Our daughter Brynn was born in March 2000 and was jaundiced on the first day of her life. We assumed this was normal neonatal jaundice and were surprised at how much blood work was being ordered by our pediatrician. By the second day of her life, a neonatologist sat down with us and explained the difference between conjugated and unconjugated bilirubin and why Brynn’s blood work was concerning. Scary phrases like "possible metabolic disorder," "biliary atresia," and "cystic fibrosis" were being hurled our way. More tests were ordered. I remember someone coming in and saying to me "Well, we’re not putting her on the transplant list YET." I couldn’t believe what I was hearing. We were devastated! 

From the beginning, Brynn’s condition didn’t seem to fit the mold of any known disease. By the time she was 8 days old, her liver was already so badly scarred that the biopsy sample crumbled apart when extracted. Because of the extensive fibrosis, it was difficult to diagnose the underlying disease, but after hearing "Congenital Hepatic Fibrosis" and "Caroli’s Syndrome", we were eventually advised that Brynn’s disease was most likely a variant and severe form of intrahepatic biliary atresia. In any case, it was obvious to everyone involved that the Kasai operation would not help her and that she would most likely require a transplant within the year. We listed her in June of 2000 at Pittsburgh Children’s Hospital as a status 3. We were told that we were really lucky that her blood type was A+. When we listed her, she was already first on the list for her size and blood type. We expected a call any day. She was not gaining weight very well, but she was playful and happy like any other baby and we just assumed that she would be transplanted before she got really sick. 

Unfortunately, her health really began to deteriorate when she was about 4 months old. She had terrible problems with blood clotting that we kept under control with weekly Vitamin K shots. She then began to develop ascites which became very severe. Her abdomen was extremely distended and varices were prominent all over her belly. It was so painful to see her this way. She was so uncomfortable, she stopped smiling and playing and basically just sat in her infant seat most of the day. In early September, she was admitted to CHP for IV Lasix and albumin to help alleviate the ascites. While we were there in the hospital, her esophageal varices burst and she started to vomit blood across the room. She was rushed to the operating room where the bleeding was controlled. 

We were then told that it was very unlikely that she would be leaving the hospital without a new liver. We inquired about living-related donation, but our transplant team suggested that we try and wait a little while longer. Dr. Reyes, the head of the pediatric liver transplant team at CHP, even confessed to us that since we had been there, he had been offered at least 3 livers for her, but he had turned them all down because he didn’t feel they were the right fit. We were stunned when he admitted this, but his confidence that the right liver would come along was very comforting. 

And thankfully, Dr. Reyes was right! A few days later, we were notified that a donor organ was available and suitable for Brynn’s tiny 10-pound body. She went into surgery at 5:30 p.m. and emerged 12 hours later a whole new baby! It was a miracle to see her recover and witness the changing of her skin tone and to finally see the whites of her beautiful blue eyes. Our most memorable moment was seeing her smiling and laughing again just one week after her transplant. 

At 8 months post-tx, Brynn is absolutely full of life! Currently, she most enjoys playing peek-a-boo and giggling with her big brother Evan. We feel so fortunate to be able to enjoy our days with this beautiful, strong and happy baby girl, and we are forever grateful to the family of the child whose liver saved her life. We are also so very thankful to the transplant team at CHP – the level of skill and commitment in that group continues to amaze us!

Update:  February 2005

Our daughter Brynn was born in March 2000 and was jaundiced on the first day of her life. We assumed this was normal neonatal jaundice and were surprised at how much blood work was being ordered by our pediatrician. By the second day of her life, a neonatologist sat down with us and explained the difference between conjugated and unconjugated bilirubin and why Brynn’s blood work was concerning. Scary phrases like "possible metabolic disorder," "biliary atresia," and "cystic fibrosis" were being hurled our way. More tests were ordered. I remember someone coming in and saying to me "Well, we’re not putting her on the transplant list YET." I couldn’t believe what I was hearing. We were devastated! 

From the beginning, Brynn’s condition didn’t seem to fit the mold of any known disease. By the time she was 8 days old, her liver was already so badly scarred that the biopsy sample crumbled apart when extracted. Because of the extensive fibrosis, it was difficult to diagnose the underlying disease, but after hearing "Congenital Hepatic Fibrosis" and "Caroli’s Syndrome", we were eventually advised that Brynn’s disease was most likely a variant and severe form of intrahepatic biliary atresia. In any case, it was obvious to everyone involved that the Kasai operation would not help her and that she would most likely require a transplant within the year. We listed her in June of 2000 at Pittsburgh Children’s Hospital as a status 3. We were told that we were really lucky that her blood type was A+. When we listed her, she was already first on the list for her size and blood type. We expected a call any day. She was not gaining weight very well, but she was playful and happy like any other baby and we just assumed that she would be transplanted before she got really sick. 

Unfortunately, her health really began to deteriorate when she was about 4 months old. She had terrible problems with blood clotting that we kept under control with weekly Vitamin K shots. She then began to develop ascites which became very severe. Her abdomen was extremely distended and varices were prominent all over her belly. It was so painful to see her this way. She was so uncomfortable, she stopped smiling and playing and basically just sat in her infant seat most of the day. In early September, she was admitted to CHP for IV Lasix and albumin to help alleviate the ascites. While we were there in the hospital, her esophageal varices burst and she started to vomit blood across the room. She was rushed to the operating room where the bleeding was controlled. 

We were then told that it was very unlikely that she would be leaving the hospital without a new liver. We inquired about living-related donation, but our transplant team suggested that we try and wait a little while longer. Dr. Reyes, the head of the pediatric liver transplant team at CHP, even confessed to us that since we had been there, he had been offered at least 3 livers for her, but he had turned them all down because he didn’t feel they were the right fit. We were stunned when he admitted this, but his confidence that the right liver would come along was very comforting. 

And thankfully, Dr. Reyes was right! A few days later, we were notified that a donor organ was available and suitable for Brynn’s tiny 10-pound body. She went into surgery at 5:30 p.m. and emerged 12 hours later a whole new baby! It was a miracle to see her recover and witness the changing of her skin tone and to finally see the whites of her beautiful blue eyes. Our most memorable moment was seeing her smiling and laughing again just one week after her transplant. 

At 8 months post-tx, Brynn is absolutely full of life! Currently, she most enjoys playing peek-a-boo and giggling with her big brother Evan. We feel so fortunate to be able to enjoy our days with this beautiful, strong and happy baby girl, and we are forever grateful to the family of the child whose liver saved her life. We are also so very thankful to the transplant team at CHP – the level of skill and commitment in that group continues to amaze us!

Update:  February 2005