Children's Hospitals Today - Summer 2016 - 32

child'sstory A dancer's distraction A team at Seattle Children's constructs a new jaw for a patient and improves her quality of life. W hen Lisa Skylynd talks about her daughter, the first thing she says is, "Well, she's a dancer." She goes on to describe a social 7 year old who half walks, half sashays from one place to the next. Dance is one of Lexi Melton's favorite hobbies. At 3 years old, it was one of the few physical activities she could participate in without endangering her life. Lexi was born with auriculocondular syndrome, a congenital condition that left her with a lower jaw that mirrors the structure of an upper jaw. There have only been 24 cases reported in medical literature. After she was diagnosed with her condition in utero, The University of Washington Medical Center consulted with Seattle Children's, where she would Lexiʼs skull Average childʼs skull be treated after delivery. Seattle Children's then formed a team and a plan that gave Lexi the best chance of surviving birth. "We are grateful for the team members at Seattle Children's because they are what kept Lexi alive and gave her the opportunities she has today," Skylynd says. Lexi was born via C-section and rushed to Seattle Children's after she was stabilized with a breathing tube. At 3 days old, she had her first of 12 surgeries to install a tracheostomy. With her breathing stabilized and her nutrition coming from an NG-tube, Lexi passed the first crucial point in her life until she was 8 days old and had a bilateral intraventricular hemorrhage, a bleed in her brain. "She almost died that night; but they were able to nurse her through it," Skylynd says. Lexi recuperated, but she had hydrocephalus and needed a shunt. When she was 6 months old, Lexi had another surgery to replace her NG-tube with a G-tube. The new parents had to learn as they went, but Skylynd says they were lucky for the strong parent education program at Seattle Children's. "Understanding is something that comes with time and experience," she says. "The first six months with the trach were a huge learning curve." While the tracheostomy allowed Lexi to breathe, it led to a series of respiratory infections and inpatient stays, leaving her parents in constant worry. "We knew if the 32 CHILDREN'S HOSPITAL S TODAY Summer 2016 Lexi Melton with her new lower jaw. trach came out, we had seconds to get it back in," Skylynd says. Shortly before Lexi's fourth birthday, Richard Hopper, M.D., division chief of plastic surgery and surgical director of the Craniofacial Center at Seattle Children's, performed a bone distraction, taking rib bone and attaching it to her jawline with hardware her parents turned twice a day to trigger new bone growth. The new jaw opened her airway enough that the tracheostomy was removed, and Lexi could participate in activities like swimming, skiing and hiking with her family. In 2017, Lexi will have another surgery to create a jaw joint, which will allow her to eat independently and improve her articulation. Hopper and his team will plan out the surgical movements virtually to minimize the dangers of working below the skull base, where two large arteries and several important nerves are located. "It's going to be a challenging surgery, and we will have to use some new techniques and invent some new ones for Lexi's case, which will benefit other kids," Hopper says. She will also need several other surgeries until she is physically mature because her jaw will not grow with the rest of her body. "Medical teams are innovating every day," Skylynd says. "There will be options that work better, more consistently and are less painful and less invasive, which I find inspiring and hopeful." To help with such innovations, Lexi's parents donated her DNA for research. Along with other patients' DNA, a team of experts from around the world, including Michael Cunningham, M.D., division chief of Craniofacial Medicine and medical director of the Craniofacial Center at Seattle Children's, used this donation to discover two genes that may have caused Lexi's condition. "It starts with families being interested in trying to identify a cause and enthusiastic about being in research," Cunningham says. "Even though it doesn't have a direct impact on the donors, they participated because they wanted to help other people." Hopper usually only gets to see children in the clinic, where they are often nervous, so seeing Lexi perform an Irish dance was a special moment. "It's like there are sparks coming off of her as she's whirling around with her Irish dress and socks on," Hopper says. "It just typifies Lexi. She gives more to the world than she takes." -Kaitie Marolf Send questions or comments to magazine@childrenshospitals.org. childrenshospital s.org http://www.childrenshospitals.org

Table of Contents for the Digital Edition of Children's Hospitals Today - Summer 2016