Quantcast

Primary Sclerosing Cholangitis (PSC) - Online Support Group

Why is diagnosis taking so long?

Hi, everyone. I'm new here, and very frustrated.

About a year ago, my husband started having stomach discomfort. We just assumed it was stress (at the time it started, I was pregnant with our very unexpected third child and he had tons going on at work) or that he was eating meals that were bothering him. He was also having dark urine, but we figured he wasn't drinking enough. He probably should have seen a doctor, but he hadn't seen a doctor in seven years and wasn't about to start going.

Then, six weeks ago or so, we both came down (we think) with a horrible stomach bug. He had several days of diarrhea, I threw up for days and got so sick I ended up in the ER with dehydration. Then, about a week after his symptoms had passed, he noticed his eyes were looking yellow and realized that he'd had itching and light stools for a while. Concerned about jaundice, he went to urgent care.

Urgent care kept him for about five hours, did a blood panel and an abdominal U/S, and sent him home with a diagnosis of hepatitis. He had a follow-up that Monday where they were supposed to go over the results, but the blood tests results were there, so they ran the hep tests again. The following week, all the hep tests were negative, and they ran some autoimmune tests. A couple of those weren't ordered and had to be re-run, and he was in the process of waiting for those when he got really, really sick. He was extremely yellow, in horrible pain, dizzy, confused, and freezing cold. I took him to the ER, and he spent 9 days in the hospital.

At the hospital, they ran a bunch of tests: U/S, CT, MRCP, ERCP, tons of blood panels. They kept changing their diagnosis, but it was hard to know what was going on, because they were extremely uncommunicative with us. They released him 3 days after his ERCP (where they inserted a stent in his common bile duct that seems to be working some but not great; after the procedure he was in the worst pain he'd been in for about 48 hours). The ERCP showed, according to his GI, that his liver ducts "looked weird" (seriously, that's what he said). From the procedure reports we now have copies of, we know that from his very first U/S at the first ER visit, he showed intrahepatic duct dilation, so we're not sure why this came as a surprise at the ERCP? The ERCP showed "beading" of his bile ducts, and the tissue brushings they took showed abnormal cells. They sent him home with a diagnosis of PSC, although nobody has confirmed it. We were told they were sending his file to "experts" and at his follow-up we'd have a definitive diagnosis and a treatment plan.

Cut to this past Thursday (a week after his release). It appears that the GI did NOTHING since his release. They had no new information for us. The histography of the abnormal cells were suggestive of cancer, but we only know this because a nice resident there was honest with us (said this GI doctor was not up for the job of handling my husband's case) and gave us copies of all the files he had access to, where we were able to read that. The GI doc, as well as his PCP's, have told us nothing. I've asked question after question, and they won't answer even the most basic ones. Honestly, at this point I feel like I know more about both PSC and cholangiocarcinoma than either one of them does, and all I've done is spend a few sleepless nights on the internet. The resident gave us a referral to a GI oncologist he says is very good, and after hours on the phone, I was able to get my husband in this Tuesday. We hope we'll know more then.

Anyway, I'm just so overwhelmed and confused. Why is this taking so long? I'm reading about people being diagnosed with PSC after routine check-ups; how could they not diagnose this definitively after 9 days in the hospital? Why is there so little communication with us? If they knew ten days ago that his brushing showed abnormal cells and this cancer is highly aggressive, why didn't they get an oncologist in to look at him sooner (like when he was still an inpatient)?

We are both 34, and we've got a 7 year old, 2 year old, and 8 month old. This is all so completely out of nowhere.

Oh, and a couple more things:

I jump into action mode when things are wrong, so I made my husband an appointment at the Mayo Clinic for May 14th. We figure that, if we aren't happy with how things are going here, we'll keep the appointment. We have a $2500 out-of-pocket limit with our insurance, so we figure we'll worry about costs later.

And, I'm really not sure how to help my husband deal with this. Most of the time, he's vacillating between denial ("The doctors are wrong. I'm fine. Everything's fine.") and despair ("I'll be dead in six months so why bother trying to do anything about it?"). I feel like I'm trying to hold the household together, keep the kids happy and calm, and navigate all of the medical stuff. I've been spending hours each day on the phone talking to doctors' offices and ordering records and getting referrals and dealing with the insurance company. I understand that he's sick, but I'm not sleeping well, not eating well, stressed all the time, and not feeling all that well myself right now. I know that everybody deals with things differently, but denial/despair is not going to cut it right now. I need, like, hopeful, determined resolve. Or something along those lines. Because there's a lot to be done.

Has anybody else dealt with this in their marriage? How did you work it out? I really need my husband to be more assertive about his care and to take ownership of this. But, I know he is the type to sit back and just not ask questions or demand answers, so I'm afraid that if I let him do that, he won't get the care he needs, and this is too serious for that.

1 Like

Hi there!

First of all, I'm very sorry to hear of all your recent frustration and pending diagnosis. I'm certainly no expert on the disease yet, but I can offer you some insight from my experience.

I'm 24 and was diganosed almost 4 years ago. When I was 14, I had internal bleeding in my intestine and was in Children's Hospital for 2 weeks -- I literally almost died. At the time, they had completed a liver biopsy and found fibrous tissue that "could eventually turn into something" -- they didn't tell me I already had PSC. I was on steroids to fix the bleeding and then for about 6 years I was reasonably health with limited symptoms.

When I was 20 I started getting pain in my side and knew right away that it was my liver (personal instinct). I went to a family doctor, who sent me to a GI doctor, who sent me to a Liver Center. I wasn't diagnosed until I was seen by a doctor in the Liver Center. From my experience -- GI physicians don't have the knowledge or experience with liver disease to diganose PSC (even though GI and liver are somewhat related).

My diagnosis was created through review of bloodwork, but confirmed through a liver biopsy.

Next, I'd like to address your concern of your husband dealing with denial and despair. You can ask anyone on this board -- it's completely natural and it's going to happen more than not at the beginning. PSC isn't really a common disease, there's no cure and limited forms of treatment. It's a lot for anyone to deal with -- especially when you're still be diagnosed. I have my days where I'm high on life and feel like I can do anything -- to the days where I'm just so sick and tired of being sick and tired that I just want it to end. Before I found this group, I was in a point of utter despair. You can talk to friends and family about how you feel and what it's like ... and they can say they understand ... but you know they don't. Until you actually talk with someone who has the same disease, you feel very alone (been there done that) -- until you can say to someone "I was up all night itching" or "Dairy completely put me out for the day" or "I slept for 6 hours even after a full night's rest" ... and they say "yeah, me too" ... you know that no one truly gets it. I would encourage your husband to read some stories on this message board and ask his own questions to help ease his mind.

From a "treatment" standpoint -- obviously that would be at the discretion of his physician. However, I can tell you a few things from my own experience. Diet helps A LOT with managing pain. It depends on his own personal reaction to things, but I find that dairy isn't always my friend. Somedays I'm ok with it -- other days I'll get horribly sick and need to sleep it off. I also avoid a lot of sugar as it makes my liver hurt -- a lot. And other foods that are difficult to digest -- lettuce, some vegetables, etc. Spicy food is always something that seems good at the time but I typically feel it in a few hours or the next day (not so pleasant).

I would encourage eating lemons. My Mom did some research and a massage therapist I saw confirmed that lemons help with liver function. So, lemon water, lemon added to meals -- it does help. My bloodwork was improved after a few months of drinking lemon water daily. Milk thistle is a supplement that helps rejuveniate liver function so you can consider that.

Initially the doctors had put me on Urso Forte for my liver -- I'm not a good pill taker ... I just always forget ... but I got a gut feeling that I should stop taking it. Then about a year later, my doctor told me a new study shows that it does more harm than help -- so it's a good thing I listened to my gut about that one.

I hope this helps a little! Good luck and feel free to ask any questions you or your husband have. Everyone is very helpful here. :)

Loriersea,


Welcome and I'm really sorry to hear about your troubles. I can certainly share your frustration. It took them about 6 months to definitively diagnose me (and I was at Beth Israel and then Mass General here in Boston... so no slouches). This was after a bout of internal bleeding, three hospitalizations, and an original assumption of lymphoma.

My sister is a doctor, and she was really helpful in coaching me through all of this. Her advice (which was good) was to remember that when dealing with a serious illness, doctors need to be absolutely sure before they begin treatment. That coupled with the rare nature of PSC makes it a really, really hard diagnosis to nail down. The people who get diagnosed with PSC in a routine manner likely have advanced stages, and normal progression, whereas you could be dealing with a variation (for instance, my version of PSC has not advanced into the large bile ducts, which is really weird).


So first off, if you aren't happy with your doctors, switch. Totally doing the right thing switching to Mayo. The people of at BIDMC were nice enough, but taking way too long. So we got aggressive about contacting doctors and identifying individuals, and that's how I ended up at Mass General.


Second, insist on dealing with attending physicians. No knock on residents (I actually prefer them during procedures as they are hungry and hard working) but they are managing lots of patients, and don't have the experience of an attending physician. Make sure the attending is looking at your case, and insist on meeting with them at every appointment.


Third, hear you on the costs. If it is PSC, find out about an FSA. I hit my deductible every year since I've been diagnosed, and spend ~120/month on pharma. Getting that money out pre-tax has saved me like $1000/year.


Fourth, find someone to talk to. My wife was great during my diagnosis, but hearing your spouse has a chronic issue is ALOT to handle. He'll need you even more once he is diagnosed and during serious treatment. I'm not a therapist, but I will say having a support system for both me and my wife (her parents, my sister, my therapist) was a life saver. Remember that you aren't alone and that people want to help and support you. You've got this site, too.


Fifth, in terms of his own issues, remember what he is dealing with. He heard PSC, googled, and saw all of the crazy webmd statistics, and people on forums complaining about how they were slowly dying, etc. It's scary stuff. My advice would be to tell him to stop reading anything online (except us, of course). I'm 30 years old, three years in. Since my diagnosis, I've given up drinking, gotten in shape, gotten married, bought a house and a dog, got promoted to a new role at my firm, and am expecting my first child in July. Modern medical treatment is amazing, and he can do this. He just needs some positive reinforcement that this isn't the end, just another phase in life.


Finally, in terms of the assertiveness thing... believe me I can help there. I'm the "type B" in my relationship. I was never sick before PSC, hadn't been to the doctor's literally in 8 years before i was hospitalized. It's a tough transition, but again, with the right reinforcement and support, he'll get that he needs to seize control of his treatment and his life, otherwise he might be in trouble.


1 Like

Also, feel free to message me directly if you'd like to continue this outside of a public forum. I'd be happy to speak to you and your husband and share what we learned during our diagnosis. Believe me when I say this is a manageable disease, and you can lead a very full and happy life with PSC.

Thanks, Stacy and David, for your input.

I think a huge part of my frustration is that they are throwing around possible cholangiocarcinoma without being really aggressive about testing for it. That makes no sense to me. If the brushings from his ERCP shows cells that are suggestive of an extremely aggressive, lethal cancer, why aren't they, like, working around the clock to figure out if that is or isn't the case? I mean, I get that the doctors are busy, but this still seems like it warrants more attention than it's getting. We're hoping the GI oncologist tomorrow will be able to quickly and definitively rule out or diagnose cancer.

And, at this point, if it's "just" PSC rather than PSC w/ cancer, the PSC diagnoses will feel like a blessing.

Loriersea,

Any update? The cholangiocarcinoma thing is really worrying - that said, they throw it out there with most PSC patients (the scar tissue is more susceptible to developing it) so it could be that the abnormal cells aren't cancerous per se.

Regardless, I think the best thing to do would be getting that second opinion. How did it go yesterday (if that isn't too forward).

He had an appointment at the oncologist yesterday. It was okay. We don't know anything else, but the surgeon did feel confident, from looking at all of his tests so far, that if it is cancer, it hasn't spread at all outside of his common bile duct. They didn't think the abnormal cells were a huge deal, because they said the ERCP brushings generally don't yield many cells and they often look "weird." It seems to me mostly the size of the stricture (3 cm) that they are concerned about and that is causing the cancer people to think it could be cancer.

He's going in for an EUS, which they say should help to confirm a PSC diagnosis and give them more cells to work with to determine if it's cancer. If it is cancer, I'm taking him to Mayo. The surgeon he talked to has never worked with a patient with PSC and cholangiocarcinoma, and from what I've read, the Mayo protocol is really the best option for anybody with early-stage bile duct cancer.

The good news is that physically he's feeling significantly better. His jaundice hasn't gone away completely--he's still yellow and has a bad taste in his mouth--but he's much less itchy, had more energy, and his pain/nausea is pretty much gone.

Hi, I can really identify with your story. My husband had his first ercp and stent placement due to elevated blood enzymes in dec '17. Results of brushings came back c4/ suspicious for cancer. Fast forward to today july '18 following 3 stent replacements in the common bile duct and further c3 brushing results still no clearer. His case is complicated by ulcerative colitis which is flaring since mar/ april. Docs won’t or can’t remove the inflammation unless it becomes c5 or his condition worsens. Also not a lot can be done for the uc due to the possible cancer diagnosis. Its like a catch 22 situation and honestly terrifying…

1 Like