Hi Steve and Nancy,
Sorry to hear about your experience over the last weeks. I'm glad you are getting a second option because as you have probably recently learned this cancer is somewhat rare and you need to make sure you have an experienced doctor.
As for the CA 19-9 it is a simple blood test. Elevation over 100 is normally considered suspicious. However with that being said, I had read stories of family members with numbers under 100 who are still very sick. My mother's was almost 400 when she was first diagnosed with a 3CM non-metasticizing tumor. ( and btw - feel free to be pushy and ask for this info). Others have had their markers in the tens of thousands range with no initial sign of a visible tumor ( the cancer was in cellular form). Generally the marker does seem to have a direct correlation to the progression. Right now there are several board members currently having chemo who have had their marker numbers reduced significantly due to their treatment. However, this one marker is only part of a collection of tools used for diagnosis, etc.
The diagnosis is usually centered around several indictors, such as the CA 19-9, visible site of a tumor on a scan, FISH staining of the ERCP brushings, history of PSC liver disease, etc).
Unfortunately the downward progression ( from what I read on caringbridge and various carepages,etc) seems to take the form of high billirubin ( failing liver) levels that do not allow the patient to continue with chemo. You can find this type of info under a separate section of this board dedicated to these specific types of questions.
Glad your are headed for the second opinion. Please keep us posted as we are hoping the best for Nancy.
Ashley from CT