In general, the long-term outlook for patients with biliary atresia is excellent. Most biliary atresia patients can expect to live into adulthood with either their native liver or a transplanted liver.
From the 1960s to the present, numerous advances in the surgical approach to biliary atresia have been incorporated into routine care. Prior to the early 1960’s, biliary atresia was considered a uniformly fatal disease.
Today — with the Kasai hepatoportoenterstomy and improvements in liver transplantation — patients with biliary atresia are reaching adulthood.
The most recent reports from the U.S., Japan, France, Germany and England have provided insight into the general prognosis patients with biliary atresia.
Overall survival with a native liver (not transplanted) ranges from 30-55 percent at 5 years of age; and 30-40 percent at 10 years of age. It is thought that approximately 80 percent of patients with biliary atresia will require liver transplantation by the age of 20. But these statistics may change as we learn more about biliary atresia and modify our treatment approaches.
Those living with their native liver and reaching adulthood, can expect to go to school, have jobs and even families, if they desire. Both young adults living with their native liver and those who have undergone transplantation can have children.
Their children do not inherit biliary atresia.
Whether living with a native or transplanted liver, young adults with biliary atresia have been described as enjoying a normal quality of life.
Beyond young adults, no one knows exactly what to expect. It is the current group of patients who will tell us what the future holds.
Since 2002, physicians and scientists from around the country and at CHOP have been dedicated to improving the lives of those with biliary atresia through The Childhood Liver Disease Research and Education Network. There are current studies that focus on school functioning, medical complications, timing of transplantation, fibrosis and inflammation.
Together, these studies give the best hope to the new generation of children growing up with biliary atresia.