I’m not yet 100% diagnosed but well on the way to a small bileduct PSC dx. In Feb I had what I thought was a nasty virus, but when I turned yellow and developed purple splotchy rash I was sent to Emergency. There I was told I was having a reaction to antibiotics, ‘flucoxacillin toxicity’ a rare but known side effect.
As the months have progressed I have had various tests including MRCP x 2 which came up clear; ultrasounds x 3 all clear; weekly blood tests which showed my liver function levels to be high to critically high and 2biopsys.
My first biopsy was horrible, done with ultrasound and a quick needle sample taken, I was then in intense pain which was not ignored but not taken seriously until the next morning I was sent for CT where internal bleeding was discovered. So I had pain for the next 10 days as my body absorbed the excess fluid in my abdominal cavity. This biopsy was clear. I am allergic to morphine, endone, fentynal and pethadine, so pain relief options are limited. My second biopsy was done under CT. this 40 min procedure was done without pain relief and involved 2 samples being taken. For each (excruciating) movement of the needle I was pushed back into the CT machine for a scan before being pulled and and the needle moved to the correct position before the sample was taken. It took nearly 3 hours for me to get the upper hand on the pain. Thankfully no internal bleeding this time! However this biopsy showed evidence of inflammation and ‘scarring’ in and around the small bile ducts of my liver.
That has instigated the suspicion of Small bile duct PSC. The only thing stopping the diagnosis is my blood tests taken only 2 weeks before my illness show perfect liver function levels. My Hepatologist is hopeful it is still just the antibiotics reaction and I’m taking longer than excpected to heal, however they will perform another biopsy in 2-5 months to see how the damage is looking in my small bile ducts.
Where this all leads I don’t really know as there is limited information on flucoxacillin toxicity, or SBD PSC.
I have lost my yellow colour although my latest bloods show my bilirubin is creeping up again. My itch is head to toe but especially my feet at night. I am extremely tired and have had to drop my full time work hours to 4-6 hours 3 days a week.
How do others manage working with this condition?
A bit more about me, I turn 39 inext week, I have 4 children 15, 12, 11, and 6.
I returned to work 2.5 years ago after our house was hit by lightning and burned down, so I work to pay the mortgage on our newly built house.
Hubby is a paramedic, which has been great from the medical side of things as he understands a fair bit.
I was studying nursing full time as well as working in an emergency dept as the data manager.
Thanks for reading my bit so brief story, I look forward to reading more about everyone’s journey
Hi Mel,
Welcome to this PSC forum. Thank you for sharing your story and journey thus far. We are here for you to help in any way we can with information and resources about PSC. You mentioned you were having a lot of itching which would be due to the increased bilirubin levels. You might ask your doctor to prescribe a drug called Rifampin. I took 300 MG twice a day and it pretty much eliminated my itching by at least 98%. Others have had success with other remedies and medication but this is the only thing that brought relief to me.
One question for you. Has your hepatologist ordered an ERCP to check your bile ducts? This is a different procedure from an MRCP. This would most definitely be able to confirm the PSC diagnosis. Take care and keep in touch.
Hi Mark,
My drs have actually put me on multiple different meds including rifampin to try and help my itching. Unfortunately so far nothing has been successful. I have always responded oddly to meds.
No ERCP yet, from what I understand my larger bile ducts are clear and it’s only my microscopic ducts that are affected. It was suggested earlier on but the second biopsy results over turned that path of investigation.
Although I may ask at my next appointment again.
Mel,
I’m no doctor but if your itching is that intense you’ve got to have a blockage in your common bile duct. Elevated bilirubin equals bile not flowing. Bile not flowing equals a stricture. I’d press them for an ERCP.
Everyone acts like these biopsies are no big deal, but like you I’ve found the pain to be out of this world. If you press hard enough they can do these while you are sedated. I went into shock after my last biopsy and this doesn’t really help to get good results if it happens during a prolonged procedure.
It sounds like your team is on the right track. Your MRCP was clean so an ERCP probably isn’t warranted in this case - they are pretty much used only for therapeutic purposes these days and you don’t have any strictures in the accessible ducts.
Small duct PSC can cause pruritus despite having no visible strictures. What meds have been attempted? Some things to try include antihistamines like Benadryl to help sleep, SSRI antidepressants, cholestyramine, ursodiol, naltrexone, UVB phototherapy (sun or prescription lights, commercial beds are usually UVA), and plasmaphoresis.
Hi Mark, I agree I feel as though there must be a blockage stoping my bile being excreted normally, however I’m not yellow (we’ll only a tiny tinge left), I will see what my next blood test reveals and ask about other investigation options available.
JTB, I’m so glad someone else found the biopsy to be horrendous, I seriously thought I was being a bit over the top in my pain response. The next biopsy I plan to demand sedation as I don’t think I could lie still knowing what’s coming.
Medication:
I was put on the highest dose of antihistamine phenergan (Australian brand) which didn’t do anything, usually 10mg knocks me out for the night and here I was tossing back 75mg like they were tic tacs.
I’ve tried this awful tasting powder, cholestyramine anhydrous, which made me ill, and naltrexone GH which was a special trial by my specialist to help with itching, also little effect but bad side effects.
I’m currently on ursofalk. I’m not sure why but they don’t want me to stop this even though there is no change physically.
I haven’t tried the antidepressant that you suggested or the phototherapy, I’m not sure if just sun is the same but I found standing in the heat of the sun increased the itch, thankfully it’s winter here at the moment so I’m not as bothered going outside, for now.
I’m currently on a wheat free, dairy free, nightshades free, no onion, garlic, lentils, or peanuts, low fat (flavour free) diet. I’m struggling to find filling meals but I have noticed a huge change in my digestion. I cheated and ate the kids leftover spaghetti with creamy pumpkin sauce yeasterday and boy today was cramp city. Lesson learnt!