Does anyone else have NRH with CVID?

[htlands2003]

It is an autoimmune condition. But I don't know of anyone else with it. The liver specialist treating me doesn't have another patient either and The Liver Trust charity has never heard of it! It would be lovely to find someone else who has this too!

[elained]

I don't have it, and hadn't heard of it.

This is a site with a description:

livertox.nih.gov/Phenotypes_nodular.html

And this is some of the content:

Description. Nodular regenerative hyperplasia (NRH) is a form of noncirrhotic portal hypertension that can be caused by chronic use of medications. NRH typically presents with the insidious or unexpected onset of signs or symptoms of portal hypertension (weakness, ascites, splenomegaly, esophageal varices) in a patient with little evidence of chronic liver disease. The diagnosis is made by liver biopsy, showing absence of significant fibrosis and presence of nodularity usually best defined by reticulin staining. On superficial review, the liver biopsy may appear normal.

Latency to Onset. The time to onset of NRH is typically long, usually 1 to 6 years, but occasionally within 6 months.

Symptoms. NRH usually presents with the insidious onset of fatigue, weight loss, abdominal distension or other signs of portal hypertension such as ascites or variceal hemorrhage.

Serum Enzyme Elevations. Serum enzymes are usually normal or minimally elevated, although acute elevations in serum enzymes accompanied by what appears to be sinusoidal obstructive syndrome may precede the development of NRH. Jaundice is rare. The early development of NRH is manifested by an unexplained decrease in platelet count.

Drugs. Typical agents that cause nodular regenerative hyperplasia include azathioprine, thioguanine, mercaptopurine, didanosine, stavudine, isoplatin, vitamin A and possibly methotrexate.

[dianesistern]

Hi not been told that I have NRH but have lots of the syptoms one being low platelet count I have had two splenectomys for this (it grew back ) and when I asked my heamotologist what was causing it she said it was my liver . Dian

[karen]

Hi,

My husband has had high blood counts and his liver function has shown to be high when he was using high doses of antibiotics along with anti-inflammatory meds. When he came back off the antibiotics his reading went back to normal. His level is currently high and again he's just finished a course of antibiotics...we hope at the next blood test this week that his reading will be back to normal.
I'm not sure if this is in anyway similar at all to your situation.

Karen

[julieb]

I have not been on the site for a while so only just saw this post. I have both CVID and NRG. Have a really distended stomach which gastro people can't seem to get under control. Had really serious blood issues last year too. I am a patient at heartlands, Birmingham and would love to know if others there are being treated by gastro team or liver team.

[dreadnought]

Hi all

any one else suffering from this delightful combination of conditions?

i was diagnosed with cvid 15 years ago and have responded well to regular 3 weekly gamma globulin infusions but 
2 months ago I started selling up like a balloon resulting in a 44lb weight increase over three weeks!( due to ascites fluid build up)

this lead to an emergency hospital admission where after a month of testing, endoscopes, gastroscopies, biopsies scans, X-rays and ultra sound scans I was given the above diagnosis.

problem is as the combination is all but unknown doctors don't know how to treat it


anyone else in similar predicament and/ or receiving treatment, if so, what?
at the moment I am on diuretics and beta blockers to try and prevent liver getting worse but fluid seems to be building up again.

all help gratefully received as they are telling me it's probably terminal but they can't tellme how long!,

at only 51 I am not quite ready to roll over and die just yet,


Andrew Paterson

[s162216]

May 9, 2014 20:49:50 GMT dreadnought said:

Hi all

any one else suffering from this delightful combination of conditions?

i was diagnosed with cvid 15 years ago and have responded well to regular 3 weekly gamma globulin infusions but 
2 months ago I started selling up like a balloon resulting in a 44lb weight increase over three weeks!( due to ascites fluid build up)

this lead to an emergency hospital admission where after a month of testing, endoscopes, gastroscopies, biopsies scans, X-rays and ultra sound scans I was given the above diagnosis.

problem is as the combination is all but unknown doctors don't know how to treat it


anyone else in similar predicament and/ or receiving treatment, if so, what?
at the moment I am on diuretics and beta blockers to try and prevent liver getting worse but fluid seems to be building up again.

all help gratefully received as they are telling me it's probably terminal but they can't tellme how long!,

at only 51 I am not quite ready to roll over and die just yet,


Andrew Paterson

Hi Andrew,

Sorry to hear that your suffering so much, and all of the uncertainty; I really hope that their able to find something effective to treat it. There are actually a couple of members on the forum who have CVID and NRH, take a look in this thread here: www.primaryimmuno.proboards.com/thread/651/nrh-cvid I'll contact the lady who started the thread as I know she's quite keen to get in contact with somebody who has CVID and NRH, and as of yet she's not been able to with anyone.

I really hope that things improve for you,

Good Luck!

Sam

[mimi1]

Hello,

I do have CVID. Diagnosis 2008. I do not have this condition. I am on 15 grams of Gammugard weekly Percutaneous IV.
I am glad to know about the antibiotics and inflammatory use from the previous post. I do worry about the kidneys and liver. When I have used inflammatory agents with Gamma TX, and antibiotics, my kidneys do hurt for awhile.

Co-morbidities are Diabetes II, Large joint pain in between TX, vertebrae stenosis, acute carpal tunnel syndrome both wrists. Right I moved to Buffalo Wyoming from Phoenix AZ to be near my grand kids, the care is not great, and there is much stigma attached to ill people. My faith is slipping to obtain good cumulative care. I had 8 specialists in Phoenix and my coordination of care was good compared to here.

I would like to hear more about the progression of this condition as you go forward.

[s162216]

Dreadnought's thread merged in

[dreadnought]

Mimi, i am sorry to hear of the lack of care you are receiving.

At least in the uk we have a free if overstretched health system. In my csse it isnt really the lack of care but the lavk of knowlege of the specialists as cvid and nrh are an extremely rare combination so no research has been done by any majorvdrug companies as there is no income for them.

I amcurrently reasessing my diet andvgennersl philosophy on life andcwould muvh appreciatevany pointers on what if anything you have found that helps or hinders.

Kind regards

Andrew

[john]

Hello Andrew, I am sorry you are having a hard time and hope things will improve soon.
Your situation is a bit like my own, I have immunodeficiency and was given IVIg three weekly for 20 odd years.
During this time my liver function tests showed my liver was starting to cause concern and I got seen by the Gastro Dept.
They did all the tests you have had and discovered that I have portal hypertension, so I was put on beta blockers. At this time I decided myself to give up alcohol!
My liver was previously described as "fatty", but they're now saying its looking more "normal".
Trouble is that the hypertension is causing sporadic gastric bleeding, which entails emergency admission and tying the bleeding varices off whilst having an endoscopy.
At the present I have three monthly endoscopies to keep an eye on things, and as another precaution
I now get my immunoglobin weekly using subcutaneous infusion, in order to avoid a large increase in blood volume at infusion time.
I do have water retension in my legs due to the beta blockers and find that wearing flight socks help.
Diet wise I keep my cholesterol very low using Benecol, porridge and statins, and although I permamently have low energy and high fatigue, I try to exercise by walking.
I volunteer at my immunology clinic and it seems that dodgy livers are not unusual amongst the patients.
Sorry to waffle on, if there is anything I can elaborate on for you, just ask.
Chin up Andrew....John

[htlands2003]

I am the person that started this thread looking for other people who have NRH and CVID. I was diagnosed 3 years ago following a liver biopsy. I am being seen by a Liver Consultant at the regional Liver Unit at the QE hospital in Birmingham. Luckily any deterioration has been slow and I am not taking any medication though I had heard that beta blocker or a liver stent to bypass blockages could be used. As this been mentioned to you?

I think it is vital that you are seen by a liver specialist rather than a gastric team. Also insist that you see the consultant not a Registrar. It is so rare that they don't know anything about it ,and worse than that, they can tell you completely inaccurate things! One told me " you do realise don't you that eventually you will have to have a liver transplant" . That left me dumbfounded as the consultant had never mentioned that in the past.

I wrote to the consultant (after my daughter had said she would be a live donor for me !) and he apologised for the fact that his registrar had not known that NRH was an autoimmune condition not a liver disease!

I am so sorry to hear that your symptoms are so severe. If you are not happy with the treatment that you are having you are within your rights to ask for a second opinion and I would ask to go to a Liver Unit to get the best possible treatment.

Let's hope they will be able to support you better. Margaret

[john]

Hello Margaret,
At the Royal London the Hepatology Dept And the Gastrology Department come under the same roof, so I am fortunate to have access to both.
It seems to me that the reason I take beta blockers is simply to reduce the blood pressure in the portal vein and thereby minimise the risk of bleeding into the gut from leaking varices.
I also take lansoprazole each day to reduce the acid in my gut, which might be corrosive.
Liver stents have been mentioned to me but apparently they come with other risks that the docs don't want to expose me to. Tying and banding obvious varices has been pretty successful for me so far, although it does mean lots of endoscpies, but this is better than having a bleed, one of which almost killed me two years ago.
I am very happy for you that things are stable, it is very scary when the experts mention the word transplant, the very last thing Iany of us would want to have!!
Enjoy the rest of the weekend
John

[karen]

Hi all,

Talking about 'dodgy livers'...this is a new phenomenon for us as Mark has been advised that his lover count is high for the second time in the last 6 months. We didn't think anything of it as he'd been on high dose Arcoxia (Cox 2 inhibitor) for arthritic pain and he had to cut it down and do another blood reading which showed more normal. However, it's happened again and for no apparent reason. I wonder if this is a new path we're going down as there seems to be many roads that CVID takes people down.

Andrew and Margaret - I do hope that you get further forward to more information on what's going on with you.

Best Wishes to all.

[jenny]

I was diagnoside with CVID in 2012 and have recently after a hepatic wedge pressure test and liver biopsy been told I have portal hypertension and NRH. I find that I am going from immunology clinic to liver and nobody is telling me what this means for my future. I have constant pain in stomach where it is so swollen and am know getting severe pain in middle of chest. Does anyone know what the future might hold regarding life expectancy. Has anyone been living with these conditions and can give me any advice?

[s162216]

Hi jenny and welcome to the forum.

I find it a bit concerning that you've been given a major diagnosis like NRH, and have not been told very much at all it seems. I don't personally have it so it's difficult for me to offer support about it, but I know there are several members on here with it, and I'll try contacting them for you to see if they might be able to be of any help.

Are you seeing your liver consultant shortly? If not then try your best to get an appointment as soon as possible, and when you see them be firm that you have questions that you want answers to. Most doctors are perfectly happy to answer questions, and this could very well have a major impact on your life so you deserve some answers; your clinical immunologist or GP may also be able to give you advice and support so you could try them as well. Primary Immunodeficiency UK (one of the patient support charities) may also be able to help, you can find their website at www.piduk.org/ and their contact details can be found at www.piduk.org/contactus/.

Regarding the pain you're experiencing though, if you're still having it shortly then I would highly recommend that you go to see your GP or other medical professional, and if it gets worst then go to your nearest A&E as you can never be too careful with things like this (as a rule we don't offer medical advice on the forum as obviously we are not doctors, and would not want anything to go wrong based on inappropriate advice - I'm sure you can understand our reasons for this).

I hope you find this information useful, and you're able to get the advice you need.

Sam (Admin)

[jenny]

Thanks Sam (Admin). I have an appointment with my immunology consultant on Weds.  This has only taken me 3 months and lots of chasing to get. I am hopeful that he will be able to give me some answers. jenny

[john]

Hello Jenny,
First of all welcome to our forum!
I have immunodeficiency of a type known as Good's Syndrome, which behaves and is treated in exactly the same way as CVID.
I have been on therapy for more than 20 years now and during this time my liver has become problematic, so now, like you I see a liver team as well as my immunology team.
The main liver problem I have is portal hypertension causing some oesophagal and gastric veins to bleed into my gut. This is a dangerous situation and I now have periodic endoscopies to check on the state of the varices (veins) and to band them off if they look like they might soon bleed.
To reduce the high blood pressure which makes them bleed I now take a twice daily beta blocker tablet, and the team are happy with the results. I gave up drinking alcohol three years ago to try to minimise further liver damage, although I was assured that my liver problems were not alcohol or lifestyle influenced.
I don't have the gut and chest pains you describe, but I do have severe gastric upsets. No-one has ever talked to me about my having NRH...maybe I should ask about that.
I am pretty well managed for my problems, and hope my life expectancy will not be compromised!!! (TOUCH WOOD!)

[htlands2003]

I'm sorry you are getting so little help. But it is so rare that it is difficult to find anyone that knows much about it, Liver charities know nothing either.

Could your pain and symptoms actually be caused by something else? I only ask because NRH is often without symptoms which makes it difficult to diagnose in the first place?

I am not a doctor and have no idea which treatments would work for you. So that is my disclaimer before you read on!

Let me tell you what I have found out over the past three years since I was diagnosed. I have had CVID since 2003. Three years ago I had liver tests done at the request of my Immunologist because my regular LFT results continued to climb.I had an ultrasound and a fibro scan which were in inclusive ( never heard of a hepatic wedge test!!)

I then went on to have a liver biopsy that conformed NRH. It is an auto immune condition, so completely different from CVID.

There doesnt appear to be any medication for this but I see that someone in an earlier posting says they are on betablockers, so ask about those. It seems that immunoglobulins help in some way to reduce hypertension. I am fortunate that I do not have portal vein hypertension( as yet!) though it appears to be inevitable in the future.

The liver texture changes over time with NRH and becomes more course in texture which leads to pressure build up in the liver that can cause hypertension. The most common treatment is a stent in the liver to by pass the obstruction, I believe, so it is strange if this or other possibilities have not been mentioned to you.

Are you being seen at a large teaching hospital or regional liver centre? If not I would ask to be referred there, as they are more likely to have the expertise to deal with this. It rarely leads to the need for a transplant my consultant has told me. But avoid being seen by a registrar!! They will now nothing. it is your RIGHT to be seen by the consultant so ask at the beginning of the clinic and they will mark your file to be seen by him. Even better ring up before hand and check he is not on leave!!

I was seen by a registrar who told me a had a liver disease, which is not true, and he told me I would need a liver transplant (also not true, but it had my daughter looking on line to see how she could be a live donor, which was something we could both have done without}

The only other treatment that I have heard of is done during an endoscopy to the stomach. The NRH can cause bleeding in the oesophagus (varices)which can cause bulging. This area is tied off somehow ( I havent gone further into this as there are some things I would rather not know about!) This has been mentioned to me however as one line of treatment.

I am told that about 10% of CVID patients get NRH. A consultant a Oxford has done some research into this, her name is Professor Helen Chapel. So you may want to look at anything she has available on the net.

I hope this has been helpful? Do post again if you want any more information and support!

We all need to support each other through the difficult times.

[julieb]

Hi. I have not been on the forum for a while, just trying to cope with all the health problems thrown at me. I continue to have distension of the abdomen but even though I have portal hypertension and NRH they do not think the distension is due to these. I do not seem able to get any good answers. I was diagnosed with chronic norovirus and I am having 'experimental' treatment for this but it does not seem to be helping any of my conditions. If anyone gets any good answers about NRH it would be great to share them.