Liver failure for any child, especially those under age 1, is an immensely complex disease. When transplant is indicated, life expectancy is usually measured in weeks. At St. Louis Children’s Hospital, one of the most experienced liver centers in the country, a team of highly trained liver specialists and surgeons offers every type of transplant option to treat all forms of liver disease.
“Our goal is to get patients transplanted as soon as possible,” says Jeffrey Lowell, MD. “Here, the likelihood that a child will get transplanted is higher, and their waiting time for a donor liver is statistically lower than the rest of the nation.” For the past 15 years, Dr. Lowell has served as director of the pediatric abdominal organ transplant program at St. Louis Children’s Hospital and professor of surgery and pediatrics at Washington University School of Medicine (WUSM).
Dr. Lowell stresses that the reason the liver transplant team can transplant kids faster is because this group has the skill and expertise to utilize all potential donor livers. “We have the full range of organ donor sources available, and we use them all,” Dr. Lowell states. This includes living liver donors, “split” deceased liver donors and reduced-size deceased liver donors, as well as standard, “full-size” deceased liver donors.
Program Depth
The surgical team includes Dr. Lowell and surgical partners Christopher Anderson, MD, pediatric liver and kidney transplant surgeon at St. Louis Children’s Hospital, and William Chapman, MD, FACS, surgical director of the adult liver transplant program at Barnes-Jewish Hospital. Dr. Chapman is involved in pediatric living donor and split-liver surgery. Two pediatric hepatologists constitute the medical component: Yumirle Turmelle, MD, medical director of the pediatric liver care and transplant center, and Alexander Weymann, MD. “We share a unique, very tight integration with all the team members,” Dr. Lowell adds. “Everyone on the team knows what’s going on with every single patient, every day.”
Patients can be seen within 24 hours, if medically necessary. Once a child comes to St. Louis Children’s Hospital for care, the transplant team becomes fully focused on that individual child. A complete range of specialists, including transplant nurse coordinators, dietitians, social workers, pharmacists, play therapists, clergy and intensive care and rehabilitation staff, brings every resource available. “We are a medium-large–to-large program,” notes Dr. Lowell, “and programs of exactly this size statistically have been shown to offer optimum care and best results for patients and families.”
Designated Biliary Atresia Center
Biliary atresia remains the number one indication for liver transplantation in children. One of the major contributing factors to a newborn’s outcome when diagnosed with this form of liver disease is a timely diagnosis, due to the rapidity with which the liver becomes damaged in the first few weeks of life. Studies have shown that patients have a much better chance of long-term survival if a child is under the medical and surgical direction of a specialized liver program.
Due to its extensive experience in treating the disease, St. Louis Children’s Hospital has become a designated biliary atresia center of excellence. “Most patients with biliary atresia will require transplantation,” states Dr. Turmelle, WUSM assistant professor of pediatrics. “Expert medical management of chronic liver disease is necessary while awaiting transplant, since the overall health status at time of transplant influences outcome after transplant. These include maximizing the patient’s nutritional status and managing complications such as ascites, electrolyte disturbances, increased risk of bleeding and infections.”
Double Listing Led to Liver for Anthony
According to Dr. Lowell, it’s often parents or a pediatrician who call about double listing. A baby in a Minneapolis suburb needed a liver transplant, so his mom called St. Louis Children’s Hospital to ask about a second opinion to double-list her son. That call resulted in a whole liver transplant for Anthony Postudensek on April 19, 2009, four weeks after he was double-listed at St. Louis Children’s Hospital. The infant had been on a transplant list since November 2008 at another hospital. “The staff was so proactive with Anthony, and he was getting weaker and weaker,” says Paula. “Everyone fought for him, and my pediatrician received updates. We didn’t think we’d see a difference in care between the two hospitals—but we did at St. Louis Children’s.”
Research Study Brings Young Emery to Children’s
For seven years, St. Louis Children’s Hospital has been one of 10 leading pediatric research centers participating in the Biliary Atresia Research Consortium (BARC), a nationwide, multi-center network that receives funding from the National Institutes of Health to study biliary atresia. “No single center has enough experience or sees enough patients, because biliary atresia is so rare,” explains Dr. Weymann, WUSM instructor in pediatrics. “The consortium was first created to study biliary atresia, but now we’re looking at other cholestatic liver diseases along with the Childhood Liver Disease Research and Education Network (ChiLDREN)—a group of doctors, nurses, research coordinators and medical facilities in the United States working together to study infants with rare liver diseases, including alpha-1 antitrypsin deficiency, progressive familiar intrahepatic cholestasis, bile acid synthetic defects, Alagille syndrome, mitochondrial hepatopathies and cystic fibrosis liver disease.”
This study is what first brought 2-year-old Emery Patterson to St. Louis Children’s Hospital. Emery was enrolled in the study in New York following her biliary atresia diagnosis. Knowing a job relocation was imminent, her parents moved to a southern Illinois town to ensure that Emery could be near a participating study hospital. Emery, who will soon celebrate her second birthday, was eventually listed for transplant. Six weeks later, she underwent a whole liver transplant at St. Louis Children’s.
The Long Term
Today, Drs. Turmelle and Weymann are working on an algorithm to help pediatricians and other physicians better understand clinical features and laboratory levels that would suggest biliary atresia or other types of liver diseases. Additionally, as part of the ChiLDREN study group, they are involved in a blind, randomized study to determine the efficacy of steroid use after the Kasai procedure, the initial corrective surgery that most children with biliary atresia undergo. A prospective field of future research includes the type and duration of immunosuppression following liver transplant. “We are very aggressive in trying to minimize immune suppression, and by six months to one year post-transplant, most of our patients are on immunosuppressive therapy with a single drug,” notes Dr. Turmelle. “Since our program started, our longest-surviving patient is 23-years-old. There’s really no limitation to their survival.”
Second Opinion Service
The Liver Care Center also welcomes requests for a second opinion. A second opinion is an important resource for every patient. For more information, call 800.678.4357.