Primary Sclerosing Cholangitis (PSC)

Posted by jacinta @jacinta, Aug 21, 2017

I’m told I need to have a partial liver transplant. I do have a Donor. My question is: should I get a second opinion ? I have an appointment with Mayo Clinic. I heard that they are the best.

I’d love to hear from people that had the partial liver transplant. Are you glad that you had it done? And are you getting a better quality of life.

Jacinta

@sandyjr

My daughter has just been diagnosed with this illness. It is also known as PSC. A couple of years ago her liver enzymes became erratic. This past fall, the readings were too eratic and her BP suggested she go to a gastroenterologist. She has had every test imaginable and her liver biopsy confirms this diagnosis. Of course I am heartbroken. She and I have read everything we can find on this. Now it is a reality and I feel frozen. I do not know what I can do. I want to load her into a plane and go to the Mayo Clinic. At this point, I do not think they would even deal with her. Can anyone give me some insight on this diagnosis and/or their experience with Mayo and when they should be contacted. Immunology has become a treatment for cancer, has it been tested on this disease? She also has Crohn’s disease.

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Hi, Sandy!
I was diagnosed with PBC (primary biliary cirrhosis) in 1992 at the age of 48, solely on the basis of elevated liver enzymes. A liver biopsy was done to confirm the diagnosis and stage the disease and returned positive for cirrhosis. At the time I lived near Chicago, so had good medical care available and the GI guy started me on methotrexate which had been one of the Mayo protocols for treatment and I understand has since been discredited as a treatment for PBC. Nonetheless, due to the drug or by coincidence, the enzymes began to fall and slowly, very slowly, returned toward normal. It turned out I had a heavy autoimmune component to my disease and it progressed very slowly, allowing my liver to accommodate and I had 25 years of extraordinarily good health while being followed for PBC. Eventually in 2017, a couple lesions showed up on a routine MRI which turned out to be hepatocellular carcinoma. That's when I first reached out to Mayo and it was the best decision of my life. First they took care of the cancer and then did a liver transplant in November of 2018. I could not have wished for better or more compassionate care.

I am doing well and hoping for many more good years. I hope this offers you some hope for your daughter. I'm now 74 years old and have led an active and full life; thanks to the Miracle of Mayo it has been extended. My best wishes for you and your daughter.
Sharon

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@sandyjr well i would say try to apply for SSi disability. And if she is thinking about Divorce then usually a lawyer can get her Medical coverage thru her husband exspecially if she can't work.

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@silverwoman

Hi, Sandy!
I was diagnosed with PBC (primary biliary cirrhosis) in 1992 at the age of 48, solely on the basis of elevated liver enzymes. A liver biopsy was done to confirm the diagnosis and stage the disease and returned positive for cirrhosis. At the time I lived near Chicago, so had good medical care available and the GI guy started me on methotrexate which had been one of the Mayo protocols for treatment and I understand has since been discredited as a treatment for PBC. Nonetheless, due to the drug or by coincidence, the enzymes began to fall and slowly, very slowly, returned toward normal. It turned out I had a heavy autoimmune component to my disease and it progressed very slowly, allowing my liver to accommodate and I had 25 years of extraordinarily good health while being followed for PBC. Eventually in 2017, a couple lesions showed up on a routine MRI which turned out to be hepatocellular carcinoma. That's when I first reached out to Mayo and it was the best decision of my life. First they took care of the cancer and then did a liver transplant in November of 2018. I could not have wished for better or more compassionate care.

I am doing well and hoping for many more good years. I hope this offers you some hope for your daughter. I'm now 74 years old and have led an active and full life; thanks to the Miracle of Mayo it has been extended. My best wishes for you and your daughter.
Sharon

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@silverwoman, Welcome to Mayo Connect. Congratulations on your perseverance and your commitment to take care of yourself. You have been on quite an amazing journey. I am happy to hear that you are doing well. I will be celebrating 10 years in April, so I invite you to come along and enjoy the post transplant life for many years! I was once told by 3 gentlemen, all transplant recipients who called themselves the Three Amigos, that the 'secret' to long survival is to take your meds and take care of yourself. I think that is good advice for us, don't you? Together these guys had 50+ post transplant years.

Here is something that I always enjoy sharing with new transplantees: Patients Share Their Best Tips and Tricks
https://connect.mayoclinic.org/page/transplant/newsfeed/top-transplant-hacks-patients-share-their-best-tips-and-tricks/

If you have not yet discovered the Transplant Pages – https://connect.mayoclinic.org/pages/
I suggest that you check out the regularly updates Newsfeed posts presented by the transplant staff members. You might even recognize some of them!

How has the adjustment to post transplant life gone for you?
What is the best part of being post transplant for you?

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@sandyjr

My daughter is having difficulty dealing with this life setback. Her personal life is a mess. Her husband is abusive and she would like to leave him but he holds the health insurance, so she is trapped in that relationship. She was a stay at home mom and has not worked outside of the home much. Her husband had his own business which she did all the office work for, but never collected an income, so did not pay into SS. Now we have to think about how she is going to finance her illness, especially when she gets to the point of needing a transplant. She may not qualify for Medicare and I do not think you can get the best of the best on Medicaid. Can anyone suggest ways of getting financial assistanace. She works part time now, but she is ill and with the days of not feeling well and with the time used for medical tests etc, I doubt if any place would hire her full time. So, not only are we dealing with the illness, we have these other issues. I read somewhere that it is suggested that you clip coupons and rely on family help. Get real….how many coupons can you clip when you cannot afford to buy anything. Can anyone tell me any of their own difficult financial situations and how they overcame them?

Also, except for her liver enzymes being erratic, and not all of them, different ones at different times, the only test that diagnosis PSC is the liver biopsy. She has had CT scans, HIDA scan, MRI, ECRP and just went for a CT to see how much damage was done to her in testing with this last Crohn’s attack. Her doctor has mentioned surgery if enough damage was done. This is awful. Has anyone else been diagnose with just the liver biopsy being the deciding factor?

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@sandyjr, Has she considered talking to a lawyer? I saw where another member has suggested this.
I hate to hear that on top of her health problem she is living in a threatening environment. She is going to need support both emotionally and physically if her health begin to fail.
Is her medical team aware of her home and financial situation? Has she spoken to a social worker?

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@rosemarya

@silverwoman, Welcome to Mayo Connect. Congratulations on your perseverance and your commitment to take care of yourself. You have been on quite an amazing journey. I am happy to hear that you are doing well. I will be celebrating 10 years in April, so I invite you to come along and enjoy the post transplant life for many years! I was once told by 3 gentlemen, all transplant recipients who called themselves the Three Amigos, that the 'secret' to long survival is to take your meds and take care of yourself. I think that is good advice for us, don't you? Together these guys had 50+ post transplant years.

Here is something that I always enjoy sharing with new transplantees: Patients Share Their Best Tips and Tricks
https://connect.mayoclinic.org/page/transplant/newsfeed/top-transplant-hacks-patients-share-their-best-tips-and-tricks/

If you have not yet discovered the Transplant Pages – https://connect.mayoclinic.org/pages/
I suggest that you check out the regularly updates Newsfeed posts presented by the transplant staff members. You might even recognize some of them!

How has the adjustment to post transplant life gone for you?
What is the best part of being post transplant for you?

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Thanks for your welcome and the shares, Rosemary. I'd love to join you for many years of post transplant life; it's such an inspiration to know that you and many like you have enjoyed so many, many years. Thank you for the shares; I will check them out. As for adjustment to PT life, not being 100% my old self yet is a bit frustrating…..not quite ready for my old pace yet. I'm trying to avoid crowds in this first six months PT, and being, by nature, very gregarious, this is a challenge. The best part is having the anticipation and concerns about the operation behind me and knowing that my family and friends have been so supportive and caring, a solid foundation of love and prayer. Life is good.

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@rosemarya

@sandyjr, Has she considered talking to a lawyer? I saw where another member has suggested this.
I hate to hear that on top of her health problem she is living in a threatening environment. She is going to need support both emotionally and physically if her health begin to fail.
Is her medical team aware of her home and financial situation? Has she spoken to a social worker?

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This is fairly new, so she has not had time to do much but go for tests and Dr’s appointments. She has an attorney, but has not been able to get in touch with him. He can guide her about who to see for disability and maybe give her some additional advice. She has no health team yet. She just has her GP who noticed the stuff with the liver enzymes and the gastroenterologist that she ended up with in the ER. She had seen her former gastroenterologist who was taking his time getting tests for her. The new Dr has gotten her a number of tests to diagnose the PSC and he is also treating her for her Crohn’s. I am liking how well he treats her and is working for/with her. We are new to this, so just finding our way. Getting away from the husband is a problem as he carries the health insurance and has told her if she leaves he will just quit his job….he really loves her, right? She does pretty well standing up for herself, but she sure does not need the stress and confusion. I have been single for 27 years and truly like my life and I think she would too, if she ever gets the opportunity.

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Hi @sandyjr and @silverwoman, it's been a while so I wanted to check in. Would love to get an update. How are you?

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@colleenyoung

Hi @sandyjr and @silverwoman, it's been a while so I wanted to check in. Would love to get an update. How are you?

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Hi Colleen. My daughter has started taking infusions of entyvio. She has been having stomach aches which are especially noticeable in the night. She stopped taking the ursodiol and has had some relief. She had her first infusion this past Wednesday. She sees her Dr on Monday, so hopefully they can get to the issue of the constant stomach ache. On your sight, I think I saw something about communicating with Mayo doctors. We live in NJ, so being that her PSC diagnosis is relatively new and in it's early stages, I do not think the doctors out there would consider her sick enough to see in person. Her doctor is not a specialist in the illness, but is far better than the specialist she was seeing who could not seem to get approval from her insurance company for the tests needed for diagnosis and the new doctor has been able to with no problem…what the heck? He wants to continue as her doctor and is very willing to communicate with the doctors at Mayo about her. Is this possible and if so, how is it done?

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@sandyjr

Hi Colleen. My daughter has started taking infusions of entyvio. She has been having stomach aches which are especially noticeable in the night. She stopped taking the ursodiol and has had some relief. She had her first infusion this past Wednesday. She sees her Dr on Monday, so hopefully they can get to the issue of the constant stomach ache. On your sight, I think I saw something about communicating with Mayo doctors. We live in NJ, so being that her PSC diagnosis is relatively new and in it's early stages, I do not think the doctors out there would consider her sick enough to see in person. Her doctor is not a specialist in the illness, but is far better than the specialist she was seeing who could not seem to get approval from her insurance company for the tests needed for diagnosis and the new doctor has been able to with no problem…what the heck? He wants to continue as her doctor and is very willing to communicate with the doctors at Mayo about her. Is this possible and if so, how is it done?

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@sandyjr, I am a PSCer who has had a transplant. I do not know the answer to your question to Colleen about communication with doctors at Mayo for PSC, but I will share my own experience with you.
I live in central Kentucky. My PCP first noted that I had elevated liver function in my routne labs. He sent me to our local gastroenterologist (GI who had some experience and knowledge of liver disease, and who also had professional relationship and connections with the liver specialists at both of the regional Liver transplant centers (Univ of Kentucky and Univ of Louisville). In collaboration with one of his hepatologist buddies he was able to diagnose and monitor my PSC. I credit him with being a major influence in me being alive today. He referred me to the liver transplant department at the Univ of KY (most convenient location – 45 minute drive) when I was ready for that step. I received excellent care there. And I would have transplanted there except that situation got suddenly complicated. It was my UK team who referred me to Mayo for treatment and eventual transplant.
I want to add that I did not have Crohns. I took URSO and it didnt cause me any issues.

Strength to your daughter. I think she has found a good proactive doctor!

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To my fellow PSCers.
@jacinta, @sandyblair, @monty49, @jenchavez, @sandyjr, @silverwoman,

Today I am thinking about all of you and since I have not heard from you for a while, I want to check on you. From my experience, I know that PSC can be a long and miserable journey.
How are you doing?

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I had an ERCP on 15th May and unfortunately it didn’t get rid of the scratching. I contacted my Nurse and Specialist. I begged them not to let me slip through the cracks. Both ignored me.
However, The Doctor that performed the ERCP had his Nurse contact me. An appointment was set up for 5/28. When the Nurse saw me, I could see her reaction to my body that was full of scabs and blood from scratching. She wanted to have blood work done immediately, but that Department was closed. She begged me not to fly home to Ireland, but I had my flight paid. I had only 90 minutes to get to my flight to check in etc. I told that Nurse that my own Nurse and Doctor ignored my begging plea to see me before I flew home and ignored me.

Anyway, I returned from Ireland, had blood drawn. I’m now waiting for an MRI on 24th June. Until then, I continue to destroy my body by scratching

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@jacinta

I had an ERCP on 15th May and unfortunately it didn’t get rid of the scratching. I contacted my Nurse and Specialist. I begged them not to let me slip through the cracks. Both ignored me.
However, The Doctor that performed the ERCP had his Nurse contact me. An appointment was set up for 5/28. When the Nurse saw me, I could see her reaction to my body that was full of scabs and blood from scratching. She wanted to have blood work done immediately, but that Department was closed. She begged me not to fly home to Ireland, but I had my flight paid. I had only 90 minutes to get to my flight to check in etc. I told that Nurse that my own Nurse and Doctor ignored my begging plea to see me before I flew home and ignored me.

Anyway, I returned from Ireland, had blood drawn. I’m now waiting for an MRI on 24th June. Until then, I continue to destroy my body by scratching

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@jacinta, Who is monitoring your PSC?

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I have a liver specialist and a nurse. The specialist never contacts her patients, nor does she participate in ERCPS. Her Nurse is useless. I just think she doesn’t know the severity of PSC. She likes to use “my chart.”

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@jacinta

I have a liver specialist and a nurse. The specialist never contacts her patients, nor does she participate in ERCPS. Her Nurse is useless. I just think she doesn’t know the severity of PSC. She likes to use “my chart.”

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I forgot to mention that my Doctor thinks twice a year for 10 minutes is enough to see me

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I was just curious if anybody out there experienced bad lower leg pain and swelling prior to transplant? Personally I experienced calf swelling and lower leg pain and then post transplant it disappeared.

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