Hi, I am new to this site and have never participated in online support or chat. I’m not quite sure what I am doing so please excuse me if I don’t do it correctly. My daughter was diagnosed recently and I feel quite alone with this. I try to not let my emotional reaction affect her in a negative way and want all the best possible space for her to be positive and well. Therefore I keep my emotional reaction mostly away from her. I would do anything to help her be well. I search for answers and directions we have not tried. I research the disease quite a lot and find that knowledge seems to help me feel some control over an enemy I cannot control. My hope is that by reaching out to others who deal with this disease I will feel connected to other people that grasp this. My daughter is an adult but that doesn’t lessen the impact this is having on me. I think this introduction sounds selfish and I apologize to all of you that are the ones who live with this disease for focusing on myself. I look forward to reading your stories, suggestions, and positive perspectives. thank you
We welcome you to this site and encourage you to ask all the questions that you need to ask. We are here for you to help and guide you in any way we can as you help your daughter in dealing with this disease called PSC. One of the most important decisions you will make right from the beginning is that you have her under the care of a transplant hepatologist. This is a physician that specializes in the diagnosis and treatment of liver disease. It is preferred that he/she be associated with a hospital that does liver transplants as they will be able to help her get on the transplant list when that time comes. A regular GI doctor is not the doctor generally to be treating her PSC, so that is one of the first things. Although there currently is no cure for PSC there are things that can be done to help ones quality of life. These involve medications like Ursodiol which helps thin the bile so that it can flow better, and then there are medical procedures that will need to be done from time to time like the ERCP procedure where the doctor will go in endoscopically and go up into her bile ducts to dilate them and clean them out good to allow the bile to flow again more freely. This is not a cure but only a temporary fix and may have to be repeated from time to time. As the disease progresses, this procedure will need to be done most likely more frequently. Again, with an ERCP you need to be seeing an advanced endoscopist that does this every day of the week, not your GI that does your colonoscopies and upper GI’s. This is an invasive procedure and if not done in very skilled hands can lead to serious complications, but it is a very helpful tool in patient quality of life care. I would also encourage you to read through all our different topic sections and then reply to any you wish further information or wish to comment on. Our members are always willing to contribute valuable advice to one another.
One final word of advice I would give, PSC is a disease that the patient needs to be very open and frank with their family about. Someone needs to go with her to each appointment to be her advocate. There is a list of questions from another recent post that I would encourage you to read of things to ask at the appointment with the hepatologist. but she will need all your support. You may not always understand why she is going through what she is going through but you need to support her. As things progress, she may not be able to stand the smell of certain foods, eating certain things, etc. This is all normal to the disease process. Try and encourage a regular daily exercise regimen even if it’s just 30 minutes a day of walking, keep her protein levels up to maintain body mass and weight as things progress as well is all helpful down the road when transplantation becomes necessary. Take care and keep in touch.
Here’s that link with the questions for the doctor - Questions for New PSC Patients to Ask
PSC 2011 / Liver Transplant 2015
Thank you so much for your quick reply. She is being treated at Cleveland Clinic. Her Crohn’s specialist is taking primary role and we met with hepatologist. The liver dr told us he did not believe she had psc although the next day when we returned to the GI Crohn’s dr who manages her pouchitis he reversed the opinion and said he is quite sure she does. I continue to hope he is wrong and thought about going to Mayo clinic for second opinion. Her dr manages other psc patients and has a solid reputation as does Cleveland Clinic so I don’t know if I am out of line to be thinking of getting another opinion. thank you for responding. I hope to get more thorough test information and data at our second visit with her dr about this next month.
Hi…not too sure if im posting in the right spot or not but here goes…here is my story. Im a 44 year old nurse and mother of 2 grown children and grandma of an 8 year old beautiful granddaughter. In april 1995 i was diagnosed with autoimmune hepititis…other than having to take meds and regular blood work i hardly knew i had the disease. In march of 2014 my LFT became elevated I had frequent fevers and severe abdominal pain. Because I was already being followed by GI I went for ct scans ercps then diagnosed with PSC. Both intra and extra hepatic bile ducts are affected and I have to go frequently for ercps for stenting and balloon dialation. Also been hospitalized twice in less than a year for an infected gallbladder and blood infections that take two or three rounds of antibiotics to clear. Feeling scared as it seems symptoms eg infections and need for stents are becoming more frequent.
Well thats my story thanks for reading. Love this site as this disease doesnt play by any rule its helpful to read about other peoples experiences
Welcome to our group. I’m glad you found us amidst this diagnosis. There is hope and we have many success stories here, so take a deep breath and know that we are here for you to help in any way we can. A couple of things to consider. You mentioned you are under the care of a GI. If you are not already, you need to put yourself under the care of a transplant hepatologist. The majority of GI’s are not fully qualified to properly treat PSC. Secondly, please if your GI doesn’t do ERCP’s every day, I’d really look into having them done at a transplant hospital or one connected with a transplant hospital. My doctor just basically refused to put stents in my bile ducts as they are so prone to infection and the PSC certainly will clog them up. These are just a couple of things to consider, and of course not knowing all your details you may have already considered these things. Please feel free to give a shout out any time. Kind Regards.
PSC 2011 / Liver Transplant 2015
Hello…i forgot to mention i am being followed by a hepatologist (nearly 2
hrs away) however it is my gi who does the ercps and he the best in my
area. Just wondering when you were diagnosed were you given a timeline as
to how soon you would need a transplant. I know everyone is different and
that the disease progresses at different rates but it seems like mine is
fairly fast. Saw images from my ercps that show extensive beading of my
intrahepatic ducts and every few months my common bile duct gets almost
completely occluded that causes infection of my gallbladder. Again im glad
to be part of this blog and i can read about others experiences and know
that there are others out there who are living with psc.
Hello Again Crystal,
When I was first diagnosed in 2011, I was not given a timeline, however I will say this. If you are having to have increased ERCP’s then things are moving along at a fairly good pace. A couple of questions. What is your current MELD score? Of that, what is your bilirubin level presently. When I got to a MELD of 12 and was feeling so much worse I asked my hepatologist to refer me over for transplant evaluation. He presented me to the committee and I received approval for testing. I was listed in January, 2015 with that 12 MELD. In April my MELD went up to 19. A month or so after that I needed another ERCP and this time the doctor said he had done about all the ERCP’s my bile ducts could stand as the disease had got way up into my liver where he could not reach with the endoscopic device. That was mid June. On July 20th I had labs again and my MELD had shot up to 36! I was then at the top of the list at Duke and was transplanted 2 days later. My point is that when you get up to a 12 or so MELD things can begin to turn bad quick. The sooner you are on the transplant list before things get worse the better shape you will be in. You can’t just jump on that list that easy and if you can go through the evaluation while you are relatively doing ok it will be much easier on your body. Do keep your core strength exercises going and your protein intake up. That’s so important come transplant.
When you do get your transplant I would make sure they are going to plan on the Roux-en-Y procedure which is where they hook your new bile ducts from the donor liver directly into your colon instead of back to the common bile duct where the possibility of recurrence is much higher. Just something to put on your list of things to ask.
Hope this helps.
My name is Amanda and I was scrolling the net in regards to my condition and I came across you it’s… I live in England in a little village and I am not sure if diagnosis are different names across the sea but after reading many posts I can relate to all of the symptoms, struggles and lows that many have shared. I have a condition called Intrahepatic cholestasis of the liver. This condition effects the liver and bile ducts from bile stones and toxins that build up in the liver and make me unwell. I had cholestasis of pregnancy 22 years ago and also 19 years ago and fortunate for me once my little bundles arrived my LFT returned to normal with the help of ursodeoxycholic. Ten years ago I began itching like crazy and had some pain around the liver site. An MRI confirmed a stone lodged in my bile duct which led to an endoscopy and removal of the stone. My life returned back to 'normal with the help again of ursodeoxycholic. I know there are mixed reviews on this medication but for me it worked. It supported the health of my liver and enabled it to function…until 6 weeks ago. Familiar symptoms arrived itching and pain waking me in the night. I also have complete lethargy this time which is new to me with this condition. My bilirubin is currently 20 which according to my liver professor is not worrying. I have been prescribed with colestyramine 4mg sachet twice a day in the afternoon and evening to allow me to take my other meds in the morning. I am also booked in for an MRI. I just want this itching to go away. My skin is scratched and damaged and I have spots all over my legs and arms. All of my sister’s have the same condition and my little sister was so much worse bless her and she had a liver transplant 4 years ago and has two beautiful chdren
She’s doing amazing . So that’s my story and I look forward to chatting with you all. Have a lovely Christmas from over the sea in England xxxx
I have lived unknowingly with PSC for over 20 years. It was only diagnosed last April, 2017 after my liver enzymes had spiked. I can tell that my discomfort is worse than it has been… sometimes I feel a heavy weight pressing into my side.
I got connected to a liver specialist from U of M so I’m glad about that. I’ll see him again in April.
I’m glad I found this site. Thanks to all of you for being here.
Welcome to the group and thank you for posting. I believe a number of us had PSC for many years in looking back but as you, we never knew there was a problem until we started losing weight, itching, high LFT panel showing up in routine blood-work, etc. We look forward to your contribution to our group in the days ahead. We are here for you should any need arise you need information or advice on. There’s a wealth of help among our members. Glad you have found a hepatologist. That will be an invaluable resource if your PSC starts to progress even more.
PSC 2011 / Liver Transplant 2015
Hi everyone, trying to navigate this site. My name is Rich, I’m 54 and have always beeen very healthy. In 2007, I was diagnosed with UC (hit me like a brick) and spent the next few years trying everything to no avail. In 2011, I had my colon removed and a J-Pouch installed. Very successful so far. I also have Polymyositis which limits my ability to hike/walk up stairs etc. I still remain active and have been off all meds for 3 years. I was diagnosed with PSC (go figure) about a year and have managed it with Ursodiol. Then about a month ago I crashed. Woke up with moderate jaundice and absolutely no energy. Liver enzymes multiplied 5x. Awaiting a MRCP and further tests. Pretty freaked out right now and I live in CA so the wait for a liver is horrendously long. Stumped right now on what to do (should I move?) and how to increase my odds of getting better. As I said, it came on quickly and now I’m sitting around like a sloth waiting, and waiting. And advice would be deeply appreciated! Nice to join the group!
Hi Rich, and welcome to our forum. You have come to the right place to find out as much information regarding PSC as you will probably find on a single web site. We are here for you and will do all we can to answer all your questions.
From the description you have given, jaundice and no energy are classic for PSC. With elevated liver enzymes you may be due for an ERCP procedure to clear out the bile ducts. They will go in and do a balloon dilation of your bile ducts, clean things out as best they can and that hopefully will give you relief for awhile. Every patient is different and some have to have the procedure done more often than others. The MRCP will give them a good indication of what’s going on, but the ERCP is the gold standard that gives the doctor eyes on what’s going on inside your bile ducts. I will caution you though, whatever you do, don’t just let anyone do the ERCP procedure on you. Don’t allow a student or resident doctor to do it. It needs to be done by an advanced endoscopist tied with a hospital that does liver transplants. Someone that does these procedures day in and day out. One of the reasons for that is that there is a risk of pancreatitis which can put you in the hospital in excruciating pain. That’s why you need an experienced doctor that does these all the time to lower that risk. I was fortunate to never have a bad case of pancreatitis over the 4 years I was having these procedures with my PSC before transplant. So just keep that in mind. Also, whenever possible, please ask them not to use stents. They are breading grounds for infection. Sometimes they have no choice but to use them, but if they can avoid it is always better.
As far as the no energy does, you will have days like this. I used to come home from work and just collapse in my chair till supper and sleep. This is normal for PSC patients, but I do encourage you to get some form of core strengthening exercise in daily, even if it’s just a good walk for 30 minutes each day. You need to keep your body in good physical shape as the disease progresses so you will be ready for transplant if/when that time comes.
Hope this helps and please feel free to chime in anytime. We’ve got a great group of folks here all willing to give you some good advice. We are the same age as well by the way.
PSC 2011 / Liver Transplant 2015
Thank you very much Mark! Good info as I thought stents were standard procedures. Believe me, when I had my colon removed and J-Pouch surgery (did it at Stanford), I had many complications all due to staff infections causing abscesses. It was brutal to say the least and almost killed me. I will do my homework and seek a good doc. Just starting out on this journey. Glad to have educated and experienced folks to answer questions etc. Thanks again brother!
Rich, my recovery from the colonectomy was much tougher than my transplant recovery. I can relate, but then I had very few complications.
While stents are somewhat common, they aren’t standard procedures. I have had several ecru’s but the doc was able to use a balloon to relieve the blockage caused by stones.
Hi Jeff, thanks for the info! Yes, my colon/j-pouch surgeries were brutal to say the least. Do you have a J-pouch? Glad to know about the stents as I figured they were the only way to keep the ducts open with inflammation. Still waiting, and waiting to see what they can do for me. Not quite ready for the whole “transplant” thing. Still trying to come to terms with it all. Thanks buddy!
Hi, ummmm, was diagnosed with PSC/ Crones Nov 8th '17 after what I thought was a gallbladder attack. Had a stint put in/out and put on Mesalamine for Crones. Since then high fever Thanksgiving weekend but nothing else. I feel fine and almost trick myself into thinking they made a mistake. but… I know they haven’t . Routine blood tests MRI and colonoscopy all good. No symptoms . Just can’t believe it. 52yrs old happily married with 3 Girls. 25 21 13. Sometimes it hits me hard sometimes I’m in denial.
Thanks for posting and I’m glad you are doing pretty well right now. I know it’s hard to believe when you get that diagnosis. Your whole life goes before your face, and you wonder, whose going to take care of my wife and children if I die, I’m so young. Yes, many of us have been there and done that. I just cried and cried with my dear wife when I was first diagnosed, but then I had to pick myself up and get educated, make sure I was under the very best of care and live life to its fullest!!! Just take one day at a time my friend, don’t pack up yet but go forward with your life, plan for the future, be ready to walk those girls down the aisle one day and hold your grandchildren in your arms. You can make it and we will be here to help in any way we can.
I was diagnosed with PSC at 47 and transplanted at 51. I’m now 54 and will celebrate 3 years with my new liver one month from today! Welcome and do let us know how we might give you assistance, encouragement, etc. along this path. I found that being very honest with my wife, children, family and close friends about my health and all that I was experiencing along the journey with PSC gave me great hope. I could not have made it without the support of all these family and friends through the difficult times. When life get’s at it’s lowest point, think of those you love most and press on another day.
Take care and again we welcome you.
PSC 2011 / Liver Transplant 2015
Thank You Mark I hope to talk to and learn from all of you here. I will also update and let every one know what’s going on with me.
Hi Julie, just read through your story. It sounds like you have a wonderful, positive attitude about your journey and a wonderful support system at home. Just wanted to see how things are going for you?
My husband John just went through his own PSC journey. He was diagnosed at age 54 and here we are now in the hospital celebrating our new liver nine months later.!! There is no set course of evolution it seems for PSC and as I’m sure you have read here some people remain symptom-free for years and others have a different experience. God bless and stay in touch.
Rose, so glad your hubby received a liver. Happy for you both.