Is cholangitis an ER-worthy event? (and my intro)

General
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I’m new, so this is an intro, but I also do have a question (hence the title) and figured I’d tackle both.

I’m 32/M and live on Long Island in NY. I was diagnosed with hemophilia (from mother) at six-months-old, and UC (from father) at 27-years-old. Apparently, like my parents, the two don’t get along well. It’s already difficult for colon-related bleeding to clot, so a lack of clotting factor does not help too much. Pretty much any major flareup involving bleeding is a life-threatening event. I was hospitalized four times for such events between 2012-2014—with the worst event landing me in the ICU for 20 days. I started Humira in 2014, and while I do have persistent issues, none have involved bleeding since. Medical cannabis also seems to help as well (score!).

Almost a year ago, I started having an intense pain in quadrant II of my abdomen. It would reoccur every once in a while, but not too often. This led to my old GI sending me for a CT scan, which led to my old GI diagnosing me with PSC over the phone/failing to impress upon me the gravity of my situation, which led to him becoming my old GI. Of course, the apple didn’t really hit me until I circumvented his advice, and visited various unsettling WebMDesque websites. (I’m a millennial, but quite good at identifying reliable sources.) I was not pleased.

Fast forward to a new GI, and a new old hepatologist, that were both part of the Mount Sinai Hospital system—which has a very good GI/liver program. They had this famous doctor guy you may have heard of: Burill Crohn. They are also fortunately relatively close. Anyway, Mt Sinai is in Manhattan, and if you’re familiar with the area, you’ll know traveling there via automobile is not ideal. So, I found one of their hepatologists that practices on Long Island. My appointment with her was two months ago—at which point the pain was much more frequent. I had an MRCP beforehand, and during my appointment, the hepatologist remarked that one of her colleagues is a PSC expert (red flag), and she’d be sharing the information with him. She was adamant that my quadrant II pain was a coincidence, and actually led them to discover the PSC. No immediate diagnosis for the pain besides a “not PSC” diagnosis. Her main theory was that I would have other symptoms other than the pain if it were a cholangitis attack. I then received a call from her assistant a few days later confirming my PSC. I was not pleased.

But, I was too angry about something else to really care after talking to several PSCers online. They had, in fact, experienced a similar pain + no other symptoms, but still = clogged ducts. SO, my pain may have been, and likely is, due to PSC after all (as per the internet).

Today, I made an appointment with a new new doctor, to occur on 1/24 (22 days), with the director of Mt Sinai’s Autoimmune Liver Disease Program. So, I’m thinking he may know a bit more about PSC, but he’s a hike (will probably take ~two hours, partially on foot through NYC).

So here’s my question[s]: over the past couple of days, I have become lethargic, and have been experiencing some itchiness. My appointment is in 22 days. It may very well be the medical cannabis, but I’m paranoid. If my ducts are in fact hardened or clogged, does every day count? Am I damaging my liver worse every day? Would I have like an emergency-ERCP if I went to the ER? (Obviously I don’t mean an emergency ERCP, but could a trip to the ER lead to admittance and ERCP.) Keep in mind: it will be a less-than-ideal situation if I hoof it to Mt Sinai ER, and they basically tell me to wait until my appointment. I would be, genuinely, not pleased.

Oh yeah, and I still have to find out how my hemophilia is going to play into… Every facet of my PSC treatment… Including ERCPs… And colon cancer screenings (biopsies are a sore subject).

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Hi there, do you mind me asking who your heptologist is? I live in Westchester and after changing my heptologist 2 times (once for the same reason that it seems you may have- a casual bomb drop of oh yeah, it’s PSC followed by me becoming Dr google and sobbing while planning my early funeral)
I also ended up at mount Sinai. It also takes me over an hour and is a huge pain to get to Manhattan so I found that the doc I wanted to see practices on Westchester as well and have been seeing her there. I have also brought up pain in my right quadrant and was told it was nothing to be concerned about… I get bad itching spells as well and it gives me major anxiiety that it’s causing long term damage. ERCPs are not an option for me since my first one left me hospitalized for 8 days with pancreatitis.
So while I can’t answer your question, I can relate to some of your Concerns, confusion and frustrations. I hope you find some clarity and answers on here!

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Spraytanner,
Hello and welcome. Thanks for your post. From everything you are describing you need to next have an ERCP procedure. The gold standard for 100% accurate diagnosis of PSC is an ERCP.

  • Make sure the hepatologist you are seeing is tied with a major hospital that does liver transplants.
  • Make sure whomever does the ERCP is someone who does this procedure day in and day out. DON’T let your local GI perform this procedure. It is a very invasive but necessary tool for the PSC patient. Once you find a doctor that does this advanced procedure, try to stay with him/her every time you need one. Over time they will become very familiar with your duct system and there is less opportunity for a mistake.
  • They are not likely to do an ERCP in an emergency context. If you will see your hepatologist and have the orders placed, any major transplant center should be able to get it scheduled within a few days if the need is great enough.
  • What is your bilirubin level currently? How’s your jaundice or itching presently?

We are here for you to help in any way we can, but it’s critical you find the right specialist to manage this disease long-term. Your transplant hepatologist will be your advocate to the transplant committee in the center you are going to to help you get listed when the time comes one day. I encourage you to always take your spouse or another family member or close friend to your appointments, to take notes and help you remember the questions needed to be addressed. You need an advocate for you that will speak up when you aren’t able to speak as well for yourself. Good luck and let us know how things go.

Mark
PSC 2011 / Liver Transplant 2015

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Spraytanner.
I recently went to hospital convinced that I was having my first cholangitis attack after having being diagnosed with PSC over 4 yrs ago.

Anyway this belief was derived by the much research I did on Dr google and in part by some of the commentary on this site (which I am gratefully for).

However as with most things they dont always pan out the way you think. Turns out I had a total blockage of my bowel in my right quadrant which is where I had previously had a colectomy bag when i first lost my colon to colitis.

So my advice is to listen to your body first and then use the info you have at hand to inform your own response. You will know best. Mark and Jeff from this forum are excellent sources of very good advice as are all contributors, however we are often talking about our symptoms and body which isn’t yours. Find a Heptologist you like and trust their competence is often hard to determine initially, because a saying here in Australia is “bluff it until you know it” and unfortunately i have found this applies to all professions. Good luck

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I also can relate. I was diagnosed the same way; had UC and was having bouts of acute right upper quadrant pain that turned into chronic right upper quadrant pain.

Now the pain is daily and every once in awhile will come with extreme itching and a feeling like my brain is working in slow motion. Guess what? None of my doctors will say they think any of my symptoms is from psc because my lfts are currently, in there words, “stone cold normal.”

I also cannot have another ERCP because my second one left me with pancreatitis which almost killed me. So now I’m left to wonder if everytime I feel I’m having some sort of acute issue, is anything an emergency anymore or should I just ride it out on my own? I’ve decided that only 2 things will make me seek emergent care: a fever which could be acute cholangitis, and uncontrolled severe pain. I know everyone’s situation is different and having additional Comorbidities such as hemophilia can complicate the picture and change what constitutes an emergency.

Hope this helps. What I think the hardest thing for me is coming to terms with the fact that none of this can be fixed and each time a new symptom rears it’s head, I wonder, is this temporary, or is this just the next symptom to show up to the party that is my failing body.

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Koriann,
Hi. I wonder if you would expand some on not being able to have another ERCP due to a bad bout of pancreatitis. The initial question that comes to my mind if you don’t mind answering, did a doctor in advanced endoscopy procedures perform the ERCP or was it your regular GI doctor? In addition, did the same doctor perform both ERCP’s? Thanks.

Mark

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I’m no expert, but from my personal experience, here’s my $.02.

1.) The pain / pressure feeling with most Cholangitis attacks is not life threatening and not usually worth an ER trip…but I would track how often it is and how bad it is and share that with my specialist. I typically start to worry or go to the hospital if I start getting high fevers. It could be a sign of Sepsis.

2.) After tracking things on my own and discussing with my doctor, we came up with the solution of perscriptions me antibiotics that I keep at home… enough for a 7-day treatment. When I get a bad attack and a fever starts I take that right away and E-mail him. If it’s a Friday night or something, this greatly accelerates my treatment and prevents it from getting to the point where I’m in the hospital. He will usually contact me the next business day and after a few days refill my perscription so I have it on hand for the next episode. We usually use Cipro.

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I had a couple of cholangitis attacks this summer. I went to the ER both times after feeling awful progressed to throwing up. The first one, I was in a remote area, they consulted with the on-call from my facility and sent me home with antibiotics. That cleared it up, but I had a second one a couple of months later. This time I was at home I came away from the ER (always on Saturday night, of course) with an ERCP scheduled for Monday morning.

I tell this story just as an example of how serious they considered it. Of course the ERCP was decided on after scans in the ER that showed blockages. It isn’t an automatic thing, since (as others have mentioned) it is a very invasive procedure.

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I echo what Bob said…everyone is different and reacts differently due to different levels of overall health and severity of infection.

For myself, i progress from first symptom to sepsis in a matter of 2-4 hours. This includes temps of 104+, racing pulse and low blood pressure. This buys me 3-4 days of IV antibiotics.

E

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What Frank said about keeping a filled prescription of Cipro on hand was what I always did as well. You need to be able to start it as soon as the infection hits to hopefully avoid an ER visit. Yes, definitely ask your doctor to write you one to have available, with refills.

Mark

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We definitely do have a lot in common! I had also selected my hepatologist based on proximity (there’s not too many Mt Sinai liver docs, so finding one practicing close by is rare), and I think that’s where I went wrong. I should have realized as soon as she told me she was going to forward my info. to her PSC-expert colleague. But, I didn’t really know there were doctors that actually specialize in PSC. While I’ve had the pain for almost a year, I just started itching and getting lethargic like three days ago, which led to paranoia that I was permanently damaging my liver. I spoke to three different people on Reddit with PSC that had the exact same pain, and no other symptoms (blood tests normal), and it was in fact the PSC. I mean, it does make sense. My bile ducts are inflamed, and I have a pain right in the area where they’re located… Duh…

Anyway, I’m not sure that I should share doctor info. for all to see, so I messaged you.

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Thank you for the reply and advice. I surely do need an ERCP, and I’m sure that’s what my new doc will recommend. I can’t get a biopsy either, so an ERCP seems to be my only choice.

  • Yes, the new hepatologist is part of a renowned liver transplant center.
  • The new hepatologist I’m seeing specializes in autoimmune liver diseases, so he may be the one doing the ERCP (not sure yet). It shouldn’t be an issue if one of his colleagues is the one who does the procedure, right?
  • Yeah, I realized that I was probably just giving myself anxiety. It seems like everyone with PSC lives/deals with the symptoms I just started having three days ago: itchiness and lethargy. I only had pain before. Whenever my UC acts up, it’s right to the ER, so that’s kind of the mentality I have I guess.
  • I don’t know what my bilirubin level is. I was just recently diagnosed, and all my blood work has been normal so far. The only thing that confirmed my diagnosis was my MRCP results. Since I just started feeling the itchiness, I’m not even sure if it’s PSC related, or in my head. I get random twinges of itchiness on random spots of my body–not typically on the same spot, but usually on my lower legs.

I did not actually know that my hepatologist would act as an advocate. That makes me happier knowing I’m getting a new one. Thanks for the advice, and I will keep updating.

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Dr Google can certainly go either way. Thank you for the encouragement though. I recently had a colonoscopy, and my GI didn’t find anything that would have led to the pain I’m having. My body is telling me that my bile ducts hurt, haha.

I always used to view doctors like they could make no mistakes, but I’ve certainly learned from that. It seems like no doctors have any clue when it comes to my hemophilia (besides actual hematologists). But, since this doctor I’m complaining about IS a liver doctor, I didn’t expect an incorrect response. This will be the third time I’ve switched some sort of doctor in the past three months, so I’m certainly taking things into my own hands now.

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Yes, it does help to hear I may not be crazy thinking this pain is related to my PSC. I’m not really used to just dealing with something being wrong, but I’m seeing that PSC symptoms are something I may just have to deal with. I certainly have not come to terms with the disease. Probably since my doctors have seriously failed to really tell me everything about the condition. I didn’t even know it led to transplant until I searched online. But yeah, I think the hemophilia gave me an ER-for-everything mentality. I had to go to the ER after cutting my pinky a few months ago… haha. My pain certainly is getting worse though. It was just every once in a while at first; but it’s stuck there nagging at me like a piece of corn in my teeth. I definitely am nervous about pancreatitis. But, I guess we’ll see if they are even able to perform an ERCP on me.

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Hi. I live in NJ. While Mt Sinai is a great hospital, the BEST liver doctor in NY is Dr Robert Brown at Wile Cornell. He is one of the top people in the field. He was referred to us by Dr.Pompeselli who at the time was at Leahy in Boston. He is also a personal friend. My son was diagnos3d with PSC in June, 2015. Dr. Brown is managing his care along with two surgeons and a host of others at Wile Cornell. They have been amazing! I could not be happier with the care he has been receiving. Now if he would do what he is supposed to (my son). But answer your original question, yes PSC is definitely an ER visit. But to the right hospital. It is also a qualification for disability! Good luck to you. Who is your doc at Mt Sinai? And yes, you really should be in the city. I know it’s a royal pain from LI. It’s also a pain from NJ!

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It’s funny I’ve had my diagnosis for 9 or 10 years now and I also have not come to grips with the whole thing. The only thing I really understand about PSC is that it’s not well understood. That gives me comfort when it comes to my pain. Especially when I hear that others with the disease have similar pains that boggle their doctors. I do have pain everyday-when it first started it was very sporadic. I see a pain specialist that my gastroenterologist set me up with and I’m not kidding when I say he saved my life. I’ve found a way to deal with the pain and keep it at a tolerable level. For the first few years after the pain started, I wanted the answer so I could find a way to get rid of it. 10 years later, no answer, and no pain relief. But I’ve learned to cope, and learned to live a full life while experiencing this chronic pain. I still work full time even though as I’m telling you this I can’t believe that’s true. Some days are really tough. But i tell myself I have only this one life to live so I’m going to do the most with it that I can. I just got back from a vacation with a girlfriend who doesn’t mind that most nights I want to be in bed at 8, or at least in my pjs :slightly_smiling_face:.

If I struggle, I know that you must as well. The hemophilia on top of the UC and PSC; some people have all the luck, right? For me the name of the game is no interventions until transplant because of my pancreatitis. I just get my yearly MRCP and colonoscopy to Check for cancer, and frequent lab work for monitoring, and take my medications. I’m honestly not sure about your case-there May be something they can do for you. I hope you find it.

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I hate to rock the boat but my liver specialist (Dr. Daniel Pratt), at Mass General, as well as other liver specialists I’ve seen over the years, told me – (aside from the invasiveness and the risk of pancreatitis… I’ve a friend who was in a coma for three months after an ERCP and ended up with necrotic pancreatitis) – that there is evidence ERCPs increase the progression of our biliary disease & further infections by introducing damaging bacteria into the biliary track. So, there is another side to this conversation.

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UPDATE: So, I went back to my current hep dr. on Friday (the one I have doubts about). I had scheduled the appointment months ago, and was interested in the results of my recent MRCP.

While she hasn’t officially done it, my dr. is contemplating rescindment of her PSC diagnosis. My MRCP showed a significant reduction of liver duct inflammation, but no reduction in my pancreatic duct inflammation. Based on this (since there is still something causing inflammation, but not specifically concentrated in my liver ducts), and the fact that my blood work has been normal, she believes there may be an issue other than PSC. It could be a pancreatic issue; or, she is leaning toward ampullary stenosis. This would make sense, because my pain has been getting worse while my inflammation has been getting better. However, my gall bladder is fine, with no stones—which are typically present with ampullary stenosis—so she’s puzzled by my case. I’m not sure if you’re familiar with ampullary stenosis, but it’s benign. It could also be causing my pain.

After doing blood work, she also figured out why I’m super lethargic: I have some sort of rheumatoid issue (or, the levels in my blood work indicate I do). This would make sense, because my lethargy has been more intense in my muscles. The itching I was experiencing must have just been placebo, because it’s pretty much gone now.

I’m still going to see the PSC expert later in the month; a second opinion may be even more important now. Clearly it’s frustrating not knowing what’s wrong with me, but I guess I can deal with that frustration if it means there’s a possibility I was misdiagnosed. I’ll keep updating after I get more fun news.

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I want your job - spray tanner in Chief. (assuming its the sun protection spray tan lotion applied on some beach with massive surf)

I feel for you with your lack of diagnosis. My story started off with a diagnosis of coeliac’s then two days latter yes you also have crohns. Treatment for years which didnt work, then toxic megacolon and the removal of the colon. Told didnt have crohns by UC instead. All good until my eye sight started going, then told had rare form of cancer which was strangling my optic nerve. Wrong again just some inflammation apparently.
Then get told I had PSC but maybe not, then 4 yrs latter oh yeah it was PSC because it hasn’t gone away.

So spray tanner do what your body tells you is right, enjoy life as best you can and stay as fit and health as you can so you can spend as much time worrying about put spray tan on and enjoying your loved ones company.
We work one third our life, sleep the other so fill the last third with what you can. I have a promise I made after my colon loss and that is never say no. I try and do things people ask not because I want to do it rather they have asked for a reason and to share my company so hell thats whats lifes about.