How Am I? An Update on Dragging Along with Primary Biliary Cholangitis (Cirrhosis)

Roughly a year ago, I said, I’m done. No more pricks or scans. Longtime readers will know that my life from 2010 to 2015 had become a gruesome slough of gastric/esophageal varices bleeds, an uncommon complication of early and mid-stage cirrhosis (not so uncommon in end stage). My labs were never that abnormal during this period, so I was diagnosed with every weird ulcer subspecies in the field guide. Not until I had a shunt [TIPS] put in my liver in July 2015 could the portal hypertension be measured, and it was high.

I announced I was going to quit writing about this, but I get occasional questions, and I may as well keep the history going.

I have had no more bleeds, and that is a very good thing. I think the encephalopathy which followed had some lingering effects. My spatial sense isn’t what it used to be; I can go to the drugstore, which requires exactly one turn, and end up going in a circle. Subtraction of 3-digit numbers is hard. Short term memory isn’t too keen. I haven’t had any recurrences, and I think this is attributable in large part to eating absolutely no red meat or pork. But the fatigue, which is the most common complaint of people with PBC, seems worse.

It is hard to explain because it is not everyday tiredness. It doesn’t matter if I sleep 12 hours, after being up an hour or so I am going to be tired. I can press through, and a really active day is one where I go out to breakfast, walk the dog, and go to the store. I’ll pay for it the next day when I will be too tired even to read. Maybe if I did more, I would feel like doing more, you might think. But 2 days on is going to mean 2 days off, and 3 days on will be 3 off, and at some point I will quit enjoying being on, so if I don’t have to, why not stop?

Remember The Roadrunner cartoons when the coyote is squashed by a boulder, peels himself off the pavement, and then an Acme truck  comes barreling down and pancakes him again? It is like that. Still the coyote keeps chasing that roadrunner.

I vacillate between gratitude for every day — for security and safety and a loving husband who picks up my massive slack —, and weariness. Facebook can get to seem like those wretched newsletters people would send out with their Christmas cards, where all reports are of visits to exotic places by people who are legends in their own minds. Good on them. But Christmas newsletters came just once a year.

Since I don’t go out much, the feeling of being an alien increases. I look at these reports from a distant place. Stay out of a big box store for a year. Then visit. Lord, how much stuff there is. Who could possibly need this much stuff? I’ve bought one pair of shoes and one dress in the past three years.

Goodness me, how much time and money people spend on food. So much of social life seems predicated on food and alcohol, which I simply treat as something in the category of never again.  The most annoying problem I have may or may not relate to the PBC, but finally I have an appointment with an esophageal expert. It’s been going on for years. I’ve had the standard three tests for esophageal motility, but results were negative, no problem. I don’t care what the results of these swallowing tests conducted only with liquids are. It is not normal way too often to be able neither to swallow food all the way or get rid of it the opposite way. My hepatologist agreed (hurrah!).  I eat a lot of refrieds with rice. Smooshy and a fair protein source.

So yes, I did go back to have another set of sticks and an ultrasound done. I decided it would be stupid not to check on the TIPS. The TIPS is functioning; “patent” is the word of choice. The real time ultrasound had been going on for 40 minutes when the sonographer left to fetch the radiologist himself. There were veins she just could not find. Apparently, I have a strange body architecture.

At the appointment the next day, the hepatologist asked that I return for a CT scan. We had planned a trip to the beach, and went ahead, and I sat on the dunes and thought, isn’t this just dandy.

Then Monday I had the CT scan, which looked as expected, except for “small” varices in the stomach. Dear god. “Varices.” I thought I would never have to see that ugly word again.

Most people with PBC don’t haven’t problems with bleeds. A tiny number who have the shunt installed do develop gastric varices. Most of this little pool do not have varix bleeds. I don’t want to be a footnote to a footnote to a footnote. Neither will I be terribly surprised if I am one.

PBC: No Varices for Me. Well, Just a Trace.

As I noted in my first post on primary biliary cirrhosis, during my annual routine appointment I was advised to have an endoscopy procedure to determine whether I had varices in my esophagus as a result of portal hypertension as a result of cirrhosis as a result of the autoimmune destruction of my bile ducts, that is, primary biliary cirrhosis.

I was surprised by this recommendation, since my blood enzyme levels had normalized and in 2007 an MRI of my liver did not reveal damage, but things can change, and the extent of damage my liver has suffered is unknown. There are four stages of PBC, with cirrhosis being stage four, but my case had not been staged, as they put it, because I have never had a liver biopsy.

When I first visited the hepatologist, we discussed whether a biopsy was warranted. He had enough other information to feel confident about the diagnosis, and the treatment he would start me on would be the same regardless of stage. So we concluded that while the biopsy would provide more information, it wouldn’t necessarily be useful enough information to warrant the hassle.

Medicine.net’s comprehensive article on PBC notes that whether a biopsy is optional

usually depends on the level of confidence in establishing the diagnosis of PBC using the liver tests, autoantibodies, and ultrasound. In the presence of cholestatic liver tests, high levels of AMA, and an ultrasound showing no bile duct obstruction in a middle-aged woman, the diagnosis of PBC can be made rather confidently without a biopsy.

Even had I had a biopsy three years ago when I was diagnosed, there still would have been only one way to know whether or not it was time to start worrying about varices. And that was to look.

Before we began, the doctor told me that there were four levels of varices. Levels 3 and 4 require treatment, starting with medication.

I asked whether even if I did not now have varices, was it not the case that sooner or later I would?

He quickly responded yes, then caught himself and backtracked, with the typical doctor’s abhorrence of an unqualified answer, noting that 60% to 80% of people with cirrhosis have varices, and in 1 of 6 cases, within a year’s time, if left untreated, the varices would burst and bleed into the esophagus.

The worst part of the endoscopy — really the only bad bit — had nothing to do with the endoscopy itself. I knew I was in for it when the tech responsible for starting the IV began complaining that I had no veins, and sure enough, the IV entry was and remained painful and I have the bruise to prove it.

A not-as-nasty as I was warned to expect spray was squirted in the back of my throat, Demerol was added to the IV port (there was no other use of it in my case), and that was that, until I woke up.

I had no sore throat and began eating and drinking immediately, although I’d nearly fall asleep between bites of cracker.

And the verdict was just a trace of varices, no need to do anything, just repeat in a year or two.