When Nate was born, his skin looked yellow. In all other respects, he seemed a perfectly healthy baby: eating, sleeping, fussing. When the yellow discoloration did not go away, however, Nate’s mother Wanwan began to worry.
Recognizing the Signs
“A lot of people told us, oh, that's normal. Newborns have jaundice very often. But this jaundice didn't go away even after the first month. His eyes were also yellow at that time. After about a month and a half, we took him to the pediatrician,” says Wanwan.
Jaundice affects an estimated six out of every 10 newborns. It is caused by a build-up of bilirubin, a yellow substance in the blood that is formed from red blood cells being broken down and replaced. Normally our liver processes bilirubin and eliminates it from our bodies. Newborn babies don’t process this bilirubin as effectively, due to their still-developing livers, until about two weeks after birth.
“At that time, we also looked at his poop, and it was on the lighter side. The pediatrician said that could be just what he ate on that day. But the most alarming sign for me is the jaundice,” says Wanwan. “Then the pediatrician said, ‘We’ll run a liver function test on him.’ That’s when we found out something was wrong and she referred us to a pediatric gastroenterologist at Columbia.”
Discovering Biliary Atresia
By itself, jaundice in newborns is harmless. In rare cases when the jaundice does not go away, like Nate’s, it can be a sign of a serious underlying health condition called biliary atresia. In this condition, the bile ducts that normally transport bilirubin become scarred and obstructed, causing the bilirubin to build up. The causes of biliary atresia are not yet fully understood. It affects about one in 15,000 births. Eventually, the damage caused by biliary atresia leads to liver failure and the need for transplantation.
“At that time I didn't even know that there's a disease called BA (biliary atresia). We went to the ER, and then we were admitted. Dr. [Steven] Lobritto, the pediatric GI specialist, ran all the tests and then decided that he needed to do the surgery. It was quite fast. We were admitted to the hospital, I believe, on Sunday. Then the surgery happened the following Friday,” says Wanwan.
Urgency and Hope: The Kasai Procedure
“I think Dr. Lobritto really pushed it because BA is a time-sensitive disease. It's not great the longer you wait. I think that the golden period of doing the Kasai procedure was within two months of his birth, but I think at that time we were two months and perhaps 10 days,” she continues.
The Kasai procedure is generally the first treatment for biliary atresia. It is not curative, but the procedure delays the rate of liver damage and in some cases may prevent the need for liver transplant in the future. During the procedure, the surgeon removes the scarred, damaged bile ducts and makes a new, patent connection between the liver and small intestine, releasing bile to flow as it should. After having just received Nate’s diagnosis of biliary atresia, Wanwan did not have much time to think before agreeing to his surgery.
“It was like a roller coaster. First of all, I needed to learn everything about BA because I had no idea. It's such a rare disease. The team at Columbia really educated us a lot. I asked so many questions. It was very scary. There was really not much time to process, you have to just make a decision. You have to really just trust the doctor,” says Wanwan. “But the signs were also really showing. The poop color was light and abnormal. The test results showed us the bilirubin was really high, so it's right in front of you, all these facts.”
“To me, it's just unimaginable. But they said, ‘This is the only way to save him. He won't remember anything. We will take good care of him.’ I think they really tried their best to lay everything out. I asked a million questions. I said, ‘Oh, what if the surgery wasn't successful? Do we need to consider a liver transplant right away?’ They were very calm, telling me that it's a step-by-step process,” says Wanwan.
“They explained to me that the Kasai has three results. One result is very good, very successful, and therefore you can go into adulthood not needing a liver transplant. The second category is good for a couple of years then you may need to discuss liver transplant. Then the third category is the most unfortunate in that it’s not successful at all, and they need to consider liver transplant right away,” she continues. “They were using very plain language to explain to me these three possibilities.”
Coping with Recovery Challenges
For now, Nate’s surgery has been a success. His jaundice went away; his bilirubin levels normalized. There was one episode of infection which required a brief hospitalization.
“Every time he had a fever, I got nervous because I was worried that it was a liver infection. We did have one episode of the liver infection called cholangitis, and we went to Columbia and were admitted for two days to have it treated,” says Wanwan. “It's something that we as his parents need to deal with, every time he's not well, it's almost in the back of my mind that, oh my God, it's related to his liver. Most of the time, it's not. Most of the time, it's just like a normal kid getting sick in the daycare.”
Only time will tell for Nate if surgery has permanently staved off his need for liver transplantation when he grows into adulthood. Around two-thirds of infants who undergo Kasai eventually require liver transplants.
“They were very honest with us. They said most of the patients will need liver transplants down the road, and I'm still trying to make peace with this fact,” says Wanwan. “But Dr. Lobritto, our liver specialist, said, ‘In 16 years, in 20 years when he is an adult, when he's at the time of needing transplant, you never know what the technology may look like. The Kasai procedure really prolonged, really earned him some time for the technology of medicine to develop.’"
Navigating the Future
Nate is two years and four months old now. He is too young to remember his time in surgery, too young to worry about the future. His mother wonders about when, and how, to best teach him about his condition.
“When we shower him, I'll say, ‘Oh, this is the scar from when you had the surgery.’ But I really don't think he understands. We'll have to wait a couple of years to tell him. I think I will tell him everything. I will treat him like a person who understands. Because eventually he needs to take care of himself. He needs to understand,” says Wanwan. “He will gradually engage in the process.”
“We see our liver specialist at Columbia quite often. I have to say the Columbia liver team is so good. Whenever I have a question, I call them, and then I right away get a doctor on the phone,” says Wanwan. “For example, when he's having a fever, when I'm not sure about some symptoms, I always call them, and they always get back to me.”
“Although I wish that transplant would never need to happen if in the future we really do need a liver transplant, Columbia will be the place for us to go. He has all his team there,” says Wanwan. “I felt like we are really in good hands.”
Related:
- Tiny Patients, Big Solutions: A Closer Look at Fetal Surgery Innovations
- State of the Union: Pediatric Surgery
- NewYork-Presbyterian Morgan Stanley Children’s Hospital Recognized for Providing the Region’s Best Care