Liver Clinic 9-19-12: Impromptu Consult

 

 Well, although stressful as usual, Else's "Liver Clinic" appt today was quite productive. We had a guest appearance/ consult by Dr. Shepherd (new director of the TCH Liver Center & renowned expert on BA) who Dr. Fishman brought into the room after I challenged him [again] on the issue of Ascites, since it does not show up on her fourth consecutive ultrasound. Well, Dr. Shepherd examined Elise and said he agrees that she does have ascites, but the fluid is likely hidden on ultrasound by her enlarged liver, "multiple spleens" and malrotated intestines.  Furthermore, he said that looking at her ultrasound and veins which are visible on her stomach, he can see that blood is being diverted away from the liver which puts stress on the vessels and further damages the liver. He said that it is clear that her liver disease is progressing and that transplant being required within the next 6-12 months is inevitible.

He went on to explain that she will do best with a left side of a split liver due to the way in which her intestines are malrotated, and it will likely take longer to find an exact match to what they want for her, which is why they have listed her 3 months ago, ahead of the game, and she is accumulating time on the list so that once her PELD jumps up, she will get offers over others with same PELD who have less time on the list. Makes sense, right?? I feel like I understand everything so much better after hearing it from the mouth of Dr. Shepherd. I like his accent too!  Anyway, it's wonderful that it seems everyone on our treatment team is on the same page.  

 

 

So now to get to the nitty gritty details....

 

 

 

Elise has not gained any weight.  In fact, she lost a tiny bit of weight since last month-- 5 grams to be exact.  In her defense, she did wet her diaper shortly before clinic. Anyway, needless to say, the team is not letting go of the NG tube. They said that they will be looking very closely at her weight gain (or loss) next month though to evaluate for the possible need for TPN/ lipds intravenously. In regards to labs, all of her liver enzymes are elevated from last draw, except her conjugated bilirubin which remains 0.0 (great!)  Her GGT has increased this month, and her Albumin is lower indicating poor absorption of nutrition. Her PT (clotting time) and INR are within normal limits though, which is good.

 

 

 

 

 

 

 

 

 

 

 

OH, and I want to be sure and point out that the "kasai' operation Elise had shortly after birth has not yet failed....which is the reason that her conjugated bilirubin is still at 0.0, and she's not jaundiced. For now, bile is still managing to drain out of her liver. Her doctors commented that she would be in a much different place right now if her "kasai" had failed. The transplant surgeon Dr. Goss, Dr. Shepherd, and Dr. Fishman are all in agreement that her liver still has a good bit of fight left, and more importantly, her little body is in good shape to fight. And she's apparently going to do it all with that precious smile on her face!! :-)