ESLD: Holding Steady

This week marks the first anniversary of what I for many months felt was a final descent. In the course of three weeks I had a massive esophageal bleed at my mother’s deathbed, had a transjugular intrahepatic portosystemic shunt (TIPS) installed, and was hospitalized with hepatic encephalopathy.

It took until April 21 for me to stop thinking about dying and to begin to think about living. The evening of the 20th I took a half-dose (by mistake) of a new sleep drug, and I had a paradoxical reaction: I could not sleep at all. I was awake for about 36 hours, and if you have ever experienced that, you know that around about hour 32, things can start to get quite interesting. It was Earth Day, and for three hours in the late afternoon I did nothing but watch the changing colors of the green leaves at the day turned to dusk.

And the next day I felt like my brain‘s reset button had been engaged.

I hit a plateau, I guess. It’s like I had been falling, falling, falling into the abyss but then the fall was interrupted, as if I had landed on an anomaly in the walls of this abyss, some unanticipated protuberance.

This doesn’t mean I can get all together out of the abyss; eventually the protuberance will erode, and the fall resume, but it isn’t happening now.

Now I have some distance from the events of the past year, perhaps I can describe some of those psycho-social aspects of my situation.

For much of last year I felt like all my psychic defenses had been breached. I was attracted by its title to Dion Fortune’s classic Psychic Self-Defense in which she describes the effects of psychic attacks by evil magicians or spirits and means of banishing them or protecting one’s self from further harm. I felt as if I had been attacked by malevolent forces – not bad magic mind you – but a free-floating malevolence.

I think magical thinking is an aspect of illness. I’m even a bit hesitant about saying things are going pretty well for fear that the saying so will make them go wrong.

I remember a particularly bad night when my daughter was hours late getting home (she had gone to the movies; phone off, etc.). I envisioned all the worst as having happened, and that here I was thinking things were pretty bad, and the Universe was going to say, “hey, you think things had been bad before but you have no idea just how bad bad can be” – a little like when you hear some particularly stupid parent tell a child who is crying to quit or else “I’ll give you something to cry about” and is then amazed that the child cries harder still.

Then there is last-time-it is. Is this my last Thanksgiving, my last birthday, my last Christmas? It doesn’t make it seem more precious: it ruins it.

Then there is the any of us could die at any time problem. While this is absolutely true, we don’t really believe it. It is a tralse — some truth, more falsity. Sometimes I feel like responding to that observation with a counter-offering: “in that case, why don’t we switch livers?” But these is no reason to offend folks who are trying to be kind.

I neglected my social life. It was easy to do so. I think I was like the spectre at the feast for much of the year, looking as exhausted and as beat as I felt. Maybe I imagined that people were pulling away from me. Maybe they were. It is an awkward situation.

It didn’t help matters when David Bowie died and the initial reports were that liver cancer was the cause (later clarified as pancreatic that had spread to the liver). Not all people who have liver cancer are cirrhotics, and not all cirrohtics get liver cancer. But cirrhosis is a definite risk factor.

I had decided long ago that if I developed liver cancer, I was not going to fight it (in fact, I think there is little that can be done when someone with ESLD gets liver cancer). I am supposed to go every six months to see if I have developed cancer, but I decided, why bother?

I will tell you why: in March we went on a vacation, and after nine months of doing nothing, I wasn’t prepared for walking a lot. After about a half hour, I would hurt worse and worse as a pain developed on my side and radiated to my back. Of course I decided well damn, I must have cancer.

So I went in for the ultrasound and blood test to see if maybe I was wrong. And I was. But I did have a severe vitamin D deficiency.

Throughout much of the night of my 36 hours of wakefulness I watched Bowie on youtube. He lived until he died. Made an album, wrote a play.

Maybe that contributed to my change of attitude, my finding the plateau. Or maybe it takes 9 months to transition from one existence to another.

I just know that when I finally did sleep, when I awoke, I decided that today I was not going to think about My Death.

And now that has become a habit.

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.

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.)

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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

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.