Thursday, March 29, 2012

Not the best news but could be worse I guess

It could always be worse...I just gotta remember that.

So I found out yesterday that I am in Stage 2 of Primary Biliary Cirrhosis...Ugh.  There are 4 stages, I was hoping for stage 1 (Or Zero- That would mean no damage at all yet, but I knew that'd be asking for too much...).


  • Stage one: Inflammation and/or abnormal connective tissue confined to the portal areas
  • Stage two: Inflammation and/or fibrosis confined to portal and periportal areas
  • Stage three: Bridging fibrosis
  • Stage four: Cirrhosis
{Warning: Medical jargon coming!}
Histologic stage — PBC is classified histologically into four stages.
As noted above, the natural history of PBC involves histologic progression along these stages, although treatment with UDCA may slow disease progression.

{Pre-UDCA [The Medicine I take]}
In a study involving 916 biopsy specimens from 222 patients followed in the pre-UDCA era, cirrhosis developed within four years in 31 and 50 percent who presented initially with stage I or II disease, respectively
The presence of cirrhosis (stage IV), is associated with a worse prognosis and identifies a group of patients at risk for development of complications related to cirrhosis including variceal bleeding and development of hepatocellular carcinoma. In a study of 256 patients seen in the pre-UDCA era, 31 percent developed esophageal varices during a median of 5.6 years of follow-up. One- and three-year survival rates were 83 and 59 percent, respectively, after the development of varices.

{With UDCA}
In another study, the presence of ductopenia {"refers to the associated reduction in the number of intrahepatic bile ducts, a process that ultimately leads to cholestasis"[bile not flowing and destroying liver]}on a baseline liver biopsy predicted histologic progression despite UDCA


In a trial of 180 patients at one medical center, UDCA led to a significant decrease in the plasma levels of aminotransferases, alkaline phosphatase, and bilirubin. Although there were also fewer deaths in the treatment arm, there was no improvement in fatigue, pruritus, liver histology, or referral for liver transplantation. In a follow-up report in which treatment had been continued for a total of three years, UDCA was associated with a significant reduction in the risk of death and need for transplantation

Similar findings were noted in a multicenter Canadian study of 222 patients. The active therapy group had lower plasma levels of the aminotransferases, alkaline phosphatase, bilirubin, IgM, and cholesterol. Fatigue, pruritus, and the rates of liver transplantation and death were unchanged. There was, however, a beneficial effect on liver histology as manifested by decreased progression of periportal hepatocellular ballooning and bile duct loss.

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{Random interesting tidbit I found.  Sucks it's still coming back to bite me after 4 years quit!}

Cigarette smoking — An association between PBC and cigarette smoking has been suggested in epidemiologic studies. At least two studies also suggested that cigarette smoking is associated with more advanced fibrosis stage. In one of the studies, never-smokers were significantly less likely to have advanced fibrosis (METAVIR fibrosis score of 3 or 4) than patients who had smoked in the past or were current smokers (16 versus 33 percent). For each pack-year increase in smoking, there was a 5 percent increase in the likelihood of advanced fibrosis.

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So Anyway, what now?  Now I get more tests! Yay! I have to get blood work drawn in 2 weeks, 3 months and 6 months.  Then see the doctor again.  Hopefully we'll see a downward trend of the numbers and can assume the meds (and diet changes) are helping.  Depending on how it's going, at some point I'll get another liver biopsy to see what stage I'm in then. 

My hope is that the meds slow down the progression and my diet changes help to start sealing my gut and EVENTUALLY (I'm talking several years here), I MIGHT see some reversal of this.  And then after even more time, maybe I'll actually beat this disease.  I mean I know it's supposedly 'incurable', but I have already met people that have cured their Auto Immune disease and read about lots more by doing what I'm doing.  So my hopes are high. 

I'm just going to stay on top of this and see where it goes.


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Now I also know to stress even more to Zoe to not smoke (which of course I would already, but here's even another reason) and to eat right.  I really don't want my little girl to get this ("familial clustering of autoimmunity has long been recognized"  "Inheriting certain genes can make it more likely to get an autoimmune disease. But a combination of genes and other factors may trigger the disease to start"). And it's not just PBC I have to worry about- "Children {and anyone} with one autoimmune disease tend to run a higher risk of developing another"- Which is what has happened to me- I got lichen sclerosis first (AI disease) and now PBC...What next??


Heredity: A child inherits certain genes from her parents that make her susceptible to a particular disease.

  • Environmental factors: The disease doesn’t actually reveal itself until it’s “triggered” by something.
  • Hormonal factors: Given that many autoimmune diseases tend to affect adolescent girls and young women, the presence or amount of certain naturally occurring hormones in the body may also play a role in when these illnesses come to the fore.

  • So this is how it could have happened with me:
    I am predisposed to get an AI; I eat Gluten all my life; I cause holes in the gut and gluten travels around my body; My immune system attacks the Gluten; Then I get my Sphincter of Oddi Dysfunction attacks; That 'triggered' my immune system some how to get confused and start attacking my body giving me LS and PBC

    [" When the immune system attacks the gluten protein it can confuse other bodily proteins for gluten due to their similar molecular structure. The term used to describe this phenomenon is “cross-reactivity”.  This cross reactivity is the cause of the immune system attacking “self”. It believes it’s attacking a toxin, in this case gluten. In some cases the trigger to attack self can be an infection"]

    Of course, there's no way to know if I've had the PBC before the LS and just didn't know about it - the LS just makes itself KNOWN!  Maybe it was the time I tried the Depo Shots that triggered it, or the hormonal changes from quitting smoking or being pregnant, or the stress of a kindey stone or gall stone, or that really high fever I had that one time- Just making a point, that there's really no way to determine the 'trigger', could be anything, which is why it's so important for me to try to prevent the leaky gut in the first place for Zoe!


    Ugh.  Just sucks to know I have probably passed the genes on to Zoe to develop AI disease(s). 

    Welcome to the world! You're beautiful! Smart! Funny! And, oh yea, here's a little something extra...

    Great job mommy!


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    Sources:
    UpToDate.com Unfortuantely, my trial is almost over! :(
    ChildrensHospital.org
    WomensHealth.gov
    ncbi.nlm.nih.gov


    1 comment:

    1. I'm sorry Tammi, that is a total bummer. But it sounds like you are, as always, proactive and on top of the problem. I promise to help you out by having plenty of gluten-free snacks for every playdate!

      ReplyDelete