Max's parents vividly remember the difficult days after their newborn was diagnosed with transposition of the great arteries (TGA), a complex congenital heart defect.
They also remember the many people at The Children’s Hospital of Philadelphia who helped them through the ordeal.
The nurses who showed them how to bathe their infant. The surgeon who took 40 minutes to explain the procedure she would perform. The parking-garage attendant who noticed Max’s mom was in tears. “He took my hand and said, ‘Everything is going to be OK. Your son is in the best hands,’” she recalls.
For Juliette and her husband, John, the support they experienced was extraordinary.
“Everyone here made us feel like we weren’t alone,” Max’s mom says. “Everyone was focused on the same goal: to see Max come out of here healthy.”
“We’re big fans of CHOP,” John says. “We cannot speak glowingly enough about this place.”
Diagnosis: Transposition of the great arteries (TGA)
When Max was born in February 2009 at Abington Memorial Hospital, his color was slightly blue. Doctors initially attributed this to the fact that the umbilical cord was wrapped around his neck (a relatively common occurrence). However, once the cord was removed, he didn’t regain color.
“They noticed he wasn’t getting pink enough,” his dad recalls. “His lower extremities were still a little blue.”
A neonatologist arrived. Juliette and John were able to hold Max for a moment before he was whisked away to the neonatal intensive care unit. The doctor there suspected transposition of the great arteries (TGA), a life-threatening heart defect in which the aorta and the pulmonary artery are attached to the heart in the wrong places.
Five hours later, Max had arrived by ambulance to the Cardiac Center at CHOP. It was the middle of the night. His dad followed by car, arriving a half-hour later.
“By the time I got there, there were well over a dozen people around Max,” he recalls. “I was in disbelief.”
Max’s mom, still recovering from the delivery, had to say goodbye to her son and stay behind.
If Max were born today, he may have been delivered in CHOP’s Garbose Family Special Delivery Unit (SDU), a state-of-the-art labor and delivery unit opened in 2008 for mothers carrying babies with known birth defects. The unit is on the same floor as the cardiac operating rooms, catheterization labs and patient units, so parents and baby stay close rather than being separated at birth.
Diagnosis and the Rashkind procedure
The diagnosis of TGA had been confirmed. Cardiac Center nurses and pediatric cardiologist Brian Hanna, MD, explained that Max needed an emergency catheterization procedure, called balloon atrial septostomy or the Rashkind procedure.
The balloon atrial septostomy procedure has saved thousands of babies around the world since created at CHOP in the 1960s by William Rashkind, MD. A cardiologist threads a catheter through the belly button or a vein in the leg, to the heart, and creates a hole between the upper chambers. The hole allows oxygenated blood from the left side of the heart and deoxygenated blood from the right side to mix, increasing the amount of oxygen in the blood pumped to the body.
For Max, this procedure meant he would be able to survive for several days, before surgery to permanently fix the defect.
By 4 a.m., Dr. Hanna had completed the Rashkind procedure.
Max’s mom arrived later that morning. Seeing her baby with so many tubes and wires was difficult. So too was the news that Max would have open heart surgery the next day.
Surgery to treat Max's TGA
The whirlwind continued. On the day of surgery, Max’s parents said goodbye to their baby at 7 a.m. They watched as anesthesiologist Susan Nicolson, MD, took Max to one of the cardiac operating rooms down the hall. The Cardiac Operative and Imaging Complex is on the same floor as the Cardiac Intensive Care Unit to facilitate safer and easier patient transport and staff communication.
A cardiac nurse accompanied Juliette and John to the office of surgeon Stephanie Fuller, MD.
“Dr. Fuller sat with us for a good 40 minutes and explained everything that would happen,” Juliette recalls.
This is standard at CHOP: as the operating room team preps the child for surgery, the surgeon explains the procedure and answers questions.
The surgery to treat Max's TGA lasted approximately eight hours. Dr. Fuller corrected the placement of the aorta and pulmonary artery and closed the hole created during the atrial septostomy.
Max recovered from open heart surgery relatively quickly. He was in the Hospital 10 more days, with his parents often spending the night in family sleep-rooms.
Parents can feel helpless when their child is in intensive care after surgery. With the nurses’ help, Juliette did everything she could to participate.
“They knew how important it was for me to do the mom things,” she recalls. “I couldn’t hold him or nurse him. But they showed me how to give him baths and take his temperature.”
Juliette met with one of the Hospital’s lactation specialists. She pumped milk for Max to drink from a bottle, and was soon breastfeeding.
The specialized pediatric cardiac nurses and the lactation consultants, in addition to the cardiologists, surgeons, social workers, therapists and many others at CHOP, are recognized for setting the national standard for excellence in the specialized care of infants, children and adults with congenital heart disease.
We understand that exceptional medical care is only one piece of the picture, and that truly comprehensive care for patients means also providing families with the resources, education and support they need, every step of the way.
Follow-up care for TGA
At 12 days of age, Max went home. Today, he is a thriving 1-year-old. Follow-up visits have shown him to be in fine health and as Max grows, he will have annual check-ups with Geoffrey Bird, MD, his primary cardiologist.
After his open heart surgery, when Max was so frail, his parents took a photo of him. They hesitated, but the nurses encouraged them, saying that once children recover, most parents are glad to have a record of their memories of such a difficult time.
Today, looking at the photo, Max’s parents can hardly believe their good fortune.
“That was one day,” his mom says. “And now we’ve had so many months of him being healthy and happy and playing. We’ve been so blessed.”
Originally posted: November 2009