When Angel Moreno was following the emergency medical helicopter in her car from Sacramento to Stanford Children’s Health last April, her mind was racing with worry. She didn’t know if her 7-week-old baby, Barbarita, would survive.
“A lot of times over the last year I didn’t know if she’d reach her first birthday,” says Angel. “Yet here I am, planning a party to celebrate her turning 1 on Feb. 20.”
Barbarita, or Barbie for short, was born with a very rare condition called neonatal lupus. Neonatal lupus is not the same as lupus that affects adults. Rather, neonatal lupus develops when antibodies in the mother’s blood pass through the placenta to her developing baby. It can occur even if the mother does not have lupus, which was the case with Angel.
While some babies born with neonatal lupus experience only a rash that goes away with time, others, like Barbie, develop more severe complications, with the most serious being a congenital heart block. In this condition, antibodies attack the part of the heart that controls the heartbeat, causing damage that’s discovered in the womb or at birth.
When Barbie was just five days old, doctors at UC Davis Medical Center, where Barbie was born, placed a pacemaker. It helped for a few weeks, but then her heart function worsened. Fortunately, UC Davis and Stanford Children’s Health have a strong collaborative relationship that brings heart transplant services to patients from the Sacramento area. Every three months, Seth Hollander, MD, pediatric cardiologist at Stanford Children’s Health, visits UC Davis to help with decision-making for children with heart problems and evaluate whether heart transplant might be an option. After a brief hospital stay at UC Davis, Barbie was airlifted to Stanford Children’s Health.
“Her doctors at UC Davis told us that Stanford Children’s Health has one of the best heart centers in the nation,” Angel adds.
The heart experts with the Stanford Children’s Health Pediatric Advanced Cardiac Therapies (PACT) program approached Barbie’s care from several angles. Barbie’s heart was failing, so they put in a second lead from her pacemaker to the right side of her heart, since the lead to the damaged left side was ineffective in resynchronizing her heart. That’s when they discovered that the right side was also damaged. They teamed up with rheumatology experts to try to lower her lupus antibodies in hopes of stopping further damage to her heart. While medications helped, they did not completely neutralize the antibodies. Barbie was also placed on oxygen and a feeding tube to support her growth.
The PACT program at Stanford Children’s Health exists within the Betty Irene Moore Children’s Heart Center—one of the leading pediatric heart centers in the United States—and treats more than 1,000 clinic patients each year, some coming from as far away as Alaska and Hawaii, and even other countries. In this set-up, the team manages heart failure and heart transplant patients as a single program. Doing so empowers doctors to provide the right care at exactly the right time—and proceed with ventricular assist devices (VAD) and heart transplantation when needed. VADs are mechanical pumps that give the heart the help it needs to pump blood from the heart to the lower part of the body. They keep babies like Barbie alive and act as a bridge to heart transplant.
The PACT team at Stanford Children’s Health is made up of pediatric cardiologists, heart transplant surgeons, heart failure and heart transplantation nurse specialists, and pediatric cardiac anesthesiologists. The clinical team is supported by a multidisciplinary team of pediatric nurse practitioners, child psychologists, social workers, nutritionists, child life specialists, physical therapists, and occupational therapists, who together with the clinical staff provide 360-degree care before, during, and after a heart transplant.
“We created the PACT program because we wanted to follow children with heart problems throughout their care journey. They often see a heart failure specialist, a VAD specialist, and a heart transplant specialist. Since they may need all these experts, we brought them together under one program and one team to provide the best possible care and outcomes for our young patients,” says Dr. Hollander. “This means Barbie received coordinated care from the same team from the day she arrived to the day she left.”
After trying several solutions, Barbie’s doctors concluded that her heart was simply too damaged to recover. She developed cardiomyopathy, a state where the heart becomes weak and stiff. Her heart needed help, and that came in the form of a VAD—specifically, a Berlin Heart, which was designed for children. The Moore Children’s Heart Center at Stanford Children’s Health is one of the first pediatric centers in the United States that adopted the use of a Berlin Heart for children with cardiomyopathy awaiting heart transplant. This is the reason why Lucile Packard Children’s Hospital Stanford has more experience using this device than most other children’s hospitals in the world.
“We like to see babies early on so we can try alternative treatments to avoid or delay the need for a heart transplant, but when a baby’s heart continues to fail, we need to place a VAD,” Dr. Hollander says.
To date, the cardiothoracic surgeons at Packard Children’s have implanted more than 160 VADs after placing the first pediatric VAD in 1998. The Stanford Children’s Health surgeons have a talent for placing VADs in the tiniest of babies, using a variety of VADs, and pioneering unique ways to implant them and address complex heart conditions.
On day 142 of Barbie’s hospital stay, her Stanford Children’s Health doctors received the call that a donor heart was available. The news was both exciting and frightening for Angel.
“When I heard they had a heart for Barbie, I vacillated between happy and excited to nervous and scared,” Angel says. “I remember Barbie was looking at me all perplexed as I was crying and smiling.”
The heart transplant surgery took nine hours to complete. During the surgery and their entire stay, Angel felt confident in Barbie’s doctors.
With nearly 40 years of experience and more pediatric heart transplants than any other institution in California (440+), the PACT team at Stanford Children’s Health is able to provide lifesaving, comprehensive care to even the most highly complex heart patients with outstanding results. The Pediatric Heart Transplant Program at Packard Children’s Hospital has some of the highest pediatric heart transplantation survival rates in the nation.
“We had constant conversations between our team and the parents about when to place the VAD. We didn’t want to do it too early and rob her of a chance to recover on her own, or do it too late and lessen her chance for a successful heart transplant,” Dr. Hollander says. “We timed it just right. And Barbie’s heart transplant went really well, with zero complications.”
Angel greatly appreciated that the PACT team kept her informed by inviting her to daily huddles to discuss Barbie’s care. Knowing what to expect, and being able to give input and ask questions, eased her worries.
Today, Barbie is doing great. After 205 days in the hospital, she’s home and receiving telehealth visits from the PACT team’s physical therapist to help her catch up on her developmental milestones.
“Today, watching her act like any other baby, it’s hard to believe the heart transplant even happened. To have her home, starting to crawl and engage with her world, is such a delight,” Angel says.
Angel doesn’t know what she would have done without Stanford Children’s Health. Today she’s planning Barbie’s birthday party and celebrating where they are now, compared to where they’ve been.
“Barbie is an extraordinarily happy and adorable child, and we are very optimistic about her future, and look forward to receiving pictures of her life milestones as she grows older,” Dr. Hollander concludes.
He gives a shout-out to the brave, anonymous family that donated a heart to Barbie. It’s the reason she’s alive today, and he, together with the rest of the PACT team, appreciates their generous, difficult choice. For Barbie, it’s the best birthday present ever.
View the NBC coverage of Barbie’s heart transplant story here.