“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.
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.