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Primary Sclerosing Cholangitis (PSC) - Online Support Group

Survival rates?


#1

Has anyone gotten any recent information concerning survival rates? Any silver linings to share? I am coming across a lot of information on the 'net, none of which seems to be detailed, reliable, or even accurate. For example....

Stat: "The median length of survival from diagnosis to death is approximately 12 years." Source: http://emedicine.medscape.com/article/187724-overview#aw2aab6b2b4 (no date) There's people on this forum that already debunk that...

Stat: "The survival rate [post liver transplant] for two or more years is about 80 percent, with a good quality of life after recovery." Source: http://www.liverfoundation.org/abouttheliver/info/psc/ (page updated September 2014) So, what happens after the transplant "shelf life" runs out?

The Mayo Clinic gives you this handy-dandy death prediction device....

http://www.mayoclinic.org/medical-professionals/model-end-stage-liver-disease/revised-natural-history-model-for-primary-sclerosing-chonalgitis

Probably the best I've found so far, but still leaves the transplant cliff-hanger:

"Studies have suggested that the median transplant-free survival of patients with PSC ranges from 9.3—18 years; however, in a large population-based study on the epidemiology and natural history of PSC that we recently performed in The Netherlands, a median transplant-free survival of 21.3 years was found. Diagnosis of PSC was commonly preceded by a period of up to 5 years when, in retrospect, symptoms or abnormal laboratory values could be attributed to the disease. The findings from this population-based review were recently published in Hepatology.

With the availability of noninvasive magnetic resonance cholangiography (MRC), which is now thought to be preferable to endoscopic retrograde cholangiog-raphy (ERC) in the diagnosis of PSC because of its much lower adverse effect profile, diagnosis is being made earlier.

As for the prognosis of patients who undergo liver transplantation for PSC, the 5-year survival rate is approximately 85%." Source: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3736784/ (Gastroenterol Hepatol (N Y). Jul 2013; 9(7): 453–465.)


#2

I am so sorry you progressed that far. How are you doing now? What treatment options are you involved in, and, are they helping? Are you looking at transplant, or have you already gone that route?

I realize the watch is ticking for all of us. What I'm trying to figure is whether or not this is a chronic issue that is lifelong management, or if it is, itself, the terminator. Are there 90+ year olds still managing?

I suppose in the long run, it doesn't necessarily matter, it's just getting your head around planning employment, finances, life, etc... Sorry, just frustrated at lack of answers. :) I really do appreciate your response!


#3

I have noticed the same thing about wildly different survival estimates. In any case, lots of people with psc live 25+ years without transplant.
I try to stay positive. I had routine blood test 15+ years ago showing high bilirubin levels. My psc was diagnosed 2 years ago. Still the same blood test values.

I believe treatment will be available in the next few years (e.g. treatment that works at least for some people).
Oral vancomycin, nor-udca, lum001, btt-1023, and few others are now in clinical trials.
Few days ago FDA designated another Orphan Drug for psc (DHA) I.e. it might potentially be treatment for psc if phase2&3 provide positive results.


#4

With respect to the differing survival ranges that you mention I have to agree with detmsh.. due to the increased availability and use of tests like MRCP's and more awareness of the condition, diagnosis is occurring much earlier and therefore, where previous studies indicated a median survival of 10 years without a liver transplant, the survival range is probably closer to 12-18 years without a liver transplant now. I now my bloods had been abnormal since 2009. I even had an ultrasound done at the time to look at my liver and nothing in particular was found. Five years on, MRCP's have confirmed changes in my bile ducts in line with PSC.

I, like many here, can completely relate to your relative's and your frustrations. We have probably All been there. All I can say to you is that for now stay positive and try not to dwell too much on the eventualities. No one knows for sure what is going to happen 10, 15, 20 years down the line. There may be a cure, there may be a transplant or there may not even be a need for a transplant... who knows. I don't mean bury your head in the sand and pretend everything is normal.. accept the diagnosis (assuming it is correct and for that I would hold tight until your meeting with the Med Coll. of WI) but do not let it take over your present.

Keeping one's life / finances etc. in order is a sensible thing to do for anyone at any stage in life... I agree, if you can get your head around planning these it may help you feel more at ease. Especially knowing that once PSC is confirmed, it is very difficult to get life insurance.

All the best in your hunt for answers. Do keep us posted on how your relative gets on with their new doctor!


#5

These are great questions…and I have a couple to add to the pile! My daughter was diagnosed about 18 months ago (at age 10) and, for about the past 10 months, has had normal liver enzymes and bilirubin. Does this mean the disease is still progressing towards “end stage” despite the apparent improvement in blood levels…or does the progress “pause” when enzymes normalize? Has anyone seen clear evidence of disease progression with themselves or loved ones without any accompanying abnormal liver enzymes? Thanks so much for helping with this!


#6

Good questions - I am going to ask at the next appointment. Here's the root of my inquiry: "Can PSC enter "remission"? What do improved blood results really indicate?"
eogray said:

These are great questions...and I have a couple to add to the pile! My daughter was diagnosed about 18 months ago (at age 10) and, for about the past 10 months, has had normal liver enzymes and bilirubin. Does this mean the disease is still progressing towards "end stage" despite the apparent improvement in blood levels...or does the progress "pause" when enzymes normalize? Has anyone seen clear evidence of disease progression with themselves or loved ones without any accompanying abnormal liver enzymes? Thanks so much for helping with this!

#7

I was diagnosed eighteen years ago, and remain symptom-free. Some people aren’t as fortunate and progress much faster, but stories like mine should offer hope to people who are newly diagnosed.


#8

Missourimedic, after 18 years and symptom-free-I wish all of us could say that!


#9

Missourimedic, are you taking anything or eating a specific diet? that is great!


#10

Came across the old thread but I figured I’d reply in case it helps someone stay positive.

I was diagnosed 23 years ago and have been symptom-free the whole time. I am definitely fortunate, but it’s not uncommon.


#11

Hi Annoninusa,
It’s great that you have been symptom free for so long! Have you taken any specific approach that may have helped you? Are you talking about major symptoms only, or do you have some minor symptoms that you ‘put up with’?
I have small duct psc and have a range of symptoms but certainly none of the more major troublesome ones, which I am grateful for.


#12

Hi Rick! Hard to know to know if anything I’m doing is impactful, but I’ve taken the approach of “if it’s not harmful and it might help, I’ll do it”. I take Urso, a large quantity of probiotics, Omega-3s, turmeric, milk thistle, and vitamin D, and I eat well, don’t drink and exercise regularly. Though I’ve been more or less diligent with all of these over the 23 years. I’d like to think some of these help on the margin, but I suspect there’s just a large degree of luck.

I’ve been completely symptom free as far as I can tell. My vitamin D levels take effort to keep up, which is likely a result of the PSC, but that’s about it. My scarring and duct inflammation is still very mild, so it’s clearly just been a very slow progression. I hope it stays that way!
The largest effects for me (beyond the regular check ups, blood tests and scans) have been the sort of underlying stress and uncertainty of knowing that things could blow up at any time. It’s taken me a long time to figure out how to balance that reality with making long term plans/goals. And I’m not sure I’ve fully got that figured out. Obviously in the scheme of PSC this is a lot to be grateful for, but it’s sometimes hard to figure out what the right benchmark is… median PSC or median person?

As far as I can tell, there are many others similar to me, but I suspect we’re usually the least active on forums as there’s usually not that much for me to contribute.


#13

For the four years I had psc, the mental aspect was tough, especially the last two.

It can be tough to stay positive, and annoninusa, I admire the fact you remain positive after having it for so long.

You seem to be controlling what you can, which is all we can hope to do as well.


#14

I think a lot can depend on whether additional complications develop with the PSC, such as cancer.


#15

Hello everyone
Today is my first day on any kind of support group.
I was diagnosed when I was 28.
I am now 46. I build steel houses for a living. I do not take any kind of meds. I work extremely hard and try my best to eat healthy. My biggest symptom is the itching and weight loss. I suffer from IBS as well. The survival “guidelines” has never bothered me until now. I guess I just realized the other day I could be on “borrowed” time. Thanks for letting me share.


#16

Hollissteel,
The best thing we can all do is to live our lives as best we can, and deal with the symptoms when and where they arise. Glad things are going ok (could always be worse) for you.

Maybe the “best” thing about psc is that is generally progresses pretty slowly.There are people who have to be transplanted asap, and there are those who live 15-20 or more years with psc. It can be a roller coaster ride.

With your strenuous work and your weight loss, I would be concerned your getting enough protein. Have you had any recent blood work?


#17

I’m going may 15th. I hadn’t been in over 2 yrs. Out of sight out of mind kind of thing.


#18

Hi everyone. To encourage you all, I was diagnosed only a year ago at age 71 but have had symptoms for over 35 years and nobody could figure out what was causing it. I had gobs of tests while living in the Chicago area - at a liver specialist. Nothing showed up. Now I’m seeing a hepatologist from U of M hospital and he believes the symptoms I’ve had for so long were all from PSC. He dx. the disease from an MRI last April.

I saw the Dr. again yesterday and he wants me back in 1 year. Meanwhile every six months I’ll have blood work and a liver ultrasound done. I feel fine, have minimal discomfort and do all the things I would normally do.

My symptoms were elevated liver enzymes (since 1982), some discomfort in the upper right abdominal quardrant, and nothing else. So, I encourage all of you, if you are not seeing a hepatologist, find one soon. Then, don’t ever lose hope…statistics can be discouraging. I know my situation may be unique, but aren’t we all? Eat healthy, excercise often, read a lot of fun books and laugh heartily. Bruce


#19

HBruce,

 So glad you finally got connected with a liver specialist. All you have described for what they have told you is in line with initial diagnosis. It doesn’t sound like much progression has occurred yet from your description. Please reach out to this group for any needs and we will do our best to help.   I wish you the very best going forward. 

Mark


#20

My father was diagnosed in his late 60s - he is now 82 - has lived relatively symptom free until this past 2 months - at one point, his liver dr was questioning whether he had “primary” schlerosing because all his readings had been in a good range - he has taken milk thistle/vitamin D from the first diagnosis and lives a rural Alaska lifestyle - lots of exercise and healthy eating -