside affected families is a moving experience.
“A few people on the board have children with the condition,” she says. “It’s been so touching and emotional to sit at some board meetings where they had just recently gotten the diagnosis. Just to see that and hear the raw emotion in their voice; they could barely even speak about it.”
Wengel has made full use of her access to the state’s top executive on behalf of these families, such as gaining a proclamation of May as NF Awareness Month in Arkansas. Gov. Hutchinson has participated in other ways, too, like throwing out the first pitch at an Arkansas Travelers game last season on NF Awareness Night.
“The kids all thought that was super cool,” Wengel says. “We had a big family night and that was a lot of fun. They all got to hang out together; it was just a great night.”
Everyone in the state organization — from the advisory board to the events committee to Oslica herself — are volunteers, which means every dime raised goes to serve families, pay for programs and fund research. And that investment is beginning to yield dividends with several promising drugs in trials, the first such medications to be developed.
All in all, it’s been a successful ride for CTF Arkansas. While a NF diagnosis is no less traumatic — and as-yet incurable — the mechanism for dealing with it is much easier for Arkansas families to navigate, thanks to the organization’s efforts. Still, to the surprise of no one who knows her, Oslica sees her mission as unfinished business.
“I want NF to become a household name, that’s one of the goals,” Oslica says. “The other thing is getting physicians and our medical field and our NF community connected nationally so that correct practices and standards of care are known to every physician in Arkansas.
“Our tagline is ‘Ending NF Through Research,’ but that’s a pretty big goal, so it’s also about what we do in the interim and what the action steps are to get there. We have made such great strides. We still have a long way to go.”