Today I took Stephen for a liver and spleen ultrasound, which I thought was just a follow-up to his labs from last week to check to make sure everything is ok with his liver. We have known all along that often times children with ARPKD also have some liver issues. And from day one in the NICU, I had been asking his doctors about it. But every response I've gotten has been favorable; that there's nothing to be concerned about regarding his liver. And after last week's nephrology appointment where I was told his labs were good, I didn't think there was anything to worry about either. But apparently I was wrong.
Sitting in that little ultrasound room watching the tech scan Stephen's abdomen was like a horrid case of deja vu. We have been through so many heart breaking ultrasounds when I was pregnant to know the signs when something isn't quite right. First, they start to take lots and lots of pictures of the same thing. Then the tech conferences with the doctor, and then the doctor herself comes in to take a better look. Once the doctor came in the room and started looking at how blood (I think) was flowing through the liver, I knew instantly something wasn't right.
Apparently, the order on the ultrasound was to look specifically for signs of Caroli's disease. All I know is that Caroli's affects the bile ducts of the liver. There's stuff all over the Internet indicating that Caroli's specifically targets those inflicted with ARPKD and is another recessive disease. Lucky us. Right now I don't know anything more than that. I don't know how severe Stephen's case is, I don't if we'll need to do something to treat it, and I don't know how it will affect Stephen. I did call the pediatrician and she instantly called me back and said she would call the radiologist herself and get some more information. I'm so thankful to have such an awesome pediatrician who is doing such an amazing job and keeping all of Stephen's doctors connected. She's going to speak with the nephrologists and Stephen's GI doctor to make sure everyone is on the same page and that all the important players are aware of this.
But I guess at least this new development isn't something that has totally blind-sided us or the doctors. We've been aware of the potential for a liver issue, but I honestly thought that since no one had really said anything, that we were in the clear. Hopefully this will turn out to be something we'll just need to watch and he won't actually need a liver transplant down the road.