Julia Wynne has dealt with a wide variety of medical issues with fortitude and grace since the day she was born. The 15-year-old from Montana was born with a complex medical condition called VACTERL.
An acronym for a series of congenital defects, VACTERL includes vertebral, anal, cardiovascular, tracheal, esophageal, renal and limb deformities. Children diagnosed with the disorder have at least three of the seven defects. In Julia’s case, she had five, including a significant heart defect in which the valve that regulates blood flow from the heart to the lungs failed to develop.
“She’s had numerous surgeries on her heart, esophagus, trachea and hand,” says Julia’s mother, Renee. “She also was diagnosed with scoliosis, but it paled in comparison to the heart and other issues we faced when she was younger.”
While she was still an infant, cardiac surgeons performed a delicate procedure called Fontan fenestration that created a tunnel from the heart to the lungs to restore oxygenated blood flow to Julia’s body. The family moved from St. Louis to Montana when Julia was 6 months old but continued to return for ongoing care. Over the years, she underwent abdominal and open heart procedures at St. Louis Children’s Hospital (SLCH).
Beginning in 2010, doctors in St. Louis and in Montana started closely monitoring Julia’s spine, which began to curve significantly as she grew older. She was referred to Scott Luhmann, MD, Washington University pediatric orthopedic surgeon at SLCH, specializing in pediatric and adolescent spine surgery. He initially recommended vertebral stapling, a procedure that involves stapling the growth plates of the vertebrae to minimize the growth rate. By 2015, however, Julia’s scoliosis had progressed from 40-degrees to 70-degrees out of alignment. Dr. Luhmann then recommended spinal fusion.
“I had never really noticed the problem too much when I was younger,” says Julia. “Then I started to notice things in the mirror, like that my shoulder was twisting forward and my back was feeling achy while sitting at school.”
If the spinal curvature continued to progress, Julia’s cardiologist in Montana felt that the scoliosis would be more disabling to her lifestyle than even the heart problems. Julia and her family decided to return to SLCH for the spinal fusion in May 2016.
Because of Julia’s heart condition, the surgery had added risk. “Julia’s Fontan fenestration helps her congenitally malformed heart work more efficiently, but it also increases her risk of blood clot or other cardiovascular complications,” explains Dr. Luhmann. “Placing her in a prone position to perform spine surgery is taxing on the cardiovascular system of anyone, but when there is a congenital heart defect, the risk of problems increases.”
Dr. Luhmann worked with multiple care teams, including cardiology, anesthesia, pediatric intensivists and surgical staff to develop Julia’s surgical plan. He also contacted Julia’s pediatric cardiologist in Montana. Significantly, Dr. Luhmann worked with the surgical team to minimize the time Julia was lying face down during spinal surgery to minimize the stress on her heart. A specialized cardiac anesthesia team also was present. Before and after surgery, Dr. Luhmann kept both Julia and her parents informed about the procedure.
“I like to know everything,” says Julia. “I liked that they talked to me and I knew exactly what would happen at all times. Even after surgery, they kept checking on me. A lot of kindness was shown throughout my time in the hospital.”
“Every person treated my daughter with honesty and respect,” says Renee. “I can’t say enough good things about the team and the care Julia received.”
Now back in Montana, Julia is eagerly looking forward to high school where she plans to get involved in speech and debate and pursue her love of reading and writing. A longtime fan of theater, she has worked at the local performing arts center for the past several years. At home, she also has returned to her other passion—baking and cooking.
Two months after surgery, Julia went biking in the countryside for 12 miles with her family. “I’m really fine now,” she says.
“It was an incredibly difficult surgery, but the team was realistic from the start, and Julia knew what to expect,” says Renee.
She adds, “It’s incredible what was done to orchestrate all of the plans and make this as easy as possible on our family. Words cannot express the gratitude I feel to the people at St. Louis Children’s Hospital and to all who have continually cared for my daughter with such competence and kindness. They’ve been an incredible blessing in my daughter’s life.”
To speak with Dr. Luhmann or to refer a patient, call Children’s Direct at 800.678.HELP (4357).