A first-time mom puts her baby girl’s heart in the hands of Rady Children’s CTICU.
Ana Castaneda was 24 weeks into her first pregnancy when a routine sonogram turned her world upside down.
“I was happy, healthy and hopeful, but this news was a complete whirlwind,” Ana recalls. “At the time, I had no suspicion anything was wrong. I was happy, and everyone said I had that pregnancy glow. Of course, I was tired because I was growing a little human, but it was my first pregnancy, so I thought everything was good,” she continues. “When my doctor saw something abnormal on my exam and sent me for another sonogram, they told me her heart and cardiac anatomy weren’t developing correctly. It was a huge blow.”
Ana learned that her unborn daughter had Pentalogy of Cantrell (POC), a group of five congenital defects of the heart, pericardium, diaphragm, sternum and abdominal wall. A complex condition with a survival rate and prognosis that vary greatly depending on its severity, POC can present with a multitude of anatomical malformations that require immediate surgery upon birth. Babies with POC also may not survive longer than a few days. Ana’s baby also had a coarctation of the aorta, meaning that her aorta was narrower than normal. This can cause the heart to lose its ability to pump enough blood to other organs, which can result in organ failure.
“They told me her sternum was not fully intact and that as soon as she was born, they would know how severe it was and if she needed surgery right away,” Ana says. “I’m a single mom. I was pushed to the brink, thinking of what kind of quality of life my baby was going to have and whether I would be able to handle it on my own. I talked to many people, and I prayed.”
Preparing for the Unknown
With three more months to go before her baby’s due date, Ana moved forward, preparing as best she could for the unknown complications she and her baby would experience once she was welcomed into the world. Still, Ana continued to feel physically well.
“I was moving around, eating healthy, going to work—I still felt great,” she says. “I stopped working three or four weeks before my scheduled c-section, but only to mentally prepare myself for how she was going to come into the world. I wanted to get all the information I could, so I could expect the unexpected.”
In September 2021, Ana went in for her scheduled delivery at a local hospital close to Rady Children’s. The proximity enables newborns with complex medical needs to immediately be transferred to Rady Children’s Hospital intensive care units.
“As much as I prepared, I was still unprepared for how huge of a surgery a c-section is,” she recalls. “But it didn’t matter. I had to get up and get moving because it wasn’t about me, it was about my little girl, and I had to go see her.”
Within a day of giving birth, Ana was visiting her new daughter, Amanda, in the cardiothoracic intensive care unit (CTICU) at Rady Children’s.
“The first time I saw her, she was connected to tubes, and it was very overwhelming to see her that way,” she says. “It was sad and depressing, but my family and friends were all there for me, especially my mom. She was a rockstar and she helped me through it.”
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 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 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 ready with all the resources and training she needed for a successful transition from hospital to home.
“When they told me she was ready, I said, ‘OK, what’s next?’ and they told me to come in for training,” Ana recalls. “They said she would go home with a feeding tube, so that was something I had to learn, but they also said that when she’s home, she’s going to be so loved that she’s going to heal so much faster.”
Amanda’s nurses taught Ana how to gauge the amount of food she needed, how to use the feeding tube, how to keep track of her caloric intake, manage her medications and what to do in case of a feeding emergency. Ana took video of it all and shared it with her parents and her siblings, so she’d have the comfort of knowing her support system was as educated as she was.
“It was so much to learn, but the nurses showed me everything and then gave me a pop quiz—I got straight A’s,” she jokes. “It was all very overwhelming, but that’s part of the process of being a parent with a baby that has high medical needs. You have to expect the unexpected. Even though Amanda came home earlier than expected—and I didn’t expect that I could handle all of this—I did, and she’s not only surviving, but she’s also thriving!”
Now two-and-a-half, Amanda is still under the care of her Rady Children’s team, which includes a cardiologist, neurologist, otolaryngologist (ENT) and dermatologist.
“We had so many different specialists and therapies, and so many people caring for us at Rady Children’s—they’re like a second family,” Ana says. “Throughout it all, they were so amazing. They are considerate and they really listen to you. Amanda was a little human who couldn’t speak for herself, but now she’s so strong and sassy and happy. She has no idea all that she went through—all she knows is what she wants and what she doesn’t want, and she’s not afraid to let you know!”
On Amanda’s second birthday, she and her mom brought donuts to the Hospital to celebrate with the CTICU team that had cared for Amanda.
“This was a thank you to the team who took care of her 24/7,” Ana says. “That day I felt it all come full circle, because she wouldn’t be here without them. We wanted to show them the significance of what they do. They truly save lives, and Amanda is living proof.”
To learn more about Rady Children’s Heart Institute, click here.