Cedar was just 5 months old when her parents got the news that she had Progressive Familial Intrahepatic Cholestasis (PFIC). PFIC is considered an “ultra-rare” liver disease as only 1 in 100,000 people have it. “The difficulty with a rare disease is after diagnosis you are not handed a lot of answers or action steps. You are
saddled with the news and essentially told, at least in our case, that your 5-month-old baby will eventually go into liver failure and will need a liver transplant at that time” explains Emily, Cedar’s Mom.
“Prior to diagnosis Cedar was always crying. Our Doctor told us that she was a colicky baby and that she would grow out of it, however, we found out later that it was this rare disease that was affecting her liver and making her itch all the time. Once we found out what it was, we had this terrible lingering feeling. This news put a bit of a damper on our parental journey, but we adapted, ya know? We were very sad; we didn’t want this for her, but what else can you do but adapt?” Says Emily.
She goes on, “Cedar had a disease that, despite treatment, would eventually progress to liver failure. From the time she was 5 months old, she went through multiple liver biopsies, freq
uent lab draws and imaging tests, multiple daily medications, and even surgery, all to try to accomplish some s
ort of quality of life with her diseased liver. Ultimately, at age 5, her doctors had done all that they could do, her disease was progressing toward liver failure. The process of getting listed was harder than being on the waiting list itself. We went through the process of listing her because we knew that she would eventually need a transplant. The doctors expected her to be waiting on that list for a while which was scary for us. We didn’t want her to go into complete liver failure and be near death. It was a constant waiting game since diagnosis, but when we got her on the transplant list it was hard to accept that that was actually what she needed. We were scared that we would put her through this transplant surgery that would change the course of her life, and we were scared that that wasn’t something we needed to do at that particular moment. She seemed like a pretty normal 5-year-old, with the exception of some fatigue and elevated liver levels” explains Emily.
“We ended up getting ‘the call’ only 2 weeks after officially being listed and we are so lucky we did. Once she was transplanted, we were able to see her old liver and it was completely black. She could have been deathly ill at ANY moment and we would have been blind-sided because her symptoms were so minimal at that time”.
Now we are able to celebrate milestones like being out of the hospital for a full year. We celebrate this because her immune system is so delicate and we know that if she is around someone who is sick, she will get sick. That is just the life of an immune-compromised child, and we are so grateful to have that life”.
Cedar loves to climb in Red River Gorge and has a great community that surrounds her. She is able to do the things she loves and be as normal as possible. “We have been able to watch her grow, watch her come into her personality, and watch her thrive. This is something we didn’t know if we were going to be able to do with her original diagnosis. We are looking forward to a relative life or normalcy thanks to our donor” says Emily.
When we asked Emily what she would say to her donor family if they were standing in front of her, she replied “As a nurse and the Executive Director of the PFIC Advocacy & Resource Network I am always in advocacy mode. Shifting into caregiver mode is more emotional. It is a gratitude that you can’t put into words. I cannot imagine how tough it was to be put in the position to have to make that decision; to say ‘yes’ to donation. To even be in that position is unfathomable. Without them saying ‘yes’ in their darkest moment, Cedar would not be here. The science just isn’t there yet for her disease group. This was the only way she could live. How do you put into words what a perfect stranger means to you? How they gave your kid life when they did not have to? The way her old liver looked at transplant, we know for certain that she would not be alive today if it weren’t for them”, explains Emily. “We are just so grateful and thankful to them for saving our girl”.