This Clinician’s View opinion piece is written by Regino P. Gonzalez-Peralta, MD, AdventHealth for Children medical director of pediatric gastroenterology, hepatology and liver transplantation.
For many years, the only pediatric liver transplant program in Florida was in Miami. At AdventHealth for Children, we knew the need for this lifesaving care was growing and wanted to ease the burden on patients and families in the central and northern parts of the state, providing them access to a program closer to home. UPMC Children’s Hospital of Pittsburgh (UPMC) had the nation’s first and best pediatric liver transplant program, and as we looked to develop our program, we decided to take an innovative approach — collaborating with UPMC to bring their world-class care and expertise to Central Florida.
In 2018, AdventHealth for Children and UPMC officially partnered to establish Orlando’s first comprehensive pediatric liver transplant program, and we accepted our first patient in May of that year. It was the second partnership program for UPMC after they launched their first in 2016 with the University of Virginia’s Children’s Hospital in Charlottesville. Today, we remain the only program of our kind in Central Florida and one of only two pediatric liver transplant programs in Florida.
Collaboration to Deliver Clinical Expertise and Patient-centered Care
UPMC opened its comprehensive pediatric transplant center in 1981, under the guidance of transplant pioneer Thomas E. Starzl, MD, PhD. It consistently ranks amongst the top in the country for patient outcomes and volumes. It also remains a leading-edge, destination program of expertise, innovation and patient- and family-centered care for transplant patients from all over the world.
Transplant surgeons and medical specialists from both UPMC and AdventHealth for Children collaborate on patient care and management for patients in Florida, performing pediatric liver transplant surgeries together at AdventHealth for Children. Experienced AdventHealth transplant surgeons L. Thomas Chin, MD; Michael Angelis, MD; Philip Wait, MD; Ryan Day, MD; and Bobby Nibhanupudy, MD, work alongside UPMC pediatric liver transplant surgeons Kyle Soltys, MD; George Mazariegos, MD; Rakesh Sindhi, MD; and Ajaj Kanna, MD. I really don’t think of it as “them” and “us” but simply as “us.”
Under the leadership of Medical Director for Pediatric Critical Care Services Glenn Stryjewski, MD, MPH, and liver team intensivist Marla Carter, MD, the AdventHealth for Children PICU team provides critical medical care for transplant patients. Additional members of the AdventHealth for Children pediatric gastroenterology and hepatology care team include pediatric transplant hepatologist Stefany Hernandez Benabe, MD; hepatology advanced practitioner Elizabeth Freeman, ARNP; dietician Nicole Dutcher, RD; transplant coordinators Karen Woodrum, RN; and Jordan Perno, RN; and social worker Awilda Medina, LCSW; as well as pediatric gastroenterologists Sridhar Reddy Goli, MD; and Lina Hernandez, MD.
After each transplant surgery, our teams remain in constant contact and thanks to video conferencing technology, round together virtually daily even after the UPMC surgeons return to Pittsburgh. While we come from two different states and institutions, we are a cohesive team with a common mission: continually improving the lives of children suffering from liver disease. We’re all in this together for one patient at a time and keep that patient at the front and center of all we do.
In addition to transplants, our world-class program has performed liver resections for children with liver cancer as well as porto-systemic shunts to treat children with elevated pressures related to blocked portal veins, the vessel that supplies most of the blood to the liver.
New Hope for Ezekiel
In the summer of 2018, Angie Lopez became concerned when her two-year-old son Ezekiel didn’t want to eat and complained about stomach pain every day for a week. After bringing him to a clinic and being told that she had nothing to worry about, she took him to a local emergency room because she knew something was wrong. They ran a battery of different tests, and then Angie received the devastating news that her son was in acute liver failure.
Ezekiel was transferred to AdventHealth for Children with severe jaundice. After a few days, his liver function began to improve, and he was discharged shortly before his third birthday. However, just one week later, at Ezekiel’s follow-up visit, his numbers worsened. He was re-admitted to the hospital, and a liver biopsy showed that most of his liver cells were severely damaged, so he was placed at the top of the transplant list.
Within a day, our transplant team had located a match, and under the care of Drs. Chin, Angelis, Soltys and Mazariegos, Ezekiel became the third pediatric liver transplant recipient at AdventHealth for Children. I see Ezekiel regularly for follow-up visits, and I’m proud to report that today he is a happy, healthy eight-year-old who loves collecting Pokemon cards and playing outside with his mom. He also takes karate lessons and joined his school’s soccer club.
Exceptional Program Growth and Outcomes
Certified by the United Network of Organ Sharing (UNOS), the AdventHealth for Children Pediatric Liver Transplant Program has grown quickly in a short time to become one of the busiest in our state and the southeast. On March 28, 2024, we performed our 50th transplant. So far this year, we have completed seven pediatric liver transplants. Most importantly, our program has achieved outstanding outcomes with a 95% overall survival rate since its establishment.
A New Destination for Pediatric Metabolic Liver Transplants
Our unique program achieved another significant milestone on April 10, 2022, when we performed our first-ever pediatric metabolic liver transplant, a unique type of transplant that requires specialized perioperative care as the new liver produces its desired effects and built-up metabolic precursors are eradicated.
We have now performed two metabolic liver transplants on infants with rare urea cycle disorders. These disorders are genetic conditions that result from a defect in one of the enzymes involved in the hepatic removal of ammonia from the bloodstream. Left untreated, the disorder can cause brain toxicity and prove fatal. Because of potential severe neurological sequelae, it can also be a burden to manage as the child grows. Replacing the liver with a transplanted liver provided the missing enzyme and completely eradicated the problem. We’ve also performed a metabolic liver transplant on a child with maple syrup urine disease, another rare, genetic metabolic condition. In this disorder, the body cannot process certain amino acids, causing a harmful build-up of these substances in the blood and urine.
The addition of metabolic liver transplants is a significant opportunity to improve children’s lives, and we’re hoping to build upon our experience and UPMC’s expertise in this area. Our goal is to become the only dedicated, integrated metabolic pediatric liver transplant program in the southeastern U.S.
Leading Through Research & Innovation
In an effort to continually improve the care provided to our patients, AdventHealth for Children’s Liver Transplant Program is one of only 16 programs in the country to participate in the Starzl Network for Excellence in Pediatric Transplantation national registry, a community of top pediatric transplant centers focused on discovery and lifesaving innovation. We also participate in the Society of Pediatric Liver Transplantation national registry.
In addition, because hepatology extends beyond transplant care, our pediatric hepatology team remains actively engaged in clinical trials focused on emerging treatments for liver disorders. We are one of only a few centers in the world participating in the International Wilson’s Disease Patient Registry and gene therapy for this disease. Our team is also actively involved in a trial investigating a new class of compounds called ileal bile acid transport inhibitors to treat progressive familial intrahepatic cholestatic syndromes, and we are active participants in the American Association for the Studies of Liver Disease Acute Liver Failure Group. Finally, we participate in an international Alagille syndrome (ALGS) registry called the Global Alagille Alliance Study (GALA) to improve outcomes of children with this rare condition.
Looking Ahead
Moving forward, the AdventHealth for Children/UPMC partnership program plans to continue to provide education and build relationships with physicians throughout Florida. Providers interested in meeting to learn more about the latest developments in pediatric liver transplantation can reach me at [email protected] or my partner Stefany Hernandez Benabe at [email protected].
