Budd chiari syndrome

[Neph]

I'm 25 yo and have recently been diagnosed with bcs, which is quite a rare condition, so I was quite shocked about having this condition. I was in a critical state two day after I had been admitted into hospital. By the time I had been transfered to a specialist hospital I had gone into renal and liver failure and my husband and my mom were told that if the TIPPS procedure didn't work there would be nothing else they could do for me,( I'm quite glad that I was oblivious to all of this). I am now thankfully back at home with my two young children and my husband. I still feel shocked and scared by all of this and I'm finding it hard to get my head round. I had my TIPPS procedure 7 weeks ago now but I still feel tired and my whole abdomen area aches. I just want to feel better so I can get back to being a mother to me children I feel as though they are missing out on so much as I am unable to do things with them because I am constantly tired and uncomfortable. Does any body know when I feel a bit more like my self?

[nickprich]

I was diagnosed with acute BCS in 1998. In my case I had a mesocaval shunt after three months of wait and see and a failed TIPPS procedure. Following the surgery, which was needed as all else had failed, I began to get better, but slowly. The aching abdomen and constant tiredness I put down to recovery and in time it went.

You had a TIPPS procedure which is relatively non invasive. Do you know for sure that it worked? The tiredness and discomfort in the abdomen do not strike me as recovery based. To be told TIPPS not Woking meant there was nothing else they could do alarms me. Was a shunt not discussed? Transplanting?

Please get some support with this . BCS can be managed with anti coagulants for years, yet the pressure that builds up in the portal vein will eventually result in shunt or transplant as the last resort.

I am more than happy to relate any information I have about BCS. Take care of yourself.

Nick

[Neph]

Hi nick, i had a scan before I was discharged and the TIPPS was working, plus my acitisis has gone, my husband and mom where told that was the last thing they could do for me because of how long I was left for. Even the TIPPS procedure was tricky for the surgeon to do, shunt was mentioned but it had gone to far for that procedure. They are still trying to find out if I have an underline condition that could of caused this. I have questions for my consultant every time I see him, I mentioned the pain I was getting and he said that I had an enlarged splen and the pain was properly from this. Thank you for your comments and reassurance my next consaultation is in December so hopefully I will get some answers.

[nickprich]

Hi Neph,

Glad to hear that the acites has gone and the TIPPS worked. Have you been referred to a Haematologist, at Consultant level? If not, try and push for this as your enlarged spleen and BCS are indicative of an MPD ( Myloproliferative Disorder ). 50 % of BCS patients have this as an underlying condition, or mutation in their JAK 2 gene. It's 'big science' and i don't get it all after all this time, but if you research it it may give you some clues or cues to discuss with the consultant when you can.

Bye for now,

Nick

[Neph in reply to nickprich]

Hi nick thank you for this information I have been referred to a haematologist but not yet had a date to meet him and discuss my bloods. Have you also got on of the above conditions that you mentioned above? If so do you have to take medication for this too or just for bcs. Thank you again for your comments you have put my mind at ease a considerable amount.

Neph

[nickprich]

Hi Neph ( not sure if this is your first or sign in name : ) )

I have an MPD called Essential Thrombocythemia which was diagnosed less than a year ago. Along with Warfarin for anti - coagulation I also take aspirin and a drug called Hydroxycarbamide to lower my platelets to an acceptable range. You will see my history if you wish look at my profile.

There is a chance that you too will have an MPD but an equal chance you won't . It may be that the MPD contributed to your BCS, or maybe it didn't. Not wanting to sound flippant, but you would have to talk to haematologists about this, but from what i know you can be screened for the MPD now should you wish ( it's for the JAK 2 gene/ protein mutation which will tell you if you possibly have ET or one of two other MPDs - Polyceama Vera or Myleofibrosis).

Please get information from a support group called MPD voice ( you can access it through this healthunlocked site ) but bear in mind it's all early days. The BCS is more than enough to be dealing with and the haematologists should screen for the MPD in good time. Your spleen issues tell me it should be soon, however. Knock heads together if you can; I believe that I should have had the hydroxcarbamide and aspirin before I did , and communication between two hospitals would have ensured this happened, but that's my opinion.

I may have had the ET for over 15 years and, on it's own, its only in the last ten months that i have had to take the hydroxycarbamide at a relatively low maintenance dose. If the condition is managed correctly it has no impact on life expectancy. For information about PV and Myleofibrosis the MPD site should tell you all you need to know.

Phew! That's a lot to get your head round, but i hope you can get tthe information you need to make an informed decision about your future treatment.

Take care,

Nick