Chris has come a long way since he was diagnosed with biliary atresia, a liver disease. His mom shares his story and how Chris has been helped by CHOP's Biliary Atresia Program.
Chris is a now a happy healthy 1-year-old, but he has come a long way in the past 10 months. When Chris was born during the summer of 2005, we had no reason to believe he would do anything but thrive.
Chris was an exceptional baby; he slept a lot and when he was awake he was alert and a joy to be around. Although he had some issues with feeding very early on, by his third week it seemed as though all was going very well.
When Chris was about two months old, his grandmother visited us. Although he looked good to the people who saw him every day, his grandmother thought that he looked a bit yellow. At her encouragement we made an appointment to see Chris’ pediatrician early on a Friday morning. Chris’ pediatrician was a bit baffled by a 2-month-old being jaundiced so he sent us to the emergency room for tests.
After spending a day in the emergency room one thing was very clear — Chris had a problem with his liver. What was not so clear at that point was what was causing the problem.
Liver questions and answers
Chris was then scheduled to have a HIDA scan the following Tuesday. The lapse of time between when we were first told we needed a HIDA scan until the time we got a preliminary diagnosis unfortunately allowed us to look at the Internet and find the worst case scenarios. At this point we really didn’t know what was going on with Chris. While the technicians were performing the last part of the HIDA scan one of them mentioned they were testing Chris for biliary atresia. Those words hit us like a freight train. Biliary atresia was one of the diseases we read about that scared us the most because there is no cure.
At this point, doctors could not make a definitive diagnosis, but they were pretty confident Chris had biliary atresia. We were very lucky to have a pediatrician who understood the severity of Chris’ condition and understood how important it was to get Chris to Children's Hospital of Philadelphia (CHOP), where they were best equipped to deal with his problems.
Journey to Children's Hospital
We were in a room at Children's Hospital within one week of Chris’ initial visit to the emergency room. This is when we were first introduced to many of the people who have given our family so much support over the past year.
The next few days were almost a blur. We arrived at the hospital from our home in New Jersey on Thursday afternoon. Chris had a liver biopsy on Saturday to help the doctors better understand the status of his liver. By early Monday morning he was rolled into the operating room where Alan Flake, MD, performed the Kasai procedure.
The first few days were not easy. After the operation Chris’ face became very puffy and he had tubes coming from his nose. It was a very visual reminder of how sick he really was and how much his little body had gone through. Although Chris’ body had gone through a lot, it mended itself very quickly. We had our happy baby boy back by the end of the week.
We left the hospital armed with a great deal of information and what seemed like endless amounts of medication. Although it was a great relief to finally be heading home, it was extremely nerve-racking. In many ways, we knew Chris would be like any other child his age, but we also were keenly aware we needed to take added precautions to keep him safe and healthy.
While most children have fevers when they are young, we knew that for Chris a fever could be a sign of a very serious infection of the liver. For that reason we were instructed to call the GI doctor on call when Chris’ temperature went over 101 degrees.
We were out of the hospital for less than a month when the first fever struck. On Oct. 29, Chris was having problems sleeping. We took his temperature and found it was over 101 degrees. We called the GI doctor on call and were told to go to the emergency room.
After spending hours in the ER, Chris was admitted into the Hospital and stayed for four days for evaluation. This meant Chris spent his first Halloween on the 8th floor of Children's Hospital instead of at home. We never got a definitive answer about what caused the fever, but by Chris’ next appointment on Nov. 7, he seemed to be doing very well and was taken off the special formula that he needed after his surgery, as well as three of his medications.
Fevers and trips to the ER
We were lucky to make it through Chris' first Thanksgiving and Christmas without another fever, but unfortunately, a few days after Christmas Chris returned to the ER. It was another fever. This time it was a short hospital stay, but it was still very upsetting. We were beginning to get back into the swing of having a healthy baby but these trips to the hospital were reminders that his liver was not as healthy as his looks and attitude would lead us to believe.
In less than a month we were back at Children's Hospital again. Chris went into the hospital on Jan. 17 with a fever. It was a short stay and we were home by Jan. 20.
After this last trip we thought that the fevers were a thing of the past and we were looking forward to the christening we had planned for him on Jan. 29. This was going to be a wonderful chance for him to see family and friends that lived far away and hadn’t had a chance to see him since his surgery.
Unfortunately, Chris’ fever returned on Jan. 28. He spent the next 12 days in the hospital on IV antibiotics. Although there was never a definitive answer, the doctors believe he probably had a case of choliangitis.
Planning for the future
Chris has now gone for more than six months without needing a trip to the ER. He seems so happy and healthy that it is sometimes hard to imagine that he has a liver problem. We know that the Kasai procedure does not always work permanently, and Chris may still need a liver transplant at some point in his life. But, for now, we are enjoying his good health.
If Chris does need a liver transplant, we are sure we will be guided through that procedure in the same caring and insightful way we have been guided throughout this process.
By Chris' s mom, May 2007