I read with interest the previous thread with a similar title and so I decided to start a separate thread. I have much to say on this topic that might be helpful to patients, new and old, and for it to be lost in the body of another thread might limit its reach.
Where to begin...
I have lived knowingly with PBC in my life for 24 years. My mother was diagnosed with PBC then. I have lived unknowingly with PBC for many many years: more than just the 24 as she had symptoms long before she was ever diagnosed.
24 years on, she is still here alive and well, 62 years old and still kicking my erm, well... moving on...
In my time since then I have met well over 2 thousand patients in many countries around the world, including on three continents. I have read more studies than I can name and have witnessed more harm done by inaccurate information on the web than I can bear to see.
I have read that people with PBC can expect to live 5, 7, 7.5, 10, 15 and 16 years from diagnosis. I am sure all of these studies were well intended. I am sure many of them might have been accurate to the best of their ability (with a limited snapshot of what PBC is, etc) albeit very much within their limitations.
Reading these studies breaks my heart because they are founded upon really limited information. They create fear, confusion, and unnecessary upset. That said, if you really are going to google "PBC prognosis", what on Earth do you expect to find?
I've spoken with people believing this disinformation and were looking to put their affairs in order before they died (which they didn't!!). I've spoken with people cancelling holidays because they didn't expect to live long enough to go. Even now, we have people convinced they are going to die too early because of their PBC because of the stuff they find on the interweb.
I am looking forward to the day these people realise they will live longer than they thought. I just hope they realise soon enough not to waste a single moment more on unnecessary worry.
In our time at the Foundation, the oldest member (that we know about) passed on at 103, and not because of her PBC. My personal record is a patient who was 88 (and 3/4 she reminded me!!) and PBC wasn't one of her issues. I have met patients who have been diagnosed 10, 15, 20, 25, 30 and even 38 years: all very much alive when I met them.
Before we deal with the facts, let's deal with the nonsense.
You have X years from diagnosis.
When were you diagnosed? Were you diagnosed when you were yellow with Jaundice? Were you diagnosed early by accidental pick-up of raised liver tests? If you were diagnosed many years ago, the chances were the former. If diagnosed more recently, the chances are the latter is closer to your experience. And there are thousands of patients somewhere in between.
In the olden days, when PBC stood for Primary Biliary Cirrhsosis, it was called that because patients were cirrhotic before they were diagnosed. They would probably have been jaundiced and would have had many years of PBC without them (or their doctors) knowing it. So, in those days, an untreated cirrhotic patient would have had a limited prognosis.
Also, many of these studies were published when PBC still wasn't treated effectively.
You were diagnosed at Y point in your PBC Journey.
So, how long did you have PBC before you were diagnosed? Weeks? Months? Years? Decades?? Maybe you've had symptoms for years that have only just led to diagnosis. Maybe you were diagnosed at the very start of your abnormal liver tests. We will never know. Sure, we can tell early PBC from progressed PBC but that still doesn't give us a start date: day zero.
If you don't know, what chance does your doctor have of knowing this? If your doctor doesn't know this, then what chance does a random researcher crunching some numbers on their laptop going to have?
The answer is none.
I have Stage X PBC so I know my prognosis.
No, you don't. You really don't. You may have stage X histology (cell change within the liver) but you do not have stage X PBC. There can be early disease and later stage disease but these are not the same.
Even with a Stage X histological finding, that number doesn't mean a huge amount in terms of prognosis. I have met patients who have been stage 4 for years (over 15 years) without progression.
I could go on and on, but shall stop here as this post is already long enough and we haven't even reached the facts yet...
Early diagnosis + Urso response = normal life expectancy
That is quite a bold statement. However, it would appear to be true. Of course, there are anomalies, exceptions and the like... However, what is coming through the data is that people who have an early diagnosis and who respond to Urso (UDCA) are living to normal life expectancy ages, equalling an age/sex matched population.
There isn't yet a scientific definition of "early diagnosis" but we know the difference between early and late PBC.
This leaves us the section of the population who do not respond to Urso. (this could be because they don't respond, because they don't tolerate, or because they haven't been given access to Urso in the first place). What happens to them?
Well, in many places around the world there is a second-line therapy available which has been deemed safe (when used appropriately) and effective in normalising or lowering liver tests. We won't know what that does over 20 years until we have been using it for 20 years, but the anticipation is that it will also prolong life expectancy.
Some places have been using fibrates, with different levels of success. Again, the long term effect will not be known until we get long-term data but knowing how successful Urso is, doctors believe that this will also have a positive impact.
Even if those therapies fail the patient, ultimately there is the possibility of liver transplant for many. Remember that these studies speak of progressing to death or transplantation.
Then we have a whole raft of new potential therapies going through the process of becoming a licensed therapy, many of which will be here in the next few years.
So, even for someone diagnosed today with PBC, we have no idea how long their prognosis is. We know it is improving. We know the majority of PBC patients respond to Urso and can have a normal life expectancy.
Looking at the old studies, there are other factors that come into play: nationality, for example. Access to medicines, transplant, insurance, etc all come into the picture.
My last point is possibly the most serious one I want to make in this post.
Where are you getting your information?
The PBC Foundation has worked hard, really really hard, to partner the best PBC minds around the globe. We reach patients in 78 countries and our information can be found in 18 languages. We have apps, magazines, an 70+ page compendium, webinars, workshops, abstracts, etc giving accurate, current and free information.
Join us. Read our information. We won't be publishing any such nonsense. We don't fill our community members full of fear (nor of false hope). Everything we publish is peer-reviewed by world-eminent multiple-time published renowned PBC experts. We do not have Dr Google on our Medical Advisory Board, and for good reason.
There is absolutely no reason why you wouldn't want to have free access to some of the best PBC information available. None. So join us: it costs you a few moments of your time. Get yourself the information you need: not just today, or tomorrow, or 5, 7, 7.5, 10, 15 or even 16 years away... but for the rest of your long lives.
We can't tell you where to go for your information. We can only support you when you get misinformation and help you unlearn what you have learned. We can't tell you who to ask or what to ask. We can only give you the best information available.
We are here to help, to serve and to improve the community affected by PBC. Use us: our knowledge, experience, expertise, access to the finest. That's all we ask.
Yours, as ever,