Budd Chiari Syndrome

[Jo3560]

Hello all,

Does anyone else on this site have Budd Chiari Syndrome combined with a myeloproliferative disorder such as polycythaemia vera?

Would love to hear from anyone else with this disorder!

Thank you

[Harrisonmatty93]

I have both yes

[Jo3560 in reply to Harrisonmatty93]

Hi thank you for replying . When were you diagnosed and have you had tips procedure? Do you take medication . I was diagnosed in 2005 and had Tips and currently take warfarin, aspirin and hydroxycarbamide . I'm sure you agree that as this condition is so rare it is hard to find people in the same position !

[Harrisonmatty93 in reply to Jo3560]

I have had stents put in my left hepatic vein, I also take warfarin and aspirin but that is all as of yet. I'm going for a liver biopsy in June as last time they tried to do one it was unsuccessful. Very rare conditions, Chelle_ helped me with a lot of information when I was really ill as she has experienced a lot.

[Chelle_]

I don't have Budd Chiari, but I do have Cirrhosis of the liver, Portal Vein Thrombosis along with Polycythaemia Vera. I am one Hydroxcarbamide, warfarin, propranolol and two type of diuretics. I am on the transplant waiting list. I do have compassionate approval for Ruxulitanib but not until after the transplant. How are you coping? x

[Harrisonmatty93 in reply to Chelle_]

Hi Chelle, not spoke in a long time, quick update though, I'm doing really well compared to what I was and I'm getting on with my life as normal! Thank you x

[Chelle_ in reply to Harrisonmatty93]

Excellent news Matt. I hope things remain smooth for you. x

[Jo3560 in reply to Chelle_]

How long have you been on hydroxycarbamide ? I've only been taking it for a month. After my initial Tips 12 years ago I have regular tipsagrams under general anaesthetic - approximately 3 times a year . The reason is my stomach swells up although I have no aesities and straight after tipsagram I loose approx 1/2 stone in weight over night by constant visits to the loo ! I am under the most amazing liver consultant at the Royal Free, but to my knowledge no one else seems to swell up as frequently as I do !!

Thank you for your reply

[Chelle_ in reply to Jo3560]

I have been on it nearly 3 years now, I did start on the interferon but didn't get on well with that. I have a blood clot in my portal vein so I am classed as a 'high risk' I was dx in Feb 14 and have been on medication since. I get ascites and am on 2 types of diuretics they seem to do an ok job. I haven't ever had TIPS, I do have endoscopies every 4-8 weeks to try and stop the bleeding on my tummy. How are you getting on with the Hydroxycarbamide?

[Jo3560 in reply to Chelle_]

The first two weeks I felt very sick but thankfully that has now settled. I am extremely tired all the time but if the tablets reduce my platelets that's just something I will get on with ! x

[Chelle_ in reply to Jo3560]

I am constantly tired too and just get on with it. I still work full time so that takes a lot of my energy. My platelets hover from about 70-140 and I get quite anaemic. To be honest because of the liver problems my MPN takes a back seat.

[TheMaestro07 in reply to Jo3560]

Hello, I was diagnosed with BCS at the Royal Free earlier this year about 40 odd days in a different London hospital where BCS and other conditions where ruled out. I have had TIPS, the week after my diagnosis, and was discharged exactly 8 months ago today. I go to see Dr Patch (performed the TIPS) and also have less regular appointments with Haemotology. I am on Warfarin, 2 types of diuretics (Furosemide and Spironolactone), Folic Acid, Vit. D and Cetirizine. I was on Aspirin as an inpatient but was taken off that 3 months ago and my target INR increased to 3.0 from 2.5.

The staff and the consultants at the Royal Free were and remain to be really good, I couldn't have asked for more from them, I count myself as fortunate to have been transferred there. I haven't had to had any further procedures and the ascites has generally stayed away, although 25 litres was drained over two separate drains in addition to the fluid passed through IV diuretics (I lost 35kg if you compare my weight when I was admitted on 31/10/16 to when I was discharged on 24/01/17), so know the weight loss feeling, although it hasn't been regular like yours. I have an ultrasound on Friday so let's see how it goes!

Shout if you want to chat, I haven't really found anyone willing to chat that has BCS in the UK but I know how helpful it was for me to talk to someone (in the States) that had undergone a very similar experience.

I hope things are well with you since you posted this, which I know was 6 months ago!

Best wishes!

[Jo3560 in reply to TheMaestro07]

Hi !

For some reason my earlier reply has vanished ! Thank you for responding . Dr Patch also did my TIPS at the Royal Free in 2005 and continues to do tipsagrams approx every 3 months. He is amazing and I won't let anyone else do them ! I also attend the joint hepatology and haematology clinic at the RF every 3 months where they check my meds and have scans. I currently take warfarin, aspirin and hydroxycarbomide which are all long term. I am due to have a tipsagram on Wednesday as once again my stomach has distended .

It's great to find someone who has the same condition as it is so rare and I'm more than happy to chat whenever you feel like it.

I hope your scan goes well

Best wishes

Jo

[TheMaestro07 in reply to Jo3560]

Hey Jo

Who knows, we may bump in to each other at the RF at some point! I'm there Friday for my US and then seeing Dr Patch in just over 3 weeks time. Will also be back in Dec for my haemo appointment where they may tell me whether or not my BCS is through a genetic mutation as there is no history really of any liver issues in my family (predominantly been the heart).

I wouldn't let anyone else perform the TIPS too, he's done a good job and I feel fortunate to have gone back to work full time 2 months ago after 2 months of phased return which was after 7 months off.

I hope the procedure tomorrow goes well - I'll try and remember to drop you a message after - wishing you the very best of luck!

Best

Mish

[Neph]

Hi yes I have both I was diagnosed in 2012 I had TIPPS procedure. I am currently on warfarin and Pegasus.