Julie, Steven.....
                         Thanks for the good wishes.
                         I think you need a cooperative oncologist who already has some experience
                         with the drug and with the company.
                         We got the approval in less than a week.
                         I feel privileged and grateful.


I am happy to report that MERCK PATIENT ASSISTANCE PROGRAM approved my application for potentially 6 months of KEYTRUDA and I received my first infusion last week. Without information on predictive markers this is a shot in the dark but I think it is worth the effort.
Wish me luck smile

Thank you for your efforts.
It would be very interesting to hear what AGIOS has to say.
How can they justify witholding essential information after submitting the patient to a hazardous and painful procedure? (risk of bleeding, infection, organ damage, tumor spread). Did they get any tumor tissue? (Often they do not) If so, are there any new mutations?
The signed consent has a total of 31 pages and  few patients will read every sentence of it unless it is specifically pointed out to them.
In a general sense I feel it is extremely important that pharma companies show transparency in their trials, sharing gleaned information in order to make progress in the fight against cancer.
It has been said that not enough patients sign up for clinical studies. Yet it will be difficult to  entice more  people to submit to the often unpleasant procedures if they feel that  their personal welfare is not being respected..
Best regards,

Thanks a lot for the info.
Just a little correction - I'm not sure how the "mom" slipped in - the person I am advocating for is myself.
: )    Paul

Steven....we did the repeat PET/CT and the results are not good. After 2 months of Ag-120 there is significant new growth, so I am off the trial.

I am now looking at Keytruda off label as a possible albeit costly alternative. This would be something of  a crapshoot  given that in my case  the  predictive tests are either negative or unavailable.  I had hoped that the tumor biopsy might reveal some new genetic alterations that could help when looking at new approaches, yet when asking repeatedly about the results of my biopsy I have only received evasive answers.
Looking carefully at the trial consent I signed, I now came across the following statement  that I had not seen before:


Could somebody please explain to me why anybody would wish to withhold from a trial patient information  that could potentially save his life?
Best regards,

Hello Steven
Thank you very much for responding.
I am due for new imaging studies in the next two weeks to see if I derive any benefit from Ag-120. My side effects have been relatively mild, mainly diarrhea, pretty well controlled with imodium.
I am aware, of course, that even under the best circumstances targeted therapies, such as Ag-120 for IGH1 mutations are unlikely to be curative. They can keep the disease stable for a while until - hopefully- some suitable immunotherapy exits the pipeline. Right now Keytruda sounds exiting but it seems only to work well in patients who are also positive for mismatch repair deficiency, if I am not mistaken.

Anyway, I'll keep you posted and hope to hear from more people on this  particular trial.

All the best for your mother.


Good morning,
I have not written for a the longest time, more than 3 years to be exact, even though I have followed the board on a regular basis. After extensive surgery, SBRT to a portocaval node, a
short course of chemo followed by re-exploration and abdominal hernia repair one year ago,
I now experienced a more aggressive recurrence of the malignancy as I am running out of treatment options. Genetic profiling had revealed genomic alterations that included IDH1.
Therefore I applied and was just recently accepted into the Agios Ag-120 trial. I do not have follow-up results yet but would be interested to establish contact with other patients who are on that trial.
Thank you for being there,

Congratulations! Well done!
A good item to place on Facebook to increase public awareness.
Thank you.

Congratulations. Your story gives us all hope.
Would you be so kind as to mention the size of the tumor and the grade of differentiation when it was discovered?
Thank you and enjoy your new life.

Congratulations, Sue.
It is persons like you that give hope to the rest of us. .
Reading through the posts on the boards can get quite depressing after a while. So, your feedback is very welcome indeed.


Thank you, Marions, for your kind words.
I just received my latest labs which show a significant improvement of the liver
values. So,considering the rarity of polyneuropathy in cholangiocarcinoma , the condition may be coincidental, after all.
Well, only time will tell.
Unfortunately, in spite of the positive news, the anxiety remains.

Best regards,

Hello to everyone.

I just recently came upon this board with loads of helpful information about this devastating illness. Thank you for being there.

I wonder if there is anyone out there with cholangiocarcinoma who experienced signs of neuropathy PRIOR to the diagnosis of the disease. (Chemotherapy can have this as a side-effect, but  this is not what I mean).

Briefly my history:
                              I had a normal physical and normal labs at the end of December of 2011. We celebrated my 70th birthday in May of 2012  (70 going on 50, both in energy and appearance, never having had a serious illness before, non-smoker, non-drinker, physically active).   
At the end of June 2012 I developed some intermittend numbness in my toes and fingertips. I first did not pay much attention to this, but as the tingling and numbness worsend over time, I went to my GP for some blood tests and a neurological referral. The neurologist confirmed a mild polyneuropathy and explained that nearly half of the cases have no good explanation. The blood tests showed mildly elevated liver enzymes, which lead to a sonogram, which lead to an MRI of the liver.The first impression of the radiologist was that of
a giant hemangioma and the GI specialist refused a biopsy since I did not have any cancer-related symptoms and negative markers. However, more refined imaging studies ruled out the hemangioma impression. So the biopsy was finally performed in the first part of September.
Diagnosis: Poorly differentiated cholangiocarcinoma.
Because of its size and proximity to large blood vessels it was difficult to find a center that still agreed to operate. I finally underwent, at the end of September, an extended right hemipepathectomy, leaving me with a calculated 28% of the liver. The postoperative course involved a prolonged stay in ICU because of massive blood loss and respiratory difficulty. The tumor was removed with what appeared to be clear margins and the lymph nodes were negative. So, there was some hope.
We did not start any adjuvant therapy, its usefulness being doubtful in this disease.
On returning home, I was still weak but did not have significant neurological
symptoms. Unfortunately the polyneuropathy has now returned full force and even though, MRI, bone and PET scan have so far been negative, I am afraid
that this can be a sign of tumor recurrence which may show up soon.

Over the last weeks I have searched the internet for similar cases, but in vain.
Theoretically, the appearance of neurological symptoms in cholangiocarcinoma can occur (it is known as "paraneoplastic syndrome" and shows up most often in patients with small cell carcinoma of the lung),yet I fail to find any real-life documentation for this in bile duct cancer anywhere.

If you have experienced something like this or know someone who has, please let me know. I would greatly appreciate it.

Thank you ever so much.