Advice please - PBC stages and healing after surgery

[jayneypickles]

Newly diagnosed with PBC in July 2016 after itching for almost 12mths. Just had the results from my biopsy that say I am stage 3 of 6 (my understanding is there were 4?!) with definite fibrosis and significant inflammation, indicative of the disease being aggressive which they do see in younger people (I'm 36) my consultant is going to leave me on urso until my next appointment in December then potentially looking at introducing a steroid as in some cases this can help to address the inflammation. Does anyone have any experience of this?

On another note I had a septoplasty in February 2016 that has not gone well, and still not healed as well as giving me a significant hole in my septum which wasn't there before. Anyway to cut a long story short I need a full nose job to put this right with cartlidge from my ear and skin grafted from somewhere else. I've seen the ENT consultant today and he says without a doubt I need the op to put it right, but my PBC means that he is unable to operate as he feels it will leave me in a worse situation as my condition will mean it won't heal. My PBC consultant is happy for me to have the op provided I didn't have cirrhosis, which I don't, before bothering her again I just wanted to see if anyone had any experience in this.

To say I'm a bit confused and feel like one step forward three steps back is an understatement.

Thanks in advance Jayne X

[Karaliz]

Hi Janeypickles

From my experience you will be absolutely fine to have the operation, provided your clotting profile is normal. I have had PBC for 13 years ( diagnosed at 38) and progressed to stage 4 - cirrhosis 4 years ago. In that time I have had 3 operations - one a major op - and recovered well. I have been extremely fortunate to be under the care of the liver transplant team in Melbourne where I live and they have taken wonderful care of me. The medical teams certainly don't take the fact that I have cirrhosis lightly but the fact is, people who are far sicker with liver disease are operated on all the time. The only hitch I experienced post surgery was more bleeding from the wound than was expected but this was closely monitored and resolved.

Regarding the use of steroids and PBC, my consultant toyed with the idea briefly years ago when I stopped responding to Urso and liver was hugely inflamed but together we decided the side effects were not worth the short term benefit. He described the use of them ( in my case) as a band aid remedy.....where I would feel better initially but would be back to where we started the moment I came off them. The ramifications for bone health while using steroids is also an issue and as there would be no long term benefit, I was reluctant to take that path.

This is my experience but I hope you find it of some use Janey.

All the best

Karaliz

[Junolee]

Yes, I was on steroids for 18 months that brought the information down, after that they put me on imuran (low dosage) between that and Urso after 4 years my bloods are almost back to normal and liver functioning tests are just slightly raised, they are not at all concerned. Have had numerous ultra sounds and MRIs and no changes since diagnosis. All is good at the moment. I think the steroids might be a good idea for you to get you back on track. I had minor surgery last year which worked out fine.

[Karaliz in reply to Junolee]

Junolee, am I correct in remembering that you have AIH as well as PBC? I know steroids and then an immune suppressant such as Imuran are the treatments for AIH... As far as I know, steroids have shown to be of little benefit in treating PBC which makes it unusual in that nearly all other auto immune diseases respond well to steroids.

Karaliz

[Junolee]

Sure but they are worth a try to bring down the inflammation.

[Karaliz in reply to Junolee]

If that was the case then surely they would be used widely for PBC. The fact that the benefit is so short lived seems to be why Drs steer away from them in PBC. I understand the benefit in AIH and other auto immune diseases is another story altogether.

Cheers

[DoreenD]

Hi Thera are only 4 stages ofPBC

Stages of PBC Disease

Stage 1: Portal Stage

Normal sized triads; portal inflammation, subtle bile duct damage. Granulomas - nodules filled with a variety of inflammatory cells - are often detected in this stage.

Stage 2: Periportal Stage

Enlarged triads; periportal fibrosis and/or inflammation. Typically characterized by the finding of a proliferation of small bile ducts.

Stage 3: Septal Stage

Active and/or passive fibrous septae

Stage 4: Biliary Cirrhosis

Nodules present; garland or jigsaw pattern.

However, the entire liver is not always uniformly involved, and a single biopsy may demonstrate the feature of all four stages simultaneously. Thus both under- and over-estimation may occur. It is crucial to have a sufficient size of specimen to minimize error

All the best

Doreen Melbourne Australia