Hi all, hope this day finds you in good form and well.
My son was diagnosed with PSC a year and a half ago. His blood tests revealed worrying results for quite a time. We have since moved to Canada and have a new consultant, who incidently is kind, compassionate and very nice! He has looked at my son’s notes and recent blood results and said it’s very unusual but things are almost at normal level. He is not going to do any further testing for another six months because he doesn’t want to ‘fix what’s not broken’. Is it unusual for the liver to show signs of normality once diagnosed? Is there a chance he may have been mis-diagnosed previously? The only med he’s on is azathioprine (for his UC), could this being helping his liver also?
PSC levels can and often do fluctuate. Some PSCers have levels that never range much from 10x the normal limits and some PSCers have them right in the normal limits. Was he diagnosed by an ERCP? That's the best way to know if PSC is at play. As to azathioprine, it's likely not helping his liver. Sorry, wish there was better news there!
Hi there Sandi, and thank you so much for taking the time to reply to me. My son had a liver biopsy in the UK and looking at the results they seemed to think there was enough scarring to confirm PSC. They advised he should have an MRCP once we are in Canada but as mentioned above, the consultant here doesn’t want to perform such a test while his blood results are so good. Hence my confusion. They seemed pretty sure in the UK that he does have PSC.
A biopsy alone isn't enough to determine PSC, so you're right to ask for more information. An MRCP is not an invasive test, so there would be no risk other than that if he has a contrast allergy (and depending on his age, they may not even use contrast). An ERCP is a more invasive procedure, but one generally deemed worth the risk by most doctors when they need to get in and see what's going on. For a PSCer, yearly or bi-annual MRCPs should be the standard of care with further intervention (like ERCP) taking place when needed.
Is your new doctor familiar with PSC? Has he/she treated PSCers before? It's incredibly important to be with a hepatologist who is familiar with PSC whenever possible. It truly does make such a difference in this disease!
His liver biopsy was apparently very unusual so the docs in the UK sent the sample to another specialist to get a second opinion, which came back as definite PSC. Reading through his notes the wording says there’s no doubt. I’d heard that ECRP/MRCP is the only true way to diagnose, hence trying to encourage he gets this test done. The consultant we’re seeing is GI, how do I get my son seen by a hepatologist? I would have thought the gastro chap would have referred him. Although he was really nice, I did feel we’d come to the end of the consultation when he said ‘keep well and see you in six months’. I feel such an idiot for not being more pushy.
No, not an idiot at all! You may want to ask your GI's office for a referral to a hepatologist familiar with PSC. Or, failing that, you can always call around to some transplant hospitals near you and ask who they'd recommend. If a name pops up more than once or twice, chances are that's a good sign!
You're right that ERCP/MRCP is considered the gold standard in diagnosis. Biopsies can be largely unreliable all on their own. This isn't to say that your son doesn't have PSC, just that if I were you, I'd want more information, too. When you do talk next with your consultant and/or a hepatologist, you may want to have some questions prepared to take in with you. Here's a list of some commonly asked ones (taken from www.pscpartners.org) that you can modify to your son's situation.
Questions You Should Ask Your Doctor
Go ahead: Just ask. Don't worry if you think your question is silly or stupid. If you're wondering, ask. It's likely someone else has already asked that question and the doctors have an answer for you. When you are open and communicative, you will develop a better relationship with the medical team.
Be sure to get the name of the person and his/her contact information so that if you think of something later on you can re-connect with your question. Ask how long you should expect to wait until you get a response, an answer to your question. PSCers recommend that you bring someone with you to medical appointments, perhaps to ask questions, and to hear what the doctor is saying. That person can take notes for you, too.
Write your questions down ahead of time; don't be rushed during your visit. You need the information to maintain your health. Here are some questions experienced PSCers developed that you will want to ask your team if you are newly diagnosed with PSC.
If you are newly diagnosed, these questions might be useful to raise with your doctor:
Working with your physician
How many cases of PSC have you managed in the past and currently?
At what point would you want me to call regarding symptoms, and for which symptoms would you want me to go straight to an ER?
Can I have my routine labs drawn before my appointments so that we may discuss the results in person?
Diet/nutrition
Are grapefruit and juice OK to eat with my medications?
Are fish oils an acceptable supplement? If yes, what dosage would you recommend?
Is it OK to consume alcohol-containing products?
Should I make any changes to my diet?
Recommendations on tests
If I do not currently have ulcerative colitis, how often should I have a colonoscopy to look for ulcerative colitis?
How many biopsies will you take to look for quiescent disease?
If I have ulcerative colitis, should I be taking folic acid supplements along with Ursodiol? If yes, what dosage?
Medications
Should Ursodiol play a role in the management of my PSC? If yes, what dosage?
Are any of my current medications problematic?
If I have a headache or musculoskeletal pain, what over the counter medications would you recommend? What should I avoid?
If I experience itching from my liver disease, also known as pruritis, what can I do to gain relief? What about phototherapy with tanning beds for my itching (pruritis)?
Some advanced questions for follow up visits:
Cancer
Upon average, when does bile duct cancer (cholangiocarcinoma: the Klatskin tumor) arise from the time diagnosed with PSC?
Does Ursodiol decrease the incidence of cholangiocarcinoma? What about decreasing the chance of colon cancer?
If bile duct cancer is suspected and brush scrapings are done, will you send the sample to Rochester Mayo for FISH staining?
Transplant
What is my MELD score?
If I need a transplant, where do you usually recommend having the surgery?
Post-transplant, should I be put back on Ursodiol to help reduce the chances of colon cancer?
Post-transplant will you start me back on my ulcerative colitis maintenance medication?
Recommendations on tests
Can I have a copy of my blood and diagnostic test results?
How often do you recommend an ERCP or MRCP?
How often should I have an abdominal ultrasound? How did my gallbladder appear?
Is there any evidence of ascites? Any suspicious lesions?
How often should a CA 19-9 level be drawn?How often should my INR/PT, Albumin, AST, ALT, Alkaline Phosphatase, Total Bilirubin, and Direct Bilirubin be drawn?
How frequently should I be checked for fat-soluble vitamins (Vit A, E, K, D) levels?
Should I take fat soluble vitamin supplements? If yes, what dosages?
How often should a bone density test be done?
What vaccinations are required since I have liver disease?
No, not an idiot at all! You may want to ask your GI's office for a referral to a hepatologist familiar with PSC. Or, failing that, you can always call around to some transplant hospitals near you and ask who they'd recommend. If a name pops up more than once or twice, chances are that's a good sign!
You're right that ERCP/MRCP is considered the gold standard in diagnosis. Biopsies can be largely unreliable all on their own. This isn't to say that your son doesn't have PSC, just that if I were you, I'd want more information, too. When you do talk next with your consultant and/or a hepatologist, you may want to have some questions prepared to take in with you. Here's a list of some commonly asked ones (taken from www.pscpartners.org) that you can modify to your son's situation.
Questions You Should Ask Your Doctor
Go ahead: Just ask. Don't worry if you think your question is silly or stupid. If you're wondering, ask. It's likely someone else has already asked that question and the doctors have an answer for you. When you are open and communicative, you will develop a better relationship with the medical team.
Be sure to get the name of the person and his/her contact information so that if you think of something later on you can re-connect with your question. Ask how long you should expect to wait until you get a response, an answer to your question. PSCers recommend that you bring someone with you to medical appointments, perhaps to ask questions, and to hear what the doctor is saying. That person can take notes for you, too.
Write your questions down ahead of time; don't be rushed during your visit. You need the information to maintain your health. Here are some questions experienced PSCers developed that you will want to ask your team if you are newly diagnosed with PSC.
If you are newly diagnosed, these questions might be useful to raise with your doctor:
Working with your physician
How many cases of PSC have you managed in the past and currently?
At what point would you want me to call regarding symptoms, and for which symptoms would you want me to go straight to an ER?
Can I have my routine labs drawn before my appointments so that we may discuss the results in person?
Diet/nutrition
Are grapefruit and juice OK to eat with my medications?
Are fish oils an acceptable supplement? If yes, what dosage would you recommend?
Is it OK to consume alcohol-containing products?
Should I make any changes to my diet?
Recommendations on tests
If I do not currently have ulcerative colitis, how often should I have a colonoscopy to look for ulcerative colitis?
How many biopsies will you take to look for quiescent disease?
If I have ulcerative colitis, should I be taking folic acid supplements along with Ursodiol? If yes, what dosage?
Medications
Should Ursodiol play a role in the management of my PSC? If yes, what dosage?
Are any of my current medications problematic?
If I have a headache or musculoskeletal pain, what over the counter medications would you recommend? What should I avoid?
If I experience itching from my liver disease, also known as pruritis, what can I do to gain relief? What about phototherapy with tanning beds for my itching (pruritis)?
Some advanced questions for follow up visits:
Cancer
Upon average, when does bile duct cancer (cholangiocarcinoma: the Klatskin tumor) arise from the time diagnosed with PSC?
Does Ursodiol decrease the incidence of cholangiocarcinoma? What about decreasing the chance of colon cancer?
If bile duct cancer is suspected and brush scrapings are done, will you send the sample to Rochester Mayo for FISH staining?
Transplant
What is my MELD score?
If I need a transplant, where do you usually recommend having the surgery?
Post-transplant, should I be put back on Ursodiol to help reduce the chances of colon cancer?
Post-transplant will you start me back on my ulcerative colitis maintenance medication?
Recommendations on tests
Can I have a copy of my blood and diagnostic test results?
How often do you recommend an ERCP or MRCP?
How often should I have an abdominal ultrasound? How did my gallbladder appear?
Is there any evidence of ascites? Any suspicious lesions?
How often should a CA 19-9 level be drawn?How often should my INR/PT, Albumin, AST, ALT, Alkaline Phosphatase, Total Bilirubin, and Direct Bilirubin be drawn?
How frequently should I be checked for fat-soluble vitamins (Vit A, E, K, D) levels?
Should I take fat soluble vitamin supplements? If yes, what dosages?
How often should a bone density test be done?
What vaccinations are required since I have liver disease?
Wow Sandi, thank you so much for this info. I shall read and absorb more thoroughly but just wanted to thank you for the comprehensive reply. It’s all so confusing in the beginning and it doesn’t help to emigrate in the middle of it all! I just want to know the definitive result so we can move forward with whatever we have to face.
Hmm...I think my reply just disappeared. Sorry if you get this twice!
You're absolutely welcome and you're absolutely not all alone out there. If you need help, reach on out. Even if it's just to vent! I promise though, things do get easier with time. It's a steep learning curve, but you'll get there! Just take a few minutes (at least) every single day to enjoy life and forget about PSC for a bit if you can!
I don't know how to send a private message in this group/on this site, but if you'd like to stay in touch, feel free to send me a note how to reach you and I'll be happy to exchange info with you. You can reach me by e-mail and/or FB. No pressure, just an offer if it helps to know you've got a friend out there who gets what you're going through! :)
Hope tonight is a great one!
Sandi Motherofone said:
Wow Sandi, thank you so much for this info. I shall read and absorb more thoroughly but just wanted to thank you for the comprehensive reply. It's all so confusing in the beginning and it doesn't help to emigrate in the middle of it all! I just want to know the definitive result so we can move forward with whatever we have to face.
Per the Stanford Cox study my daughter has been taking 500mg oral vancomycin (OV) 3x / day for 7 weeks now. Her clinical symptoms have normalized - 1-2 solid stools/day; gained 6 pounds. She just had a colonoscopy and the GI doctor has decided to keep her on the OV for 6 more months to see if there is further improvement in the histology of her colon. We will have her blood panel taken in January and will keep you posted. Let me know if you want more information.
Per the Stanford Cox study my daughter has been taking 500mg oral vancomycin (OV) 3x / day for 7 weeks now. Her clinical symptoms have normalized - 1-2 solid stools/day; gained 6 pounds. She just had a colonoscopy and the GI doctor has decided to keep her on the OV for 6 more months to see if there is further improvement in the histology of her colon. We will have her blood panel taken in January and will keep you posted. Let me know if you want more information.
Cactusgirl, Is insurance covering your daughter treatment? Did you buy liquid form for oral use? I want to approach my GI to try vancomycin, but not sure how doc would react .
She is taking OV (oral vancomycin). It is a generic drug so my insurance charges generic rate. I understand the liquid is cheaper but pills are much easier. The pills are expenisve if you don't have insurance. Let me know if you need more information or help. you can always call me or email me.
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