Intro and questions

[Guest guest]

Hello all,

My name is , and this last Nov. we discovered that my 8yr old son Nick had low enough white blood and platelet counts that we immediatly were sent to University of MO. Columbia for a ton of labs, CT w/ contrast, and a bone marrow test. His spleen was enlarged 4cm past his ribcage, and his liver was also enlarged. He also had portal hypertension.

We had one initial visit to the pediatric liver specialist through MU two weeks later and his spleen was enlarged 8cm and to the midline of his chest. He was put on vitim K until a liver biopsy and an upper and lower GI could be done on him. This was around Xmas and after a month of waiting I still wasn't even on the books for an appointment.

I contacted Children's Mercy Hospital in KC, and four days later we were there having MRI, Upper and lower GI, liver biopsy, and sweat chloride test. I might have even left some things out. Nick received platelets while he was there. After a week in the hospital we were told his liver showed signs of scaring. Dr. was really leaning towards PSC. He was put on Ursodiol twice day, and omeprezole (anacid).

A few weeks later we were back having an ERCP done. After a bad bout of pancreatis the Doc then tells us that he has severe liver disease, but they don't know which one it is. He said it was not PSC. It was somewhere on the spectrum between PSC and Auto immune. What does that mean? Is that what it means when I have read other posts from people have over (lapping) PSC and Auto immune?

Anyway, Doctor says Nick's small bile ducts are scared and a lot of them are gone. He is calling what Nick has secondary liver fibrosis with portal hypertension. They told us his spleen will not go back down to size either. He also has some small veracies in his stomach.

They say they will just have to track his symptoms and changes over the next several months to a year to figure out what disease it is.

No mention of staging yet. Just that he has significant scaring of the small bile ducts. His last visit was Mar. 6 th and they said his plateletts have been running between 47-57 the whole time. I have a print off that says his alt was 53units, his ast was 50 units,his bilirubin is 0.5mg/dl what ever that means. Not sure if that is high or what. He doesn't look jaundice. They told us he can wait an come back in June unless there are changes. A few times he has had small amounts of blood in his stools, but that has only been one stool per incident. Doc said not to worry unless it happens more than once or is a lot. Then I need to check his levels again.

Just wanted to say I have lurked here since Dec. and have learned a lot. Not sure if I belong here or not, really. Please let me know any info you have or whatever about my son's issues. I have done some reading that says that portal hypertension and enlarged spleen are signs that the disease is in later stages. I don't know why I haven't ask my doc. I prob just was too scared to. Any ideas?

Sorry for my long novel. I'll keep it shorter from here out. Thanks so much for your posts and responses. I have learned a lot by reading them.

son Nick newly 9yrs. w/ liver disease 2/09