Rylynn Riojas, a typical 5-year-old, loves horsing around at her family’s ranch. But her life has been anything but typical.
Like many parents-to-be, Andrea and Gilly Riojas anticipated the day they would find out whether to plan for pink or blue. But what was supposed to be a simple anatomy scan at 20 weeks gestation turned into much more. Unexpected news of a serious heart condition changed their focus to saving their baby girl’s life.
Rylynn was born with hypoplastic left heart syndrome, a condition causing the left side of the heart to be severely underdeveloped. As planned, Dr. Joseph Forbess, division director of Cardiothoracic Surgery and co-director of The Heart Center at Children’s Health, performed the first of a three-part open-heart surgery series when Rylynn was 4 days old. This allowed the single right ventricle to pump blood to the lungs and body. At 4 ½ months old, Rylynn headed back to the operating room for her second surgery – bidirectional Glenn shunt procedure – which allows most of the upper-body blood to flow directly into the lungs. At this point, she was closely monitored with the hopes of waiting until age 2 or 3 for a final surgery when her lungs were further developed.
Thirteen months later, an echocardiogram revealed declining heart function, and Andrea and Gilly started the transplant listing process which included moving from their ranch in Lampasas to be closer to the hospital.
“It was heartbreaking to take Rylynn away from our home and her horses and cows,” Andrea said.
Rylynn’s PRA (panel reactive antibody) levels were high, meaning her chance of receiving a match was very low. Throughout the next couple of months, Rylynn’s worsening heart function caused her to be moved to status 1A on the transplant list. She desperately needed a new heart.
On the brink of liver failure, doctors at Children’s Health knew they had only one option to keep her alive until a donor heart became available – implanting a Berlin Heart Ventricular Assist Device to help pump blood from her heart to her body.
“Sometimes when tiny patients are waiting for a heart, like in Rylynn’s case, medical therapy isn’t enough, and the Berlin Heart is their only option for survival,” said Dr. Kristine Guleserian, surgical director of Pediatric Cardiac Transplantation at Children’s Health.
But the FDA had not yet approved the device at the time of the crisis. Dr. Guleserian contacted the FDA seeking emergency access to it on a compassionate-use basis, a request the agency approved.
“After Berlin Heart implantation, we saw an immediate improvement – her liver had drastically decreased in size, her eyes were less puffy, and the pressures inside her heart and lungs came down, giving her the chance she needed to wait for a donor heart,” Dr. Guleserian said.
A Thriving Kindergartener
In October 2014, Andrea and Gilly celebrated a special milestone with Rylynn – her third heart “transplanniversary.”
“Three years later, it is wonderfully typical in our world,” Andrea said. “Gilly and I are busy working while Rylynn is attending kindergarten in a class full of new friends. We could not ask for more. Her belly and chest bear the unique and perfect scars of such a painful journey, and we do our best to explain how special she is because of what she has been through.”
“Today, like every day, we are just happy to be with our sweet girl.”