Tag: PBC

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.

 

 

Always Bleeding from the Same Scar: Bleeds 9 and 10

There’s a new series of posts I am planning, but first I need to catch up on my bloody misfortunes. It’s been a year since my last post on a bleed, caused by portal hypertension, in turn itself caused by damage to the liver, in turn caused by my autoimmune illness, primary biliary cirrhosis. But I have had two since then, and am now up to 17 transfusions.

First, let me say something about these transfusions. I wouldn’t be here tonight if it weren’t for people who give blood. Sometimes when I’m wandering around, I ask myself: was it her? Or him? Whose blood is in my body? I cannot know, but I thank anyone who has ever given — or even just tried to give — blood. These are rare and strong and generous people.

Back to these bleeds. Number 9 hit in September, when I was in Miami, trying to help my 84-year-old mother, and was a 2-transfusion bleed, as was bleed 10, that waited until February, less than a month after I moved my mother out of Miami. This one occurred a year to the day after my beloved collie died of hemangiosarcoma, a canine cancer that causes sarcoma to develop. Mine was on my dog’s spleen. It ruptured, and he bled out.  

The doctor who performed the endoscopy here couldn’t find the source of the bleed.

But a month later when I went to see my hepatologist at University of Alabama’s Kirklin Clinic, he found an actively bleeding varix at the fundus, where the stomach and esophagus meet. The site of multiple bleeds of mine has been at that little crook to the left of where the arrow is pointing.

File:Illu stomach2.jpg

In fact, this is the same site as my first — and still worst — bleed, the one that that the GI who saw me in the ER thought to be a bleeding ulcer and that for a while my hepatologist thought might be a Dieulafoy’s Lesion or a Cameron’s Erosion.

But after reviewing my records, now the theory is that the same area keeps bleeding because it was weakened by the three clips (like tiny clothes pins) put in as an emergency approach to shutting down the 4-transfusion bleed I wrote about back in August 2010.

Banding, essentially using the equivalents of rubber bands, to cut off the supply of blood to a bleeding varix or one that looks like it could become a bleeder, is the preferred approach.

The clips are a last resort.

The reason I keep having these bleeds may then be because the delicate walls of the esophagus have been compromised by the clips.

One thing others with primary biliary cirrhosis should know is that having these bleeds is extraordinarily unusual. I asked my hepatologist if other PBC’ers have similar problems with repeated bleeds, and he said, no, he has never seen or read of a comparable case.

I think then that it is best we end with the inspiration for the title of this post: David Bowie’s “Always Crashing in the Same Car.”

 

 

Cameron’s Erosion Erupts (Again): Bleed the Eighth

Update: The current (November 2014) hypothesis among my doctors is that whatever they were called in my past posts — Dieulafoy lesions, Cameron’s erosions, or bleeding ulcers — all these bleeds have their source in the portal hypertension which comes from cirrhosis which is caused by my auto immune system attacking my bile ducts, that is, my primary biliary cirrhosis.

Back when I first decided to write about having primary biliary cirrhosis in November 2009, I never figured that this would become my bloody blog. I have neglected the blog for months because if I am going to follow through with my plan to write an account of living with this auto-immune illness, then I have to, once again, post about another bleed. Each has its own special moments, however, and here I have two warnings for you, and a comment from my gastroenterologist.

Once again, as in bleed 7, the culprit was my Cameron’s Erosion (or Lesions), an ulcer “in the hiatal sac of patients with hiatal hernia,” which is how Wiipedia’s 2-line article describes the thing. You know you have a rare condition when Wikipedia has next to nothing. I described what I learned about Cameron’s after bleed 7.

The link to PBC is that because my liver is compromised by the disease, it is too dangerous to repair the hiatal hernia.

I’m getting better at identifying the onset of these bleeds, anyway. This time I even drove myself to the hospital. Signs were clear: black BM and the taste of iron in my mouth.

Now for the three things that made this bleed memorable, and some advice.

1. Do not let a doctor put in an IV. There is some incompatibility between all the things that I might need intravenously during a bleed, and so I end up with IVs in both arms. I had a great nurse in the ER who inserted the first IV as painlessly as I can remember. Then this doctor or resident drifted in. I could tell he hadn’t been on the job long — and thought he was God’s gift to the world — because he was wearing a V-neck scrub top that let me see way too much of his curly chest hair way too close up. He wanted a little practice with IVs, I guess. So he tried to start the second line. And failed, miserably. Blood spurting and me doing the vasovagal response — that is, fainting. Finally the nurse guided the doctor’s every move and the second IV was inserted.

She was a great nurse, and I asked her later, how do you stand that — doctors coming in and thinking they can do all you can, and you having to deal with the aftermath. Diplomatically, professionally, she responded that at least that doctor will think twice before he gets snippy when a nurse has trouble with an IV.

2. It hurts like hell to have your stomach pumped. And it’s worse when there’s no reason to do this. My bleeds sometimes have two parts: black stool and vomiting. The vomiting always happens, but when both occur, usually comes an hour or two later. This time, I got to the ER before I vomited. All that I needed was time, but for reasons best known to himself (and that itself is a problem), my ER doctor decided that instead of letting things take their course, he would pump the blood from my stomach.

Never again. I would have been better off sitting outside the doors of the ER until I threw up.

I always imagined stomach pumping would involve a tube down the throat, turning on the pump, and whoosh, all done.

It isn’t like that.

This is what it is like to have your stomach pumped: A tube is inserted up your nose and down your throat. If the first nostril tried gives the nurses trouble, then they start over with the second. They keep giving you water to drink so you swallow, and swallow down the tube. Maybe it was just me, maybe the tube was just lodged against a nerve, but it hurt like hell the entire time the tube was up my nose and down my throat. 

And the entire time isn’t a matter of minutes. It’s a matter of hours. The pumping is slow and not constant. You watch the content of your stomach (in my case, red blood followed by black) slowly proceed down this thin tube. Sometimes it starts flowing backwards. 

I supposed most who OD and have their stomachs pumped are out of it. I can’t imagine that anyone who has had it done would risk OD’ing twice. I was not out of it. Other than a local anesthetic sprayed in my throat, I had no recourse but to lie there in pain between 1 and 5:30 in the morning and watch my blow flow out of my stomach.

I talked to my floor nurses about this, and each said, you always have the right to refuse a procedure. And refuse I shall. 

3. Don’t go out during lightening storms. This was her advice when I asked my gastroenterologist how often people have both PBC varcies and Cameron’s lesions. First she suggested buying lottery tickets, and then backtracked, since having bad luck doesn’t mean having good luck too. (I spent an hour at the Harrahs in Cherokee last week and never once was ahead.)

Actually, her advice misses the mark. Not going out during lightening storms is an action I can take to avoid without fail being one of the rare people struck down.

But there is no way I can avoid without fail the next bleed.

My PBC: Collateral Damage

I’ve described the gory consequence of my primary biliary cirrhosis: gastrointestinal bleeds. There’s also collateral damage, for want of a better term, that isn’t so dramatic and isn’t life-threatening, but like the disease itself, slowly does lasting harm.

Primary biliary cirrhosis [PBC] is an autoimmune illness in which the body’s own immune system destroys the bile ducts of the liver, leading to cirrhosis and liver failure. Most research suggests the disease’s progress can be slowed by years, even decades, in patients diagnosed early who respond to medications that thin the bile.

I started out as one of these lucky ones. I was diagnosed in December 2006, responded immediately to ursidol, and my liver chemistry values largely returned to normal.

I ignored my disease 364 days a year. On the 365th, I went to the Liver Center at University of Alabama-Birmingham. I didn’t even tell most people about it. It seemed abstract, really: just a matter of numbers.

Some of the collateral damage is to other parts of my body. My hepatologist tells me that his patients frequently complain that their teeth are crumbling. My salivary glands don’t work properly.  My mouth is like Afghanistan: dry, bloody, and hopeless. You know that question mark shaped thing that dentists hook over your teeth to suction fluid while they work? They don’t need to use one with me.

But then the bleeds started, with the first and worst in early August 2010, followed by another a year later, a third in early March 2012, and, since October 5, 2012, four more. Even after the first and second bleeds I could convince myself that with more frequent endoscopies, these could be prevented.

And then came this fall.

I have been rather spectacularly unlucky.

I should make it clear especially to others with PBC that my experience is not typical. The bleeds are like a side effect of PBC. Some people have them, and many don’t. My hepatologist says it is very unusual for someone to have as many bleeds as I have had so close together.

When you have four hospitalizations in 16 weeks, it’s hard to ignore that things are not going too well — and you can’t keep the problem to yourself. People start worrying.

And the soul-lacerating collateral damage mounts.

Because of this illness, I am not as good of a mother, wife, daughter, friend, sister, aunt, neighbor, or even pet owner as I like to think I otherwise would be (this was going to be the year I kept up with the collie’s coat).

I don’t have the energy that others do. After bleed 7, I had three transfusions, but still I am anemic.

I need to visit my 83-year-old mom, but I’m scared to fly. The thing about these bleeds is that there are only two warnings I have experienced: fatigue and loss of appetite in the 12 hours or so before one starts. But lots of things can cause that. The bleeds are stoppable if IV fluids and drugs can be administered in a timely fashion, but what would happen if I started throwing up blood on a plane? Moreover, how much use would I be to my mom if I began a bleed while visiting? I drove a half day away to see my son for his 23rd birthday. He spent it visiting me in the hospital.

My children were 14 and 20 the first time. None of us knew what was happening. They saw it all, as did my husband: the blood, the shock, the ambulance sitting in the street for 15 minutes before even starting for the hospital.

I had four transfusions that time and rebounded quickly. We thought it was a one-off. For a year it seemed it would be.

But not now.

The thing is, the bleeds aren’t painful. Gross but not painful. The worst part is the IV sticks. 

What is painful, though, is knowing that what is happening to me is taking a toll on those I love — and there isn’t a thing I can do about it.

PBC Bleed 7. The Vesuvius Within Me. Crashing in the Same Car.

Good thing I finally got around this month to describing November’s bleeds 5 and 6 in this continuing realtime account of me and my PBC (primary biliary cirrhosis) because now it’s already time to move along to bleed 7.

This was a rather dramatic one, occurring in the wee hours of January 18, 2013. My hemoglobin [hgb] dropped to a personal worst of 6.1, I landed in the ICU and then on the cardiology floor, and required 3 transfusions. A couple of days ago my hgb was 10.5, mildly anemic, but I feel great.

Why the ICU and cardio unit? Because when your hemoglobin drops that low (normal for post-menopausal women = 11.7 to  13.8), it means none of your organs — including your heart — are getting enough oxygen.

What went right this time was that the Asheville ER got a gastroenterologist in to perform an endoscopy immediately, while I was still actively bleeding. Except for my first bleed, in Huntsville the doctors waited 20 hours or more to ‘scope, after drugs and IV fluids had stopped the bleeding.

You can’t be sure of the source of the bleed if you don’t see the bleed.

The Asheville GI theorized what I had this time around was a Cameron’s Erosion. This is erosion in the stomach near the diaphragmatic haitus which is a hole where the esophagus passes into the stomach. The junction should be below the diaphragm, but if you have a hiatal hernia, as I do, then it is above the diaphragm. Usually, hiatal hernias cause no bother other than indigestion. But I have other things going on as a result of the PBC, namely protal hypertension and gastric and esophageal varices. (If you search “cameron’s erosions” + “portal hypertension” + pbc, Google comes up with 75 results, which in the Googleverse is close to zero.)

Now as it happened, I had an endoscopy and visit planned with my hepatologist, Dr. Brendan McGuire, at University of Alabama-Birmingham’s Liver Center for January 22 and 23. So immediately after leaving the hospital on the 21st, we headed south.

Dr. McGuire scoped me Tuesday morning and reported he agreed with the Asheville doctors. He described the area as not unlike a scrape on a kid’s knee that scabs over, but before it gets a chance to heal completely, keeps getting banged up. He didn’t see the site of my first bleed until a few weeks had passed but thinks this one was in its vicinity if not the same place.

So it could be like I keep crashing in the same car, having the same bleed over and again. Since I wasn’t scoped during bleeds 2 through 6, we’ll never know.

Why not fix the hiatal hernia? Too risky: its position, the sites of the erosion and varices, the amount of scar tissue, the thinness of the veins — lots of reasons.

What can be done: double the dose of beta blockers I’m taking to slow heartbeat and of antacids to reduce stomach erosion. And hope that the Vesuvius within me remains dormant.

And what about my primary biliary cirrhosis? I’m doing just fine there, holding fairly stable. It could be years and years before it is bad enough to warrant a transplant. There is something called a MELD score. Normal people’s is zero. Those near dead of liver failure have a score of 40. I’m at 8. Bleeds don’t factor in.

So all I have to do is hope I don’t erupt.

But what we need now is a little relief from the dreariness of reading about me going on about vomiting blood.

I suggest a segue to youtube to view some loveliness: David Bowie singing “Always Crashing in the Same Car.”

You can choose between this one with a particularly happy Bowie, or the GQ Awards show where Bowie wore sandals with socks, or this with a sassy Bowie around 3:18. Or all and more (like here, where he isn’t playing the guitar and seems not to know what to do with his arms).

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PS: Although it doesn’t have much about PBC, this site has a cool diagram of possible diagnoses related to liver trouble.

PBC, Bleeding Varices. Bleeds 5 and 5.5. Or 6.

Yes, it has been months since I last posted, and, yes, my next-to-last post was on this same topic (Primary Biliary Cirrhosis [PBC], Portal Hypertension, My Perfect Endoscopy Results and My Fourth Bleed) in my continuing series of what my experience with PBC is like. I’m beginning to understand why there aren’t so many real-time chronicles of progressively worsening illnesses.

You may recall that back in early October 2012, I had a routine exploratory endoscopy with perfect results, followed a few days later by a bleed I was told was from a tear in the esophagus which was patched up, and no other problems noted.

Bleed 5.

November 6 I drove from Asheville, NC back to Huntsville, AL to spend the 8th with my son on his 23rd birthday. Before dawn on the 8th I started throwing up and defecating black blood. I really, really did not want to go to the hospital. I didn’t want to ruin my son’s birthday. I had so looked forward to this. But even less did I want him to discover me bled out, so I told him, and he insisted I go to the hospital. We ended up visiting in a room of the same hospital where we were 23 years previously, to the day.

Next day, the 9th, another endoscopy. And behold, the GI discovers two varices needing banding. He also reports that he saw no evidence of the tear I was told was the cause of my October 5 bleed, with repeated emphasis on the word tear. Huntsville GI added he wasn’t impressed with the Asheville GI’s work and that I had a good deal of scar tissue. Because my hemoglobin creeps up to 9 (12 is normal lower limit), I’m not transfused and am released that day.

Bleed 5.5 or 6.

Between November 8 and November 20, I felt worse and worse, like I was practicing being dead. I didn’t read or write or sleep, just stared out the window.

I was with my daughter at her doctor’s on the 20th when I started throwing up blood. Again. This time was a bit different; t some red mixed in there with the black, not quite as much as in past bleeds, but enough so I am on the verge of losing consciousness.

It was two days before Thanksgiving, and I had really, really been looking forward to spending it with my family and new friends.

The GI on call from the practice I visited to schedule October’s exploratory endoscopy paid me a visit in the ER. This made the sixth I’d seen from that practice. I never will believe that I had a spontaneous tear after the exploratory and not a nick, but GI #6 is simply preposterous. He claims that with PBC patients, low hemoglobin, as low as 8, is preferable to the normal 12-14 because less blood means less likelihood of portal hypertension. How stupid does this man think I am? He’s the doctor and I am not so I refrained from telling him his job or the role of oxygenated blood in maintaining life. But man, why don’t we just take a few pints of your blood, drop you to 8, and then let’s discuss quality of life.

Fortunately, a new GI did the next day’s endoscopy. She reported that I didn’t have varices that bled. Instead, the bleeding was caused by ulcerations around the two bands placed by the Huntsville GI (who was, you’ll recall, unimpressed by the Asheville group’s work).

So was this Bleed 5.5 or Bleed 6? It wasn’t a varices bleed. But then again, neither was Bleed 4 (the nick bleed).

There was a good thing about Bleed 5.5 or 6. It got me some blood. My hemoglobin had dipped to 6.7, an all-time personal worst for me.

I thought after the doctor visited Thanksgiving morning that I would be able to go home when the second transfusion ended. But a nurse said it would take 12 hours to wean me off the IV which was delivering a drug to help stop internal bleeding.

Despair. In comes Thanksgiving Day hospital turkey.

However, when the hospitalist came around, she said since this was not a varices bleed, I didn’t need to be on that drug, and so didn’t need to be weaned from it. I could leave.

Joy.

No one can give me an answer beyond bad luck for how in hell two varices burst on November 8 when on October 2 and 5, there were no signs of developing varices.

I’m going to Birmingham to see my hepatologist at UAB in 10 days. It is worth it to me to drive 6 hours for an exploratory endoscopy with someone I trust.

Image

Thanksgiving 2012. My left arm. The other looked about the same.

Primary Biliary Cirrhosis, Portal Hypertension, My Perfect Endoscopy Results and My Fourth Bleed

This post updates my last one, Primary Biliary Cirrhosis, Portal Hypertension, and the Frustration of Knowing There’s No Way of Knowing What I Need to Know, in which I discussed what a relief it was going to be to have an exploratory endoscopy in which either I would discover that I had no varices on the verge of bursting and causing a life-threatening bleed, or if I did have varices, they would be banded and so I would not be at risk — for the time being — of a bleed.

Well, here’s what happened. (Fair warning: This blog isn’t for the weak-stomached today.)

Tuesday morning: endoscopy. Great news! No varices! The GI suggested that maybe I could go a year before the next scope.

Wednesday: Normal life, until evening, when it was a struggle to stay focused enough to watch the debates.

Thursday: I felt really poorly, headache, no energy, unable to focus or think or read, light-headedness. I thought, Gee, it’s a good thing I had the endoscopy Tuesday or else I’d be sure I was starting a bleed.

Friday, 1 am: Urgent need to use the toilet. Expelled globs of old digested black blood, then started throwing up black blood. Simultaneously. Really disgusting. Yelled for help, husband came, call into 911, off I go in the ambulance.

Now, this bleed wasn’t as bad as bleeds one, two, and three because my blood pressure never dropped low enough so that I lost consciousness. I was even able to talk the EMT out of starting an IV in my rolling, uncooperative veins en route to the hospital along winding and bumpy roads.

So what happened? How did I manage to go from A+ to F in the esophageal health department?

Next day the founder and boss of the GI practice did a much slower endoscopy. He found a tear in the esophagus and repaired it with two clips.  He wants to call it a Mallory-Weiss tear, which can follow extreme retching. But the only retching I did was sudden and swift vomiting of blood. In fact, no retching was involved. More like spouting.

He can call it what he wants. There’s no way of ever knowing what caused this tear. But the hospitalist, the nurses, anyone without a vested interest in it being a Mallory-Weiss, is likely to agree with me: I got nicked during the Tuesday morning endoscopy.

It happens. I’m not irate. I know that endoscopy is an invasive procedure and that there are risks. According to the Mayo Clinic, tears happen in “an estimated 3 to 5 of every 10,000 diagnostic upper endoscopies.” It is a good thing that I was conscious, coherent, and creditable, and returned to the same hospital where the exploratory endoscopy had been done. Without a history to work up a diagnosis, this could have been as bad as a burst varices bleed. The mortality rate is 20% when the esophagus is already compromised and because

the diversity of clinical symptoms and signs combined with a lack of individual experience [among doctors] regarding this particular condition may impede rapid identification of this potentially hazardous situation. Accordingly, delayed diagnostic work-up may hinder timely and appropriate treatment with a negative effect on patient outcome.

Well, that’s not real encouraging, is it?

So now what? What do I do in six months’ time?

It is, you see, a classic damned if you do and damned if you don’t, between a rock and a hard place, etc. situation: in trying to eliminate the risk of a burst varices bleed by exploratory endoscopy, I incur the risk of a bleed from the endoscopy itself.

Well, I’ll tell you. I’ll have the scope. But I’ll either have it with the hepatologist at UAB even though that means a five or six hour trip, or maybe I would have it here — but only if the bossman himself does it.

Someone who can do the math can combine these odds, just for fun. Incidence of having PBC in the first place: 2.7/100,00. Incidence of tears in upper endoscopies: 3-5/10,000. Incidence of having PBC and having  a tear following an endoscopy = ???

Third Time Around: PBC, Portal Hypertension, and a Routine Bleed

This one’s for that small audience of folks with primary biliary cirrhosis or other conditions that lead to portal hypertension, which can lead to burst varices usually in the esophagus or stomach, which result in a disgusting and alarming expulsion of fresh red or older black blobs of blood. I’ve received comments from people with PBC that they have looked for information on what these bleeds are really like. I’ve been chronicling my experience with PBC for a couple of years now, so here’s an update on this emerging story.

It happened to me again Sunday. This one wasn’t too bad — just two transfusions and two days in the hospital.

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PBC, Portal Hypertension, and a Bloody Inconvenient Route

My last post before my world changed on August 28 was “Another Bloody August. Mysteries and Muddles. And Hospitalists” about my second sudden gastric bleed in two years and my second misdiagnosis by Huntsville Hospitalists and GIs who insist I simply have bleeding ulcers and refuse to consider portal hypertension, a common complication of liver failure, which is the end result of primary biliary cirrhosis.

If you are new to this blog, you are likely confused. One of my recurring subjects is my life with primary biliary cirrhosis, an autoimmune disorder that causes the bile ducts to self-destruct; the back-up of bile leads to cirrhosis, and cirrhosis to liver failure. At the top of my homepage I recently added a link to a permanent page listing both my postings on this subject and some of the most useful websites I’ve come across. I’ve considered pulling these posts and putting them in their own blog, but for now, they are staying here.

I write about this because it is a rare enough disease that firsthand accounts of living with the illness year after year are hard to come by. The blog format gives a new opportunity to illness diarists, a sort of real-time history of our decline. Readers can extract only the posts tagged PBC and see a sort of sped-up version. Like in my case, my first post about having PBC was two years ago, November 13, 2009,  Varicose Veins in my Esophagus? when I was surprised on my annual visit to Kirklin Clinic to learn I’d have to start having endoscopies to check for esophageal varices. Then I decided it was time to write about this illness because I wasn’t going to be able to ignore it forever.

I was right about that; in August 2010 I had a bleed requiring 4 transfusions, and this year another requiring just 2 transfusions. That one landed me in the ICU, however (two weeks later that creep beat the hell out of my daughter, by the way. He knew I’d been very sick. Prince of a fella, that one. I digress).

This August, the Huntsville Hospital staff GI did an endoscopy 20 hours after the bleed and decided I had ulcers. After all, I had had them last year. Only I hadn’t.  In September I visited another GI in Huntsville who determined that portal hypertension, not bleeding ulcers, was the culprit; he started me on beta blockers. Then in October I took a little break from the domestic violence battles to have another endoscopy by the good man Dr. Brendan McGuire of UAB down at the Kirklin Clinic. He rubberbanded several large varices that could have burst, essentially destroying the vessels.

So yesterday, fresh from the non-trial debacle, it was time to return to Kirklin for my annual November pow-wow and bloodletting with McGuire.

I was worried that I was going to find out that my PBC had advanced since I had had a second portal hypertension burst varices bleed.

But no, my blood chemistry remains stable for a person with PBC.

So why the bleeds?

It’s a mechanical issue, according to Dr. McGuire. The blood wants to get up to the heart and lungs. It wants to go through the liver, but the PBC has blocked its usual routes. It could decide to take stronger, wider vessels in my back, for example, as its detour route. But no, instead my blood has chosen to detour through my stomach and esophagus, taking routes that can’t handle that volume of traffic, stressing vessels not meant to handle such loads. When these routes are stressed to the breaking point, they break, and the endoscopy road crew has to come and clean up the mess and patch up the leaks, About the only thing otherwise that can be done is routine maintenance — that is, endoscopy to check out potential problems and band the varices — block the routes — before they have a chance to burst.

So April I’ll have another endoscopy. And who knows, maybe I won’t have a third consecutive bloody August. That would be nice.