Another doctor, Another hospital and confirmation of Portal Hypertension

Email August 29, 2014

Yesterday we met with the chief of the division of pediatric gastroenterology, hepatology, and nutrition at the Children's Hospital of Atlanta....pretty intimidating but I haven't felt so informed in 3 years and feel this is the most aggressive plan we have ever had. Our new doctor took time to explain and diagram everything...as most of you know I do better with visuals! The facilities in Atlanta were pretty awesome and despite knowing we
were at a hospital it was such a happy place.

-as far as the doctor is concerned Patrick does not have liver disease but does have a liver issue

-definitive no on congenital hepatic fibrosis right now(so no need to discuss transplant right now!)

-feels the scarring doctors have seen is the shadowing from portal hypertension

-Patrick does have portal hypertension but manifests in his vessels and not the liver; he has some sort of clot in his portal vein which is causing the back flow into his spleen and most likely down into his intestines area; Patrick's body has made collateral vessels to help carry the blood but noticed these are bulging and becoming 'hot' (?)

-liver was enlarged yesterday dropping down but is not concerned; spleen has gotten bigger again even from a few weeks back in comparison with imaging

-Andy's mom had mentioned that two of her brothers have Factor 5 (blood clotting issues) and they were extremely concerned with this...ran blood panels for this and hope to have them when we head up to Duke Hematology/Oncology on 9/11 - this could be the reason for the clot in his portal vein and further clots could manifest

-Patrick is on track for height but is severely underweight (more than likely due to diarrhea)

-portal hypertension can cause problems within the esophagus/intestines and our biggest concern will be bleeding 

-We will return to him but before then we need to have an endoscopy and small regular bowel biopsy (basically biopsy the intestines and lower stomach areas).  

-During this time we also will see local and Duke liver doctors and Duke hem./onc.  If we notice signs of internal bleeding we need to rush him in.

-After the biopsy and endoscopy he will be able to see where the varices are and will begin to discuss banding these (basically taking a rubber band).  At the beginning of our visit he talked about this possibility in the future and that we could do this locally....by the end of the exam/history he decided he wanted to be the one to do it.  He also instructed us (even in the discharge notes) to not have anyone remove his spleen...our MUSC doctor was very adamant on this as well as it is the only indicator of what is going on...and yesterday he said that this organ is the 'victim' not the cause.  Plus, in the future when the PH worsens there is a procedure they can do but can only do if the spleen is still there.

I kind of had written off the appointment in Atlanta thinking we would get the same instructions to just wait and see what happens.  Our appointment had been scheduled with the division chief but really figured we'd have some other doctor in there so was humbled by him walking in and telling us that our little boy has a lot of problems and he was here to help us figure them out.