NORMAN — A tangle of lifesaving wires and tubes makes it too cumbersome for Elliot Burr's parents to hold her. So when baby Elliot's first Christmas arrives Wednesday, her parents will have to stroke her tiny foot or fragile hand.
Born with a rare and complex heart defect, Elliot is recovering in a Dallas hospital from the first of a series of surgeries she will face. It's the most delicate stage on the road to helping her heart work as if it were whole.
Her condition — hypoplastic left heart syndrome — means the left side of her heart is critically underdeveloped, and doctors will essentially reroute her circulation so the right side of her heart can take over. Thirty years ago, the condition was a death sentence, explained Dr. Kent Ward, a pediatric cardiologist at The Children's Hospital at OU Medical Center.
“Once we diagnosed it, we couldn't do anything for them. We gave them to their moms and let them hold them for a week or so,” he said.
But with advances in medicine, Ward said hypoplastic left heart syndrome patients now have an 85 percent chance of survival after undergoing three crucial surgeries, the first about 2 weeks old, a second about 4 to 6 months of age and a final surgery between 2 and 4 years old.
Elliot's first surgery, called the Norwood procedure, was performed Dec. 7.
“We were trying to stay positive about it and realize this is what needed to happen. It was really the only course of action we had,” said Elliot's father, Dan Burr, of Norman.
Lacking a specialized surgeon to perform a Norwood procedure, the OU Medical Center has been referring patients to medical facilities in other cities such as Dallas, Ward said. But the hospital recently hired Dr. Harold Burkhart, a pediatric cardiothoracic surgeon at the Mayo Clinic, to join the staff in Oklahoma City, meaning babies like Elliot, and their families, won't have to uproot to another city for treatment any longer.
“Not everybody has the knack to do this operation. It just takes the right mindset, the right hands, the right skills ... these surgeons are not a dime a dozen,” Ward said.
Not the norm
Burr and his wife, Mandy, spend each day standing over their tiny baby's incubator, talking and reading books to the girl. Just six weeks ago, they brought home a healthy, 6-pound, 12-ounce baby with no indication something was wrong.
An ultrasound scan at 17 weeks of pregnancy didn't find any abnormalities, Burr said. Dr. Ward said that's not the norm. More than half of babies with hypoplastic left heart syndrome are discovered in utero.
A couple of weeks after they brought Elliot home, the Burrs noticed she was having bouts of labored breathing. On Thanksgiving, her health had worsened and they took her to the emergency room.
Elliot was diagnosed that day and referred to Children's Medical Center in Dallas. The first major step in her treatment was the Norwood operation, which reconstructs the aorta and connects it to the heart's lower right chamber. This allows the right ventricle to pump blood to the lungs and the body, according to the Mayo Clinic.
“It's one of the most difficult operations we do in pediatric cardiology and is one of the highest mortality risk operations,” Ward said.
While their daughter's heart is constantly monitored in the hospital, the Burrs are living in housing with Ronald McDonald House Charities. The doctors say it could be weeks before Elliot can leave the hospital, and then she will need to stay in Dallas for a few more weeks in case urgent care is needed.
Michelle Hernandez, Dan Burr's aunt, is helping to raise money to support the Burr family while Elliot is in the hospital. Neither parent has any income right now, and the expenses — doctor bills, housing at $15 a night, train rides to Norman — are piling up.
She set up an account at Tinker Federal Credit Union where people can donate.
Being in a hospital room day in and day out is difficult for the Burrs, and the feeling is compounded by being away from their 4-year-old son, Owen, who is staying with family in Norman.
“When things are going well, they are optimistic. When things are not going well, it's pretty close to fragile,” Hernandez said. “It's a roller coaster.”
Dan Burr said their hope is to be transferred to OU Medical Center as soon as possible. The next major step in Elliot's journey is to undergo a bidirectional Glenn procedure, which reduces the work of the right ventricle by allowing it to pump blood mainly to the aorta.
She'll face a third surgery, the Fontan procedure, between 2 and 4 years old. That will allow the rest of the blood coming back from her body to go to her lungs.
Basically, the operations reverse the blood flow in a person's body.
“It's not the optimum circulation. But you can survive with basically a two-chambered heart,” Ward said.
But the heart, a muscle, can't always keep up. Many hypoplastic left heart syndrome patients experience heart failure after 10 years or so of the right ventricle doing the work for the left. At that point, a heart transplant is their best option, Ward said.
However, Burkhart, the hospital's new pediatric heart surgeon, has been researching ways to help these patients' left ventricle continue to grow and, eventually, support a normal circulation. He'll continue that research in Oklahoma, Ward said, and it could lead to newer treatment options for babies like Elliot.