Tag: portal hypertension

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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Primary Biliary Cirrhosis, Portal Hypertension, and the Frustration of Knowing There’s No Way of Knowing What I Need to Know.

I haven’t had a post recently on primary biliary cirrhosis. One feature of this blog is the occasional post on what it is like to have this autoimmune condition.

In the past two years, I had three bleeds as a result of esophageal varices bursting as a result of portal hypertension, itself a result of liver damage caused by damage to the bile ducts.

The first was in August 2010 and I required four transfusions. A year and two weeks later I had my second bleed in August 2011. Then at the beginning of this March, six and a half months later, I had my third. After the second and third bleeds, I had two transfusions.

As you might guess, when August of this year rolled around, I worried. I got through August fine, and September as well, that is, no bleed. Tomorrow I am having an exploratory endoscopy to band any varices that look like they could cause problems.

Twelve hours, and I will have dodged a bullet. Or maybe I will be informed that there was nothing to worry about, that no new varices have developed. But this doesn’t mean varices won’t develop later this month. Or maybe not til November or December. Or maybe never.

This is what I mean by the frustration of knowing there’s no way of knowing what I most need to know.

There’s no predicting these things. You can catch them before they happen with endoscopies — and luck.

Endoscopies aren’t painful, but they require twilight sleep anesthesia, and they are invasive procedures with the usual risks. It just doesn’t make sense to have one every week, or month, or two months. How about every three months? Four? Where to draw the line?

When the varices burst,  there are no warning signs until it is too late to do anything but react. With the third I had an inkling that I had started a bleed when I began to feel faint for no reason. Within 15 minutes I was vomiting blood. When the paramedics arrived, they were hopeful they could get me out the door to the stretcher. It didn’t happen. I stood up, and next thing I knew I was in the ambulance with IVs adding fluids to raise pressure to keep blood going to the organs.

When there is no science, magical thinking takes over. You look for patterns to make sense and end up thinking that if in the past two Augusts you’ve had a bleed, why won’t the pattern continue? If the time elapsed between the first and second was roughly a year, and between the second and third was six months, does that mean the fourth will be six months after the third? — or three months? — or maybe never or maybe there is no pattern.

It’s hard not to think that each day that passes without a bleed simply means you’re one day closer to the next one. Or that every time you don’t feel quite right the next thing to expect will be blood.

And there are places it would be worse to be than others. On a hike in a remote region, alone on the road on a rural highway, up in the air mid-flight. You start to curtail yourself, and let the threat define the parameters of your life.

Whether varices are found tomorrow or not, I’ll be safe from this threat — for a while, anyway. But how long a while?

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

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

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

(more…)

PBC, Portal Hypertension, and a Bloody Inconvenient Route

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

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

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

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

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

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

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

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

So why the bleeds?

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

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

 

Another Bloody August. Mysteries and Muddles. And Hospitalists.

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.

Silly me. I look back on my post from earlier this month, An Uneventful Day, Unlike Last August 2, and wonder. There I wrote about how on August 2, 2010, I had an arterial lesion burst where the esophagus meets the stomach, and how nice it was that this August 2, 2011, was so comparatively uneventful.

Little did I know that just 11 days later I’d be back in the ER. Circumstances weren’t quite as dramatic this time around — no ambulance — and I got only 2 transfusions compared to last year’s 4. But in some respects, this episode of vomiting blood was worse.

Why am I inflicting this on you? Actually, I am writing primarily for my readers who like me have primary biliary cirrhosis. There’s more not understood about that disease than is known, and I feel it might prove worthwhile to use the internet as a way to compile and compare histories.

I accept last year’s diagnosis of a Dieulafoy’s lesion, which I attained after consulting my hepatologist, not content with the GI’s conclusion that I had a spurting gastric ulcer. This year the diagnosis is gastric erosion, but I’m not convinced for some of the same reasons I wasn’t last year. While swallowing is a problem some times, once food hits my stomach, all is well — spicier the better.

It seems too weird I can go a year without a stomach-ache while having such a torn up gut that I end up in the ER — too weird considering I am at risk for esophageal varices as a result of portal hypertension as a consequence of PBC.

My intuition tells me the PBC was at least a contributing factor to the Dieulafoy’s lesion, and to this latest event as well.

So this is what I want to know: has anyone else out there with PBC landed in the ER vomiting blood not from varices?

And Now for the Muddles. And Hospitalists.

I keep copies of my medical records. Last year I discovered from the hospitalist’s history that “I got dizzy, and came to the emergency room.” When I complained that this wasn’t an altogether accurate way of describing arriving via ambulance, lights and siren, IV’s in both arms, on O2, my objections were dismissed. This year, I found out that I had been vomiting blood for 2 days when I came to the ER. Wrong again. First, I’m not an idiot. Second, I told the zillion people who had to have the day’s events recounted, that I had vomited twice that afternoon before coming to the ER.

Why can’t these hospitalists listen?

And what, you ask, is a hospitalist anyway? If you are a slow learner like me and it takes you years to realize your [now ex-] general practitioner is too indifferent to bother with hospitalising her own patients, you are stuck with a hospitalist “managing” your case. In my experience this year and last, they are, at best, obstructionists.

This year’s example of foolishness:

In the triage room, I felt really bad, clammy and sweaty. Then I was on a gurney being rushed to the ER trauma room. It wasn’t like fainting, because it wasn’t gradual (when I’ve fainted, usually things turn black but I can still hear what is going on, and then I’m down). I wasn’t out for long, and the ER staff started getting fluids in me as quickly as it could. Seems to me an obvious case of hypovolemic shock. This happens with blood loss.

But the hospitalist saw things differently. He wanted to know about my history of seizures. I don’t have one. What, this has never happened before? Well, yea, once, last year, when I was throwing up golfball-sized clots of blood. Ah ha, so you do have a history of seizures. No I don’t.  How I hate being in no position to resist.

Next thing I knew I was wheeled away and my head was in the damn doughnut — a brain CT, looking for the cause of my “seizures.” CT was normal (duh), so the next day in comes the EEG woman to stick wires on my head. EEG normal — well a bit of excess in the betas, probably related to anxiety (does exasperation count as anxiety?).

This year, 20 hours passed before I had an endoscopy to look for the cause of the bleeding, compared to last year’s 4. Meanwhile, my hematocrit kept falling. Finally, when it hit 7.3, 32 hours after my arrival, I was transfused, compared to pretty much immediately last year.

Was there a connection between the relative slowness of dealing with the problem (blood loss) this year and the the hospitalist’s obsession with seizures?  I don’t expect to ever know the answer. It was, however, most definitely an annoyance I didn’t need.

At Risk for Esophageal Varices and I Nearly Bleed Out from a Gastric Ulcer: How Weird Is That?

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.

If you’ve read any of my posts on primary biliary cirrhosis (PBC), you were probably surprised by yesterday’s post that there wasn’t some sort of medical muddle involved. Wonder no longer: there was. It’s just that my emphasis was different so I left it for today.

I’ve written about being at risk for esophageal varices as a result of having PBC. These are swollen veins (like varicose veins), caused by portal hypertension (itself caused by cirrhosis [in the case of PBC — there are others. Click through to a site devoted to patients’ experiences with it]) in the esophagus. Left untreated these could “burst and bleed into the gut.”

But I had an endoscopy just this past January that showed only a trace of varices, and in such cases, 96% of people are trouble-free for at least 1-2 years (then they do another scope).

Of course, that means 4% of people aren’t.

So as soon as I regained consciousness in the ambulance, I alerted the chief EMT to tell the folks in the emergency room about this. And when I got there, I told them myself.

I will refrain from elaborating on how it feels while being transfused to have to repeatedly explain primary biliary cirrhosis and spell Urso Forte [the drug I take for it] to the ER nurses, and later my floor nurse and abdominal ultrasound technician.

However, following the endoscopy performed there in the ER trauma room, the GI who did the procedure reported that I lost enough blood to require four transfusions not because any esophageal varices burst, but because of a “gastric ulcer spurting blood.” He repaired it with three hemostatic clips and put me on pantoprazole.

But how weird is that? To be at risk of burst esophageal varices and have a gastric ulcer burst instead?

I reviewed my endoscopy report (high marks to the GI, who actually gave me a copy of my own medical report!) and found the location of the ulcer to be the cardia. Googled that, and discovered it is right where the esophagus becomes the stomach, and, in fact, for many years there was debate as to which organ it belonged to.

Now then, there is a new kid on the block at the hospital: the hospitalist. This person is sort of in charge of patients who come in through the ER and whose regular doctor doesn’t admit or have any role in their care. Like me. It took me two days to get someone to tell me who was really in charge of my case: the GI who did the procedure, or the hospitalist.

When the hospitalist visited me, I explained about my surprise that my bleed was gastric and not related to portal hypertension [PBC]. I told him that I didn’t have a local GI, but that I was under the care of a hepatologist at the UAB med center.

So the next day he returns, and says, “Good news: you don’t have to have a liver transplant.” I thought yeah, duh, but let him continue. He told me he had set up an appointment with the hepatologist who would do another endoscopy. And I said that sounds great, but what about this report from the GI deeming the cause of the bleed to be a gastric ulcer? The poor guy looked confused. I suggested he go back and have another (an initial) read of  my endoscopy findings.

But the hepatologist’s office and I agree that I should be seen by him. I have so many questions: can a burst esophageal varice adjacent to the cardia be mistaken for a spurting gastric ulcer? Is it really possible to have such an awful gastric ulcer and no abdominal pain? Can portal hypertension cause a gastric ulcer? Will this happen again? And will I have no warning other than feeling steam-rollered before it does?

And just how weird is it, if it was a garden variety gastric ulcer, for this to happen to a person who has to worry about bursting varices?

Stay tuned.

Melena, Hematemesis, Hypovolemic Shock — and A Lot of Love

Gore alert: Medical terms in title refer to situations involving blood, blood, and more blood.

Were mine a glass house, what you would ordinarily see are four people: Mom, Dad, 14-year old Daughter, and 20-year old Son sitting in separate rooms, staring at separate screens. Even holidays aren’t so different; each year they come chugging along with annoying regularity — and I still can’t figure out how to get on board. Orchestrating Hallmark Moments, creating those Special Memories your family will cherish for a lifetime, all that is beyond me. No surprise then that Mother’s Day barely registers on our screens.  But I’ve something better than a lifetime’s stack of cards (and why do people give greeting cards to people they live with, anyway?): my fractured memory of this past Monday afternoon.

Last Saturday night we returned from a 3200-mile+ roadtrip out west, so Sunday I wasn’t surprised to be really tired. Monday wasn’t any better, and I had no appetite but no stomach pains, ate a banana and some soup.  I wasn’t even that surprised when I had the most horrible black diarrhea. I put this down to culinary karma — what did I expect after eating a sausage pizza at a truckstop in rural Arkansas? But Husband was concerned and called to get me a doctor’s appointment. The nurse said I needed to get to the ER, asap. I learned later that this was blood I’d passed, blood mixed with stomach acids: melena.

I resisted. I’d just have to sit there for hours. Besides, I couldn’t even get to the refrigerator and back without having to lie on the floor to rest. Looking back I see how odd it was to think that a reason not to go to the ER. I relented, but told my husband, who needs a total hip replacement, that Son would have to help me to the car.

We hadn’t reached the door when I said I needed to rest, so Husband went to get the air going in the car.

And then I started vomiting up huge black clots of blood. I wasn’t seeing what was going on at this stage, but I was hearing it. Husband called 911 for an ambulance. Dispatcher heard collie barking, said to get him secured. Rascal wouldn’t leave my side, but Daughter and Son together pulled and pushed him out to the backyard. Then both returned and as Husband talked with dispatcher the two of them followed her instructions, keeping me on my side as I continued vomiting up this foul black matter (hematemesis). They told me later I was flailing around, maybe convulsing or seizing, with my eyes wide open but my pupils not right. I certainly wasn’t seeing anything. I remember their stroking me, kissing me, telling me they loved me.

The EMTs and fire truck arrived in minutes. The kids say that the first guys in backed away, until the woman in charge came in and told them it wasn’t trauma (did they think I’d been shot?). Because of the position of our door, deck, and steps, getting a stretcher in wasn’t an option, so they had to haul me out to a gurney placed on the sidewalk. I remember being rolled into a blanket or something. And that’s it, for a while.

Although they worked on me outside, I remember nothing til I was loaded into the ambulance. My guess is I’d lost consciousness, but they started oxygen as soon as they got me out the door so by the time I was in the ambulance I was aware of talk about my blood pressure, getting needles in both arms, hearing the sirens when after 15 minutes or so I was stabilized and we got moving, and I have a few visual memories of the Head EMT and inside the vehicle. I think at some point I must have been between stages 3 and 4 of hypovolemic shock.

In the ER I soon was given two transfusions; later I was to receive two more. I remember the Head EMT telling the nurses they had a very anxious husband pacing in the waiting room who need to be allowed back as soon as possible. Then she was gone.

I became alert enough to be interested in the trauma room. There are posters on the walls telling RN 1, RN 2, RN 3 — up to 6 or 7, I think, exactly what to do and even where to stand relative to the patient’s bed. Someone cut my housedress off, just like on TV ER shows. I complained about the pain the large IV needles were causing me, about being thirsty, about needing to get the blood out of my hair. That was really gross. Even though I couldn’t move my arms I could feel that blood was stuck in my hair, and when I looked at my pillowcase, it was totally red. Not a priority, however.

I ended up having a spurting gastric ulcer repaired via endoscopy, and was home less than 48 hours after the ordeal began.

And what was going on while I was in the ER? Son was cleaning blood off the carpet and Daughter was cleaning everything else in sight.

When I came home, my collie was waiting on the deck in the 102° heat, somehow knowing I was on my way. My bed was made with fresh linens, and Daughter had imposed order on the clutter of the bedside table. She spent a good hour getting all remains of adhesive off my badly bruised arms.

Ever since I got out of the hospital and home with my family, I’ve been uncharacteristically cheerful, bouncing off the walls buoyant.

Of course.

Varicose Veins in my Esophagus?

“And if the dam breaks open many years too soon…”


I was doing a pretty good job of cultivating indifference toward my bad luck at having somehow acquired primary biliary cirrhosis [PBC], taking my 1000 mg of  Ursodiol most days (sometimes I forget), and not scouring the internet hourly looking for the article that would tell me my future, confining that process to just the week before what had become an annual appointment at the University of Alabama at Birmingham liver clinic.

After all, my alkaline phosphatase levels had dropped from the high 400s to the upper limit of normal, 117,  indicating the Ursodiol is doing its job, increasing bile flow from the damaged bile ducts, thereby theoretically slowing the progression of liver damage caused by backed-up toxic bile.

Typically it would make sense to judge a treatment’s effectiveness  not quite so objectively, but in this situation, I’ve only the numbers to rely on.

Do I feel better than I did in December 2006 when I started on the Urso Forte (my favorite brand name for a drug, roughly translatable, or so I claim, as Strength of the Bears)?

Not dramatically. The only symptoms of PBC before the liver is damaged significantly are fatigue and itching, and I never experienced the itching and who among us doesn’t complain of fatigue?

This week the only bad marks on my blood tests were for ones I’ve scored poorly on for at least four years: low white blood cell count and low platelet counts. In the past, these scores were considered not bad enough for alarm, just an anomaly, perhaps.

So I was surprised to be told I should have an upper GI endoscopy to detect whether large varices, or swollen veins (like varicose veins), caused by portal hypertension (itself caused by cirrhosis) are in my esophagus. Left untreated these could “burst and bleed into the gut.

But I gather that an association has recently been made, or at least suggested, between portal hypertension and low white blood cell count (leucopenia), and/or low platelet count (thrombocytopenia). Moreover, in 2006, on the long and wearying road to the PBC diagnosis, an ultrasound showed a very much enlarged spleen, and hypersplenism is another red flag.

My first impulse was to say no to this test based on a strong and irrational desire not to submit to another test, a resistance that I couldn’t explain and thus ignored.

Afterwards, after I started processing this new information, that impulse seemed less irrational: if I do have varicose veins in my esophagus, it probably means that I can no longer tell myself that my mostly normal blood test numbers  mean the PBC was diagnosed  and treatment begun in time to escape cirrhosis, at least for a while. It will mean that  my liver is already dying.

Or so it seems to me. Surprises during doctor appointments frustrate me: I need time to think to know what questions to ask.

 

Resources for the curious or those in a similar fix:

A simple intro to PBC is at PBC Foundation

A much more thorough one is at MedicineNet.com

The “bad luck” link in the first sentence of this post refers to the rarity of the disease. In “Food for Thought about Primary Biliary Cirrhosis in 2006,” an essay on the website of the UC-Davis Immunology Lab, there’s this statement:

PBC is thought to be more commonly found in specific areas of the world. In particular, disease frequency varies between 10 and 400 cases per million population. It has been suggested that PBC is more common in northern countries, including England, Sweden, and Northern American states.

MedicineNet says:

Studies indicate that the number of people with PBC at a given time (referred to as the prevalence of disease) ranges from 19 to 251 per million population in various countries.