Watch little Alexis (Lexi) Melton, 7, Irish dance across a stage, or effortlessly ski down a slope, and you would never guess the hurdles she’s had to overcome in her short life.
When Lexi was born, her family didn’t know if she would survive. Her extremely rare craniofacial condition literally makes her one in a million. But her story isn’t one of heartache, it’s a story of hope.
Lisa Skylynd, Lexi’s mother, never let anything hold Lexi back, not even her complex medical condition. She always told her,
“If you want to dance, you should dance. If you want to run, you should run.”
And that’s exactly what she’s done.
“Lexi is not only alive today because of the care she received at Seattle Children’s; she’s living her life to the fullest,” added Lisa.
The beginning of a long journey
Skylynd’s pregnancy took an unexpected turn around 27 weeks. When many parents would be placing finishing touches on their baby’s room, their family was rushing to the hospital. Amniotic fluid was building up in Skylynd’s belly, up to three liters a week. To add to the complications, ultrasounds revealed there was something wrong with Lexi.
“Until that point, it had been such a seamless pregnancy,” said Skylynd. “The only thing we knew from the ultrasounds was that she had a really tiny jaw. It was overwhelming.”
At 34 weeks, baby Lexi was born at the University of Washington Medical Center. A team of doctors from Seattle Children’s were there to assist with the high risk delivery. The collaborative teams knew Lexi would need assistance breathing as soon as she was born and would likely need a tracheostomy because her tiny jaw made it impossible for Lexi to breathe on her own. When Lexi was stable, she was transported by Seattle Children’s transport team, national leaders in safe newborn transport, to Seattle Children’s Level IV Neonatal Intensive Care Unit (NICU).
Lexi was clinically diagnosed with a rare condition called auriculo-condylar syndrome, which caused the malformation of her jaw. Through detailed image analysis,discovered that her lower jaw was a mirror image of the upper jaw and it was fused to her skull.
“Lexi’s condition is extraordinarily rare,” said Dr. Michael Cunningham, medical director of Seattle Children’s Craniofacial Center. “The prevalence is less than one in one million. Without treatment it’s life threatening. Before Lexi, we’d only seen two other children with a similar genetic condition.”
For two months, Lexi stayed at Seattle Children’s. The diagnosis was a lot to take in for the family, but thankfully Seattle Children’s Craniofacial Center was there to help provide support. Seattle Children’s Craniofacial Center is the largest, most comprehensive craniofacial center in the country, and one of a select few centers in the U.S. that has every discipline necessary to provide customized care to meet the needs of any patient with a craniofacial diagnosis. From reconstructive surgery to craniofacial pediatrics and orthodontics, the center includes more than 50 healthcare providers from 19 specialty areas.
“It was very emotional,” said Skylynd. “At the time it was hard to understand how compromised her airway was or the complexity of her condition. She literally didn’t have an airway and could not breathe without the tracheostomy. If the tracheostomy were to ever come out, there was a possibility we could lose her. Living with that reality, and not being able to travel or do activities we loved like camping or hiking was really challenging. We knew it was going to be a huge impact on what we envisioned our life would be like, but we were also so very grateful to have Lexi in our lives.”
Dr. Richard Hopper, surgical director of Seattle Children’s Craniofacial Center, Lexi’s surgeon and one of the many doctors who cared for her added, “A tracheostomy is like only being able to breathe through a big straw that’s prone to having problems at any time. That burden and worry on a parent is unimaginable.”
Hopper, along with the rest of Seattle Children’s craniofacial team, went to work devising the best treatment plan to improve Lexi’s quality of life, giving her the ability to live without a tracheostomy and with a new jaw.
Forming a new jaw
When Lexi turned 3 years old, Hopper met with the family to talk with them about reconstruction, a process that would eliminate the need for the tracheostomy. The procedure, a trans-facial mandible distraction, would literally grow a new jaw for Lexi and open up her airway.
“Mandible distraction is pretty common,” said Hopper. “But the severity of her case and the amount of growth that was required was very different.”
The first hurdle: Lexi needed more bone for her lower jaw. Once that occurred, pins could be placed through her facial bones and lower jaw, and a distraction device could gradually stretch her bone and create a jaw that didn’t exist previously.
“We began by using one of Lexi’s lower ribs to add bone to the back of her jaw,” said Hopper. “After adding the distraction device, it works like the winding of a clock. Twice a day her parents needed to turn the device and it gradually formed new bone. We essentially took her old jaw and made it into a new jaw.”
Hopper added, “The true success and joy of this procedure, however, was helping Lexi be able to breathe and finally live without the tracheostomy.”
By the time Lexi was 4 years old, her jaw had grown into place and she was tracheostomy free. She was finally able to breathe on her own, and also laugh and speak for the first time.
“Living without a tracheostomy changed our lives,” said Skylynd. “Things like taking a bath were life-threatening before the surgery. Now she’s taking swim lessons.”
A new lease on life
Today, Lexi is a fun-loving, energetic 7-year-old that can be found on hiking trails with her family, on the dance floor, on the ski slopes or in the pool. She’s embracing what life has to offer and isn’t letting her condition slow her down.
“I still remember the day her mom sent me a photo of Lexi on the ski slope,” said Hopper. “To actually see this young girl who has overcome so much doing the same activities my children enjoyed when they were younger, it was incredible. As a surgeon, I’m glad the surgery and distraction was successful. As a father, I see it differently. I see the joy in Lexi’s eyes and it brings me joy. It’s why we do what we do.”
“She’s been through so much, but you’d never know it,” said Skylynd. “We’re so very grateful for Seattle Children’s. We never stopped believing. It can feel like everything is falling in on you, but there is so much hope. You have to be strong.”
Lexi will need to undergo another reconstructive surgery in 2017. The surgery will hopefully allow her to reach the next milestone of being able to eat on her own for her first time. For now, however, she’s simply enjoying life.