Time to Transplant: Bella's PSC Story

Written by Tracy Pacini, Bella's mother. Edited by CC Cunningham, CKF Program Coordinator.It all started when Bella Pacini was in first grade. She was diagnosed with Crohn’s disease, an inflammatory bowel disease in which the immune system attacks the gastrointestinal tract. At the time of diagnosis, Bella’s doctors noticed that her liver enzymes were elevated and she would have to undergo additional testing and a liver biopsy to properly diagnose the issue. At seven years of age, she was officially diagnosed with primary sclerosing cholangitis (PSC), a progressive liver disease that causes inflammation and scarring of the bile ducts (and the same disease that Chris Klug had which led to his transplant). The only known cure for PSC is a liver transplant.For many years, Bella’s disease remained dormant—the PSC did not appear to be progressing and the Pacini family was optimistic. However, at age 12, Bella’s doctors noticed signs that her disease was becoming more serious. She developed esophageal varices (extremely dilated veins in her esophagus), which were caused by hypertension within the central vein of her liver. This meant that blood was getting pushed away from her liver into smaller blood vessels in her esophagus. It is most commonly due to increased scarring of the liver. Bella needed to be hospitalized every couple of months to get “banded”, a procedure in which rubber bands are placed over the varices to alleviate pressure and stop the bleeding.It wasn’t long before Bella’s doctors discovered that Bella’s spleen was enlarged: three times its normal size. It was around this time that Bella began to experience itching, a common symptom for those with liver disease. As her symptoms became worse and harder to endure, it was clear that something needed to be done.Bella is now 13 years old and beginning the transplant process. As she waits for her new liver, Bella remains positive and strong, while looking forward to putting PSC behind her. She hopes to become a doctor when she is older, to help children just like her.Bella’s mother has set up a Facebook page so others can follow her daughter’s story. Follow the link and “like” the page on Facebook if you are interested in staying up-to-date with Bella’s transplant journey.