In May of 1995, Ruth Patras realized that something was wrong with her 5-week-old daughter, Ciara. Initially happy and healthy, about a month after Ciara was born, the whites of her eyes started to turn yellow. Over the next few days, the color deepened, and her appetite diminished. Patras took Ciara to her pediatrician, who sent the family to Children’s Hospital of Philadelphia. Tests revealed that Ciara had biliary atresias, a rare liver disease in which the ducts that pass bile from the liver to the gallbladder and the first section of the small intestine become blocked. Bile serves two functions in the body, helping to digest fat and carry waste out of the liver. When trapped, the excess bile damages liver cells, eventually leading to liver failure.
Doctors told Patras that the only hope for Ciara was a complex surgery known as the Kasai procedure, in which the gallbladder and bile ducts are removed and the liver is connected directly to the small intestine. The Kasai procedure is hardly a cure, though: It’s only successful 30 to 50 percent of the time, and when it fails, patients need a liver transplant as early as age 1 or 2; even when it works, around three-quarters of patients still require a liver transplant by their 20th birthday. Read more
BY Danielle Elliot