Tag: primary biliary cirrhosis

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.



How PBC Became PBC

I haven’t updated since October of last year. I get comments now and then, asking how things are going. To catch you up if you are new here, I have  been writing about my battles with  Primary Biliary Cirrhosis. While I haven’t had any more esophageal bleeds or episodes of hepatic encephalopathy, this condition continues its destruction.

Although my bilirubin and albumin are worse than they were this time last year, it’s the psycho-social effects that have been devastating this winter. I intend to write more, but shorter, posts on those aspects of End Stage Liver Disease.

But first up is that I no longer have Primary Biliary Cirrhosis, according to the international health community.

I have instead Primary Biliary Cholangitis.

Cholangitis isn’t an altogether accurate a term to take the place of cirrhosis for this illness. I suppose cholangitis  was assumed a good enough switch since in both cholangitis  and primary biliary cirrhosis, the bile ducts are compromised.

But there are some very significant differences. Simple cholangitis is usually caused by a bacterial infection; primary biliary cirrhosis (cholangitis) is an auto-immune condition. The prognosis for simple  cholangitis is good if caught in time. There are a variety of treatments.

Primary Biliary Cirrhosis or Primary Biliary Cholangitis has one drug that may slow the progression of bile duct destruction. It will lead to cirrhosis and end-stage liver disease. How long this will take varies, but it will happen. The only fix is a transplant, and, since this is an auto-immune illness, it isn’t unusual for it to recur. The name made sense: primary (firstly), biliary (bile ducts destoyed), cirrhosis (inevitable effect in the long-run).

But at least the same letters apply, so PBC can become PBC.

Why change to a less accurate name?

Ignorance and prejudice. Although there are many conditions that can cause cirrhosis, the biggie is excessive alcohol use (of course, there are daily drinkers who do not reach end-stage liver disease) and hepatitis (and one of these can be caused by using dirty needles).

And so cirrhosis is a huge trigger word: this person’s lifestyle has caused her condition. For many, the social stigma is as bad as the condition itself, and the medical community decided these people have enough to deal with. They are not even close to fixing the disease, but lessening the instant self-righteousness of the unafflicted is within their range.

Say “I have primary biliary cirrhosis.” Most people hear something like “I gobblledly gook blab blab cirrhosis.” Next, they likely speculate on what vice is the cause of the problem.

It’s a rare enough illness that I have had to explain it to first responders and ER nurses.

You can feel what isn’t said: the emergency personnel have scraped up enough people killed by drunk drivers to have no sympathy for heavy drinkers.

Believe me, there are no heavy drinkers among those with PBC whose livers are failing. If the liver can no longer handle red meat, it isn’t going to be up to processing alcohol.

Maybe we once drank cheerfully and heartily. Maybe we were teetotalers. Neither would have made any difference.

At least the next time I’m hauled in my biggest problem with listing my medical history will be remembering how to spell cholangitis, and not dealing with all the baggage cirrhosis drags around.


Protein, Ammonia, Cirrhosis, and Hepatic Encephalopathy: What I Learned

My last post, “Hepatic Encephalopathy: On Knowing I Didn’t Know What I Knew I Know,” ended with me trying to cope with an episode of confusion, temporary loss of lifelong memories and inability to form new ones a week following the installation of a Transjugular Intrahepatic Portosystemic Shunt (TIPS) to deal with the portal hypertension resulting from cirrhosis, a consequence of Primary Biliary Cirrhosis (or Cholangitis) (Regular readers will be used to the growing “This is the house that Jack built” nature of my introductory sentences.).

I believe I have regained all I had lost of my memory. The treatment was (and continues to be as a preventive) an extreme laxative called Lactulose that “works by drawing ammonia from the blood into the colon where it is removed from the body.” It is a sickeningly sweet liquid that makes my intestines feel as if they’ve been invaded by frolicsome ferrets, but this transient discomfort is trivial compared to the frightful alternative of hepatic encephalopathy.

After the TIPS was installed, I was told to avoid fried food and red meat and I complied (by the way, pork is not “the other white meat” when it comes to ammonia: it is a red meat). However, because of the events of the two weeks preceding my encephalopathy (days at the nursing home, my mother’s death, 300 mile each way trip to UAB, two endoscopies with conscious sedation, general anesthesia with the TIPS, etc.) I had become very irregular, backed up as it were.  I was not getting food processed and through my digestive system efficiently, and certainly not quickly (now the Lactulose helps with that).

My understanding of why I should avoid red meat and eat small amounts of protein over the course of the day rather than in two or three sessions was slow in coming.

After release from the hospital, my family and I started a hunt for low-ammonia foods. No luck — just try to find the low ammonia diet. There are horrifying stories about “pink slime” and the use of ammonia to control e coli in food processing plants (remember that? The “pink slime” Wikipedia entry hasn’t been updated since 2013, which doesn’t mean the stuff isn’t still around.). Red meats and rind cheeses were listed as items to avoid if you suffer from cirrhosis, but there wasn’t a clear indication of why. Finally, I contacted a food scientist I knew who provided the key.

I was not going to find low ammonia foods. Ammonia is a by-product of the breakdown of proteins, whether they be animal or plant-based. The thing is, red meats and rind cheeses (and to a lesser extent, chicken and fish) are more protein-intensive than vegetable-based proteins, so more ammonia is produced during their digestion.

For most people this isn’t a problem. The liver takes care of the problem, as it handles other toxins consumed.

But a cirrhotic liver isn’t up to the task. Think of all the drug labels and commercials that tell you to consult with your doctor before using if you have an impaired liver.

Red meat should contain a similar warning.

From the 1950s (at least) into the first years of this century, cirrhotics who had experienced hepatic encephalopathy [HE] were told to eat little protein, period, and malnourishment was common among cirrhotics. As their bodies consumed their own muscle and fat for protein, ammonia was released, and thus HE was not avoided by not eating meat.

Now the advice is for cirrhotics to consume more protein than than non-cirrhotics, and I have been told that while it is most important to get enough protein — to eat chicken and fish if I need to — non-animal sources of protein are less of a challenge to my liver. I am still looking for studies on whether vegetarian or vegan diets are superior for preventing HE in contrast to those that include some animal protein. If it were possible to eliminate the need for Lactolose or rifiximin (an antibiotic used to prevent HE), I would go vegan.

It was easier to give up red meat than I would have imagined. Back when severe anemia was one of my major issues,  I was a staunch defender of meat-eating, arguing that for some of us, a vegetarian diet was not an option, that we couldn’t otherwise get the iron needed to raise our hemoglobin from the 8’s to the 12’s without transfusions or infusions. And I still think that universal vegetarianism isn’t practical, unless you want to require all who live in regions inhospitable to agriculture to eat processed foods shipped in from other climes (are there any Sami, Inuits, or Eskimo thriving on locally-produced vegetarian foods?).

Now I eat far less chicken and fish; they don’t dominate the plate but are supplements to the grains and vegetables, and I eat them only a few times a week. I eat a lot of beans and rice, oatmeal, grains. No fast food — and I don’t miss that at all.

There’s a relief to being forced to do what I’ve known for a long time I should do but have been too lazy to bother doing.

So far, I’ve had no more problems with HE. There are other theories about what causes hepatic encephalopathy, but the prevailing one is that when the liver can’t handle the ammonia that is a by-product of protein digestion, the ammonia crosses the blood-brain barrier, and the brain becomes a sink for this noxious toxin.

Still, it is amazing to me that cirrhosis has been recognized as a disease for hundreds of years and yet so many questions remain about its treatment and effects, but I suspect that may be because it is largely linked to alcoholism, and the stigma of cirrhosis as a lifestyle and avoidable disease.

My 13th — and Perhaps Final — Portal Hypertension Bleed

At the end of my previous post, My Mother’s Last Three Days, I announced that I had had my thirteenth portal hypertension bleed at my mother’s death bed.*

It had happened. A bleed. A big one. I was taken to the hospital by ambulance.

I think that I have never been nearer to a complete breakdown than I was that night in the ER. Why a bleed now? Even if she had had enough opiates to cloud her memory, my last memory of my mother will be this: not being there for her.

The usual thing, the IV’s, the history, the whole admissions rigmarole proceeded. I told the GI on call that night if I wasn’t going to be scoped in the morning when there may still be a chance of finding the source of the bleed, that I wouldn’t consent at all to an upper endoscopy. The gastroenterologists here have repeatedly delayed up to twenty hours between the start of a bleed and their looking for its source, and then are surprised when they find none. A bleed can stop on its own, and the IV medications, as I have explained before, aid this.

I was taken for the endoscopy at 8:30 or so in the morning.

When I was returned to the room, my husband was there. He had his news, and I had mine. His presence was enough to tell me what his was: my mother was dead.

Mine? The GI who did the endoscopy said that what she had seen was something she could not fix, and neither could her colleagues. I needed to get to a teaching hospital as soon as possible.

I had a stray blood vessel resting over two large varices. Normally, if I understood her correctly, this vessel could have been cauterized, but as it was positioned, there was a risk of burning through the vessel and into my varices, causing a massive bleed.

Things had gone for bad to worse. I was too distressed to be surprised, quite frankly.

So we got in contact with my hepatologist at the University of Alabama in Birmingham about six hours away, and an appointment was set so that he could have a look for himself first thing Monday morning.

Here are the pictures from the July 9 endoscopy clearly showing the problem in my esophagus. I think the doctor said the purple things were large varices that couldn’t be banded because of the blood vessel that couldn’t be cauterized because of its position.

In my next post, I’ll explain how things got worse — again.

*(To catch you up if you are new here, I have Primary Biliary Cirrhosis (or Primary Biliary Cholangitis), and since August 2010, I have had some minor and a some awful episodes of vomiting blood —  hematemesis — because of bursts varices in my esophagus. There have been lots of co-diagnoses along the way, from ordinary ulcer upper GI bleeds, to Cameron’s Erosions, to Dieulafoy’s Lesions, but as will become evident, whatever you want to call the spouting body, the source was likely always the same: the portal pressure in my portal vein in my liver measured 15 on July 16, 2015,  dangerously high. It is now 2 to 4. More on that later.)

3 in a row 4 in a row ang1 arrows angi2 angie3 angie-4

Bleed 11, An Exploratory Endoscopy, Bleed 12, Injectafer Again

My trials with gastrointestinal bleeds continue. Simply go straight up and click Primary Biliary Cirrhosis or Portal Hypertension Bleeds if you are a late arrival to this wearisome party. At this point I think I keep chronicling them so I can remember myself, have I had 11 bleeds or 12? How many transfusions so far?

The Halloween Bleed 2014

I was trundling down the tracks uneventfully — how lovely an uneventful life can sometimes be — when I derailed on October 28, 2014. I started with melena, and so my husband drove me to the ER; I knew from the metallic taste in my mouth the upper GI bit would begin soon.

As one friend said, some people will do anything to  win the Halloween costume contest.
As one friend said, some people will do anything to win the Halloween costume contest.

Now every bleed has its moments, and this was during the Ebola scare. I thought I could perform a useful service to the Ebola response team every US hospital was throwing together last fall, so I told the triage nurse that within minutes, probably no more than an hour, I would be throwing up blood. She stopped me. Had I been to Africa or been around anyone who had? No and no. However, I continued, since I posed no infectious risk, I was the ideal test: get a team suited up, handle me as though my emesis had an unknown cause, and see how well they did with avoiding getting any bloody vomit on exposed skin. She scurried away and passed me to a different waiting room for labs.

The tech just about had time to get the needle in when up came the red blood, a good bowl full (I had brought my own bowl). We were crammed in a tiny room where another nurse was charting. The two looked horrified. I said, I told triage this would happen.

On a scale of 1 to 5, with 5 being worst yet (the first, early August 2010), this bleed ranked about a 2, and only because it was both a melena and emesis event. I didn’t need transfusing. The Injectafer iron infusions I had had back in April stood me well.

But then I made a mistake. After each upper GI bleed, the GI on call for the practice that has a monopoly in this town does an endoscopy to ostensibly find the source of the bleed. Since these scopes occur on average 18 – 22 hours after the bleed, and since in the meantime I have received bags of IV octreotide that stop GI bleeding, these are really Cover Their Asses exercises so they don’t discharge me with an active bleed. They have never once found the source of the bleed. Once you stop a bleed in the GI tract and it has had time to clot, chances of finding it are too small to bother with.

Back to the mistake, and the second memorable moment. The GI visited my room and said, while he didn’t think he had found the source of this bleed, he did find some oddity. “I stared at it for a long time.” This was what he had to say about the oddity he decided to call an ulcer and to put a clip on (another scar!) even though he said it was in an odd place, wasn’t bleeding, and wasn’t the color or shape expected. But to answer my questions he repeated, “as I said, I stared at it for a long time.” (How very reassuring!) He wanted to stare at it again in 3 months.

And like a fool, like an idiot, I foolishly, idiotically agreed to return February 3, 2015, so he could stare at it again.

February 3, 2015: The Exploratory Scope

So in I go as an outpatient, in fine fettle. Hg on January 21 of 13.1. Very respectable.

And the mysterious it? Vanished. The doc found the usual Cameron’s Erosions. I had a look at the pictures from his scope, and said, well, guess I’ll be back here soon. He said, on the contrary, all looked fine, no banding needed.

February 12, 2015. Bleed the Twelfth.

Nine days after the unremarkable exploratory I was in the ER again. This was a major bleed. On my new, devising as I go along, scale of 1-5, I’d say maybe a 4.0 to 4.5 based on projectile emesis and brief loss of consciousness. I still haven’t seen the stair stretcher or whatever it is the EMTs use (when I’ve needed it, I’ve been too far gone to see it).

My biggest fans will know two things seem familiar here. One is the date. A year ago, Feb. 12, 2014, I had had a bleed on the first anniversary of my collie’s death. Now it was the second anniversary. Rascal bled out, by the way. Undiagnosed tumors burst on his spleen. Well, that is just coincidental gothic bad luck.

But I’m not so sure about the other coincidence. The last time I had an exploratory scope in this city, I had a bleed about 64 hours later. 

That’s it. All done. I will have exploratories at University of Alabama-Birmingham (med school) Kirklin Clinic, but not here.

And I may refuse any post-bleed scopes here as well. But that is a story not yet completed. For now let’s leave it at this: February 3, 2015, as an outpatient in good heath I had Managed Anesthesia Care (meaning a nurse anesthetist is present throughout the procedure). On February 13, 2015, as an in-patient who had lost an estimated 3 – 4 units of blood less than 24 hours previously, I did not.

Back to the Injectafer Infusions

I left the hospital after Bleed 12 with a hg of 9.1. I had not been transfused. Two weeks later my hg had dropped to 8.7, and so I had my iron reserves checked:   My iron was at 28, % saturation 6, and ferritin 4. So the hematologist this time ordered two sets of Injectafer (4 infusions).

I should feel better by April 10.

At least I am now caught up on chronicling my crises.injec

Injectafer infusion

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).


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