MRCP & 2 HIDAS Advice Needed

I'm 30, history of common variable immunodeficiency, growth hormone deficiency, PCOS.  6 weeks ago had nausea, fever 102, vomiting, diarrhea, abdominal pain for 5 days.   After vomiting/darrhea stopped, had constant nausea for 4+ weeks, lost 10 lbs in 2 weeks and another 5 lbs since.   Had 2 more episodes of vomiting (after eating fatty foods).   2-3 weeks ago severe gas, abdominal pain, nausea and fever 1-2 hours after eating fatty food.   Prior lab results had shown UTI so next day was given Cipro.   Later in week had back pain between shoulderblades, went to ER because dr worried about pyelo but they ruled it out.   I had upper endoscope, mild gastritis, biopsies not back yet.  Had HIDA scan Fri, tracer did not leave gallbladder, could not see bile duct and intestines 'questionably faintly seen after 4 hours'.   Had U/S & MRCP, both normal, bile duct was 3 mm.   Had repeat HIDA today, again could not see CBD or intestines after 3 3/4 hours.   They did the EF (after I signed a waiver), and it was 94%.  Could my body not be making CCK on its own but work ok if given exogenously?  Or could it be sphincter of oddi problem since I've been on opiates for chronic pain for 15+ years?   I'd appreciate any advice, I've missed over 2 weeks of school.   The GI told his nurse he thinks I still may need GB out or exploratory surgery.    Being on gluten/lactose-free diet and the double PPI/H2 blocker have helped, pain & gas was much worse before those but still constant RUQ pain/pressure - feel tugging in RUQ..  Thank you.

[Christopher R Watters, MD]

First, I am very impressed with your thought process. I have had a particular interest in hyperkinetic biliary dyskinesia. Now that a commercial CCK test is available I plan to look into this further. i have had the same thought. Namely, is the high ejection fraction representative of the intrinsic situation or could there actually be low CCK with hypokinesia but up regulation of CCK receptors such that administering a constant amount of CCK results in an overly exhuberant contraction.

Second, a HIDA with CCK is very unreliable in the face of an acute illness. It would be very unlikely that a functional gallbladder issue would produce fever. However, the combination of systemic inflammation and chronic narcotics would significantly alter the measured motility of the biliary tree.

[warrenl7372]

I should also mention I've continued having fevers - 2-3 weeks ago when things got aggravated after the bridal shower, fever of 101-102.  After the week of Cipro, fever is now mid 99 (99.3-99.7).  

[warrenl7372]

Thank you Dr Watters, I'm actually a 3rd year medical student, so I've been trying to research all of this since I've been missing rotations.   Is there any way to prove low CCK with hypokinesia - is there a blood test to measure CCK?    

And do you think it's possibly sphincter of oddi dysfunction possibly due to medications (or exacerbated by meds).  I'm on multiple anticholinergics in addition to the opiates, which I know can slow motility.    Also is 94% EF indicative of spasm of gallbladder - I thought normal range was 35-75%, so is anything done if level is higher than normal?    I know my GI is thinking about sending me for cholecystectomy, is it worth doing if I might have a sphincter of oddi problem?  Or should I have ERCP with manometry done first?

Thank you so much for your help.  I'm tempted to come to Duke to see you (I'm in NJ).

Sincerely
Beth

[warrenl7372]

For your interest Dr Watters, I found this article on CCK and possible deficiency.   http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2718729/

[warrenl7372]

Also patient with autoimmune polyglandular endocrinopathy with CCK deficiency.    http://www.nejm.org/doi/full/10.1056/NEJM200101253440405

[Christopher R Watters, MD]

It is rare to be able to diagnose SOD with an intact gallbladder. When the morbidity of sphincter of Oddi manometry is actually higher than that od a lap chole it is generally worth the cholecystectomy first. This has been improved recently with the development of solid state catheters for manometry. Yes, CCK can be measured. My pathologists tell me that it is sent out, I think to Mayo, but it is available.

As  for SOD due to drugs, I'm not sure that I would label it as true SOD if it is an expected side effect of the medication. The fact that most people that take the meds do not have these problems would argue that there is more at play.

[warrenl7372]

I wasn't sure if opiates would be more likely to cause gallbladder spasm or SOD problem.    Is 94% ejection fraction ever considered a problem, if normal function is 35-75%?   Or is it just considered normal?  (I didn't know if it could indicate spastic gallbladder).

The one thing I'm worried about is if I have it removed, and do have sphincter issues, I know those people tend to still be sick or get worse after surgery.   I don't know if a surgeon would put in a stent at the same time as removing the gallbladder.

Thank you.  I'm not sure what to do, or if I could even find a surgeon to operate without a low EF and normal ultrasound/MRCP.

[Christopher R Watters, MD]

One of the questions is how the CCK was infused. There is substantial literature supporting a 45-60 min infusion while the most common seems to still be a 3 min infusion that has been shown to have a 30% false positive rate. A 94% EF would not be out of the range of mean + or - 2 standard deviations if a 60 min infusion was used. As for the GB vs sphincter issue, the results are mixed. In my experience, patients with EF's > 90% with symptom reproduction with CCK have a 90% chance of symptom resolution with cholecystectomy. This is based on about 40 patients. Theoretically, if the real issue is SOD symptoms could be made worse since the GB could be viewed as a "blow off" for back pressure. I haven't seen this clinically and believe that loss of the bolus effect is the predominant effect seen.

[warrenl7372]

I know the CCK was infused over 15 minutes (that's what they said anyway), and took pictures for 30 minutes.   But I don't know if my gallbladder was more ready to dump it more quickly since they waited almost 4 hours before giving me the CCK.  (and also like we discussed, if I had low CCK with hypokinesia but infusion of CCK upregulated the receptors causing hyperresponse).

A family friend who is a surgeon said he thinks 30% of people get worse after having gallbladder out, 30% have no change and 30% get better....do you think that's wrong?  (I believe he's referring to if there's no evidence of gallstones or low EF).

Do you believe it's worth going to a another GI specializing in biliary issues - is there any additional tests they might do?   And what's the best way to tell a doctor if you decided to go elsewhere (although he was planning to refer me to a biliary specialist anyway but I had ended up going to a GI closer to my home and wasn't sure how to tell him when he asked why he didn't have copies of tests I mentioned.    He was rarely in his office and I got frustrated that he didn't seem interested in my history and felt like he's just brushing it off as stress.  (I'm a lot more stressed after this than I was before, because of how it's affecting school).   I don't want to alienate him since he's near my school and I may have to interact with him in the future.

Thanks so much.   Also, what do you think of the articles I posted, have you seen them?

[Christopher R Watters, MD]

What happens to the remaining 10%? I know that you have been reviewing the literature about this. What has your research shown? By and large, if there is strong clinical suspicion of gallbladder disease and objective evidence of dysfunction - low EF, high EF, especially with symptom reproduction with CCK, delayed visualization on HIDA, the results of cholecystectomy have been quite favorable. I think that it is simplistic to think that the gallbladder is an isolated abnormality in dyskinetic patients but symptom reduction is the norm.

As for second opinions, remember that you are the customer. You don't need to worry about hurting anybody's feelings by seeking another opinion, especially when your case is complex and not clear.

[warrenl7372]

Hi Dr Watters,

Unfortunately just visited my Internist/ID and he wants to 'wait and watch' since my symptoms have improved a little on the PPIs.   He says he's worried about post-cholecystectomy, and thinks not seeing the bile duct/intestines after 4 hours means nothing as long as they saw the gallbladder.  He said it may be GERD...I originally felt like he believed me, now I feel like he's focusing on normal test results and not sure he believes me.    He says he doesn't feel I need surgery unless I get worse.   (and he brushed me off as soon as I mentioned about low CCK, telling me to stop researching.   I'm a medical student, I think it's normal for me to look into causes.  He says it's more likely to be an unusual presentation of a common disease, not anything rare.    My parents are very concerned and have come in with me and I feel like sometimes that turns doctors off, even though it's normal for parents to be concerned/protective.  I'm a little frustrated because I am a little better, the past 2 days I got through most of the shifts, but have kept having to go in late because I feel so bad in the AM until the Zofran starts working.

I really appreciate your help.  My parents said if neccessary we'd come to Duke to see you, we don't know where to go next or what to do.  I feel I can't perform at my best on rotations, even if I can force myself to make it through, I'm not thinking well or doing the best I can for my patients.  

[Christopher R Watters, MD]

I can understand the frustration. In reviewing your posts, I don't think that gallbladder dyskinesia would explain the original presentation. It would be bizarre to see a febrile, acute illness due to dysfunction. HIDA with CCK tests have to be viewed in the context of the clinical presentation, they are now useful as screening studies. In your situation, your gallbladder could certainly be adding to the misery but I doubt that it has been the primary problem. The other malady that seems to be quite common in these folks with hyperkinetic dyskinesia is alkaline reflux gastritis. Although there is no good test for it many of your symptoms would support this. If you were sitting in my office I would favor a month or so of sucralfate with or without metaclopramide as a presumptive treatment for alkaline reflux gastritis. Only after this failed would I consider cholecystectomy.

[warrenl7372]

I was on Reglan for a few days to a week when I first got sick, it didn't seem to make a difference, and I've been on double dose PPI & H2 blocker for the past 2-3 weeks...it has helped somewhat but still having trouble functioning.   Is it possible that first week really was gastroenteritis, and since then it's something else?   (I was on my peds rotation when I first got sick).  The reason why I've been so suspicious of the gallbladder is that this is my 3rd or 4th attack in 10-15 years, and I believe all of them seemed to come on after eating fatty food (one was when I was in my teens on a cruise ship, after eating the rich sauces for a few days, then could barely eat for the rest of it).    And would alkaline reflux explain why the common bile duct and intestines weren't seen after almost 4 hours on 2 HIDA scans, or does that not mean anything since the MRCP was normal?   Thanks so much for your help.  

[Christopher R Watters, MD]

The initial presentation sounds much more like gastroenteritis than anything else. The fact that your LFT's are normal and the MRCP was also would argue that the HIDA finding of CBD not showing up well and a delay in duodenal visualization are not significant findings. If it were to be alkaline reflux all the polypharmacy to reduce stomach acid could actually worsen the problem. That's where the sucralfate comes in.