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.

Continue reading “Third Time Around: PBC, Portal Hypertension, and a Routine Bleed”


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.

Two Novels and Two Liver Transplants: Peter James’ Dead Tomorrow and Abraham Verghese’s Cutting for Stone

I’ve posted several times about primary biliary cirrhosis, a condition for which the only certain cure is a liver transplant, and recently by chance listened to two audio novels in which liver transplants function as plot devices. How weird is that? In all other ways, Peter James’ Dead Tomorrow and Abraham Verghese’s Cutting for Stone couldn’t be more different.

Continue reading “Two Novels and Two Liver Transplants: Peter James’ Dead Tomorrow and Abraham Verghese’s Cutting for Stone

PBC Re-cap by Comment

I like most things about the WordPress template I use for this blog, but if you read posts on the home page and don’t click through to the page for the individual post, you’ll miss the comments.

And I had such a thorough response to one of my posts, PBC: No Varices for Me. Well, Just a Trace, that I didn’t want you to miss it. You can read all of it here, but I want to respond to some of dsars’ remarks because I think her comment sums up a lot of the points I’ve tried to make  in this series of posts.

Missed Diagnosis:

I can’t stress enough how much we need to doublecheck our own lab reports and to question our doctors. I told you how in 2006, the year I was diagnosed, I went back and got hold  of  as many lab reports as I could from the past  and found that  in April 2001 my alkaline phosphatase was through the roof. Here is dsar’s story:

I, too, could have been properly diagnosed years before I was actually diagnosed. I went to see a dermatologist for darkening patches of skin and itching. I specifically mentioned to the doctor that I realize that some skin conditions are indicative of a more serious disease, and that I wanted to make sure I wasn’t going to die of something. I was diagnosed with having hypo and hyper pigmentation (I lost some pigmentation from scratching so much), and was prescribed an old anti-depressant (pre-SSRIs) that has a side effect of having anti-histamine qualities. The doctor ordered blood tests, as the medication is processed by the liver…and she didn’t even mention whether there were abnormal numbers in the lab results. Years later I went to my family doctor for jaundice.

The Connotations of Cirrhosis:

Seems it’s not just me who’d rather not tell people what I have if it means suffering their assumptions:

 Also, the potential social stigma of having the word “cirrhosis” in the name of the disease and the likelihood of people to associate it with alcohol or drug issues has kept me from sharing with some friends/co-workers about this disease.

Here’s a modest proposal. We can keep PBC; we know what it means. We can tell the rest of the world we have Primary Biliary Condition, that is, PBC. If they are sincerely interested and search for “primary biliary condition,” they are going to reach “primary biliary cirrhosis,” and then the objective authorities can explain to them about the cirrhosis bit.

All of a Sudden, Varices:

Commenter dsars, whose biopsy places her at stage 4, writes:

I’m seeing my second Gastroenterologist, as I recently switched insurances. Having been treated for PBC for about 2 years, endoscopy and varices was never explored with me. All of the sudden, this new doctor is telling me about this big bloody blow up mess, gives me the literature on the procedure, sends me off on my merry way and goes on vacation.

Frankly I don’t understand why as soon as her biopsy indicated stage 4 dsars wasn’t sent for an endoscopy since the varices come, if  they do, with cirrhosis, and the “big bloody blow up mess” is not a surprise you want.

Get Critical About Your Care:

I’m grateful to dsars for having written since it is good to know the work I’ve put into these articles has been of some small use. She concludes:

Reading your blog also made me realize that perhaps I need to connect with some of the support systems available via the internet (I’m willing to bet that nobody else has the same diagnosis in the itty bitty Northern California town I live in).

Yes, dsars, you should visit And there maybe you can find what hepatologists others in your area are using.

I’m willing to bet that you are right: in your “itty bitty” town you are the only pbc-er. And you know what that means? You are your gastroenterologist’s only pbc-er.

I fear you could be hours and hours from San Francisco, Sacramento or Portand. I’m lucky enough to be just a 100 miles from a major university medical center. I was ready to yell at you:  get a hepatologist — but now I’ve stopped myself.  If she could, she would.  I wish I could help.

PBC: No Varices for Me. Well, Just a Trace.

As I noted in my first post on primary biliary cirrhosis, during my annual routine appointment I was advised to have an endoscopy procedure to determine whether I had varices in my esophagus as a result of portal hypertension as a result of cirrhosis as a result of the autoimmune destruction of my bile ducts, that is, primary biliary cirrhosis.

I was surprised by this recommendation, since my blood enzyme levels had normalized and in 2007 an MRI of my liver did not reveal damage, but things can change, and the extent of damage my liver has suffered is unknown. There are four stages of PBC, with cirrhosis being stage four, but my case had not been staged, as they put it, because I have never had a liver biopsy.

When I first visited the hepatologist, we discussed whether a biopsy was warranted. He had enough other information to feel confident about the diagnosis, and the treatment he would start me on would be the same regardless of stage. So we concluded that while the biopsy would provide more information, it wouldn’t necessarily be useful enough information to warrant the hassle.’s comprehensive article on PBC notes that whether a biopsy is optional

usually depends on the level of confidence in establishing the diagnosis of PBC using the liver tests, autoantibodies, and ultrasound. In the presence of cholestatic liver tests, high levels of AMA, and an ultrasound showing no bile duct obstruction in a middle-aged woman, the diagnosis of PBC can be made rather confidently without a biopsy.

Even had I had a biopsy three years ago when I was diagnosed, there still would have been only one way to know whether or not it was time to start worrying about varices. And that was to look.

Before we began, the doctor told me that there were four levels of varices. Levels 3 and 4 require treatment, starting with medication.

I asked whether even if I did not now have varices, was it not the case that sooner or later I would?

He quickly responded yes, then caught himself and backtracked, with the typical doctor’s abhorrence of an unqualified answer, noting that 60% to 80% of people with cirrhosis have varices, and in 1 of 6 cases, within a year’s time, if left untreated, the varices would burst and bleed into the esophagus.

The worst part of the endoscopy — really the only bad bit — had nothing to do with the endoscopy itself. I knew I was in for it when the tech responsible for starting the IV began complaining that I had no veins, and sure enough, the IV entry was and remained painful and I have the bruise to prove it.

A not-as-nasty as I was warned to expect spray was squirted in the back of my throat, Demerol was added to the IV port (there was no other use of it in my case), and that was that, until I woke up.

I had no sore throat and began eating and drinking immediately, although I’d nearly fall asleep between bites of cracker.

And the verdict was just a trace of varices, no need to do anything, just repeat in a year or two.

You Give Your Blood, You Pay the Bill, So Get the Results!

Do you know why I could report my lab scores in my last post? Simple: I tell my doctors I want a print out of my bloodwork results. For years I never considered doing this, but at some point I got fed up with throwing out my arm for yet another needle, getting the bill for the copay –and having nothing concrete to show for it.

Moreover, I felt that going from doctor to doctor there was a risk of no continuity, no history, no comparisons, no full use being made of the data available. And there were some oddities I felt needed investigating and explaining.

I’ve had conscientious doctors and I have had blunderers. And I’ve finally recognized the obvious (well, when it comes to adults, anyway):

No matter how good your doctor is, no one, and I mean no one, cares about your health as much as you do.

So get your results and hold onto them. It would be nice to have a Permanent Record, like the legendary thing that people my age always heard about in school (better not do x,y, and z: it will go on your Permanent Record), with all your health info, all that data collected at great expense, readily available, but there is no such thing. The best you can do is get hold of the few scraps the medical system is willing to give you — or to let you purchase.

You may not understand all of it (I sure don’t), but you can learn a lot on the net. The big thing though is to note any Hs [Highs] or Ls [Lows] or highlighted or checked lines, look at the normal range, and if your numbers are way off,  ask your doctor, what do these scores mean? If she says it is something to watch, then you watch it too.

This is what can happen if you don’t:

During my PBC diagnosis odyssey (see last post), I remembered some weirdness that went on when I was hospitalized with pneumonia five years previously in spring 2001. Gall bladder tests were ordered — for pneumonia? I never got an explanation and was too sick at the time to pursue it with either the family doctor who admitted me or the pulmonologist who took over. I had follow-up visits with both after my release, and neither discussed the tests. So I mentally filed it under weirdness. My family doctor quit practicing within the next year or two.

In 2006 I  decided it was time for a closer look. I went to the patient records archives at the hospital I’d been in, and I paid for records of my visit.

This is what I learned: Way back in April 2001, when I had pneumonia,  my alkaline phosphatase (ALP) reading was 445 (norm 39-118). Remember, elevated ALP is one of the, if not the, biggest red flags for PBC. What the doctors I saw in the hospital did was order an ultrasound and CT scan to check out the biliary tract and a HIDA (Hepatobiliary Imino-Diacetic Acid) scan to evaluate the gallbladder. The doctor’s notes for my last day in the hospital allude to waiting on HIDA results and considering an outpatient bone scan, but I never heard anything about these results or further studies.

These wouldn’t have diagnosed the PBC, but that’s not the point: when tests don’t show what the doctor is looking for, then those results should  prompt further investigations. Good doctors don’t just shrug their shoulders and forget the problem that caused them to order tests in the first place.

Moreover, I was never told my alkaline phosphatase levels were out of whack.

Five years would pass before I first heard or saw the words alkaline phosphatase.

What does this mean:

It means I could have been diagnosed with PBC — I could have started treatment –– in spring 2001 rather than winter 2007.

I like to think if I had left the hospital with copies of my labs, rather than returning there 5 years later to get them, I would have asked the right questions: What is this Alk Phos total? Why is it so abnormal?, and that I would have pestered someone until I got some answers.

By the way, the charges for the diagnostic tests no one followed up on: Nuclear Med Diagnostic: $891.25. Ultrasound: $189.25. Nuclear Med: $120.75, CT scan: $1,169.75 = $2371.00. Complete waste.

High ALP + High GGT + High IgM + AMA = PBC

So how did I end up getting diagnosed with primary biliary cirrhosis (PBC) three years ago? It wasn’t easy, believe me.

Stop 1: Family doctor, July 2006

My complaint was that I was more tired than usual. The previous year I had had iron deficiency anemia due to female problems 😦 leading to a D&C. So anemia was what I expected as this year’s verdict as well.

Wrong. My family doctor, Dr. Cindy McAdams, paid close attention to my lab work (in another post I will explain why I think that worth mentioning. Suffice it to say, I have had doctors who haven’t).

My serum alkaline phosphatase (alk phos or ALP) was 410 (normal is 25-153), AST 74 and ALT 91 (0-40 norm).


These are liver enzymes. Abnormal  AST and ALT are linked to a number of conditions and frequently occur in connection with the use or misuse of scores of medications. A mild to moderate elevstion, like I had, is seen in PBC, but is not a crucial bit of information.

Markedly elevated alkaline phosphatase is, and a score of 410 when 153 is top normal is not good. From MedicineNet:

Abnormally high blood levels of alkaline phosphatase may indicate disease in bone or liver, bile duct obstruction, or certain malignancies. The enzyme is often elevated in the leukemic cells in chronic myelogenous leukemia.

A repeat of the bloodwork with additional tests added yielded more bad marks, including a 68 Westergren sedimentation rate (upper norm of 20) and low platelets and white blood count, and I was packed off to a haematologist (specialist in blood disorders).

Stop 2: Hematologist, August 2006

This stop proved useful to rule some things out. The hematologist started with an ultrasound, and that revealed a much enlarged spleen — and enlarged lymph nodes. Things had taken a grim turn indeed, and a CAT scan was ordered to establish whether there was activity in these enlarged lymph nodes. Now I am not a medico, but I know what activity in the lymph nodes could have indicated: cancer.

I was lucky, however, and not sorry to be sent along from that office; the hematology and oncology units were housed together, and it was a place I didn’t want to belong.

I did leave with some new findings:

  • Markedly elevated GGT (585 when normal is 7-32). GGT stands for gamma-glutamyl transferase. High ALP with normal GGT means that the trouble is a bone disease; high ALP and high GGT narrow it down to bile ducts or liver.
  • Immunoglobulin IgM was 810 (norm 40-230). This is what I found out: “Increased serum immunoglobulin concentrations occur due to polyclonal or oligoclonal immunoglobulin proliferation in hepatic disease (hepatitis, liver cirrhosis), connective tissue diseases…”

Stop 3: Rheumatologist, September 2006

I had enough findings to make lupus or rheumatoid arthritis a possible if not exclusive diagnosis, and so I visited the rheumatologist and left behind who knows how many vials of my blood. My ALP was now 481, and other bad marks were added to my lengthening list:

  • Actin (Smooth Muscle) Antibody of 115 units when norm is 0-19. Enter a new possible diagnosis: autoimmune hepatitis.
  • Presence of antimitochondrial antibodies (AMA), and “between 95 and 98% of patients with primary biliary cirrhosis (PBC) have autoantibodies (antibodies to self) in their blood that react with the inner lining of mitochondria.”

Stops 4 & 5: Gastroenterologists, October to November 2006

At this point I should have seen a hepatologist, a specialist in liver diseases, but since there isn’t one locally, a gastroenterologist was the fallback.

Two stops here because the first GI I visited ordered his own bloodwork, gave me an appointment date two weeks later, and when I arrived, he was not. Yes, I know emergencies happen. But when his staff offered to reschedule me in the new year, his first available appointment since he would be out of the country in December, I said no way, just give me my records and I’ll find someone else. They told me to hold on a minute, and came back with the news that another doctor in the practice would see me in an hour.

His approach was to pursue the possibility of autoimmune hepatitis (AIH). We were pretty much down to two choices: that or PBC. And 7 of 10 people with AIH go into remission after 3 years’ treatment. People with PBC don’t.

So the plan was to give me prednisone, and if I responded to this steroid, it was likely I had AIH. If not, then we were left with PBC.

So I took prednisone for 6 weeks.

Maybe you’ve seen it in old British movies: back when that country still had capital punishment, if the judge was going to announce a sentence of hanging,  he entered the court he wearing something like a black piece of cloth atop his white wig. GI2 may as well have been wearing one of those when he came into the examining room. My scores were worse.

Then he did the best thing and the dumbest thing he could.

The best was that he set me up an appointment with a hepatologist at the University of Alabama in Birmingham Medical School. The dumbest was he simply stopped the prednisone.

When you’ve taken 40 mg of prednisone daily for 6 weeks, it is not a good idea to go cold turkey, to abruptly entirely withdraw it. I spent the next month practicing being dead.

Stop 6: Hepatologist, December 2006

I first visited the hepatologist the week before Christmas. All the puzzle pieces were ready to put into place:

An elevation of the aminotransferases alanine aminotransferase (ALT) and aspartate aminotransferase (AST) may be identified in most patients with primary biliary cirrhosis, but significant elevations of the alkaline phosphatase (ALP), g -glutamyl transpeptidase (GGTP), and immunoglobulin levels (mainly immunoglobulin M [IgM]) are usually the most prominent findings….AMAs can be found in 90-95% of patients with primary biliary cirrhosis, and they have a specificity of 98% for this disease.

  • Elevated ALP √
  • Elevated GGT √
  • Elevated IgM √
  • Elevated ALT √
  • Elevated AST √
  • Presence of AMA √
  • The hepatologist started me on 1000 mg of Urso Forte a day.

    January 2007

    A month later my ALP, which at its highest was 481, had returned to the upper limit of normal (on this lab’s scale [they vary]): 117.

    November 2009

    My ALP is still 117. Just how meaningful this is remains to be seen.