Hi, anyone else here with a diagnosis of Nodular Regenerative Hyperplasia of Liver?

[Tootingalong]

It would be good to hear from anyone else who is living with this condition. My partner was diagnosed after a massive bleed at home from oesophageal varices (that he was unaware he had!) 5 years ago. On admission to the emergency dept. of hospital, the blood loss and trauma to his body was so great that after several days in intensive care followed by a week in H.D.U. and finally on the general ward, the Consultant came and sat with us and told us that they hadn't expected him to survive. He also apologised to me as when he and another doctor had questioned me about his drinking habits and I had said that "he doesn't drink and never has because he's one of a few people who doesn't like alcohol!", they had dismissed this information as he had presented to the medical world with burst varices and they had made the assumption that it was alcohol related. During this chat at his bedside, he also confirmed that after extensive tests and observation, they realised what a (previously) healthy and fit man he was and that it was, indeed, this fitness level that had contributed greatly to his survival. So, skip forward a while and they've carried out tests for every non-alcoholic liver disease imaginable (P.B.C, Wilson's disease etc). Eventually, a biopsy was sent off to a Professor at Newcastle from the liver unit at Glasgow and he was given a diagnosis of Nodular Regenerative Hyperplasia of Liver. I suppose it could be said that he's fairly lucky to have this diagnosis as its normally diagnosed at post-mortem! So, here we are 5 years down the line, he still has banding under sedation every 6 months (the usual life-long procedure), EXTREME fatigue, portal hypertension, splenomegaly, awful oedema of legs and scrotum (thankfully this comes and goes as its painful and uncomfortable), leg cramps and awful itchiness over his body. He takes carvedilol, spirolactone, furosemide, atarax and questran. He's also advised to follow a very low-salt diet and drink lots of tonic (for the cramps). I don't know why I've put this in the "Question" section, it's more of a blog; apologies. I think I really am just wondering after all these years if there is anyone out there with the same?

[Bolly]

Hi Tooting. I didnt know about this condition before your post, so looked it up. I see from research articles on PubMed its described as a 'very rare cause' of the symptoms you describe.

I hope someone with personal knowledge comes along and reads this and gets in touch. It must seem quite lonely. How knowledgeable is the team you partner is being monitored by? My hepatologist trained at the Freeman Newcastle, they seem to be a good place for information - he still gets in touch with his mentors there when he's uncertain about me (I'm a not very common overlap between 2 liver diseases) even though he no longer works there.

[Dreadnought]

Hello Tooting, I was diagnosed with NRH on 10th April and was told rather like your partner that it was a rare and life threatening condition. In my case my emergency admission to the Chelsea and Westminster hospital London was as a direct result of my diagnosis of large ascites, (build up of fluid in the abdomen) in my case the fluid had built up quite suddenly during a 3-4 week business trip to the Middle East during which time I went from 66kg to over 86kg!

For over a year I had been suffering from a variety of gastro-intestinal problems including giardiasis,campylobacter, salmonella and various others which had all combined to bring me down to the lowest weight I had been for almost 35 years. I assumed that these various stomach upsets were largely due to my travels in the developing world where I had been designing and building schools, clinics etc in some fairly off the beaten track locations in Pakistan and East Africa.

At the same time as all of this I have been receiving regular treatment for common variable immuno deficiency (cvid) which consisted of a blood transfusion of antibodies every three weeks or so as my schedule allowed. This was first diagnosed in the summer of 1999 after repeated cases of pneumonia, pleurisy, bronchitis, sinusitis and similar respiratory tract infections going back to the early 1980s. The immunoglobulin treatment had been a great success with no major infection of any sort in 15 years as opposed to pre diagnosis when I suffered multiple infections, almost continuous use of antibiotics and regular hospitalisation for 15yrs plus.

My immune consultant from the Brompton hospital is sure that this antibody treatment is in someway responsible for the NRH and the ascites, which went on during my month in the C&W to develope into huge swelling of the scrotum, oedema in both calves and ankles and great pain, numbness and pins and needles in both my upper thighs, this pain was particularly bad in the Middle East and left me unable to walk more than a few yards or so on many occasions.

Good news is that following the insertion of a drain and the removal of 5 gallons of fluid over 5 separate procedures, most of the discomfort and swelling subsided. Like your partner I am now on beta blockers, carvedilol diuretics , spironolactone and furosemide as well as general antibiotics , iron and calcium supplements as well as folic acid, although as a 51yr old male I think it highly unlikely that I am pregnant.

During my incarceration I had several endoscopes including varices banding which will be monitored every 6 months or so going forward. Since my discharge I have been weak as a kitten, tired and nauseous and have put on just over 3kg in about a month although at least some of this is due to returning ascites build up. So far I have been lucky and have yet to suffer the range of your partners complaints, I hope this is not the calm before the storm!

I spite of the initial rather dismal prognosis of months rather than years of survival, it now seems that the doctors are researching just what can be done to safely suppress my immune system to give the liver a chance of respite and reduce the risk of hypertension, in the meantime they hope to be able to stabilise me at the current position as they continue their research.

Your partners survival of 5 years gives me great hope and I am doing what I can to stay positive, change my diet and listen to my body for the first time. Any other hints and tips would be gratefully received. Again, like your partner, I have never been much of a drinker so can't even claim to have enjoyed the build up to this bloody condition!!

Regards

[Dsmyle1 in reply to Dreadnought]

Dreadnought! I think you just made my heart skip a beat! My fiance was diagnosed with CVID after experiencing gastrointestinal issues and now nodular regenerative hyperplasia with portal hypertension. Your story is his, but only he resides in Texas. I am crying as I read your story, as he thought he was along. I am hoping you are still around and can reply to my comment or email me directly at [e-mail], as he would like to know he isn't alone.

These diseases and their symptoms are truly the most heart-wrenching thing I have ever watched a loved one go through. He is on a drug called Stelara and is being followed by the National Institutes of Health in Bethesda, Maryland.

I know it is of no consolation, but I no longer feel as if we are fighting a battle with this illness alone knowing there is another just like him out there!

[SummerDawn in reply to Dsmyle1]

I've recently created a couple of places for us (my husband) NRH'ers to be able to go for info/support:

The public page (for general info) is:

facebook.com/Nodular-Regene...

The PRIVATE group (more intimate/detailed discussion, support, etc), is:

facebook.com/groups/2038203...

We've felt VERY alone since 2001, when he was diagnosed, and only recently decided to try to reach out more to find others!

[SummerDawn]

Hi there! YES! My husband is 35 and was diagnosed in 2001 with it. He also never drank. He has had no major symptoms (other than portal hypertension and an enlarged spleen - he also had esophegeal bandings annually, as needed) until late-May, when he started having upper GI bleeding. We went through a similar experience as yours (ICU for 19 days - he shouldn't have lived, did rare surgery to install side-by-side spleno-renal shunt to relieve pressure, etc...), and are on the other side of it - he's doing well! Would love to connect. ~Scott & Summer ([e-mail])

[dchammond]

Hello,

I am 51 and was diagnosed with NRH three years ago. I didn't even know I was sick until I had a significant bleed that laid me out on the floor. My doc said if I had lost much more blood that first time it would have been over. I had 2 more minor bleeds in the coming months. It took a long time to finally pin this down and no one can say how or when this disease started.

I am a health economist by training and spent a lot of time researching NRH. I quickly discovered that not much is really known for certain. Whether it is misdiagnosed, undiagnosed or, as in my case, completely symptom free NRH is frustrating to say the least. The first year was absolutely the worst and I felt like I had a dagger hanging over me.

I am on all the standard meds and that didn't work. I was told never to lift anything over 10 lbs. which is impossible to abide by. I now get scoped for vein banding every 3 months with 3-6 bands per session. As of a couple of weeks ago I am being moved to a transplant list. Because of the degree of liver damage (est. 70-90%) the TIPS isn't likely going to work for me so we're apparently skipping that step.

A transplant is no picnic but if I can get an appropriate donor it will double the survival rate and could add some years. With or without the transplant the clock is ticking and I've decided to stop being worried all the time and return to a more normal life. I just had the best summer of my life with camping, fishing and kayaking. No regrets.

[alpacasweet]

I know this was posted long ago but I have just received this diagnosis and would love to connect with others. I am in the US though. My problems started with acute hepatitis after taking nitrofurantoin (antibiotic) for several years. My doctors expected the liver issue to resolve and thought I would be okay. I am much better but imaging showed what looked like cirrhosis which they didn't understand. They ordered another biopsy and from that have diagnosed NRH. No idea what my future will be. It's so scary.

--Robin

[Onstage2013 in reply to alpacasweet]

Hi alpacasweet,

I know how you feel. I was diagnosed over a year ago after ending up in the hospital with bleeding varices. The doctors were confused because I didn't have any symptoms or risk for cirrhosis. I haven't had a biopsy, because of low platelets, but my doctor thinks it's NRH. It is scary because little is known about prognosis.

[alpacasweet in reply to Onstage2013]

Hi onstage2013,

I am also frustrated. My doctor tells me that no one knows the prognosis for this condition. There is a study going on at our NIH about the natural history of NRH but there are no interventions because there are no cures or treatments of the condition at this point. I'm going to investigate the study but it seems to require more invasive tests and I don't think I really want to risk that.

Were they able to treat your varices okay? I hope you are feeling well now. Have you found anyone else with NRH?

Best,

--Robin

[TiaWheeler in reply to alpacasweet]

Robin,

I too am in the US, although I lived in the UK. At age 30, I was diagnosed with NRH over 10 years ago at the Mayo Clinic, following hospitalization for a bleed and acute liver failure. I have done fairly well managing with betablockers, diuretics, and proper diet, but it continues to progress - my lymphedema is advanced and it is time for a liver transplant. 

If you or anyone else would like to connect by email for support or sharing information, I can be reached at [e-mail] 

Tia

[alpacasweet in reply to TiaWheeler]

Hi Tia,

How are you doing?

I sent an email to your gmail account.  I hope you saw it.

--Robin

[Onstage2013]

Hi Robin,

Yes, they have been able to treat the varices with a beta blocker and banding. I had been going every 3 months for banding, then went to 6 months, and the last time my doctor said 8 months. She thought I could go a year between procedures, but to be cautious, she wanted me to do another in 8 months. I haven't needed any bands placed the last few procedures, which is good. I feel good, and wouldn't know there was something wrong with me.

The diagnosis was traumatic. As I said, I ended up in the hospital bleeding. My liver enzymes and electrolytes were and are normal. My bilirubin is sometimes just slightly high but usually in the higher end of normal ranges. Are your blood results all normal? I don't know anyone who has it. I try to find information and studies online, but there isn't much.

Cheryl

[alpacasweet in reply to Onstage2013]

Hi Cheryl,

I'm glad to hear you are doing well.

My case started differently. I started feeling ill with all kinds of GI symptoms and had no idea why. It wasn't until I was critically ill that I finally found a doctor who figured out that I had acute hepatitis and the cause was an antibiotic, nitrofurantoin, that I had been taking prophylactically for 2 years. So I got off that drug and my enzyme,s which had been very elevated, went down. It took over a year for them to get back to normal and the liver images they did after 6 months were showing what looked like cirrhosis. My images at the time of my diagnosis looked normal. So, the doctor was puzzled and I finally decided to have a second biopsy a few weeks ago. The results show NRH. The doctor tells me that he doesn't know why it happened and he doesn't know what my prognosis will be. I do have a somewhat low platelet count. I've had one endoscopy which showed no issues and I have to have them at least yearly now. So I went from being a very active healthy 56 year old to someone with what could be a serious chronic illness. Very traumatic for me as well.

In case it interests you this is the link to our NIH trial. I'm sure you are too far away to participate but it's something to keep an eye on anyway. clinicaltrials.gov/ct2/show...

I would be happy to stay in touch if you like. Is there a way to send a private message on here?

--Robin

[Onstage2013 in reply to alpacasweet]

I was very sick initially but feel pretty good now. Sometimes, a few mild stomach issues. I hope you're feeling better too. I don't know how to private message on here. I just joined to respond to this thread.

[51chevy]

for the cramps i used hot towels on the muscles involved,i tried everything else,tonic water did that nothing,try this see if works,any other questions,i have been through it all..

[1969bean]

My mom was just diagnosed with NRH yesterday. She's 77 and was diagnosed with colon cancer in Feb 2016. I think her NRH was caused by Folfox, one of the chemo drugs. Though I'm sorry to hear about everyone's condition, it was somewhat reassuring to find this group on google (since everything else about NRH is a medical research paper). She had to stop chemo in July after 7 of 12 chemo treatments due to multiple complications (high sugar, anemia, blood transfusion, bad port causing blood clots, etc.). She was hospitalized in August for colitis and while there had heart failure, low ejection fraction, defibrillation, lung collapse, extreme edema throughout her body (she felt like a memory foam mattress) and had to learn to walk again due to lost muscle mass. I finally moved her from Reno to California and set her up with doctors here. Things were looking better and her heart recovered and her kidney function went up from 19% to 29% (she has had polycystic kidney disease since her 30s). She was noticeably thin (CLUE) when she moved her in Nov 2016 and mentioned extreme itchiness (CLUE)and bloating. Dec 26, 2016, she went into the ER to get 5 gallons of fluid drained. PET scan showed 2 cysts, one which was "lit" and thought to be cancer. GI doctor through fluid was due to liver issues. She had a biopsy of the cysts and liver scheduled in early Jan 2017 and ended up hospitalized due to mysterious bacteria in her blood (klepsiella) and cyst (streptoccocus). Drains were installed in her abdomen and 2 cysts. 2 weeks hospitalization plus 4 weeks in a nursing home for IV antiobiotics. Once the drains were removed, her alkaline phosphatate numbers were extremely high (CLUE). She's been dealing with a balancing act of fluid retention and kidney function since being home after drain removal but her energy is good. I hope her journey will help provide some encouragement (?) to others to know that there may be some parallel experiences. It helped me to see that others dealt with similar issues and meds. Once I saw everyone else posts, I thought "yeah, same here!". Lasix and spronolactone together are affecting kidney function, so we tried Lasix alone with the same results. Now we're trying spronolactone by itself with a low potassium (and low sodium all along) diet. She has been taking Atarax for itching as well. I hope everyone is managing well. My heart goes out to all of you who are living with NRH as well as walking along side those who are are living with it.

[krufinwe]

I was diagnosed with NRH. I think a medication mercaptopurine caused it which was prescribed to improve my digestive health. Now I suffer from hepatic encephalopathy due to a stent in my liver. Had esophageal varices and now take lactulose, furosemide, spirolactone etc. A complete mess.

[BillWeston]

I have just been diagnosed with nrh. After a liver transplant to cure BCS, caused by PV. acronym lottery on the NHS!

[SummerDawn]

I've recently created a couple of places for us (my husband) NRH'ers to be able to go for info/support:

The public page (for general info) is:

facebook.com/Nodular-Regene...

The PRIVATE group (more intimate/detailed discussion, support, etc), is:

facebook.com/groups/2038203...

[alpacasweet in reply to SummerDawn]

Hi SummerDawn,

Why did you create another private FB group since there already was one? I saw you posted in the one that was already there.

Just curious.

[SummerDawn in reply to alpacasweet]

I did it just before I saw the (closed/private) other - so glad someone had taken the initiative to set one up, looks like about two years ago years ago!