How a collaborative medical effort saved a baby’s liver—and her life
Gastroenterologists, rheumatologists and other specialists at Stanford Children’s Health banded together to address one baby’s rare condition.
By WP BrandStudio
Like most parents, when Bay Area couple Natasha and Steven* learned they were going to have their daughter, Coralynn, they felt a mix of nerves and excitement. Unlike most parents, they had a potential complication to worry about: Natasha had lupus nephritis, an autoimmune disorder that attacks the kidneys. Lupus antibodies can pass from mother to child during pregnancy, so there was a small chance that Natasha’s lupus could affect Coralynn’s development in utero and even lead to neonatal lupus, a different form of the disease that affects newborns.
Natasha faced almost no complications during pregnancy, and when Coralynn was born at Lucile Packard Children’s Hospital Stanford, part of Stanford Children’s Health, she looked perfect. The couple thought things were fine, Natasha recalled. But about 24 hours later, things took a turn—Coralynn became lethargic and her blood sugar was low, so she was whisked away for more tests. Natasha and Steven were devastated.
“We put our guard down before she was born, so we weren’t expecting it,” said Natasha. “[When] we had to go back in, I was a complete mess, in tears. It was very emotional and stressful.”
The doctors and nurses at Stanford Children’s Health constantly kept the couple abreast of the situation, and after several days in an incubator, the cause of Coralynn’s symptoms still wasn’t clear. Natasha experienced a lupus-flare up during her pregnancy and took steroids to manage it. Could those medications have affected Coralynn’s vitals? Or did she just have jaundice, as many newborns do?
Coralynn received test after test, and after a while, lab results showed that her liver was failing. With the possibility of neonatal lupus becoming more likely each day, Coralynn’s doctors decided to pull in other pediatric specialists to give their thoughts on what could be done to save her life. This included a cardiologist, who could help interpret tests that had been done on Coralynn’s heart; a hepatologist and a gastroenterologist, who were consulted on what to do about Coralynn’s liver; and a rheumatologist, Dr. Christy Sandborg.
The moment she came aboard, Sandborg was almost certain what was going on.
“When we saw her there, our first inclination was to think, ‘What else could be causing this but neonatal lupus?’” Sandborg said. “We needed to do something or she–or her liver–may not have survived.”
We put our guard down before she was born, so we weren’t expecting it. When we had to go back in, I was a complete mess, in tears. It was very emotional and stressful.
Natasha
Grappling with a rare disease
Neonatal lupus with acute liver disease is a condition caused by lupus autoantibodies entering a baby’s bloodstream through the placenta during pregnancy. It’s very rare—one out of every 20,000 children are born with it in the United States. And because it’s so rare, there is no standard way of treating it.
At Stanford Children’s Health, a unique multidisciplinary approach to care is helping to change that. The hospital is known around the world for its commitment to innovation, especially in the realm of collaboration—which is a key part of the hospital’s ability to treat difficult cases, according to Dr. Sohail Husain, chief of pediatric gastroenterology.
“[We have] the ability to think innovatively about how we interact and address problems as a team of complementary experts,” he said.
Coralynn’s treatment team was facing two major pressure points while innovating a solution that would work for her. One was the need to move quickly—there was no telling how much time Coralynn had before her condition would become even more critical, so her doctors didn’t have many opportunities to hesitate.
There was also the crucial need to preserve her liver. A liver transplant was an option for Coralynn, especially since Stanford Children’s Health has one of the largest pediatric liver transplant programs in the country. But it would have been hard on her; the bleeding and infections that sometimes accompany a major surgery can take a toll on a baby. On top of that, all liver transplants come with the risk of rejection—which is what happens when the body doesn’t recognize the new liver, and the immune system starts to attack it. It’s an experience that is taxing on anyone, but especially so for a newborn.
“Our transplant surgeons are expert at working on very tiny babies, but it’s a big surgery,” said Dr. Noelle Ebel, a pediatric liver specialist (also known as a hepatologist) at Stanford Children’s Health who helped treat Coralynn. “If we could have spared her that, then we wanted to do everything possible [to do so].”
A precedent for others to follow
To save Coralynn’s liver—and her life—her treatment team, including Ebel and Sandborg, decided on an exchange transfusion, which they hoped would clear the lupus autoantibodies from her blood. Those antibodies, passed on from her mother during pregnancy, were the culprit for her condition. If eliminated, Coralynn’s body could create its own, lupus-free antibodies as she grew, reducing the effects of the neonatal lupus over time.
Ebel and Sandborg paired this treatment with anti-inflammatory steroids, which helped to reverse the effects of her liver failure. And they gave Coralynn IVIG therapy, which provided her body with additional immunoglobulin to boost her immune system and help fight off infection while she built up her own healthy antibodies.
All these treatments were commonly used in lupus patients, but they had never been used in conjunction to help a baby’s liver, which Ebel and Sandborg knew. But they also knew they needed to think fast and try things that had never been done before if they were going to avoid putting Coralynn through a liver transplant. So the two doctors made the decision to try the new approach on Coralynn—and ultimately, it was what saved her life.
We have at our disposal programs such as integrative medicine that other hospitals might not be able to provide.
Dr. Sohail Husain, chief of pediatric gastroenterology
“If Coralynn was at another hospital that didn’t have [close interactions between] pediatric rheumatologists and hepatologists, it would have been tricky to have come so quickly to this treatment,” said Sandborg.
Coralynn, now a year-and-a-half old, is growing like a weed, and healthy enough to chase the family dogs and play in the dirt. She won’t remember what happened with her at Stanford Children’s Health, though she’ll have mementos to remind her of her journey, including a few scars and an interesting birth story to share when she’s older. Moreover, she’ll have the knowledge that her case helped create a blueprint for treating neonatal lupus across the board. The team at Stanford Children’s Health plans to publish a paper about her experience, in the hope that it can help other kids like Coralynn make it through neonatal lupus using the same form of care.
“It was important to tell others, ‘This is how we saved this child’s life,’ so no one will ever feel like they’re in a situation again where they’re trying to innovate rapidly when the stakes are so high,” Ebel said.
Needless to say, Natasha and Steven are more grateful than anyone that the treatment worked—and they’re beyond relieved to have Coralynn’s health restored. Steven recalls spending months being anxious, even after Coralynn’s condition started to improve. It took a long time for the family to truly feel “in the clear,” he said. But now, things are at ease.
“New parents sometimes complain about their sleep. With us, we’re like, ‘Oh, we’re just so happy to have the child home,’” Natasha said.
*Natasha, Steven and Coralynn’s last names have been omitted to protect their privacy.
Source:
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3437607/
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