portosystemic encephalopathy and transplant

[pennycat]

Hi  I hope you could help me, I am a 30year old woman in UK with extra hepatic portal vein thrombosis since age 12 when I had oesophageal varicies bleeding.  All that was treated fine and until my child's birth 2 years ago I was ok, now I have daily periods of inability to speak or move, personality changes, day-night reversal and now a periodic shaking/jerking (but fully concious) of arms, legs and head.  EEG excess of slow waves and no epilepsy.  I seem to be getting so much worse every month.  Dr's think its PSE but I am on protien restriction and lactulose. (Tried neomycin twice, helped a bit) Liver function is ok and only other thing is biliopathy.

Would a transplant be considered for a person in my situation?  I am not a candidate for any TIPS due to location of thrombosis.  We're finding it hard to get any doctors to take my situation seriously here in UK, (I need daily care to keep myself and child safe) they don't know what to try, and we are considering coming to USA to find real experts!  

Thank you so much for reading this you must be so busy and I really appreciate it.

[Thomas D Schiano, MD]

you should not be having hepatic encephalopathy as in theory the liver should be normal.  sometimes however the liver can become cirrhotic after long term portal venous thrombosis.  With long standing portal hypertension, spontaneous plenorenal shunts can form which can lead to encephalopathy.

we are now using xifaxan (rifaxamin) as an adjunctive agent to lactulose instead of neomycin.  I have a colleague named Kaushik Agarwal at King's College Liver Unit who is excellent and who I might recommend you see.

[Thomas D Schiano, MD]

if the liver is non cirrhotic then closure of such a splenorenal shunt may be helpful.  there is no reason to perform a TIPS procedure.

[pennycat]

Thank you so much for taking the time to review my post, I do indeed have considerable amounts of shunts and so it helps me to know that I should be keeping an eye on my liver function, thank you so much.

I am also grateful for knowing about your colleague at Kings and will be making enquiries as if he would be able to see me.  

I am really grateful for your advice and feel now able to proceed with discussions with the team at Birmingham Liver unit about a possible way forward.

Many thanks and best wishes