Budd chari syndrome

[ANGELPrater]

Hello everyone Im new to this site I really just need someone to talk to, Im 27, and have advanced liver disease, I have budd chari syndrome and it's very rare only 1@million peoplepeople have it so ive been living with this almost 3 year's and Im wondering if anyone has this disease?

[Hidden]

Hi.

I too have budd chiari and liver damage as a result of it. I am female and was 36 at the time I was diagnosed which was over 7 years ago.

I suddenly started feeling very ill with a swollen stomach. It took around 3 weeks to diagnose what the problem was - got told it was a stomach ulcer, then ovarian cancer before a CT scan confirmed it was my liver and that all the main veins in my liver were blocked with blood clots. I had a stent fitted to try and open up the least badly blocked vein in my liver which failed as by this point it had been blocked for some time and couldn't get enough blood flow through it.

I then had a TIPS shunt done which fortunately worked and eased the pressure in my liver and stomach by allowing blood through it. I was bright yellow at this point and not at all well and close to requiring a liver transplant. My liver was permanently damaged.

I started to feel better after the TIPS. The ascites drained out of my stomach and I could breathe again. I returned to 'normal' life relatively quickly. My legs were still swollen by the end of the day, I got tired easily, I was on warfarin to stop further blood clots in my liver and I itched like mad. I went back to full time work and other than not drinking alcohol at all and giving up holidays to remote destinations (outrageous insurance costs plus not wanting to be far away from decent medical facilities just in case), I continued my life as before.

The cause of my budd chiari was investigated - I was referred to the haematology dept and eventually it was confirmed I had a dodgy jak2 gene and PV. My blood was producing too many platelets which had caused the clots. I still go for 3 monthly blood tests to check my platelet levels. Currently my levels are ok but this is because I am anaemic and my body doesn't have enough iron to produce extra platelets (or red blood cells). Living with anaemia ok apart from more tiredness, sometimes feel bit dizzy and breathless but better than further blood clots.

About 2 years after the TIPS it blocked. I felt similar symptoms to when I was first diagnosed - swollen stomach and being out of breath easily, which I recognised and contacted the hospital. Somewhat miraculously over the 2 weeks it took to get a scan, my liver compensated itself and instead of blood flowing through the shunt it started to pass through lots of smaller veins in bits of my newly regrown liver. My liver is swollen as a result because it's got lots of blood flowing slowly through it but is actually doing its job remarkably well - the itching stopped too which was a bonus.

I still have regular check ups - liver scans, endoscopies to check for varices, blood tests, warfarin level checks.

My local hospital is St James in Leeds which is a transplant centre with a full team of specialists who I can't praise highly enough for the care and treatment I've received.

I realise I'm incredibly lucky compared to some of the people on this forum as I'm able to lead a relatively normal life.

This forum is a great place to get information from people who've been through similar problems. Ask questions and there will be someone who can help even if it's only words of kindness or support. Get better advice than just googling budd chiari although we've probably all done that too.

Sorry I went on a bit. I've always intended to put my experience of liver disease on here but never got round to it.

Julie

[Neph]

I have budd chiari too. I was 25 when I was diagnosed, I'm now 28. I also find it very hard to live with this, because it is so rare. I also had TIPS procedure as I was in liver, renal and heart failure. Because my GP left it so long before even doing blood tests. I also am JAK 2+. Feel free to contact me if you would like to chat. X

[Jjjo1 in reply to Neph]

What were your symptoms for this condition.

[Neph in reply to Jjjo1]

Hi my symptoms were tiredness, acities and I couldn't eat or drink due to my liver being so enlarged.