Non-cirrhotic portal hypertension

[katiegardenhead]

Hi all,

I'm hoping to hear stories or advice regarding non-cirrhotic portal hypertension. My dad is currently in our local hospital's HDU recovering from a huge variceal bleed caused by non-cirrhotic portal hypertension that landed him in intensive care for 5 weeks. Prior to this, he had been in and out of hospital for about a month because he kept having variceal bleeds that meant a visit to A+E, investigation via an endoscopy/gastroscopy, and then a banding procedure...however, unfortunately this process didn't really work, which led to the huge bleed. We are very lucky that he's still with us. He is now doing much better and recovering - however, the root of the problem (the portal hypertension) still has not been 'fixed' and his consultant keeps telling us that it is a very rare case because his liver is absolutely fine (which we expected) and the banding procedure normally works on 95% of people with non-cirrhotic portal hypertension. I was wondering if anyone here has any similar stories, or has a similar condition? And if so, did you have a procedure or plan apart from banding/'gluing' the varices, and medication to prevent it?

Dad's consultant wants to refer him to a liver and gastro specialist in another hospital, which we are all very keen to have happen. The worst part is the waiting in between that - dad is still in hospital and hasn't had a bleed in 5 weeks, but his bleeds are so unpredictable and the waiting is really awful, especially as they want to move him to a normal ward where his blood pressure/hemoglobin will be monitored less. Basically I just want to hear stories of living with this condition, and results/procedures that have worked for you. Please let me know if any of this doesn't make sense! My brain is a bit all over the place at the moment. Thanks in advance

[Faithfull]

Just to let you know thinking of you and all the best.

[katiegardenhead in reply to Faithfull]

Thank you

[jojokarak]

Have they mentioned a stent at all? This would slow the blood down through his veins and take the pressure off

[katiegardenhead in reply to jojokarak]

Hi, they have mentioned a stent but also said that they don't know if it would help in my dad's situation - when we had the conversation about the stent it was when my dad was very critical, so the consultant just said it wouldn't be a possibility for a long time and left it at that. He also had a heart attack caused by the bleeding whilst in ITU, so I think that has affected his chance of having the stent any time soon we're hoping to speak to his consultant tomorrow regarding future treatment now that dad is stable, and I'm definitely going to bring up the stent again!

[jojokarak in reply to katiegardenhead]

I just seen your other post as well ... Ask about beta blockers as well they put me on them for a time but I have asthma and they didn't suit me but a lot are on them for variceal bleeds. I was in ICU in a coma after a bleed that's when they fitted my stent and it saved my life my family were concerned about them moving me to a general ward but I didn't suffer any ill effects from it they will monitor him so closely I was getting my blood pressure checked every 4 hours it's great for the patients state of mind as well I know it's awful living with the chance of a bleed could happen at any time but most that do are sorted out they will figure out a solution and they will give him the care he deserves x

[katiegardenhead in reply to jojokarak]

Thank you so much, this is so so helpful to read. We've all felt a bit isolated because dad's condition seemed so rare (I hadn't even heard of varices before and when I mention it to other people they look confused!) and then the heart attack happened and was a huge setback because obviously treatments you use for heart attacks often thin the blood...it was horrible. Every four hours is the same as they're checking it in HDU, so that's really promising! I just want to make sure he's moved to the right ward (and the ward that they want to move him to recently received a patient from ITU who suffered a cardiac arrest on their ward and they didn't pick up on it for 20 minutes :/ so now he's back on ITU). Did you have the TIPS procedure? I'm just trying to tell myself that if he has a bleed they'll catch it but they didn't before which keeps stressing me out! Really we want him to be moved as an inpatient to the specialist hospital, and I think his consultant will push for that...fingers crossed. How are you doing now? I hope you're doing well and have recovered well from your time in ICU! Thanks again for your message, it helped loads xx

[jojokarak in reply to katiegardenhead]

I did have the tipps procedure and for 2 years was fantastic for me I didn't have another bleed but I already had cirrhosis but I had a transplant a year ago so am a hundred times better ... It will feel impossible at moment but I have always put my trust in the consultants and here I am after 5 years alive and well 😀 it's more worrying for family members rather than the patient x

[katiegardenhead in reply to jojokarak]

Oh I'm so glad to hear that! And so glad to hear you're doing well now you're right - the doctors keep telling us that this is the hardest part for dad now, because he's been through the worst of it fully sedated and doesn't remember so has nothing to compare it to, whereas we've been through the worst bit for us, but I think we're still more worried than dad is now! ITU have been amazing so I trust them, but dad's case is so rare because his varices/hypertension are non-cirrhotic so they've never really seen anything like this before, whereas hopefully the specialist will have! He hasn't had a bleed in 5 weeks and I'm hoping that as long as they monitor it he will be okay until they figure out the long term plan xx

[Eurotrash in reply to katiegardenhead]

Hello. I'm sorry your dad is unwell. Have they established the cause of the portal hypertension? I've heard of blood clots causing it. It's positive to hear he hasn't got cirrhosis. All the best.

[katiegardenhead in reply to Eurotrash]

Hi, so sorry for late reply, my dad was discharged from hospital so have had a very busy couple of weeks! They believe the cause is related to his pancreas, as he has pancreatitis a few years ago. His liver is absolutely fine which, as you said, is really positive! His type of portal hypertension is really rare, though...we're trying to set up a meeting with a specialist of NCPH in Birmingham asap.

[jojokarak in reply to katiegardenhead]

They will and in a way it's a blessing it's a rare case it's going to get a lot of interest from guys or gals in the top of their field I know it's hard but try and stay positive 😀 where are you based?

[katiegardenhead in reply to jojokarak]

Hi, so sorry for late reply, my dad was discharged from hospital just before the new year so have had a very busy couple of weeks! You're right - all of the top specialists from around the country have been putting their heads together and we should be having a meeting with the top NCPH specialist in a week or so...fingers crossed! Dad has a gastroscopy just before he came out of hospital and there were absolutely no signs of varices in his throat or stomach, thank god, so I feel like we can relax a little bit, but obviously the hypertension is still there. We're based in Northampton, England! How about you?

[jojokarak in reply to katiegardenhead]

Oh that's great news and glad you are feeling better x. You are near my father in law and he under liver doctors as well lol I am in Manchester x

[katiegardenhead in reply to jojokarak]

Thank you! Oh, so not that far just wanted to let you know that my dad has an appointment with a NCPH specialist at Birmingham in just over a week, we're so happy! I thought I'd let you know because your messages really helped when I was feeling a bit despondent and hopeless about everything.

[jojokarak in reply to katiegardenhead]

Fantastic news he being sent to one of the best hospitals I am positive you will get somewhere now and I glad I could help a little bit thanks for letting me know and fingers crossed the appointment goes well xx

[katiegardenhead in reply to jojokarak]

Thanks! He's going there today, I'm really nervous! Will let you know what they say fingers crossed!

[jojokarak in reply to katiegardenhead]

Good luck xx

[jojokarak]

I noticed you have a post on BLT and ICU steps x I on both as well

[katiegardenhead in reply to jojokarak]

Great! I'm going to have a read through your posts

[Splodge60]

I’m not sure of some of the terms you use eg. What is a varices bleed? What is portal hypertension? I am bleeding from various orifices at present but I don’t know why? Have NASH with very high liver enzyme results. I can understand your concern. Let us know how he gets on.

[jojokarak in reply to Splodge60]

Varices bleed is veins in our oseophagaus being swollen like varicose veins on your legs

[Hidden]

Hi. I have had a variceal bleed and was put onto a normal ward straight away, even though I was on oxygen and the machine for my drip kept going on the blink. After about 3 days, I was sent home to recover. That was nearly two years ago now. I was taking a beta blocker (although I can't take the full dose), and had recently had an endoscopy with banding. So I'm afraid these things are not always easy to predict. I also have portal hypertension without cirrhosis (although that is borderline). My fibroscan and biopsies do not indicate that I should have portal hypertension, so I too am a rare case. (I do have an auto-immune problem)

Our latest visit with the consultant meant that the explanation we got for such an aggressive reaction from my body, despite my liver state indicating otherwise, was that it does matter where the initial attack on your liver occurs. However, even if they knew where the initial attack on the liver occurred, they would not change the meds.

In short, I am on a beta blocker and have regular endoscopies (once a year) as well as other meds for my auto-immune condition. So far I have not had any further bleeds.

I do hope that things improve for your dad as he sounds as if he has been hit pretty strongly. I have not been offered TIPS and I don't really think I would want it. I have lived normally since the bleed, although other aspects of my condition have caused me other problems. However, bleeds have not, thankfully been one of them. Hang on in there and if the hospital won't give you a referral, get one through your GP. My GP referred me to the QE after I took advice from a consultant other than my own.

Good luck and hope this helps

[katiegardenhead in reply to Hidden]

Hi, so sorry for late reply, my dad was discharged from hospital just before the new year so have had a very busy couple of weeks! Your message helped so so much, I wish I could get across just how much. It's made us all feel so much more hopeful. We should be having a meeting with Dr. Tripathi at the QE in the next week or so!

[Hidden]

Hi there katiegardenhead. I'm so glad that my post was actually of some use to you and also that your dad has now been discharged. I remember quizzing my husband about what happened during the initial part of my stay (i.e. when I had the bleed) and discovered that it wasn't as terrible as I had imagined it in my mind. Having spoken to a consultant recently about the prospect of future bleeds, they have re-assured me that through the continual endoscopies and meds (carvedilol in my case) that this should be avoided. Also, it is not the bleed itself that it is the major issue, it is the time afterwards because of the possibility of infection, so hopefully his prolonged stay in hospital has made sure that this is not an issue either.

Thank-you so much for getting back to me and I really hope that all goes well for your dad. I do hope that you get the answers that you are looking for at the QE. My experience with them so far has been very positive and because I like straightforward and to the point, that is how they have been with me.

All the very best to you and yours

[katiegardenhead in reply to Hidden]

Thank you so much my dad has his appointment with Dr Tripathi at the QE in just over a week, we're so happy!

[Hidden in reply to katiegardenhead]

Excellent katiegardenhead. Make sure you have all the questions written down. I am absolutely positive that with your caring attitude and cool head, everything will go well.

[AileenHolden]

Hi Katie, I just signed into this website to hopefully find out some information for my self when I came across your post. I skimmed through the comments. I wanted to let you know that I have NCPH too. I am having the TIPS done at the end of June this year. Ive had a few bleeds, and I survived (and baby too) a pregnancy. Sorry to hear about your dad.

I'm still overwhelmed with all this information and wanted to find others to see if I can help them, or they help me!

I'm taking Nadolol and beta blocker too. Hope this info finds you all well.

Aileen

[Pankajsinghrawat14]

I am exactly in same situation and after bleeding 2-3 times finally went for TIPS procedure 2 days back. I hope your father is doing well. Has he gone through TIPS. Please update me in case any root cause of PH is found at your end.