Arlaya's Story

Arlaya was born on 7/13/21 at 5lbs 14oz. Brian and I were beyond grateful to have the most beautiful baby girl. The day of getting discharged, we were informed that her newborn screening had slightly elevated levels of bilirubin which is a sign of jaundice. The following day, we went to her newborn appointment where her pediatrician wanted us to complete another newborn screening as a precaution. This second screening showed a slightly higher level of bilirubin and referred us to a Gastroenterologist (GI) specialist at Children’s Hospital. Once we met with the GI team, the first step was doing a full lab draw to get a panel of her liver levels. They did a full physical exam and while doing so, the doctor noticed that the color of her stool was pale like clay. As first-time parents, we were always asked if her stool was the color of mustard, but nobody specified the kind! This was a bit alarming since it was aligning with the symptoms of a disease called Biliary Atresia. Biliary atresia is a rare liver disease where the bile ducts are damaged or non-functioning which causes a blockage causing long term damage and even liver failure. There is no cure for this disease but there is a procedure called Kasai that would help increase the chances of a normal functioning liver. It would entail the removal of her bile ducts and bypass her intestines directly to her liver so the bile can naturally flow through. However, this procedure is only 30% successful and is meant to prolong a liver transplant. Moving forward, the next steps were to complete a liver biopsy to gather more information about it. The results came back and showed there were already signs of scarring. They also completed an ultrasound that showed she was born with no gall bladder. With the color of her stool, lab work, and the biopsy, the doctors determined we would be moving forward with the Kasai.
At 20 days old, Arlaya underwent the Kasai procedure. After the longest three weeks of recovery, we were finally back home, and the waiting game began. There was a three-month window to see whether the procedure worked or not. November crept up on us quickly and we completed the blood work necessary to see whether her liver was able to function with the bypass. Unfortunately, her bilirubin levels were still rising, and our doctors began planning the liver transplant process. We were scheduled two full days of evaluation at the end of December where we completed a CT scan, an ultrasound, an EKG, an echo, and extensive blood work. We also met with a full team of social workers, coordinators, transplant nurses, pharmacists, and of course the surgeons to get all the necessary information for transplant. Arlaya was officially listed on the UNOS database with a PELD score of 16. (To find out more about what a score is, please visit: https://unos.org) After a month of being listed, her liver function has gone down rapidly. The liver disease has caused fluid buildup called ascites and began putting pressure on her organs to where she’s now constantly uncomfortable. We’ve also been admitted due to infection and the increase of her ascites. She is also on total parenteral nutrition (TPN) which is a method of feeding to bypass directly into her blood stream to maximize the amount of nutrition to prepare her for success in transplant. She has a central line IV that has been placed on her chest for her to receive TPN 24 hours a day up until transplant.