Bailey and James were excited to learn they were expecting their first child, a baby boy they would soon name after dad. However, a routine sonogram around 26 weeks turned their excitement into fear. Scans showed the baby had a congenital heart defect, called transposition of the great arteries (TGA). Baby James would be born with this condition in which the arteries carrying blood from the heart are reversed. Without surgery in the first few days of life, the condition is lethal and causes babies to turn blue and impacts breathing.
Doctors first thought he may not ever play sports, run or swim because of the impacts … some didn’t think he would survive at all.
“It was terrifying,” Bailey says. “We were shocked, scared and in disbelief. One doctor said he may not live a year. I recall the doctors encouraging us not to go online and search his condition.”
The family decided against that and did some research on their own and found James Quintessenza, M.D., at Johns Hopkins All Children’s Hospital in St. Petersburg, Florida.
“We knew he was the best,” Bailey says. “I felt in as good of hands as we could be.”
Quintessenza met with the family to discuss James’ condition as well as what to expect before, during and after delivery, including a surgical procedure called an arterial switch to correct TGA. The procedure consists of dividing and transferring the major arteries entering the incorrect heart chambers and moving the small coronary arteries connected to the aorta so that the baby would have oxygenated blood.
“When we have a fetal diagnosis with significant cardiac problems that will probably need surgery, we usually sit and answer the family’s questions and they hear about the surgery and what the outcomes might be, help them understand and put them a little at ease,” says Quintessenza, co-director of the Johns Hopkins All Children's Heart Institute and chief of cardiovascular surgery at Johns Hopkins All Children’s Hospital. “We want to provide hope but want to be realistic and accurate as possible so expectations align with reality. Most of the time there are ways to deal with these problems and many patients can have quite good quality of life.”
Shortly after learning more about what would come next, Bailey had a cesarean section at 36 weeks and baby James was whisked away to the cardiovascular intensive care unit (CVICU) at Johns Hopkins All Children’s. He was just 4 pounds – one of the smallest babies in the CVICU at the time. Quintessenza, who has performed this procedure for 30 years now, decided it was time for James’ surgery at just 8 days old.
“Bailey was recovering so it was a trying time,” says James Sr. as he recalls the first few days in the hospital. “We were surrounded by family that morning and in walks Dr. Q, confident as always and he assured us the procedure would go well.”
James Sr. and Bailey recall the procedure went extremely well and the team came out of surgery early, but baby James would spend 30 days in the hospital.
“We couldn’t touch him or hold him at first, but we played Mozart for him every day and read The Little Engine That Could,” recalls Bailey. The family says while those days were tough, they kept a positive attitude, just as the book inspired them to do. In the days and weeks that followed, James’ breathing tube was removed, the family was able to hold him and he was excelling at his feeding therapy and getting stronger everyday thanks to the team at the hospital.
“From the cardiologists, perfusionists, operating room nurses, physician assistants, ICU staff and other therapists, it takes a village to care for these babies early on, and all that has to be done with experience and gentleness for these tiny babies,” adds Quintessenza.
Fast forward to 10 years later and his family says James is defying all odds. He’s into baseball, golf, swimming and his favorite sport – lacrosse.
“I want other kids to know that you can still play and prove people wrong,” James says.
His lacrosse coach, who also happens to be the hospital’s director of sports medicine, didn’t even know until halfway through the season that James was born with a heart condition.
“When I heard his story, I was amazed at what he had been through,” Patrick Mularoni, M.D., says. “Seeing his level of activity and his approach to the game is incredible knowing all of the adversity he faced early on and the challenges that he has overcome to get on the field.”
James also impressed his heart surgeon.
“He looks pretty good on the field,” Quintessenza says. “It’s really satisfying to know that our patient who came in with a condition that was not compatible with life, is now playing sports and has a really bright future. That’s awesome.”
Mom and Dad continue to be amazed. They are thankful the Heart Institute team is in their community if they ever need the team again.
“When I was pregnant with James, we were so worried about his heart and him just being healthy and surviving,” Bailey says. “Now, his heart is one of the most beautiful things about him. Everyone says how kind, empathetic and loving he is. His heart isn’t one of the first things on my mind anymore. His heart is perfect. If something ’technically’ goes wrong I have all the confidence we will get it corrected, and he will be fine. I know we are in good hands.”