Biliary Atresia: Causes, Signs, Diagnosis and Treatment at St. Louis Children’s Hospital

What if you knew you were going to have a baby? Would you be excited? Happy? Jubilant? What if after waiting for what seems like years, your baby was born, but she you knew something wasn’t’ quite right? What if your baby was jaundice? What if your baby’s diapers look different? What if you took your baby to the pediatrician and you found out she had a life-threatening disease that was fatal without treatment? My son was born with Biliary Atresia. My daughter was born with Biliary Atresia, a liver disease that affects children. Biliary Atresia is a condition where an infant is either born or develops shortly after, where the biliary system, which are the bile ducts coming out of the liver are blocked. So because there’s blockage and no drainage of bilirubin, there is progression of liver disease and eventually, if nothing is done, children will end up with a scar liver and will probably die within two or three years. Early on, a lot of babies are jaundice, which means they’re yellow. Yellow the skin, yellow the eyes. Which is common in a lot of newborns, but for some, it doesn’t mean normal, it means that they have a liver disease. Another thing you have more of a pale color stool. Not completely white, but pale, clay color, light yellowish is abnormal. So at the current time there’s still little known about what causes Biliary Atresia. Approximately 300 children in the United States are born with it yearly. We were really scared at first. We had very little information on what the disease was or long-term prognosis of his disease. From diagnosis to the time of his first surgery, was within days. From the time of his first surgery to actual liver transplantation was only months. We went to the Internet and just started Googling Biliary Atresia and we would create forums and post. We went and saw the doctor and he said okay we’ll need to have the surgery this week. I’m thinking we’re coming here for an appointment, I had no idea that my baby was going to have surgery so I immediately just went into tears. The initial treatment for Biliary Atresia is a surgical procedure known as the Kasai procedure. It’s really just a mechanism used to try to buy time to when a transplant will be needed. After Kasai procedure, eventually 70% of children will require liver transplant. Of these, 50% occur before the age of two. That in itself was difficult because we knew this would not be the last surgery she would have to have. He was really doing fantastic for months and then all the sudden he got sick with what we thought was a stomach bug. We brought him into the hospital. Really everything was caused because the liver just deteriorated really rapidly and he needed to be listed for a liver transplant. So Jack was listed whenever he was nine months old. He waited for approximately ten weeks on the waiting list and got a full, whole liver by a deceased donor. Alexander had a liver transplant at five months old. He was on the waiting list for approximately ten days. In Alex’s case, we did not have a lot of time and I became his living liver donor. We were in the process of looking at living donors. My wife was being evaluated and even my younger brother. We got the page that they were ready to transplant and she was the recipient of a non-living donor. There’s a few reasons why our waiting time for a liver is shorter than the national average. One includes the technical ability of our surgeons. They are able to perform all these types of surgeries, whether it’s a whole liver or a cut down or liver related and that we have all those three options at our center. Another one, the region where we get the donors from just has a shorter waiting times so there’s more donations than some of the other regions and the rest of the United States. Overall, the success rate is really good for a child that has Biliary Atresia. But, two-thirds will end up requiring liver transplant. But that the outcomes, the success, being alive after live transplant are excellent nowadays. Throughout the nation within our own center we are looking at one-year and five-year success rate in the 90s. Kennedy is seven years old now. It’s been six years since the transplant. She’s been doing very well. She’s currently not even on medication. She likes to sing and she like to spend time with her cousins and her brother. We are now four years post liver transplant. Alex is four-and-a-half years old. He enjoys the Back to the Future trilogy, especially the DeLorean. He enjoys Thomas the Tank Engine and very much enjoys playing with his friends at school. Jack’s almost two years old now and he’s a year post-transplant. Everything from Jack’s SGI doctor to the surgeons to the nurses, they have taken such good care of not only Jack but our family. We go to the clinic once a month roughly right now and we come in for labs about every week. Organ donation is so critical. Awhile back, somebody said to me that you don’t need your organs in heaven and God knows we need them here and it’s just absolutely true. I mean, it’s lifesaving and Jack has a second chance at life because somebody made that decision.

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