Jacob and Lukas Swanson, 3, are the only known twins in North America with the life-threatening liver disease biliary atresia, an incurable disorder that ultimately necessitates a liver transplant. After waiting two years for a liver to become available, Jacob underwent a transplant at Children’s. Lukas is still waiting for a new liver.
Less than 15 years ago, most young children diagnosed with biliary atresia died before the age of 2, because they were considered too small to receive a liver transplant. Today, infants with this condition are routinely transplanted at Children’s with high success rates. At birth, Jacob and Lukas seemed healthy, although Jacob had a slight case of jaundice. A couple of months later, tests indicated that not only did Jacob have elevated levels of a substance suggesting liver disease, Lukas did as well.
Their parents, Robyn and Scott, were referred to the liver specialists in the Division of Gastroenterology, Hepatology and Nutrition at Children’s. They met with Peter F. Whitington, MD, who is a pioneer in the treatment of children with serious liver disorders. He and Riccardo A. Superina, MD, (fellow co-director of Children’s Siragusa Transplantation Center), studied the boys’ biopsy results before arriving at a diagnosis.
Biliary atresia is a progressive liver disease that results in blockage of the organ’s bile ducts, damaging the organ and affecting normal digestion and growth. The cause of the disease is not known, although research suggests that some children may have a genetic tendency to develop it.
Dr. Superina, Head of Transplant Surgery, performed procedures on both Jacob and Lukas to create a new pathway for bile to drain from their livers. If successful, the procedure can delay the need for a transplant until children are older. Although both boys initially responded well to the surgery, six months later Dr. Whitington recommended placing Jacob on the transplant list.
Shortly after Jacob was listed for a donor liver, Lukas developed internal bleeding, and ultimately underwent two minimally invasive endoscopic surgeries. A month after his brother, Lukas was added to the list for a new liver.
In August 2011, Robyn and Scott, who live in Chicago’s northwest suburbs, received a phone call from a member of Children’s organ procurement team with good news. “She said, ‘We have a liver for Jacob. Can you get to the hospital by midnight?’” says Robyn. “I said, ‘We’ll be there in two hours.’”
Children’s pediatric liver transplant program is one of the world’s largest, and its specialists have performed more than 300 liver transplants since 1997. Dr. Superina performed the six-hour transplant operation on Jacob the following evening.
“Almost immediately we noticed a change in Jacob’s energy level,” says Robyn. “He was like a new kid, running and jumping around. We were very happy, yet at the same we couldn’t help thinking, ‘Yes, but what about Lukas?’ He still needs a liver desperately.”
Jacob has faced additional challenges. In December he was hospitalized due to a rare complication from his immunosuppressant medication that caused a non-cancerous form of lymphoma. His treatment has included surgery and chemotherapy neither of which has slowed him down. “It’s amazing,” says Robyn. “To see him you’d hardly know what he’s gone through.”
Because twins with biliary atresia are so rare, the entire Swanson family is participating in a research study. “They are unique study subjects, and sorting out their genetic background will be extremely useful in understanding what pathways might be involved in a predisposition to acquire biliary atresia,” says Dr. Whitington, who is heading the study.
Despite everything the family has been through, Robyn prefers to focus on the positives.
“You don’t ever want your children to be sick,” she says. “But knowing that we have a wonderful facility like Children’s right in our backyard, with wonderful caregivers like Dr. Whitington and Dr. Superina and their teams, has been a fantastic ‘security blanket’ for our family.”
Story originally appeared in the February 2012 issue of Heroes Update.