Stage 4 PBC

[Suzie38]

This question has been asked before, but I'm looking for people who are diagnosed with stage 4 (cirrhosis) or people who can tell how long this stage can remain stable without the need of transplantation.

[Karaliz]

Hi Suzie. I've had cirrhosis for at least 5 years and the damage is widespread - perfectly obvious on ultrasounds and MRI reports which state -" multiple hepatic lesions in keeping with advanced PBC".

The question you ask seems difficult to answer - as long as cirrhosis is "compensated ", one can potentially live for a long time without a transplant. However, once cirrhosis becomes "decompensated", I believe one is on a downward spiral towards eventual transplant.

For those of us with cirrhosis, the picture is more complicated than for those without. I have never found the mantra -" likely to die with PBC than of it " very useful... I think we all know that ! The challenge - for me at least - is how can I live my very best life while dealing with the serious, exhausting ramifications of a chronic liver disease ? To live, work, look after my family and contribute to our world in a meaningful way is what I and others endeavour to do daily - I try not to dwell on the inevitable but hope for the best.

I don't believe my cirrhotic liver will last forever but it's already lasted longer than the medical team predicted so who knows - it's a wild ride !

We only live once but if we do it right, once is enough. ( Mae West)

Karaliz from Downunder

[GaelOB in reply to Karaliz]

As a fellow Stage 4er since 2015, your words lift my heart! Each new day always brings a special treat, be it a laugh, a call from a friend, a chance to help. It is a wild ride, in so many ways. And to know that others are sharing that ride, and holding on tight, seeking the adventure and not the fear is, well, inspirational. Thank you.

[Karaliz in reply to GaelOB]

Thank you Gael - your words meant a great deal to me. My 19 year old daughter gave me book of Mary Oliver's poetry which includes one of my favourite passages that I strive to live by - "What are you going to do with your one wild and precious life?"

Karen xx

[Pheebe in reply to GaelOB]

Are you still doing well ?

[Suzie38 in reply to Karaliz]

Hi Karaliz, thank you very much for your uplifting response! May I ask how old you are and how long it's been since you were diagnosed?

[Karaliz in reply to Suzie38]

Hi Suzie. Thank you for your lovely reply. I was diagnosed with PBC 14 years ago when I was 38 and the youngest of my 4 children was only 5 - I remember saying to the GP -" How can I be diagnosed with a disease I've never heard of?".

I was so busy working as a nurse and raising my family, I almost forgot I had PBC although only partially responded to Urso.

I was recruited into the OCA trial 5 years ago when I became very unwell and my disease process had advanced to cirrhosis. The OCA ( still not available here in Australia) has certainly slowed down the rate of decline which I'm very grateful for.

I work as a nurse/case manager in the community 3 days a week, look after my large family ( I have a wonderfully supportive husband) and hope for the best.

How are you feeling Susie ?

Karen xx

[gwillistexas in reply to Karaliz]

I am glad to read your positive response to ocaliva. I haven on it since October. I have so far responded well. I hope you continue to stabilize.

[Suzie38 in reply to Karaliz]

Hi Karaliz, so good to hear that you are responding to OCA and that you got the option to participate in the trial! I really hope that you remain stable.

I'm 66 and diagnosed last fall with cirrhosis due to PBC. I was seeing my GP for an UTI and after he said that my liver was enlarged, I've been reffered to a hepatologist for an US, fibroscan and blood tests and that's how I got diagnosed. I'm not suffering from a lot of symptoms, only feeling extremely fatigued sometimes. My specialist told me the same thing as yours: it's not likely that my liver will go on for ever in this stage, but how long exactly is difficult to predict. Because I'm already 66 I'm sometimes quite worried about the future when it comes to transplantation. The scientifical papers about PBC aren't so optimistic when cirrhosis is already established so I recognize what you are saing about the quote 'likely to die with PBC then of it'

How are you feeling at moment? It sounds that you have a very busy life and that for a long time together with PBC!

[Karaliz in reply to Suzie38]

Hi Suzie - thank you. Yes I'm extremely fortunate to have access to OCA and while it can't undo the damage already wrought, it has definitely slowed down the speed at which my liver damage was progressing.

Don't lose heart about your age Suzie - there are all sorts of things to consider with transplantation and a person's age is only one aspect. Have you talked with your specialist about this concern specifically? You must have received a dreadful shock to be informed of your stage at the time of diagnosis - I think you are doing very well tackling such news so well.

Like you, the fatigue is my constant companion and the most challenging symptom for me to deal with. Otherwise I chug on and really do love and appreciate my many blessings.

Great to chat and I'm here ( opposite time zone !) anytime.

Karen x

[Pheebe in reply to Karaliz]

Are you still doing well

[ILik3Pizza]

I was diagnosed in the fall of 2016 at stage 4. After picking myself up off the floor, I got on with it. Daily life is not greatly impacted and sometimes I even forget I have this disease — until I eat something fatty or have a glass of wine! There are so many other ailments that would be more difficult to cope with — I’m glad it’s only PBC! With my advanced age (68), a transplant won’t be an option. In the words of our Shannon: “Stay Strong!”

[Pheebe in reply to ILik3Pizza]

How are you doing now ? I did not know there was an age cut off for transplants. Is that still the case ?

[ILik3Pizza in reply to Pheebe]

Last time I spoke with my doctor she told me there isn’t a cut off for transplants anymore (I’m nearing 70!). She also told me that I wouldn’t need a whole liver, that I would be able to re-generate a whole liver with a one lobe donation. However, because PBC is autoimmune, the donated liver would also develop PBC. My PBC was stage 4 but I now understand that my stage of cirrhosis is different — that is “only” about a 2.5! I believe de-compensation starts to show at 3 and end-stage liver disease is 4. But as I said, I usually forget I have this disease. I bruise more easily and they last longer, I get tired more easily and fatty food really does a number on me. Overall though, life is good! Life is so much better when we can turn our focus off of ourselves and seek to serve others!

[LeeKitty]

I was diagnosed in November 2017. My liver biopsy shows stage 4. Urso has brought my blood tests into all normal range. I have had other procedures. No ascities. No vericies. No fatigue. No itching. No pain. No nausea. Does this happen?

[Suzie38 in reply to LeeKitty]

What does your doctor say about it? And do you have to go for check-ups? Nice to hear that you're doing so well with the using urso!

[LeeKitty]

He quietly dropped the stage 4 bomb after we talked about all the good results. He said that in spite of the cirrhosis my liver is functioning well. Ultra sounds and blood work every 4 months. I don’t need to see him for a year unless I get symptoms or something shows up on the tests. I am so blessed and thankful that I feel great. It is like I am waiting for the next big shoe to drop. I guess it will take time to get used to it. I appreciate what Karaliz wrote above about compensated and decompensated. That made sense. Thanks for writing back.

[Suzie38 in reply to LeeKitty]

This may also be a useful post: healthunlocked.com/pbc-foun...

[Suzie38]

What I have understand from others on this forum is that the cirrhotic stage can last for years and remain stable, especially when it is compensated, but i can understand that is difficult to accept... Nice to hear that you have a good doctor who will keep an eye on you!