Cedar

Cedar was diagnosed with PFIC 2 when she was 5 months old. We were traveling visiting family when she developed random bruises on her back and a swollen shoulder out of nowhere. We took her to the ER and she was in full blown liver failure. Nobody could understand why, but they were able to stabilize her and we were sent home to Kentucky. After one month went by, in and out of the hospital, blood work came back and she was diagnosed with PFIC 2. She was jaundice, super itchy, and cried all the time. Her doctors started her on a lot of different medicines and we went to the hospital every 3 months for blood work and ultrasound. When she was 15 months old, the itching was so unbearable that her doctor recommended surgery. She had a partial external biliary diversion (PEBD) at that time. At first, it didn’t really work. Now she was very itchy and had an ostomy bag that she was always scratching off of her belly. It was very frustrating, we were hoping for relief. Another year went by and we all had adjusted to the bag, continuing with our 3 month checkups with a hospitalization here and there to correct her clotting times which had increased because of her inability to absorb vitamin K. Her itching finally got better at that time, so right around age 3. Whether she grew out of it, adjusted to it or the bag finally did help to control her bile build up we’re not quit sure, but it was much better. Things were going very well for the next 2 years, but at age 5 we started seeing more signs that her disease was progressing. Her spleen was becoming enlarged, which started to trap her platelets and other blood cells., so she was living with a very low platelet count. Her vitamin levels became chronically low, so low that she was at risk for complications even though she was on the highest dose of supplements. She was also getting more and more tired during the day, which was hard to see. We felt like she was a ticking time bomb, so her doctors and us decided it was time to list her for liver transplant. The process was difficult to accept, but we felt like it was the right thing to do. She was transplanted way quicker than we thought, only on the list for 2 weeks. She recovered extremely quickly from surgery and we were home within a month. So far, we have had one episode of rejection but it was very manageable. After we saw the picture of her old liver, we knew we made the right choice. She is currently happy and full of life, and we couldn’t ask for anything more.