Three generations of strong women: Amanda with her mom, grandmother and aunt.
A LONG ROAD AHEAD Amanda’s birth was just the beginning. Now, the real work began. Upon birth, Amanda’s doctors discovered that she had a sternum, but it wasn’t fused together. She needed surgery right away to correct that, as well as her narrow aorta. “Amanda had a complex syndrome called PHACES syndrome,” explains Jose Honold, MD, a neonatologist at Rady Children’s and clinical professor of pediatrics at UC San Diego School of Medicine. “This syndrome consists of several anomalies including a coarctation of the aorta. She also had ectopia cordis, a rare congenital condition in which some or all of a baby’s heart doesn’t have the typical coverage of the breastbone, and instead lies beneath a layer of skin and appears to be outside the chest. She also had facial hemangioma, which is a vascular tumor in the face.” Luckily, in Amanda’s case, all these conditions were reparable. Within a week of her birth, Amanda underwent surgery to repair her aorta with John Nigro, MD, a cardiothoracic and heart transplant surgeon, Division Chief of Cardiac Surgery & Cardiac
home just five weeks and one day after she was born! It was a surprise, but a very pleasant one.” According to Harjot Bassi, MD, a board- certified pediatric intensivist, the respiratory director of the CTICU at Rady Children’s, and an associate clinical professor of pediatrics at UC San Diego School of Medicine, the key to Amanda’s success was her ability to quickly regain normal airway function. Airway abnormalities are common in patients with complex congenital heart disease. “Amanda did really well from her intracardiac repair, especially based on the complexity of her sternum and the amount of surgery she required,” Dr. Bassi says. “What stood out to me about her case was her airway. We were worried that extubating her would be challenging because of her short neck and the challenges with her sternum. But in the end, we were able to provide enough respiratory support, and she surprised us by doing so well after such a complex surgery. She progressed forward from a nutritional standpoint and didn’t have any complications.” HEADING HOME TO HEAL Because Amanda was ready to head home so soon, her mom needed a crash course in caring for an infant with complex medical needs. Thankfully, the Rady Children’s team was at the
The first time I saw her, she was connected to tubes, and it was very overwhelming to see her that way. It was sad and depressing, but my family and friends were all there for me, especially my mom.
Transplant Services at Rady Children's Hospital and clinical professor of surgery at UC San Diego School of Medicine. Within a week, Dr. Nigro performed a second surgery to fuse her sternum together. “It was a lot for her little body to go through within a few short weeks of her life,” Ana recalls. But Amanda was strong, and she did it, faster than anyone could have predicted. “From the start, her cardiologists said Amanda could be in the hospital anywhere from three months to a year,” Ana says. “She surprised us all when she was ready to go
SPRING 2024 HEALTHY KIDS MAGAZINE 17
Made with FlippingBook Online newsletter maker