Beating All Odds
After overcoming multiple serious complications related to pregnancy, Amy Burk will never forget the moment when she found out the grim prognosis of her “Sunshine.”
“I was 26 weeks pregnant when we were told that Ella not only had hypoplastic left heart syndrome, but she also suffered from a highly restrictive atrial septum defect,” Amy said. “If she made it through the remainder of pregnancy and birth, she would have a 20 percent chance of living.”
Finding the Right Place
Ella’s diagnosis was extremely high risk, and she would need immediate specialized care. Doctors in their hometown of Oklahoma referred Amy and her husband, Jon, to Children’s Health in Dallas to give Ella the best chance at life.
“We couldn’t bear the thought of losing her,” Amy said. “Our baby Ella, whose name means light, was already so very loved and wanted. There aren’t words to describe the heartbreak we felt.”
When the couple met with the team at Children’s, for the first time since Ella’s diagnosis they were hopeful.
“We just felt like we were at the right place,” Amy said.
Children’s Health, the only pediatric hospital in the country that received disease specific certification in fetal heart monitoring from The Joint Commission, provided Amy and Jon with comprehensive care. Amy received a fetal heart echocardiogram, and the couple met with Ella’s cardiologist, cardiothoracic surgeon and fetal heart coordinator. Ella’s heart was monitored for the remainder of the pregnancy, and the team coordinated treatment with Ella’s other caregivers to develop a birth plan.
The goal would be to deliver Ella and transport her to the operating room in 20 minutes.
Highly Specialized Care
Ella was born at 39 weeks gestation, and Amy and Jon were able to touch her hands before she was rushed to the Children’s Health Pogue Catheterization Lab. Dr. Alan Nugent, director of cardiac catheterization at Children’s Health, performed a highly specialized advanced transcatheter procedure to open her atrial septum. After the successful procedure, Ella spent her first week of life on a ventilator before being wheeled to the operating room for open heart surgery.
But as Ella was prepped for surgery, she became unstable and Dr. Joseph Forbess, division director of Cardiothoracic Surgery and co-director of The Heart Center at Children’s Health, knew that she would not make it through the planned procedure. Instead he placed bands around her pulmonary arteries to drive more blood to the body and less to the lungs. This allowed her lungs and kidneys to improve and stabilize for one week before the Norwood procedure which allows the single right ventricle to pump blood to the lungs and body.
“We put the flag in the ground and showed the rest of the world how to treat this diagnosis with placement of pulmonary artery bands after transcatheter septostomy,” Dr. Forbess said. “Now we use this approach with every patient who comes to us with Ella’s condition.”
Ella’s next open heart surgery was at 5 months old. Dr. Forbess performed the Glenn shunt procedure to allow most of the upper-body blood to flow directly into her lungs. Ella underwent the final surgery of the three-part series, the Fontan, when she was 2 ½ years old. This improved her blood’s oxygenation.
‘Ella is our miracle’
Amy recounted many moments she will never forget when the Children’s Health staff went above and beyond to help Ella and her family through the ups and downs of her journey. As for now, Ella is back home in Oklahoma and makes trips to Dallas to be monitored regularly by Dr. Matthew Lemler, pediatric cardiologist at Children’s Health. Amy said they are taking it one day at a time.
“Our sweet sunshine is 4 years old and every day we are reminded that she is a miracle,” Amy said. “She is a little performer and loves ballet and tap dance classes. She tells us she loves us all the time. We are completely in love.”
Just like most parents, Amy and Jon have hopes and dreams for their daughter as she grows older.
“We hope Ella will inspire those who come after her and bring awareness to the Congenital Heart Disease world, and we hope that she gets to live a full life.”