Overview of my partner’s experience with hilar cholangiocarcinoma
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Elizabeth….recalling the numerous communications between us, please take comfort in knowing that you tried moving mountains in effort to conquer this cancer.
Hugs,
MarionHi Marions,
Thank you for the kind words. I miss Peter so much, but I am healing.
And yes, agreed! I’m not done filling out all the questions – am hoping just to find a few more documents – but I’m so glad the registry is happening. I hope we find more answers.
Hugs back to you,
ElizabethElizabeth….thank you for taking the time and post this important information. You are a kind and caring person and I hope for your heart to heal in little steps, one day at a time.
Your posting contains the type of information desperately needed for inclusion in the International Cholangiocarcinoma Patient Registry:
http://cholangiocarcinoma.org/professionals/research/patient-registry/Thanks again, dear Elizabeth, my thoughts are with you.
Hugs
MarionHi All,
I know some of you are seeking out patterns in people’s medical histories. In case it’s helpful, here is a fuller version of Peter’s medical history, as written up by the Mayo Clinic during his last hospital visit there before returning to Alaska (interventions once we returned to Alaska were minimal).
[From 14-Jul-2015]
Age: 29
Sex: M
Patient is a 29 year old male who was admitted post ERCP (Endoscopic retrograde cholangiopancreatography) and PTC (percutaneous transhepatic cholangiography) for observation.He has the following oncologic history
1. ~2007: Diagnosed with ulcerative colitis, previously treated with Imuran, Asacol, and a single course of prednisone. Not currently on medication. Controlled with diet.
2. ~2008: Diagnosed with primary sclerosing cholangitis, not currently on immunosuppressant therapy. Controlled with diet.
3. October, 2014: New jaundice, pruritus, and fevers. Received antibiotic therapy.
4. March 4, 2015: Local Gastroenterologist recommended abdominal ultrasound due to rising CA 19-9 levels. This showed central intrahepatic bile duct dilation.
5. March 20, 2015: ERCP showed left main hepatic and right main hepatic duct with multiple diffuse stenosis. Stents placed. Biopsies with brushing performed which showed abnormal glandular proliferation. Development of post-ERCP pancreatitis resulting in hospitalization for one week.
6. March 31, 2015: Presented to Mayo Clinic, Department of Gastroenterology, Dr. LaRusso; recommended further work up.
7. April 1, Ultrasound of the abdomen showed subtle isoechoic central hepatic tumor, likely cholangiocarcinoma, results in intrahepatic bile duct dilatation involving both the right and left lobes. Stents are noted in the extrahepatic bile duct. Hyropic gallbladder.
8. April 3, 2015: MR Elastogram performed which showed an approximate 5 cm hilar mass obstructing both right and left lobe bile ducts (both massively dilated), highly suspicious for cholangiocarcinoma. The biliary stent seen on outside CT is below the level of the mass and isn’t decompressing the biliary tree. There appears to be direct invasion of the mass into the left portal vein near the bifurcation, and at least 6 distinct lesions in both hepatic lobes suspicious for metastases The hilar lesion has increased from about 4 cm on 3/21/2015 to 5cm on today’s study.
9. April 6, 2015: Transplantation consultation with Dr. Poterucha; deemed ineligible for liver transplant.
10. April 7, 2015: ERCP performed which removed one stent from the biliary tree. A biliary tract obstruction was noted, secondary to what appears to be a mass, in the bifurcation of the right and left hepatic ducts; bilateral biliary systems were trained with stents (2). Dilation and brush cytology performed which was negative for malignancy. FISH for biliary tract malignancy was abnormal indication a high suspicion for malignancy or high-grade dysplasia of the pancreaticobiliary tract. Biopsies taken at the hilar stricture which showed minute detached cluster of atypical epithelial cells in a background of ulceration and squamous metaplasia with acute inflammation.
11. April 8, 2015: Medical Oncology consultation with Dr. Grothey; recommended systemic chemotherapy, gemcitabine and cisplatin, once bilirubin levels decreased.
12. April 29, 2015: In Alaska, ERCP showed the hepatic duct bifurcation contained multiple stenosis. Dilation with a 6-mm balloon dilator was successful, and two additional 7-Fr by 12 cm plastic biliary stents were placed.
13. ~May 2015: He started to present with fever and chills, and he underwent placement of an external drainage catheter. He seemed to have been started on meropenem for possible recurrent cholangitis (though this is unclear) with no planned stop date.
Subsequently post PTC insertion his bilirubin does not appear to be improving following placement of the percutaneous biliary drain. Therefore plan was to have the stents removed with an EGD and place a percutaneous biliary drain in the right duct. He therefore was planned to undergo a right side PTC and a left cholangiogram to make sure the left percutaneous biliary drain is in position.
Today he underwent an ERCP in which:
-Four stents were removed from the biliary tree.
-A localized biliary stricture was found. The stricture was secondary to primary sclerosing cholangitis.
-One plastic stent was replaced into the left hepatic duct.There was a plan to perform RFA, however given the bleeding from the duct post stent removal and necrotic looking mass at the hilum it was deferred. He then subsequently underwent PTC.
Under interventional radiology he underwent
1. Cholangiography
2. Placement of a 10-French Cope biliary drainage catheter in the posterior right biliary tree
3. Injection of the 10 French Mac-Loc drainage catheter in the left biliary tree. The left side looked to be draining appropriately.On arrival to the floor, he was well. Had no acute complaints. His vitals were stable.
[Written 15-Jul-2015]He then had a repeat procedure on 7/15 where there was:
1. Conversion of the right hepatic lobe biliary drain to a 10F BCL with locking loop in the duodenum across the ampulla. Additional sideholes fashioned to extend across the obstructing tumor.
2. Conversion of the left hepatic lobe biliary drain to a 10F BCL with locking loop in the duodenum across the ampulla. Additional sideholes fashioned to extend across the obstructing tumor.These 10F tubes were found to not drain appropriately and his percutaneous biliary drain was noted to drain a milky white purulent fluid. He was taken back to IR and both the right and left transhepatic biliary drains were then externalized and exchanged for 8 Fr. Locking BCL catheters with pigtails repositioned in into the peripheral dilated ducts.
He felt well after the externalization procedure, and remained afebrile. His leukocyte count was noted to be 23, increased from 15 the day before. During the admission we also consulted ID given the fact that he was on meropenem. They recommended:
1. Please continue Meropenem 500mg IV every 8 hours to complete 14 day course ending 7/28.
2. If he is still in town after finishing IV therapy, please call Infectious Disease and set up a follow-up appointment.
3. If he returns home before his IV antibiotic course is complete, please arrange followup with Infectious Disease with his home doctor.
4. At this point we would recommend eventual transition to oral suppression with alternating weekly courses of Moxifloxacin and Augmentin. This may be adjusted through his outpatient ID followup.
[Note from Elizabeth: Peter remained on Meropenem – fevers returned whenever he was taken off of it]Past Medical/Surgical History:
1. Metastic hilar cholangiocarcinoma
2. Primary sclerosing cholangitis
3. Ulcerative colitis, not currently on treatment
4. Recurrent cholangitis 2/2 #1Family History:
Unremarkable for any GI malignanciesFollowing his discharge from the hospital, Peter twice had outpatient paracentesis to drain excess fluid from his abdominal cavity. He passed away on August 11th, 2015 under at home hospice care.
If you would like information on the medicines Peter received while at Mayo, a more extensive writeup of an ERCP, Stent Extraction, and Single stent placement procedure he had while there, or the records from an ultrasound of his abdomen he had prior to his diagnosis, please feel free to message me. Excluding what is below, this is the totality of the records of his that I have.
Below, I’ve included pictures of Peter’s bloodwork from when he was at the Mayo Clinic in July as well as some bloodwork from that March, before he was diagnosed. (keywords I’m including so that people can find this post if they’re searching for these words: Hemoglobin, Hematocrit, Erythrocytes, MCV, RBC Distrib Width, Leukocytes, Platelet Count, Hematology AG CBC Aggregate, Hematology AG Auto Diff, Neutrophils, Lymphocytes, Monoctyes, Eosinophils, Basophils, Coagulation AG, Hematology AG Auto Diff, Coagulation AG Coagulation Tests, Prothombin Time, INR, APTT, Chemistry AG, Blood Chemistry 1 AG, Sodium, Potassium, Glucose, Random, Alk Phosphatase, AST (GOT), ALT (GPT), Bilirubin, Total, S, Bilirubin, Direct, Blood Chemistry 2 AG, Creatinine (w/eGFR), eGRF-Non African American, eGFR-African American, Albumin, BUN (Bld Urea Nitrogen), Chloride, Bicarbonate, P/S, Anion Gap, Enzymes, Amylase (S) )
Dear Elizabeth, I am so very sorry about Peter. Your post, I am sure will help a lot of members on our Board and what a wonderful way to have everyone remember Peter and his journey with CC by also helping others. In time all the good memories will come through and crowd out the others and that is the way Peter would want you to remember.
Death is nothing at all. It does not count.
I have only slipped away into the next room. Nothing has happened.
Everything remains exactly as it was. I am I, and you are you,
and the old life that we lived so fondly together is untouched, unchanged.
Whatever we were to each other, we are still.
Call me by the old familiar name. Speak of me in the easy way which you always used.
Laugh as we always laughed at the little jokes that we enjoyed together.
Play, smile, think of me, pray for me.
Let my name be ever the household word that it always was.
Let it be spoken without an effort, without the ghost of a shadow upon it.
Life means all that it ever meant. It is the same as it ever was.
Why should I be out of mind because I am out of sight?
I am but waiting for you, for an interval, somewhere very near, just round the corner.
All is well. Nothing is hurt; nothing is lost. One brief moment and all will be as it was before. How we shall laugh at the trouble of parting when we meet again!
By Henry Scott HollandDear Elizabeth, first, I am sorry in so many ways for how everything went. This is an older person’s cancer is what I was told, and 28 is certainly way too young. My path was very different, and my lucky breaks came one after another, I live in Cleveland, good for hospitals, bad for sports franchises, I was lucky to be referred to a surgeon who was one of the best, late in his career, had written 11 books on liver surgery and transplant. He was very good, I could understand him when he spoke, My hilar cc was found early, with a tumor that formed and grew in a very unusual way. It created a back up of bile in the left side of my liver. This makes the liver degrade and become “sick” , discolored and mushy. The assistant surgeon , a resident I assume, told me the left half of my liver appeared this way. One of my lucky breaks was to only travel less than twenty miles to get to the hospital or to see a doctor. Alaska is beautiful, and he must have seen a lot of beautiful things to talk about at the end of each day. I am also sorry you only had 4 months. Most stories don’t go like mine, I was 49 and I healed well, experienced minimal pain, and I remember hating sleeping on my back for weeks, and how nice it was to sleep on my stomach again. You are right, a skilled hand is everything, and now, a little more than ten years past surgery, I am doing well, and for the most part sans a big scar that looks like the mercedes star, You would not know anything happened here. Once again, I am sorry for the loss of your Partner, God bless ,Pat
Dear Elizabeth,
I am so sorry that you lost Peter to this horrible disease and at such a young age. All losses are heartbreaking, but when I see people around my daughter’s age diagnosed, it seems even more unfair.
Thank you for taking the time to share the good and bad of what you went through together. It is a tribute to Peter, and will help many more in the future.
I wish you peace and comfort as time goes by.
Take care of yourself,
CatherineDear Friends,
Below are the things we learned over the course of my now late partner’s illness. If you have any questions, please do not hesitate to ask.
**I’ve written many details below, but I think if I could tell my past self one thing it would be this: Find one of the best doctors in the country for your partner’s type of cholangio. Go and consult with this doctor. Cholangiocarcinoma is rare. It would be difficult for every doctor to know all the information out there about every rare disease. Even a nice, competent doctor will likely not be well versed in all the best treatments in cholangio, and even a nice, competent doctor will likely not tell you this. So find a great doctor, and go to a major cancer center to at least make a gameplan for your doctor at home if you wish to be treated there.
I really wish I’d known all this. One other note before I get into details: My friends and I determined who the best doctors for my partner’s type of cc might be by doing general internet searches, seeing who’d published frequently and recently on the type of cc in question, and by looking at cholangiocarinoma.org lectures and discussion boards. There might be more efficient ways.**
Context (course of illness/more details in upcoming post): My partner was diagnosed with hilar cholangiocarcinoma at age 28 after being jaundiced, fatigued, and itchy for 6 months. His tumor was too large to be eligible for a transplant. After diagnosis, he lived for 4 months. He had been diagnosed with ulcerative colitis and primary sclerosing cholangitis (PSC) approximately eight years prior.
Some things that worked:
-FoundationOne gave us full financial aid for genomic testing due to our income level. For those of you not familiar with genomic testing: some cancers have genetic abnormalities that make them more responsive to certain drugs. We were trying to see if some drugs might be better than others for my partner.
-Medical Marijuana helped my partner with pain more than any other drug did (e.g., Oxycodone, Percoset, Dilaudid). It also improved his appetite. We consulted with Dr. Frankel at http://www.greenbridgemed.com for dosing instructions.
– Using “gravity balls” (aka positive pressure ball, IV medicine ball) for at-home infusions of IV antibiotics. My partner needed them for high fevers that kept returning unless he was on meropenem, which only comes in IV form. I mention this here because our first few doctors didn’t know at-home infusions of IV antibiotics were possible. The choice they gave my partner was either stay in the hospital indefinitely or stop the drugs and let the fever return. Eventually, we met a doctor who put us in touch with an at-home infusion center.
-Using meropenem to fight fevers that did not respond to anything else. Meropenem is such a strong drug they tried to take my partner off it twice and replace it with weaker antibiotics – each time high fevers that left him weak and unable to concentrate returned. They tried to take him off with good reason – if a body becomes resistant to meropenem there are basically no drugs left that will work to fight infection, but it was such a strange occurrence (that only one drug would work for him), I thought I’d note it here.
-Keeping in contact with the Rochester, Minnesota Mayo Clinic from our home in Alaska. Overall, our experience at Mayo was amazing. Although it sometimes took a few calls (they’re busy there!), the doctors were kind and responsive when we came across questions or issues that our doctors in Alaska could not help us with.
-Always politely asking “when do you think I’ll hear back” before the end of a conversation about results or scheduling, and then calling back if that date passed. CC progresses so quickly, and medical centers can be so overwhelmed that this felt important.
-Using skin prep protective barrier wipes before taping on bandages – my partner’s skin became very fragile and was damaged when tape was removed. A nurse introduced us to these wipes we never went back – if skin is prepped with them, adhesive is much more smoothly removed. **And, in general, asking if there aren’t solutions to small problems like this – often a solution is out there that wouldn’t occur to a non-medical professional, but the medical professionals around will assume someone else has taken care of it/it’s not a problem unless asked. Also, sometimes it takes asking a few people.**
-Asking for help from friends on research (and everything). There is so much information out there and so much to do! Before we realized **the best route is to find a top doctor (because a top doctor would have known almost all if not all of these treatments better than any of use ever could have)**, a friend of mine set up a shared research google doc where I could post questions about CC/treatment options and my friends would do research and post responses whenever they had time. Here’s a link to the document we created on the off chance it might be of use. Please note, it was last updated in summer 2015 and was specifically for my partner’s situation. The theme of the document is: which of these therapies might be useful for my partner?
https://docs.google.com/document/d/1npxFUhFL4d1RKZ-TsM0yuwEz3vNKCvXTpWhVsJSy3Ik/edit?usp=sharing-Telling each other about any beauty we’d seen at the end of each day. This was my partner’s idea. I don’t think it’s for everyone, but it helped us. I’m glad I wrote them down, and that I still have a list of the things we found beauty in.
One other note before I move onto the next section: We were introduced the idea of using Medical Marijuana by a friend of ours who was using it for her intrahepatic CC – she says anything could be responsible for her getting through many more rounds of chemo than any doctor predicted she ever possibly would, but she got through 18 rounds of gem/cis chemo tumor growth free with a tumor that was already at 12cm. Her instinct is that the Medical Marijuana likely did this. She also consulted with Dr. Frankel.
Things I wish we’d done differently:
-I know I said this first one above, but just to go into more detail it because it’s the most important message I have: I wish I’d found one of the best doctors in the country to treat my partner’s cholangio and gone to see him/her immediately. Going into this experience, I foolishly trusted all doctors. I did not understand how much more up-to-date and experienced some doctors are than others, especially with rare diseases. I did not understand how much of a difference that could make to patient outcomes (it’s a statistically documented one). I think we felt the pain of this especially hard in Alaska – with such a low population, doctors have very little experience with a disease like this. In our case, I wish we’d gone to see Milind Javle at MD Anderson. Had my partner’s tumor been resectable or he’d needed a liver transplant, or had a different type of CC, another doctor may have been more appropriate – I don’t know.
I know the choice to leave home isn’t for everyone – not everyone can or wants to travel once they’ve been diagnosed with this disease, BUT, it’s possible to take a short trip out, get a consultation, and return home with a good treatment plan in hand. Sometimes the transfer of information from one office to another requires calling/coordination, but as long as one is conscious of this and has a doctor who is willing to work with others (some poor doctors are not), it is very possible.
-From the very beginning, I wish we’d acted with more speed. It seems to me that our oncologist’s office was very slow to get my partner in initially and to resume his chemo treatments once he returned from a trip to the Mayo Clinic. At the time, I did not know enough about CC to understand how quickly progressing it was, that a delay in the start of treatment might’ve cost my partner time.
A treatment we almost received:
-RFA from Drs. Kahaleh and Gaidhane at Weill Medical College of Cornell University. When we told the doctors at Mayo we were thinking of doing this, they said they could perform it there – they began, but once in, they found the tissue around my partner’s tumor too damaged (necrotic, mushy, friable) to proceed. Dr. Kahaleh said that given his high level of experience with the procedure he would feel comfortable giving my partner the treatment, but before we flew to them my partner’s health became too poor for that much travel/treatment. The clinical trial for the treatment with Dr. Kahaleh is below. To me and my partner, the studies on RFA made it seem very promising as something that could give a few additional months:
https://clinicaltrials.gov/ct2/show/NCT02166190?term=kahaleh+rfa&rank=2Palliative Treatments we did receive:
-Plastic Stents (replaced once)
-Three percutaneous biliary catheters: my partner felt that these were painful, but the medical professionals around us said it was more likely my partner’s liver itself was the source of the pain. Either way, it appears there are different types of stitches that can be done when the catheter is placed – sounds like some are more painful than others. Another problem with these was that fluid leaked from one of the incisions from which a catheter exited my partner’s abdomen. Because his acites was so bad towards the end, this was a considerable amount of liquid. Eventually, we placed a modified ostomy bag around it, but engineering this properly took some doing and it would leak sometimes anyway. All in all though, these catheters most likely helped my partner live longer – they drained areas of the liver that the internal stents did not help with.
-Chemotherapy: My partner’s bilirubin was extremely high by the time he was diagnosed (13-23), so he could not do gem/cis. Our doctor in Anchorage then put him on cisplatin monotherapy. I could find no studies showing this was an effective treatment, and when we talked to the doctors at Mayo about this, they conferenced with our doctor in Anchorage and requested she put my partner on mFOLFOX (with some of its components at reduced levels due to his poor liver function). It is my understanding that we could not determine whether chemo was working because there was an error in the timing of his scans (I could go on about this for a while, but I won’t – just, again, if you can get treatment at a better center or at least get your gameplan determined at one, it’s worth it). My partner spent about four days fatigued after treatments, and he thought the steroid (dexamethasone) given along with them gave him really terrible mood swings (he really hated this part, but once he figured out it was the steroids, not him, he felt better). Otherwise, apart from potentially the lightest hint of peripheral neuropathy in his right arm, he had no side effects.
[6/27/16 edit] Paracentesis (also known as a “tap”) to drain excess fluid from Peter’s abdomen (it was severely distended). I’ve added this long after writing the original post, and cannot remember how long the effects of this lasted, but do remember getting off so much water weight (several pounds) helped Peter move better in his weakened state and made him more comfortable.
I think that’s all I have at this point. In a few days I’ll post a more detailed list of all of his procedures/issues for those who are interested [Edit: These are about four posts down in this thread]. Again, please feel free to post about any questions you might have. Also, please let me know if there’s a better board to post this on.
I also wanted to say: I’m thankful for all the support I received on the message boards and that I had them to read and guide me.
Sincerely,
Elizabeth
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