When overnight breathing treatments and a trip to their local emergency room offered no improvement, their pediatrician called for an ambulance to rush them to The Children’s Hospital of Philadelphia (CHOP).
Quinn’s mom, Emily, recalls “that’s when we got really worried.”
For hours, CHOP Emergency Department doctors worked to figure out what was causing his symptoms. Quinn had a chest X-ray, an electrocardiogram and an echocardiogram, among other tests.
“We didn’t know what was wrong,” his mom remembers. “And that was the worst part.”
Quinn was admitted to CHOP’s Evelyn and Daniel M. Tabas Cardiac Intensive Care Unit (CICU), one of the largest and most sophisticated in the nation. It was the middle of the night. Emily and her husband, Keith, dozed off next to their baby.
Very early the next morning, Stephanie Fuller, MD, one of the cardiothoracic surgeons on the CHOP Cardiac Center team, visited Quinn's parents. She brought difficult news: Quinn’s labored breathing was an early sign of heart failure. He needed emergency open heart surgery to fix a rare form of congenital heart disease called anomalous left coronary artery from the pulmonary artery (ALCAPA).
In a normally functioning heart, the left coronary artery connects to the aorta and feeds oxygenated blood to the heart tissue. In ALCAPA, the left coronary artery is misplaced and the heart doesn’t get enough oxygenated blood. Without enough oxygen, the heart tissue can begin to die.
Quinn would need immediate open heart surgery to stay alive.
Dr. Fuller drew a picture to explain the ALCAPA defect and the surgery Quinn would require to survive.
“She said, ‘This is what’s wrong, and this is what we’re going to do to fix it,’” Emily recalls. “She said there were going to be a lot of doctors and nurses in the operating room and they were all going to be there to take care of Quinn.”
Though Dr. Fuller broke tough news, Emily and Keith remember her visit as a positive turning point.
“The whole night was so scary,” Emily says. “We were so upset and worried. When Dr. Fuller explained things, it was such a relief. She is the best. She’s so nice and calm. I didn’t feel scared anymore. I knew they were going to fix Quinn’s heart.”
CHOP’s cardiothoracic surgery team is one of the most experienced, performing a high volume of procedures to treat rare and complex heart conditions, such as ALCAPA.
The day of surgery
Emily and Keith had Quinn baptized and said goodbye, handing him to the cardiac anesthesiologist who brought him to the operating room and stayed with him through his open heart surgery.
CHOP is one of the only pediatric hospitals in the country with a dedicated anesthesiology team for cardiac patients, who may react differently to anesthesia than other patients. Parents have peace of mind knowing this expert team will be there not just for heart surgery, but for any procedure over the years.
Four hours later, the operation to treat Quinn’s ALCAPA was over. Dr. Fuller and a nurse visited to tell them Quinn was doing well.
Recovery after surgery for ALCAPA
Quinn faced a long recovery after his ALCAPA surgery as his heart slowly healed. He also fought a respiratory infection that slowed his progress.
But Emily never felt alone. “Every day we were there, people went out of their way to make sure Quinn was OK,” she recalls.
The Cardiac Center has an extended team to help patients recover, monitor their development, and keep them occupied. An occupational therapist brought a play mat into Quinn’s room for special belly exercises to help him regain strength. A music therapist taught a class in the Cardiac Center playroom, tailoring it for every age present.
Emily has special praise for the Cardiac Center nurses, who were with Quinn nearly every moment during his stay in the CICU. One nurse helped them set up a CarePage, a webpage that helps parents update friends and family. Another taught them to use the naso-gastric feeding tube Quinn would have for several weeks.
The nurses were always there to answer Emily’s questions. “They always listened when I was worried,” she says.
Finally, 12 weeks after open heart surgery, Quinn was able to go home. For nearly a year, he had home visits from occupational therapists and physical therapists to help him catch up on milestones such as rolling over and crawling.
Coordinated follow-up care
Quinn, now an active, happy 4-year-old who loves superheroes and playing with blocks, visits his cardiologist, Chitra Ravishankar, MD, every year as part of his ongoing follow-up care for ALCAPA.
Emily is grateful for the well-coordinated and thoughtful care Quinn receives at CHOP.
"The team is very empathetic and understanding of what you're going through," she says. "Before you even know what would be helpful, they offer it."
Quinn will need to see a cardiologist regularly for the rest of his life, and Emily takes heart knowing the Cardiac Center offers lifelong care for children with ALCAPA, including ongoing monitoring through the Cardiac Kids Developmental Follow-up Program.
If Quinn requires surgery later in life, he could have the same surgeon: Dr. Fuller is also part of the surgical team at the Philadelphia Adult Congenital Heart Center. This joint program between CHOP and Penn Medicine helps patients transition to the adult program, where the team is specially trained in the complex anatomy and heart function of adults born with congenital heart disease.
“We’re so lucky that we live here and that we have such great people to take care of him,” Emily says. “I trust everything they tell me. I know that they’re always going to be taking care of him, and he’s always going to get the best care. I don’t have any worries.”
Originally posted: February 2012