I wrote here and here about the issues I faced preceding my diagnosis of Primary Biliary Cirrhosis. The most complicating issue during the diagnostic phase was the fact that I actually have two separate liver diseases. Neither my treating physicians, nor the hospital pathologists, had ever treated a patient with both of these diseases, which made my initial prognosis more grim than it is now. As they looked at the samples retrieved in the biopsy, they assumed they were looking at a more advanced stage of Primary Biliary Cholangitis. (They staged me at a 2+ with stage 3 inflammation/hepatitis.)
In April of 2016 I was accepted into the hepatology program at NIH, because I met the rare criteria of having both CVID and Primary Biliary Cirrhosis. (And also because an amazing friend fiercely advocated for me to get in!) NIH treats the largest cohort of patients with both conditions (the largest cohort – IN THE WORLD!) and it was their team of pathologists and hepatologists who realised that I have Primary Biliary Cirrhosis (PBC) AND Nodular Regenerative Hyperplasia (NRH). The silver lining thing was the fact that they “backed up” my staging of PBC to a stage 1. And they feel the NRH is in an early stage as well.
And each disease does it’s own thing. PBC causes scarring of the liver. Nodular Regenerative Hyperplasia causes Portal Hypertension. Neither is fun. According to the American Medical Library, “Nodular regenerative hyperplasia (NRH) is a form of noncirrhotic portal hypertension that can be caused by chronic use of medications.
(Hmmm….that sounds like me.) 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”
My hepatologist believes that my NRH is caused by the chronic, severe infections which have necessitated chronic use of powerful antibiotics. Remember what I wrote about CVID and the persistent infections I’ve had? I was the perfect “host” for this complication – as are many CVID patients.
So – what’s next?
It’s hard to say. There is no medication/treatment for NRH, other than management of symptoms – and finding the root cause of the infections – and for me, that means finding a cure for the chronic uti/kidney infections.(There she goes, talking about tinkle again…) I will go into the uti/kidney treatment process in another post. But suffice it to say we are very hopeful that we’ve isolated the problem and have begun turning it around. Currently, my only symptoms of NRH include a decreased platelet count, enlarged spleen, and the evidence on the liver biopsy. I am- so far – free of esophageal varices (bleeding veins in your esophagus) and ascites (the collection of fluid in your abdomen). Because we are in the early stages of this diagnosis, I will update this as I learn more. I am one of a handful of people with both PBC and NRH….and so very lucky to have found the team at NIH. (I think I need to do a whole pages on my many abbreviations. CVID, PBC, NRH, IGG, NIH….)
And again….I reiterate the importance of self-advocating and pursuing a diagnosis. When I received the recalibrated staging, I felt as if I’d stepped off of death row. People can die “with” PBC rather than “because of it” as long as they continue to respond to the ursodiol, which prevents further scarring of the liver (and so far, I am responding really well.) The NRH and CVID complicate my case, but all things considered, I’m doing well, under my new criteria for what counts as a “good day.” Under my “Plan B.”
Below are some resources for Nodular Regenerative Hyperplasia. There are virtually no published studies completed on patients with both NRH and PBC (other than the one I’m in). And there are no (ZERO. ZILCH) blogs written by someone living with NRH. Until now. It’s really important to know that if you have CVID, you are susceptible to both of “my” liver diseases. You will hopefully walk the line between being informed
and having your computer taken away from you because you’ve googled too much and being neurotic. Today’s troubles are enough for today, carpe diem, and all that!