Goodbye to all that: No more sticks. No more scans.

I never intended for this to become an illness blog, but once I became symptomatic for the Disease Formerly Known as Primary Biliary Cirrhosis, I could find no narratives of what to expect, and so I decided to chronicle my mess.

Last month I told my hepatologist we were done here. No more scans. No more sticks. There is no point in watching the numbers because there is only one way to possibly delay dying: a transplant.

Don’t fret; there is no reason to believe I won’t be around a while. But I have spent too long seeing my life as a slo-mo train wreck.

I felt tremendously free after announcing the end to tests. He said now my MELD was 15, it was time to start thinking about getting on a transplant list. I told him no, it was not.

I’d known this day was coming for 11 years. I had been ambivalent about it, but these are the reasons I told him no. (He said I can always change my mind). I don’t advise anyone else to take this route. If my children were younger, I would not.

  1. There’s a fair chance that after a year or so, the autoimmune illness PBC could come back with a vengeance.
  2. I don’t want to spend the rest of my life on immunosuppressants.
  3. Some programs require you to re-house your fur people. Our household includes 3 cats, 2 dogs, and 3 ferrets. Those cats and dogs aren’t going anywhere. (The ferrets stay on the top floor with my daughter.)
  4. Even if all went perfectly (not the way they usually do for me), a transplant would start at $150,000. They can easily end up costing $800,000+ range. Even if my insurance coverage doesn’t change in October, it would only pay for 80% of the cost. So we are looking at a minimum of $30,000. While we could swing this, we could not do the upper range without accruing enormous debt. My husband would be willing to do whatever it took, but I am not willing to see his life or our daughter’s (who is still in school) complicated by such a huge gamble.
  5. I’m not entirely sure that on a spiritual plane donors are dead.

I think my hepatologist might have been relieved because it saved him telling me I may not be a candidate since in between this and my previous visit I had been diagnosed with a severely enlarged left atrium. Hearing this was a bit of a relief since it means that I could drop dead and miss all the liver failure gore.

So how am I? I’m tired, all the time. This could have much to do with the heart and little with the liver, or both working against me, not to mention a whacked auto-immune system.

The worse thing is likely unrelated, although it remains unexplained in spite of every test in the box. Too often I have “steakhouse syndrome,” even with macaroni. So even eating is not pleasurable any longer.

But I can still read, so I am fine. I don’t get out much but I have an active cyber-life.

I’m sick of being sick. If anything interesting happens, I’ll post. Otherwise, I’d rather write about something–anything–else or not at all.

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This Thing Called End-Stage Liver Disease

This is another post about primary biliary cholangitis (cirrhosis) [PBC]. In my last post, I used the term End-Stage Liver Disease [ESLD]. It’s a very vague and barely useful designation. 

The good news is that my portal hypertension — once the subject of so many gruesome posts — has been effectively controlled by the Transjugular Intrahepatic Portosystemic Shunt [TIPS] I had installed in July 2015. I haven’t had a bleed since, and an exploratory endoscopy in the fall showed I now have a nice, smooth, pinkish esophagus instead of one with red streaks of varicose veins.

But I haven’t felt better. When I look back on what I could do in 2014 or 2013 and compare it with how I’ve felt the past nine months, I know I am at best holding steady.

So at my last hepatologist appointment, I asked the doctor straight out: Do I have ESLD? He looked taken aback, like this was an acronym that doctors use among themselves not with patients. But he said, yes. And so I said, how much longer do I have? Months? Years? And he said he expected years.

When the bleeds happened, they were potentially life-threatening emergencies. Still, I’d get patched up and leave the hospital the next day. Once they started coming much more often, anemia became a big issue until I got Injectafor iron infusions. I avoided remote locations and long airflights, but as nasty as they were, I now look back on the Bleed years (8/10 to 7/15) as the good old days.

I also haven’t had another bout of hepatic  encephalopathy [HE], one of the scariest ordeals of this whole PBC ghastliness. 

But there is a very low grade (comparatively) of HE, and especially when I am even more fatigued than usual, I can tell that I’m having trouble with short-term memory and learning new things. For example, my husband and I went to Washington DC last month, and I simply could not grasp the subway system. It’s hard to remember what day of the week it is; then again, there is little to distinguish them.

So what is ESLD? It’s odd. You will find a lot of sites with information on End-Stage Liver Disease, but there’s little mention of when Beginning becomes Middle goes to End. I’m not sure there is a Beginning or Middle variety. End-Stage Liver Disease [ELD]  itself is most commonly mentioned in discussing MELD scores; the M refers to Model, and a MELD score is a complicated and flawed scoring system for transplant urgency. The higher the score — and 40 or so seems to be the cap, the worse off you are. I’m at 12.

More often, I’ve found articles using a different terminology. The NY Times offers this simple comparison.

  • Compensated cirrhosis means that the body still functions fairly well despite scarring of the liver. Many people with compensated cirrhosis experience few or no symptoms.

  • Decompensated cirrhosis means that the severe scarring of the liver has damaged and disrupted essential body functions. Patients with decompensated cirrhosis develop many serious and life-threatening symptoms and complications.

But then there’s another approach using four levels.  

Here’s one that does it in three: inflammation, scarring, failure. I wonder if, since the liver is the only major organ that up to a point can regenerate new cells, it would be possible to get out of inflammation back to normal.

But cirrhotic cells are scarred and ruined; they aren’t going to come back to life. And my PBC continues to destroy the bile ducts, and this blockage continues to damage liver cells.