The Start

Hello all - this is my first post to the group. The forewarning is that the post is likely more of a general venting more than any questions, but hopefully sharing part of the story will comfort others under the concept of misery loves company.

My family member has been "diagnosed" with PSC. I quote the verb as it forms the basis of the story.

Last summer, my family member presented to an emergency room with upper abdominal pain and swelling which he considered extreme gas, possibly due to moderately recent abdominal surgery (approximately a year prior). Due to his age prior surgery and blood tests, the ER thought possible gallbladder issues. He spent over 4 hours in the ER. He was released after MRI's and other scans, and told to follow up with his GI specialist due to some elevated liver enzymes and suspect gallbladder as they believed the gallbladder wasn't emptying entirely, causing the problem. This was the first indication liver enzymes. All other scans - including liver - appeared normal.

Follow up blood testing and endoscopy, the GI specialist believed the gallbladder was the likely suspect. In January this year, blood tests still showed elevated liver enzymes, however, the GI specialist believed that was due to a potentially "spastic" gallbladder. He explained that, in layman's terms, there are two kinds of problem gallbladders. One is septic, and requires immediate removal. The other is considered "spastic," or acting up inconsistently, not fully emptying and therefore agitating surrounding organs. He recommended elective removal if there were continued problems and follow up with family care doctors in the meantime.

In March/April, he returned for a routine physical including blood work. Liver enzymes were again, "slightly elevated." The office suspected Hepatitis C, and asked for another sample. In June, a second sample was submitted, elevated enzymes, but no clear diagnosis for Hep. C. Referral to "the liver guy" recommended.

In July, "the liver guy" conducted the first MRI since last year's ER visit and eventual endoscopy. "The liver guy" complained that the MRI wasn't clear and wasn't properly done, that he thought there "could be spots indicative of cancer" but again, since the images weren't clear, he had no idea. In comes MRI #2, complete with gastrointestinal (GI) series (barium swallow). This yields no results, with the MRI techs complaining that they couldn't get "good" images.

On November 29th, we progress to a liver biopsy. The biopsy results come back on December 4th. On December 4th, at 4:30 p.m., a diagnosis is made. A nurse calls and, over the phone, says "you've got primary biliary cirrhosis." The nurse spells the words, and, to our shock, indicates that the life expectancy for this 30 year old is less than the family dog.

On December 9th, we present for a follow up with "the liver guy." Liver guy calls in sick. Sorry. Come back tomorrow? *sigh* December 10th: oops, sorry. Primary sclerosing cholangitis. No idea why the nurse gave you the incorrect information. Don't worry about the problem, doesn't matter what you do/don't do, what will happen will happen. Less than five minutes of conversation.

Here's the series of questions that went badly:

Q: are you sure this diagnosis is correct?

A: I've only had one other PBC patient who didn't have the blood markers, and that's not the case here, it's not PBC.

Q: how many PSC/PBC patients do you have? If it's two, we're at a 50% error rate.

(no response/changes topic)

Q: is this terminal?

A: no, unless you get cancer.

Q: what can we do to minimize risks and symptoms?

A: nothing.

Q: if he needs a transplant, can a living donor donate?

A: no, the donor will die.

Q: what are the blood levels at now - what about bilirubin?

A: everything's the same since last time.

Q: which last time? This started over a year ago - have you reviewed those records?

A: what happened then? It doesn't matter; the levels are the same.

Q: are there any medications he should take?

A: no, the medications that have been tried make things worse. I went to this talk this one time where this guy said… (yes, that is a direct quote!)

Needless to say, we have an appointment with the Med. College of WI.

Thank you all for reading the start to this journey. I apologize for the length, but the frustration and worry are causing many a sleepless night and it helps to share.

Sounds like you need to put that doctor's office in your review mirror. It is hard enough trying to get healthy without dealing with idiots like that one.

That stinks. I'm so sorry. I think many have a long story of how they got to their final diagnosis, and to a qualified medical team. And I second the recommendation about records. It is amazing how much you can learn by going through the paperwork! Good luck, and don't hesitate to ask if you need support!

sorry to hear about this, however elly didnt your relative show any signs earlier , or for that matter was he having ulcerative colities or crohns or irritable bowel syndrome ,normally there are high chances such diseases progress to PSC.

Oh LillyAnne I am so sorry to hear about all this. How frustrating this all must be for you! Yes you definitely need to find a physician that you trust. PSC is such a roller coaster disease you need to have all of your questions answered clearly. I hope you were able to get some better answers with the Medical College of WI. Good luck. Hope the rest of the journey goes better than the start.