This summer, 12-year-old Ryan Marusich plans to do normal kid stuff, like playing Little League baseball and hanging out with friends. Last summer he could do neither of those things. His liver was failing, and his only hope for long-term survival was an organ transplant at Ann & Robert H. Lurie Children's Hospital of Chicago, where he has been treated since he was a baby.
"Ryan didn’t have a summer last year," says his mom, Debra. "He couldn't keep up with his friends. We would go to the pool, and his friends would be jumping off the diving board and having fun and all Ryan could do was just sit there and watch."
When he was three months old, Ryan was diagnosed with the rare genetic disorder Alagille syndrome, which causes malformed bile ducts in the liver, affecting the organ's ability to eliminate wastes from the bloodstream. He was treated by members of Lurie Children's hepatology (liver) team, which is part of the Division of Gastroenterology, Hepatology and Nutrition, ranked second in the nation by U.S.News & World Report. His primary clinician was Peter F. Whitington, MD, co-director of Lurie Children's Siragusa Transplantation Center and the Sally Burnett Searle Professor of Pediatrics and Transplantation.
For his first ten years, Ryan was treated with a regimen of medication, vitamin supplements and a high-fat diet. Because of digestion issues, he was small for his age, though Debra says he was still able to keep up with friends and play sports. Two years ago. Ryan's liver function began to decline, and in May 2014, Dr. Whitington told Debra and her husband, Jay, that Ryan needed to be placed on the national patient waiting list for a donor liver.
"We knew things were bad, but we didn't realize how dire Ryan’s situation was until then," says Debra. "I think we were in denial, thinking we were managing everything through diet and medication."
At the end of July, Ryan was admitted to Lurie Children's, where he would remain for nearly 100 days. His kidneys also began to fail and he needed dialysis. Both Debra and Jay were able to work remotely from Ryan's room, so he always had at least one of his parents with him.
"Everyone at the hospital was amazing," says Debra of Ryan's care team, which included experts in nutrition, physical therapy, neurology, and Child Life specialists. "The nurses are so caring, and the staff went way above and beyond for us. When Jay and I were at our wit's end, they would say, 'You need to get away and go outside for a walk.'"
In September -- two and a half months after Ryan was added to the list for a donor liver, a suitable match became available, and he underwent surgery by a team led by Riccardo Superina, MD, the Head of Transplant Surgery and the Robert E. Schneider Chair in Transplantation. Due to complications, Ryan needed two additional surgeries and several other procedures. Finally, at the end of October, Ryan was discharged from Lurie Children's. He and his parents spent ten days at nearby Ronald McDonald House transitioning to go home, which Ryan finally did on November 3.
Today, Ryan is thriving. He's growing, putting on weight and is regaining his strength and flexibility. He has regular follow-ups with the members of the liver and kidney teams, but the visits are less frequent and the number of medications he takes has been reduced.
"The transplant has been transformational," says Debra. "You can just see that he feels better. He's light-hearted, isn't scared of doing physical activities and is acting like a kid again, playing baseball and having fun with his friends."
In April, Ryan got to throw out the ceremonial first pitch at a Chicago White Sox game, attended by members of Lurie Children’s transplant team in recognition of National Donate Life Month, and about 50 of the Marusich's family and friends. "That was a wonderful afternoon," says Debra.
Lurie Children's hepatology team has a robust agenda of basic and translational liver and liver transplant research projects, led by Dr. Whitington and Estella M. Alonso, MD, Medical Director of the liver transplant program. Ryan was enrolled in several research studies, and his old liver, which contained an unusually high level of copper, is currently the focus of one of Dr. Whitington's studies.
"If participating in a research project might take these studies to the next step and help other kids with Alagille syndrome avoid liver failure and the need for a transplant, we're happy to help," says Debra.