Hello

Hi everyone, I am 26 years old and I was diagnosed with PSC just after my 19th birthday. I had issues all my life and no one ever tested my liver function until about 18 when i ended up in the hospital in a lot of pain. The doctors thought it was my gallbladder but after being sent to a hepatoligist I was diagnosed with PSC after a liver biopsy, MRI, ultrasound and I cannot even count the amount of blood vials drawn. The doctor started me on Urso but when I lost my health insurance I could not afford it and was told it was just experimental anyways.

Its been 7 years and since then I have been diagnosed with microscopic ulcerative colitis and arthritis. I have had at least 6 doctors appts ever since and kept up on the lab works.

I just recently had some blood tests done and my ALP had jumped to 782 from my last result of only 476 in june. I have been having a lot of joint pain and a lot of shoulder pain and lost a lot of weight. I am unsure of what this all means and once again am just kept waiting for an appt.

Does anyone know what could be going on? I am trying not to worry but its all I can do with not having an appt.

Any help or encouraging words would be appreciated.

It seems to me that the US has more information about PSC and I am trying to do my best to research and find things out. I do have some background knowledge because I am a nurse but I feel like I know next to nothing about this disease because I feel like the doctors have only a limited knowledge base on it.

I have never been considered to have a "flare up" and am not sure if that is what is going on but I am worried. I am unsure if I am going to have to have more tests but I am assuming so.

I’m in the waiting game too and it is a hard place to be. There are a lot of great resources online, here included. Don’t get discouraged, attitude and staying positive are key. I know sometimes it’s hard but it helps with the down time.

I am so sorry for your trouble with PSC. My partner was on URSO for a bit. URSO is experimental and his gastroenterologist( who has done a good deal of research with PSC and knew the results with URSO were not all that favorable) allowed my husband to choose to try it or not. He did and like the research shows, it mainly seemed to just mask the elevated liver function test results. When my husband tried for those 6 months he ended up with these nasty white bumps all over his body. Others have reported this issue too. He quit taking it. Basically, his enzyme counts did go down, but symptoms were all still there. I think I would not fret too much about not having the URSO.

I concur with MACH 4 that the liver functions go up and down based on a number of factors and it does not necessarily mean something has suddenly gotten worse. We have seen this in the 5 years since diagnosis. Good to check in with your doctors and ask question to others who are dealing with PSC etc. My husbands function tests go up and down too and are definitely worse during flare ups/cholangitis episodes. Taking care to keep your stress in check, eat mindfully for PSC (see those related discussions in this forum as they are very helpful) and again, good communication with your team of doctors. If they don’t seem to answer your questions maybe look for a more knowledgeable and supportive group. There is a lot of info out on the web.I have found some very helpful stuff on pscpartners.org .

I know it is nearly impossible, but try to manage stress and carry on with your life as normally as you possibly can… And also, go easy on yourself. It is a tough road to have to be waiting, but again, try not to obsess on the numbers so much. Go more by how you feel. Fever, chills, headache, increased upper right quadrant (URQ) pain is important to catch sooner than later. Since cholangitis (infection in the bile ducts) is the pits and can put you in the hospital. A round of antibiotic in a timely manner can help to avoid all of that. My husbands gastroenterologist set up with my husbands local GP doctor to prescribe an antibiotic that he can have on hand at all times in the event that cholangitis is starting. There isn’t any doubt when it is coming on, and his physicians know that he will not take it unless he is sure. Taking it when there is doubt is certainly dangerous in the long run since overuse of antibiotic can make it less effective.

Again, so sorry for the stressful time you are having. My heart goes out to you.

What medications are most people on to manage the symptoms?

I am currently only on Mobicox to help with bone and joint pain. I have already been told that I have osteopenia.

Glass Flower you mentioned that you have someone local to administer antibiotics if needed...my doctor is about 2 hours away and if there is an issue she expects me to go to the hospital she is at which isn't always possible...which antibiotic is your husband on for when he has flare ups?

Hi Briarrann

I am so sorry to hear about your struggle. Like others my numbers went up and down. Have you been referred to a transplant team yet? It may be a good idea to get a referral soon. They will also be able to provide information and work with your other docs. I agree with the supplements for nutrition. I am osteoporosis and take calcium, D, Strontium, and once in a while a combination for bones. I take these separate from my other vitamins as they are best absorbed on own.

I am curious about where you are from? You mentioned you lost your insurance. Financial stress on top of everything else is a factor. If from Canada check out CareToHope.ca for info on resources.

Ruby



briarrann said:

It seems to me that the US has more information about PSC and I am trying to do my best to research and find things out. I do have some background knowledge because I am a nurse but I feel like I know next to nothing about this disease because I feel like the doctors have only a limited knowledge base on it.

I have never been considered to have a "flare up" and am not sure if that is what is going on but I am worried. I am unsure if I am going to have to have more tests but I am assuming so.

It’s cephalexin, 500 mg for 10 days. Again, only when certain it is cholangitis. The gastro dr. Is 2 hours away for us. So, he has communicates all his reports to our local GP who basically follows the gastro dr. plan. Hospitalisation, though, we go to the specialists, for sure.




briarrann said:

What medications are most people on to manage the symptoms?

I am currently only on Mobicox to help with bone and joint pain. I have already been told that I have osteopenia.

Glass Flower you mentioned that you have someone local to administer antibiotics if needed…my doctor is about 2 hours away and if there is an issue she expects me to go to the hospital she is at which isn’t always possible…which antibiotic is your husband on for when he has flare ups?

Transplant team? Could that be premature? Thought you needed a fairly high MELD for that and would not do a transplant until you absolutely had to due to the risks involved. Is there a MELD score that you should begin to see a transplant team? Sounds like briarran might not even be close to thinking about that.




Ruby said:

Hi Briarrann


I am so sorry to hear about your struggle. Like others my numbers went up and down. Have you been referred to a transplant team yet? It may be a good idea to get a referral soon. They will also be able to provide information and work with your other docs. I agree with the supplements for nutrition. I am osteoporosis and take calcium, D, Strontium, and once in a while a combination for bones. I take these separate from my other vitamins as they are best absorbed on own.





I am curious about where you are from? You mentioned you lost your insurance. Financial stress on top of everything else is a factor. If from Canada check out CareToHope.ca for info on resources.



Ruby











briarrann said:

It seems to me that the US has more information about PSC and I am trying to do my best to research and find things out. I do have some background knowledge because I am a nurse but I feel like I know next to nothing about this disease because I feel like the doctors have only a limited knowledge base on it.

I have never been considered to have a “flare up” and am not sure if that is what is going on but I am worried. I am unsure if I am going to have to have more tests but I am assuming so.

Hi Glass Flower

Interesting question and may be different for different systems? I made the recommendation because Briarrann mentioned travelling 2 hours to see his doctors who are not very knowledgeable about PSC.

I was referred to the transplant team in Vancouver right after diagnosis to get on their radar. MELD score about 7. Then I was seen every 6 months until meld score jumped (which can happen suddenly). PSC does not have predictable course or linear degeneration.

I was rear ended and within 2 months getting an assessment for the active transplant list. Talked to the Transplant Docs and they mentioned how people wait too long for an initial assessment.

Maybe it is a difference between systems? This is a whole discussion in itself, but may be helpful across systems. eg: Canadians are benefiting from the protocols for Lyme disease in USA. In Canada treatment is a joke.
Ruby

what is this MELD score people are talking about?

I do have health coverage like most in Canada but that doesn't pay for medications. I am currently on employed part time and because of this I do not have drug coverage. I live in Ontario.

I was under the impression from the doctors that there is not contact or mention of a transplant until you are pretty much end stage liver failure. Am I incorrect about this?

I know it may seem pretty petty but I am young and most of my friends are huge drinkers, I am lucky that my boyfriend does understand and has no problem with not drinking. I am wondering if anyone else is worried about the struggle with not drinking? I have never really been a big drinker and I don't need to drink but its such a social thing at my age and I am concerned about this and how to stay away from it.

my hepatoligist is actually at toronto western.

I am waiting to hear about my appt because they want it moved up and my doctor is away right now.

I am going to ask about this MELD and see if they have a number for me. Thank you for helping with it. and now I will have to look into the liver transplant and what that is all about. Can you get a family donor? and how sick do you need to be...seems kinda backwards to me...

Hi
Agree - stay away from alcohol. A good way is to adopt an - I am allergic to alcohol - because your body really can not process it. Hopefully your friends will understand.

I have learned the hard way that there are a few other seemingly harmless meds to also stay clear of. They include Ibuprofen, ASA, and Aleve. They all thin the blood and can cause bleeding and for liver disease this is not good.
Tylenol is OK but not over recommended dosage and never mix with alcohol. The combination can cause liver failure in healthy folks.

This all came up when I had some internal bleeding and the emerge doctor gave me some ibuprofen like medication by IV. I was back in 24 hours and needed a blood transfusion. My GI was miffed! Become informed because it is such a rare disease and so many doctors know so little about it.

It is a hard blow to have this disease and very scary. You sound alone with this. Take in the support and information here to help.

Ruby

For years, if I had one /drink/beer a month, that was a lot for me. So when I got diagnosed with PSC, making the decision to not drink alcohol again was a no brainer, and easy to live with.

But I do miss my premium long island iced teas.


Briarrann,

The MELD score is based on several components of your bloodwork. It is not based directly on how healthy you are. That is a disconnect, I think, with the MELD score, but a doc can appeal the score if there is information that the doc thinks should be considered to justify a higher place on a transplant list. Backwards, to a degree.

There are two sources of liver, from live donors and deceased donors. Finding a live donor is largely up to the patient (folks, correct me if I am wrong). but has fewer physical restrictions like finding a kidney match.


briarrann said:

my hepatoligist is actually at toronto western.

I am waiting to hear about my appt because they want it moved up and my doctor is away right now.

I am going to ask about this MELD and see if they have a number for me. Thank you for helping with it. and now I will have to look into the liver transplant and what that is all about. Can you get a family donor? and how sick do you need to be...seems kinda backwards to me...

Hi Jeff, Briarrann

I had a donor for my transplant and can answer the main questions.
A donor comes forward and you can direct them to the transplant team. You can not actually be seen to influence the donor. If you think about it, ethically, this makes sense. No Bribing or coercing etc…

The donor needs to be a comparable blood type (my son was the donor, he is O-, I am O+)There are also other blood matches I believe.
A couple of my sisters also came forward, but the younger, taller and leaner, the better. The Docs loved my son and called him the perfect donor.
The donor also has to match structurally in terms of size of ducts and where they line up for the surgery to work. Livers do not have a uniform structure like kidneys. They take a large chunk of the large right lobe for transplant.

The recipient can accept a live donor or not. I had to consider ALL the risks moving ahead. My son was so firm in his decision. My brother (MD) drilled him on a the risks and possible outcomes (which I appreciated.) Research suggests that donors have a profound experience, unless it does not work out. On the other hand, if I turned it down and died, my son would have been so angry, were talking years of therapy.

So I went ahead. My son’s liver grew back in 12 weeks! I still struggle, but he is fine. My son and I have such a special bond. My daughter was one of my support person in Vancouver so she is also in on the experience. It was harsh, but we are all doing well.

A couple of donors have not done well, it is major surgery. But on the other hand donors should be considered to be given their chance to save a life if they are drilled on the risks and there is a very big chance of a positive outcome.

I hope this helps.
Let me know if there are any questions.
Ruby

this all helps so much ... my poor doctors when i go to my appt because i have a huge list of questions and new things to bring up ... you have no idea how much this has helped me right because i am trying my best to stay positive but no one around me understands what I am going through or understands how i am feeling ... im such an upbeat person and boy this knocks you down...

Dear Briarann,

How are things shaping up. I had not been around for awhile and am only now catching up on posts. Although I am not on the same journey as yourself I do understand how difficult this must be for you. I read posts like this so I can empathise and possibly relate to what my daughter may face in the future.

It is funny that you bring up alcohol, because I often wonder how she will deal with that. I hardly use to drink before and then gave up drinking when I was pregnant. Then being a mother "on-call" all the time I opted for not drinking and must have drank on three occasions in the past 9 years. Recently I attended a family gathering and everyone was drinking except three of us. One of my cousins kept telling me to take a drink and relax and how I was missing out on all the fun. I told her that I was still having fun and this was nothing as my daughter would never be able to drink. I told her besides, I may take a drink now and then learn in the future that because of that drink I may not be able to help my daughter. I know it seems hard, but you have to do what is best for your long-term health. I have given "gluten", "dairy" and array of things up for my daughter. so she will see that Mummy doesn't eat, drink...... I am hoping it will lessen the load for her, but it still worries me how she will deal with it all. I said to one family member, "I can choose not to drink, but Denisa will never really have that CHOICE!!!".

Take care.

PrincessD's Mum

hi there PrincessD's Mum,

Things are going alright, honestly feeling quite alone because no one understands what I am going through, everyone thinks "oh thats great your getting a transplant" or "at least they are helping you" but no one understands how scared I am. If my boyfriend is not here to stay over i cry myself to sleep worrying about what if I don't wake up after the surgery, or what if they don't find someone in time, or how can I ask a family member to go into surgery and do something so drastic for me. My mom keeps saying that they would do anything for any one if anybody else were in this situation and I do have a huge amazing family and I don't doubt for a second any of them would but still thats a huge sacrfice. I am waiting to go for my MRCP but seems like it is taking forever.

I don't know if I am the only one but because I don't drive I have to rely on people and i feel like a huge burden sometimes, actually most of the time. When I need a 2 hour drive to the drs, when someone asks me about it and it takes and hour to explain everything going on, when people wanna go out and drink and then realize that I can't.

i guess you could say I am feeling a little down which is not my usual personality and I am trying to hide it the best way possible.

PrincessD said:

Dear Briarann,

How are things shaping up. I had not been around for awhile and am only now catching up on posts. Although I am not on the same journey as yourself I do understand how difficult this must be for you. I read posts like this so I can empathise and possibly relate to what my daughter may face in the future.

It is funny that you bring up alcohol, because I often wonder how she will deal with that. I hardly use to drink before and then gave up drinking when I was pregnant. Then being a mother "on-call" all the time I opted for not drinking and must have drank on three occasions in the past 9 years. Recently I attended a family gathering and everyone was drinking except three of us. One of my cousins kept telling me to take a drink and relax and how I was missing out on all the fun. I told her that I was still having fun and this was nothing as my daughter would never be able to drink. I told her besides, I may take a drink now and then learn in the future that because of that drink I may not be able to help my daughter. I know it seems hard, but you have to do what is best for your long-term health. I have given "gluten", "dairy" and array of things up for my daughter. so she will see that Mummy doesn't eat, drink...... I am hoping it will lessen the load for her, but it still worries me how she will deal with it all. I said to one family member, "I can choose not to drink, but Denisa will never really have that CHOICE!!!".

Take care.

PrincessD's Mum

Dear Briarann,

I know it must be hard for you. Yes, I know you must be scared. It is only natural. I know people will tell you not to be scared, but it is because they care and don't want you to put added stress on yourself.

I know how you must feel because I don't drive so I take the bus, train and the subway to take my daughter to her appointments. She is only seven so I sometimes still stick her in the buggy because I know how draining this must all be on her little body.

I don't know if it is just me, but sometimes people ask how she is and then half way through the narration I feel as if they glaze over and tune out!! Do you feel like that too?

Do what I do. Go for a drink. A coffee or something. Ask people to meet you for lunch etc instead. It may not always work, but that way you won't always feel as isolated.

This is a long hard journey and you will have your good days and bad. People tell me I am always smiling and can't believe when I do finally tell them what my daughter faces. I have a "bubbly nature" too, but we are human. If you ever feel low, please email me. You are strong and have to believe that you will get through this. I know you see yourself as a burden, but you are not. You are loved dearly and people want to help and will help you.

Take care for now.

PrincessD's Mum

Hi Briarrann-

So very sorry to hear about your diagnosis. My son is 17 and was diagnosed at 15. There are a few older teens and young adults with PSC in your general area - might it help to connect. In fact, there's a really great young man who has survived two transplants and is thriving. He's very active in outreach and education about liver issues and organ donation. We met last year at the PSC Partners conference. If you'd like to feel less alone, let me introduce you. Also, do consider if you're able to attend the conference this spring - there seems to be a fantastic group of young adults there to act as a support system for each other. Finally, please read up on the vanco protocol and talk to your doctor about it. Be prepared to be very well-informed and know that you would need to advocate hard for it if this is something you want to try - it is not standard treatment in Ontario, but it is not unheard of.

Please feel free to send me a message if you want to try to connect with some local young adults!