Category: Portal Hypertension Bleeds

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.

 

 

Hepatic Encephalopathy: On Knowing I Didn’t Know What I Knew I Know

This post assumes you’ve read the two preceding ones: “My 13th — and Perhaps Final — Portal Hypertension Bleed” and “In Which a Transjugular Intrahepatic Portosystemic Shunt (TIPS) Is Installed in My Liver.” This post is my subjective, and given the subject, patchy account of a few dreadful days. I will share what I later learned about hepatic encephalopathy in a future post.

For well over half a century, I’ve known the answer to the question: What is your birthday?

Even when I was missing my four front teeth and saying “December twenty-six” was a twister, I could answer that question: It’s the day after Christmas.

On July 23, 2015, one week after my TIPS procedure, I couldn’t find the answer. I knew I knew it, and that knowing—that is, knowing this was happening—that I had lost a nearly lifelong memory—was horrifying.

I was suffering from hepatic encephalopathy.

My liver could not handle the ammonia it normally filtered, the ammonia had crossed the blood-brain barrier, and I was unable to make new memories or access information long stored and always before easily within reach.

I left the hospital Sunday, July 19, 2015. The installation of my Transjugular Intrahepatic Portosystemic Shunt (TIPS) was deemed a success. My portal hypertension, which was 15 (dangerously high) when the radiologists began the procedure, had dropped to a normal range of 2-4. At first my liver function numbers were all over the place, but that was to be expected. My ammonia levels were high, but had steadily dropped. My digestive system seemed to have woken up from the general anesthesia. I complained of constipation, but I did have a bm.

I know that Monday I slept all day, and I expect I did on Tuesday and Wednesday as well. I had had a rough two weeks.

The first weirdness was  pre-dawn Thursday. I could not get warm. I like a cold room when I sleep, and keep a lot of covers handy year-round. But I could not warm up.

At some point Thursday morning my husband noticed I wasn’t making sense when he asked if I wanted anything to eat or drink. I was somnolent, very hard to rouse. I have no memory of this.

He told me he called my primary care physician and gastroenterologist. It was only a few days later that I remembered anything at all about having been at the GI’s. Leaving the house, I walked right past a huge display of flowers that had arrived that morning from my brother: I had no memory of them when my husband mentioned them later.

I have a vague memory of either getting into or out of the car at the doctor’s, of looking up and seeing my daughter had joined us in the consulting room, and of sitting in the room (I guess that I wasn’t lying down because they didn’t want me to sleep). I’m told that the physician’s assistant offered to send us along with a prescription for Lactolose, a strong laxative which I’ll discuss in the next post, but since my husband and daughter wouldn’t have known what to do if I didn’t respond to this drug or what to expect or danger signs, they—I believe quite rightly—chose to take me down the street to the ER.

I have no memory of getting there or of the usual procedures (and I’ve been in the ER enough to know), like signing in, waiting in the first waiting room area, going to the triage nurse, and having vitals taken. I do remember being in the second waiting area, and I guess all the action had woken me up enough so that I realized what was happening.

Hepatic encephalopathy is the complication of a TIPs I feared the most because it involves your ability to think and remember. At its worst, it can lead to coma, sometimes reversible, sometimes not.

To the other people in the waiting room, I must have looked fine – no ice packs, bandages, vomit bag, etc. But each moment that passed I believed I was closer to losing my mind. At some point I must have asked for a DNR (Do Not Resuscitate) bracelet because much later I noticed I was wearing a purple bracelet I’d never seen before.

At some point—an hour? two?—I was taken back into the examining area.

After a while, I said to my husband, aren’t they going to do anything? They haven’t even started an IV. They always start IVs. He said, look at your hand.

I looked down, and saw I had an IV. I in fact had what I’ll call a double-barreled I on the back of my hand—a painful place for a stick—and I am one of those people with small veins that roll, etc.

Was I too somnolent to feel an IV going in? Did I feel it but was unable to remember having felt it? Was I, in other words, unable to form new memories?

Then came the questions.

  • What is your phone number? I hadn’t a clue.
  • What is your birthday? I know this, I do, I know this. I couldn’t find it.
  • What month is it? 12?
  • Try again. 6?

How strange. I have this very vivid memory of not knowing these answers.

(Later it occurred to me that the oddness of my response – 12, 6 – rather than the names of the months was because I was still searching for my birthday (12/26) and could not make the leap to the new question. My phone number I simply had to relearn, and it took til Sunday morning before I got it right routinely.)

The rest of Thursday is vague to me. I don’t know if I had anything to eat that day. I can’t tell you if it was day or night when I got into a room. This might not seem unusual, except that here going from the ER to the in-patient hospital requires an ambulance ride across a street. But I remember being able to get from the gurney to my bed without help.

Then it was Friday. I remember very little of it until the evening. Then it was time to start trying to figure out what had happened.

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

My Mother’s Last Three Days

Easily the most read and commented upon post on this blog is one that I wrote after my father’s death, How to Know When Death is Imminent, Signs Someone is Dying.

My mother died six weeks ago on July 9, 2015. I called my father’s death “hard work.” It was the first for which I had kept vigil, and now I know his was an easy one, at least for me. He suffered from Parkinson’s, dementia, and Alzheimer’s and had been in and out of consciousness for days prior to his death. He couldn’t talk, so I did not know if he was in pai or scared. He rallied to smile once, although I could tell from his eyes he was aware that people were with him.

My mother only lost consciousness at the very end, helped along, I think, by generous doses of morphine.

She had been totally bedridden for 18 months, and full assist (had to be fed) for the last six or so.

But she never became demented.

My mother was disabled by rheumatoid arthritis, and weakened and finally defeated by kidney failure. The last year of her life she was assaulted by edema. Using diuretics compromised her kidney function. Not using diuretics meant her arms especially would look like half-full water balloons, and every time she would be touched, she was in pain. Even the seam allowances on the insides of her sleeves tormented her. This is what we could see. Edema also puts a strain on the heart.

Through careful monitoring, the doctor was adding the diuretics as needed, then pulling back when the kidney function numbers started looking alarming; then repeat and repeat.

My mom had a decent quality of life, primarily because of the graciousness and professionalism of the staff of Pisgah Manor Nursing Home, who I will never be able to praise highly enough. My mom loved her meals, and she raved about the food. I’d leave Pigsah Manor hungry when I’d come to feed her. Let’s stop and consider that: Have you ever heard any visitors say they left a nursing home hungry? And my mom loved to bird watch. We had several feeders going outside her window, and I have never spent so much time bird-watching. This spring and summer brought so many goldfinches.

Then at the end of June, I had a call that my mom was hallucinating horrifying scenarios, like her blankets were on fire and George W. Bush’s dog was bleeding out on her bed (really). The nursing home suspected a urinary tract infection, and the specimen confirmed this; within a few days she seemed much better, but for the first time wasn’t eating every morsel on her plate. I heard a strange sound from her lungs, like a cat mewling.

Monday, July 6, I went out to the home; they were about to call me anyway. My mother had a gurgling sound in her lungs now, but no other cold, flu, or virus symptoms. She obviously did not feel well; she had no appetite and would only drink with persuasion.

When her morning CNA left, she reported that my mom had produced no urine. I knew we were likely in trouble then; I had learned that when my dad died.

That night, I made calls to those I needed to. There was a contingent who thought I should have her transferred to the hospital. They were certain that once there, she’d get some medication that would fix her up just fine. I decided, since she was fully cognizant, to ask her what she wanted to do.

Tuesday a catheter was inserted. After her bladder was drained, it was obvious she was simply not making urine. I told my mother she was very sick, and asked if she wanted to go to the hospital. She declared she would fight this problem at the nursing home among people who cared for her.

The next two days, my mom was frightened and in pain. She said over and over and over again: “I can’t breathe.” And over and over I would tell her that yes she could breathe, if she could not, she couldn’t talk. She had supplementary oxygen; we would tell her to breathe through her nose calmly and slowly and out through her mouth. It didn’t work.

“I can’t breathe.”

She sounded like — and likely felt like — she was drowning. Edema had spread to her lungs.

Still no urine: we were coming to the end. The nurses did not hide this from me. Always there is the caveat that no one knows for sure, people will surprise you and so on, but there was no reason to believe this would be one of those times.

I was encouraged to go home and rest. Wednesday morning when I walked in I immediately noticed two changes. My mom was ashen, especially on her upper lip area, chin, and the region of the face around her mouth.

I am not trying to be poetic when I use “ashen.” It is used for a reason. The color is much the same as ash.

I’m also not trying to be poetic when I say that the light had gone out of her eyes. This is hard to explain, but there was a haziness or dimness. Perhaps only someone who knew my mother before would have seen the difference. She was, however, still fully conscious, and still panicking and saying “I can’t breathe” and “Help me.”

I asked her again about going to the hospital, and she refused. I told her she was very, very sick.

She asked if she might die. I said yes. When? No one knew.

She seemed genuinely surprised by this. Here she was, 6 weeks shy of 86, completely bedridden and disabled, was refusing food and water, not urinating and didn’t think she could breathe — and yet she was surprised.

I asked if she wanted me to make any calls so she could talk. No. The TV had been off for a few days now, and we were down to her caregivers, me, and her.

She said she was scared. I said what of. She looked at me like I was a total fool, and said, “Dying, of course.” She agreed when I offered to call a chaplain.

The rallying that was so dramatic with my father took a different form with my mom. My mom was exceptionally determined. She had strong opinions, and this blog is named after a phrase of her that could strike fear in those on her list (I’m going to have a little talk with him. . .”) while spectators would get the popcorn popping and wait for the fireworks. She was also pretty good at denial.

When my daughter and her boyfriend came by, she brightened up; she loved visits from my daughter. But after ten minutes, my mom told the two to get along with their evening and go out and enjoy themselves, that she had no intention of dying that night and that she would see them tomorrow. And the next day. And the next.

She tried the same with me, and I went outside to make some calls. Coming back in, I encountered the Perfect Daughter, there five days out of seven, who told me my mom bragged that she had “sent them all away.”

That was the last liveliness I saw from her. The night went on. There was no more conversation, just  “I can’t breathe” and “Help me,” and me watching the clock for when she could have more morphine. The intervals were less, the dosages greater.

My mother died around 9 am Thursday morning. I was not there. She was still conscious when my husband arrived to take over, and she was worried about me.

I was in the hospital waiting for an upper endoscopy.

Midnight I had gone to get the meds nurse; she had delivered the shot, and I was feeling a bit woozy, but blamed it on tiredness and stress and leaned my head against my mom’s railing. The next thing I knew, I was looking down on myself, and I was covered in blood, the chair was covered in blood, and there was a pool at my feet.

My mother was crying out,  “I can’t breathe. . . Help me.”

I had to tell her I could not, that I needed her to press the call button. She couldn’t. So I staggered into the hallway and yelled for help.

That was the last I saw of my mother, and the last she saw of me.

I had had my thirteenth portal hypertension upper GI bleed at her deathbed.

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

How It Feels to Have an Injectafer Iron Infusion

Today (8/25/16) I sent an e-mail to American Regent, the company that markets Injectafer in the US, with a link to the comments page. On the contact page, there is an email address for reporting Adverse Drug Events, if you would like to contact them yourself.

I have ceased having bleeding from my varices and my blood counts are normal. I am glad that I was able to tolerate Injectafer well enough to get my body the jump-start required to bring my iron reserves to normal levels.

———————-

The usual caveats about this not be medical advice, etc. all apply to the following. My purpose in writing this post is to provide something I couldn’t find when I was scheduled for and after I had an Injectafer iron infusion last April: a personal account. There are a number of resources for studies of Injectafer’s safety and effectiveness, but that is not all I want to know.

Injectafer was approved by the FDA in July 2013, so it is fairly new. My understanding is that the older types of iron infusions are a lot more problematic, side effects were more common, and it took hours to receive the infusion. But I have no experience with that.

If you are here, you probably already know that delivering iron through an IV is an alternative when oral iron supplements haven’t worked in alleviating iron deficiency anemia.

Followers of this blog will know that, as a consequence of primary biliary cirrhosis and portal hypertension, I have had 10 gastric bleeds from burst varices and 17 transfusions since August 2010. The past (nearly) 14 months have been good ones, with only a single 2-transfusion bleed in February 2014.

However, in spite of the 2 transfusions, I continued to feel run down and fatigued. For one thing, although the normal hemoglobin range is 12 to 15 (or 11.7-15.5. or 11.3-15.2 — depending on the lab; I have results from 3 labs here), transfusions after a blood loss, which usually adds about 1 point to the score per transfusion (or so I think — again, an ordinary person’s understanding and not a hematologist’s is all you are getting here) are usually stopped once the patient gets into the 10s. One reason is that with every transfusion, the possibility of having a reaction to the next is more likely. Another is that usually the patient’s body will bring itself up to normal levels in a few weeks either through diet alone or with iron tabs.

The first of April, about 6 weeks after my 2 February transfusions, I had my labs done and my hg was 9.7. I can cope in the high 9’s, but coping is about all. Basically, being anemic means being oxygen deprived. If you want a similar experience, go hiking at sea level for a few hours, and then go above 12,000 feet and do the same. In another couple of weeks I had crept up to 10.5 on the 12 -15 scale; better, but not great. My family doctor sent me to a hematologist. My iron level  was 31; normal is 40-160. Two Injectafer infusions were scheduled for a weak apart for early May.

Hematology and oncology go together, and I know that since I don’t have cancer I should have nothing to say about this other than how grateful I am. But the truth is, it is really a sad and sobering experience to have to go to an oncology infusion center. Most people are there for chemo. Of course I knew I was the lucky one in the room, but you know, it is still depressing. In fact, I think the worst part of the whole process was when I was waiting to schedule my little infusions and the man ahead of me broke down crying at the desk. The doctor came out and said this time it wouldn’t be so bad. It didn’t help much.

Getting the infusions was quick. Each took about 20 minutes for the little bag to drain into my veins. It hurt a bit, but not badly; it’s an IV and those are rarely for me painfree. The iron looks like you might expect — brownish with a bit of red.

I thought the infusion would have immediate effects, that I would leave the building infused with energy.

It does not work that way.

Most surprising was that I actually felt worse for several days after the first infusion. I felt like I had been run over by a steamroller that had then backed up and done it again. I felt like I had reached the end of hope, that for the rest of my life I would never again have energy.

When I mentioned this to my hepatologist in September, he said he had heard this from others, so it is not just me. The thinking is that any time the body is hit with a sudden infusion of something it has never had before — like a load of iron all at once — it is going to have to process it, get used to the idea, make some internal adjustments, and that is fatiguing,

I didn’t feel much of anything after the second.

But about a week later, I  noticed that I wasn’t dragging around so forlornly.

After another week, I stopped eating ice.

A Connoisseur of Ice

I used to eat ice all the time. I mean it, all the time. In the middle of the night I would awaken to eat ice. Simply sipping water wasn’t enough; I’d have to go to the freezer for ice. I’d never leave home without at least one tall glass full. When I traveled I carried a small ice chest, full of ice. I decided I would probably not ever enjoy going to Europe again because I remembered the one time I went to the UK, 35 years ago, getting ice was hard, and I simply liked it then. I had my favorite source of ice: Sonic. One of the things that made hospital stays tolerable was that hospitals have good ice. The only thing I missed when I moved a few years ago was the crushed ice dispenser on my fridge door. I craved ice.

Then I didn’t. I still am thirsty all the time because I have Sjorgen’s syndrome which means my salivary glands are also under attack by my immune system, so my mouth is always dry, and my teeth have gone to hell, but I don’t eat ice. I drink fluids.

Eating ice is a pica — the consumption of non-nutritional substances, like chalk, soil, or sand as well as ice. Frankly, for an anemic, it seems it would make more sense to crave soil, but I’m glad it doesn’t, and of course, talking what makes more sense among things that don’t make sense is senseless itself.

But no one knows why people with anemia crave ice.

My Normal hg

On June 2 had an hg of 13.9, up from the 9.7 hg of April 2: a significant improvement, and my iron reserves zoomed from 31 to 163 at the end of August. So now I have stored iron with which to make new red blood cells. On 9/11 my hg was 13.6.

So my experience with Injectafer: excellent.

I wish I had known it would take weeks to work and I’d actually feel worse for a while after the first infusion, but now, if you are wondering what to expect, you have one report from the field.

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.