Riley Kid Harper Gray is Standing Strong
With sunshine peeking through the clouds on a Friday in February, Harper Gray, teetering with her arms outstretched, took one step, then another. First steps are memorable for any family, but for Harper and her parents Brittany and Austin, these first little steps—five in all—were monumental. Just more than a year ago, Harper’s legs were conjoined from the pelvis to the ankles, a characteristic of infants born with sirenomelia or “mermaid syndrome.”
The Grays found out about Harper’s condition during a routine ultrasound in the fifth month of Brittany’s pregnancy. Sirenomelia is a rare congenital disorder, occurring in approximately one in 100,000 births. In addition to full or partially fused legs, newborns with sirenomelia can have serious abnormalities, including deficiencies in or the absence of major life-sustaining organs. Most babies born with the condition die shortly after birth.
“The scariest thing we were told was that she might not survive,” Brittany said. “It was shocking and upsetting, and all we could do was just start praying.”
Following the initial ultrasound, the Grays were referred to a team of specialists at Riley Children’s Health. Using advanced fetal imaging, Riley physicians were able to tell the Grays that Harper had one small kidney, which hadn’t been detected in earlier exams. The imaging also showed that it was likely her body would be unable to remove waste. Born on May 8, 2021, Harper immediately underwent a colostomy at Riley Children’s and spent the first 33 days of her life in the neonatal intensive care unit. While she did
remarkably well, there were ups and downs during those first six months. Challenges with feeding and two tears in her esophagus—one that resulted in life-threatening complications—pushed back the anticipated surgery to separate her legs until April 2022.
“Harper’s situation emphasizes just how important it is to have a place like Riley that offers all of the expertise and services that are critically important, not only in her survival, but also getting her through these complex reconstructive surgeries,” said Gregory Borschel, M.D., division chief of pediatric plastic surgery at Riley Children’s. “So much had to happen to get Harper to the point where she could be eligible for this operation on her legs.”
Dr. Borschel, who came to Riley Children’s in early 2021 after more than a decade at The Hospital for Sick Children (SickKids) in Toronto, Canada, is an integral part of a team of Riley experts in plastic surgery, orthopedics, urology and pediatric surgery that managed Harper’s care. Led by Dr. Borschel, the surgery to separate Harper's legs is one of the few such surgeries ever performed in the world.
“Many of us at Riley have global connections, and I was fortunate to be connected with a plastic surgeon in Italy who had done an operation like this about 10 years ago,” said Dr. Borschel, a graduate of Johns Hopkins University School of Medicine. “We talked through what went well and what could have been improved. Leveraging that experience was very helpful in coming up with a unique plan for Harper.” That plan included preoperative imaging evaluating the blood vessels in Harper’s legs to determine if arteries and veins would need to be reconstructed. The imaging revealed that each leg had its own blood supply, which made the separation more feasible. To help avoid scar contractures that would limit Harper’s mobility, Dr. Borschel used a series of zigzag incisions. The operation took seven hours and required about 1,000 stitches. In addition to separating Harper’s legs, Dr. Borschel did some surgical preparation for reconstructive procedures when she is older. With her two legs wrapped in bright pink bandages after surgery, Harper took it all in stride.
“She was sitting up and smiling the next day,” Brittany recalled. “She kept looking at her legs and kicking them; it was crazy how well she did.”
Austin, an occupational therapist with skills that have helped with Harper’s therapy, added that Dr. Borschel did a great job of keeping the couple informed, sending updates out to them during the surgery, explaining what he did and what they could expect.
“For as stressful of a situation as it was, it went very smooth,” Austin said.
Following a four-day stay in the hospital, Harper began physical therapy, working to build strength in her legs. Three months after surgery, she was bearing weight on her legs and pulling herself up.
“When Brittany sent over the first videos of Harper standing on her legs, it was like Christmas for our team, seriously,” Dr. Borschel said.
Much of the movement was possible thanks to Dr. Borschel’s meticulous work on Harper’s sciatic nerve during the leg separation surgery. Instead of splitting off into both legs at the pelvis, her sciatic nerve branched off at about knee level, an anomaly that to Dr. Borschel’s knowledge has never been clinically documented.
“To address this surgically, we worked under magnification inside the nerve, dividing it up meticulously from knee level all the way up to her pelvis,” he explained “We were trying to preserve every single important piece, and fortunately, we saw that she was moving her legs.”
A fellowship-trained pediatric plastic and reconstructive surgeon, Dr. Borschel specializes in pediatric nerve injuries. He pioneered the corneal neurotization procedure to treat neurotrophic keratopathy, a blindness-causing condition in which the nerves in the cornea are either absent or damaged. People with neurotrophic keratopathy lose feeling in the cornea, making it vulnerable to repeated injury and damage. Riley is the world’s busiest center for pediatric corneal neurotization, a minimally invasive surgery in which nerves are redirected from other parts of the body into the cornea to help prevent blindness. Dr. Borschel’s lab at Indiana University School of Medicine is currently focused on studying the causes of neurotrophic keratopathy with the goal of uncovering new and better treatments.
“While we were at Riley with Harper, we heard the stories about how Dr. Borschel helped restore vision to someone who couldn’t see,” Brittany said. “He can make miracles happen; he’s done some really incredible things.”
Now, more than a year after the surgery that made Harper’s first steps possible, the Grays are filled with gratitude for their little miracle. While Harper is still under the care of Riley specialists for dialysis and is facing future surgeries, Brittany and Austin say she has always been a very happy girl. They are in awe of how far their daughter has come, how strong and resilient she is and how thankful they are for the world-class care they are receiving at Riley Children’s.
“We try and live to the fullest and make the most of every day with her,” Brittany said. “We just feel so lucky that we’re where we are and have access to Riley.”