Our sweet Bentley Christopher was born March 15, 2023. We began to notice he was jaundice at approximately 4 ½ weeks old and lab work at that time was concerning for a problem with his liver. That first hospital stay with our newborn baby lasted 10 days and was filled with numerous tests, labs, a liver biopsy and so much uncertainty about what could be going on. We left the hospital with a lot of information about what it wasn’t, but without an answer for what was going on and even more questions about the future for our sweet baby boy. Genetic results would come back the following month and give us our diagnosis of Progressive Familial Intrahepatic Cholestasis type 2 (PFIC2) and start us on this pathway to liver transplant.

PFIC2 is a rare condition that affects the liver. People with this condition generally develop signs and symptoms during infancy, which may include severe itching, jaundice, failure to thrive, portal hypertension and an enlarged liver and spleen. PFIC2 generally progresses to liver failure in the first few years of life. There is also an increased risk of developing cancer of the liver.

The last 11 months have been filled with medical appointments, lab draws, and frequent hospital stays. Bentley required tube feeds for weight gain through the summer, and though we no longer required the NG tube, he was still not gaining weight prior to the transplant. He was also unable to absorb necessary vitamins due to the liver disease.  Bentley’s other main issue was the buildup of bile that made him severely itchy. It is described by older children as an itch from inside that cannot be relieved, and he scratched constantly to the point of creating scabs all over and making himself bleed. It was absolutely heartbreaking to see him so uncomfortable as it affected him all the time. He would often cry, throw himself back and forth and was simply unable to get comfortable regardless of his position. Because of this, he did not sleep well. The hope for transplant is to take away all of these symptoms and allow him to be a normal active toddler.

In November of 2023 we had Bentley’s official evaluation for transplant at The Cleveland Clinic Children’s Hospital. Our medical teams, both at UH Rainbow Babies and at CCF, are amazing and have worked closely together to provide the highest level of care for Bentley. He was officially placed on the UNOS Transplant List January 11, 2024. Bentley received his liver transplant on February 19, 2024 from an anonymous living donor. Words cannot even begin to express the gratitude we have toward this person’s selfless, most generous decision to donate a portion of their healthy liver to help our sweet baby boy. Because of their decision, Bentley will be able to have a new life free of these horrible symptoms and be able to grow up a happy, healthy boy. The transplant process has been a whirlwind up to this point, and we have a long road ahead of us. We would love for you to follow along here with us as we continue this process. 

The reality of the financial burden a pediatric organ transplant puts on a family is one I hope none of you will ever have to experience. We have had the time to absorb this information and have come to the realization that it is ok to ask and accept assistance in any way that may present itself. We have partnered with COTA to help us with this. Donations made to COTA in Bentley’s honor will help assist with transplant-related expenses and ease some of the financial burden this transplant imposes so that we may focus on Bentley and helping him to recover. Donations are tax deductible to the fullest extent of the law for the donor, as well.  We thank you for reading Bentley’s story and are so very appreciative of the generosity and support we have been shown.

The Children’s Organ Transplant Association (COTA) helps children and young adults who need a life-saving transplant by providing fundraising assistance and family support. COTA is the nation’s only fundraising organization solely dedicated to raising life-saving dollars in honor of transplant-needy children and young adults. 100% of each contribution made to COTA in honor of our patients helps meet transplant-related expenses. COTA’s services are free to our families, and gifts to COTA are tax deductible to the fullest extent of the law.