One thing to keep in mind is that there are very few trials specific to cholangiocarcinoma, but there are some that still accept cholangio patients.  For example, some trials target a general category of "solid tumors"

Here are two trials that are recruiting in Victoria that only require solid tumors:

http://clinicaltrials.gov/ct2/show/NCT01292655

http://clinicaltrials.gov/ct2/show/NCT01986218

I am very sorry and frustrated that you can't get better help finding and better access to clinical trials.  Without cholangio patients participating in trials, making progress fighting this disease will be very slow.

Jason

Thanks for the chuckle Duke!

Rohrbork,

My wife experienced a significant amount of "referred" pain in her right shoulder shoulder.  She had a tumor growing in the dome of her liver that was pushing against and irritating her diaphragm. 

The way it was explained to me was that the nerves from the diaphragm attach to the spinal cord in roughly the same place as the nerves from the right shoulder.  When the diaphragm nerves send out a pain signal, the brain interprets the pain as coming from the shoulder (Mr. brain is saying something like "no way could the diaphragm be experiencing pain, so all that nerve excitement must be coming from the right shoulder." )  That is my lay understanding of what is going on anyway.

Maybe when your stents clog the bilirubin goes up, but that also causes some irritation to nearby areas, and this irritation is picked up as shoulder pain??

If you are experiencing referred shoulder pain, I definitely think you should get it checked out.  It was the main symptom for my wife's tumor growth.

Jason

Cholangotango,

My understanding is that drug companies race to get FDA approval, ANY FDA approval, because that opens up the "off-label" market.  I believe the "off-label" market is very lucrative. 

My guess is that Merck will have lots of Keytruda available pretty quickly for off-label use, because this is exactly why they were racing to be first out with an anti-PD1 drug (and they only have the advantage until some other company, like BMS, gets their PD1 drug approved).

What I don't know is how you can get an insurance company to cover the costs of using a drug off-label.  Do insurance companies do this??

Hopefully someone with more experience with insurance companies can chime in here.

Jason

I just looked back at this and realized how much had changed since that last update.  They talk about the roller coaster, and it has been so true for us.  Unfortunately, the roller coaster has been mostly going downhill since the last update.

Starting in late June, Andrea started to have pain in her shoulder.  It turned out that this pain was "referred" pain.  The pain was really from one of the liver tumors pushing on the diaphragm, but her brain interpreted the pain as coming from her shoulder.  Andrea got another scan in early July that indicated there were now 3 tumors, and the biggest was 3.5 cm (the one causing the pain). 

The shoulder pain had been steadily increasing, and with the tumor growth the decision was made to switch from xeloda to GEMOX.  The hope was to shrink the main tumor away from the diaphragm to allow for easier local treatment.  Unfortunately, this did not work. 

About a three weeks ago, started to get bloating in her belly.  We first thought this was just constipation caused by all her pain meds, but eventually we figured out that she had developed ascites.  Not a good development.  Our oncologist said there were basically two possibilities for the ascites.  One was that the fluid was a reaction to the tumor that was irritating the diaphragm (this was the leading hypothesis), and the other possibility was that the cancer had spread to the abdomen. 

Andrea had a paracentesis procedure where they removed 3 liters of fluid.  They tested the fluid and determined that the fluid was malignant.  This basically meant that the cancer had left the liver, and progressed to somewhere in the abdomen.  Malignant ascites is not a good development.  About 11 days after her first paracentesis, she had another where they removed 5.2 liters of fluid.  The fluid build up is very uncomfortable, so she is now on a weekly schedule to try and keep it better controlled.

What is the plan?  Well the number of possibilities seems to be much more limited.  She has switched from GEMOX to FOLFIRI.  This has hit her hard after just one treatment.  Heavy fatigue and painful cramping.  It has helped the shoulder pain though, so perhaps it is working.  We will see what the tumor marker shows next week.  Andrea also had a biopsy done that was sent off for genetic testing.  We should get the results for that on Oct. 6.  I am hoping that the genetic testing finds something that we can either target with an off-label treatment, or qualify for a clinical trial.

So that is the update.  Lots of twists and turns.  So many have been negative recently, we are really struggling to try to stay positive.  Hopefully we will get some good news soon.

Best to all,

Jason

The drug in testing is ADI-PEG20

Here are the trials I found searching for this drug:

http://clinicaltrials.gov/ct2/show/NCT0 … amp;rank=1

Jason

Here is a link to the news article:

http://www.neomatica.com/2014/08/21/chr … -eats-dna/

and the research article:

http://www.pnas.org/content/early/2014/08/12/1404171111

Jason

For me...

1st choice -- Does it need to be a week?  could you take the day 8 treatment and move it to day 9 or 10?  That would not impact much of anything.

2nd choice -- I would add an extra week between treatments and treat it as a mini "chemo break."

I can't really point to any medical data or anything other than shifting a day or two and chemo breaks happen all the time.

All of the options are probably about the same to be honest.

Jason

Hi Cazgirl71,

I am not a medical professional, but I have done some research and talked to researchers in the field.  Based on that, my understanding is that cholangiocarcinoma is unlikely to have a strong inherited component.  Very very few patients have other family members that have had cholangiocarcinoma.  It is not impossible, but at this point, CC doesn't show the characteristics of a strongly inherited disease like some other cancers.

Most (all?) cancer cells have messed up genes that cause them to reproduce uncontrollably.  A person could inherit the messed up (or nearly messed up) genes from their parents, or they could have normal genes that mutate into cancer genes during their lifetime.  This mutation process could be just a random event or could be encouraged by certain risk factors (e.g. inflammation of the liver from parasites, etc.) 

Again, my understanding is that CC is thought to likely be from genetic mutations the person acquires during their lifetime and not something that they inherited from their parents.  At this point, it seems like there could be an inherited component, but because so few patients have family members with the disease, the inherited component is unlikely to be that large.

I hope that helps.  Keep in mind, this is just the crude understanding of a caregiver that has done some looking into the topic, but is in no way an expert.

Best,

Jason

10

(68 replies, posted in Suggestions)

Just checking...

According to my system, It has been a day and a half since anyone made a post.  That almost never happens.  I figured I would make a test post to see if everything was working.

EDIT - Well, the post showed up, so it looks like everything is working ok.  I guess it is just quiet on the forums this weekend.

Jason

My wife's experience:

Tumors completely inside the liver ==> no pain

One tumor on the edge of the liver that was growing and pushing on the diaphram ==> severe pain.

Pain can certainly be from the cancer, but it seems to really depend on each individual tumor location.

Jason

Hi Jane,

Cholangiocarcinoma is usually found at a late stage because symptoms often only develop at a late stage.  However, if an extrahepatic tumor causes an early bile duct blockage, then that could contribute to finding it earlier. 

My understanding is that you are right in that surgery is the only procedure that is performed with "curative intent."  I don't think chemo + chemoradiation is generally thought to be curative.  However, I believe this is a pretty common approach, and is part of the protocol that the Mayo clinic uses as a preparation for a  liver transplant.  You could search for "Mayo transplant protocol" to learn some more about that.

I would also echo others in encouraging a second opinion.  So few doctors have CC experience, that it is a good idea, in my opinion, to try and find at least a couple of experienced teams and see what they recommend.

All the best,

Jason

It looks like they are starting up this trial at many of the major cancer centers.

The page on clinicaltrials.gov has the other centers (although everybody currently shows not yet recruiting.)

http://clinicaltrials.gov/ct2/show/NCT02150967

Thanks so much for letting us know how things are going.

Jason

14

(18 replies, posted in General Discussion)

Duke,

Andrea and I had some issues with the process as well.  First, let me say I completely agree with your point...Don't over think it!  This is where many of our problems came up as well.  Anyway, I talked with Nasra (the RN at Mayo that is supporting this process) and I think I have the correct answers to my questions.  I thought I would pass along the things that I learned that were not obvious to me at least:

Where should I get the procedure done?

We tried to get the blood drawn at Andrea's cancer center (UCSF), but we could not get them to do it.  They didn't know how to bill the procedure for insurance purposes (more on that later), and the fact that the request came from a different institution (Mayo) did not help.  With effort, we probably could have gotten this to work, but we found it easier to just use a third party lab to do the blood draw.  We used Quest Diagnostics.

Does this cost me anything?

No, but...

The easiest way to get this procedure done is to pay for it yourself, and then send the receipt to Mayo (Nasra) for reimbursement.  I think this is the way most people end up doing it.  If this is a problem, your best bet is to get the lab in contact with Nasra.  Nasra can send over an invoice that should allow the lab to bill Mayo directly.

The problem here was really one of communication.  Andrea and I didn't realize we needed to pay and then get reimbursed.  We were thus stumped when the cancer center asked us "Who is paying for this procedure?"  Our answer should have been "it is patient paid," but we didn't know that...

When should I get the procedure done?

If you live in the U.S. or Canada, you need to plan on making an appointment between Monday and Thursday.  Mayo sends you a prepaid overnight Fed Ex box to send the sample back to them.  However, because the sample needs to arrive at Mayo on a business day (Mon-Fri), it needs to be given to Fed Ex on Mon-Thurs.  If you are not in the U.S. or Canada, be sure to talk with Nasra about when you should schedule your appointment.

Do I need to do anything before I go?

Yes.  You need to find the freezer pack that is in the package.  The freezer pack is under the sample collection box (both of which are inside the return Fed Ex box)  You should bring a frozen freezer pack to your appointment (at least 4 hours in the freezer is required)

What other issues can come up?

The clinic asked us if we had an "order" for the blood draw.  It turns out there is the equivalent of an order in the sample collection box.  If you are asked, say "yes, we do have an order!"

Let the clinic know that they need to give the materials to Fed Ex.  If you try to give the box to Fed Ex yourself, Fed Ex may not take it since it contains blood.  If Fed Ex doesn't normally pick up at your clinic, you or the clinic can call them and they will make a special stop.


Those are the things that tripped us up. 

If you tried participating in the Mayo study (successfully or unsuccessfully), please please please add any other things that you found confusing or challenging to this thread.  As I understand it, the plan is to take all of this feedback and use it to improve the Mayo process.  Part of that improvement will be a guide or FAQ on this website to help patients navigate potential problems.

Thanks!

Jason

15

(57 replies, posted in Members' Cafe)

Percy,

I just wanted to say thank you. Thank you for all the time you have spent making hundreds of posts that have helped so many people.  Thank you also for the information and thoughts you have given me in person over the past year. 

The disease seems to ebb and flow, but the main arc is pretty consistent for all but a lucky few.  This does seem to be changing, but change is coming ever so slowly.

You have done so much for so many.  Be at peace my friend,

Jason

Marion,

Any thoughts on the issue of forcing patients to terminate an active trial in order to qualify for hospice?

Thanks,

Jason

Andrea and I are also planning on attending.  We are not going to be able to stay overnight though.


Should be fun.  See you there Marion!

Jason

Willow,

What an unfair and even cruel system!  Clinical trials generally make you wait until you have failed to respond to chemo in order to participate.  Then, when you do participate, you have to pick between a potentially helpful trial and the needed comfort offered by hospice?  That just does not seem right. 

I am not sure what to suggest.  Perhaps you could get a hospice "consult" to see what they would suggest without actually going on hospice?  You could also try to contact one of the other oncologists at UCSF.  Dr. Vinook was the name given to us as the backup for Dr. Kelley's patients when she was out.

You must be exhausted trying to support your sister and navigating an out of network clinical trial.  You are doing an amazing job.  Hang in there,

Jason

The whole pd-1/pd-l1 was very confusing to me, but I found this article helpful in understanding the pd1 / pd-L1 interaction:

http://www.medscape.com/viewarticle/810688_3


If I read this right, MK-3475 is a pd-1 inhibitor.  There are also trials looking at pd-L1 inhibitors.  The Merck drug for anti pd-L1 is MSB0010718C.  A trial with this drug is described here:

http://clinicaltrials.gov/ct2/show/NCT01772004

Both approaches are trying to stop the pd-1/pd-L1 interaction (which turns off the immune response).  All very very exciting.

I hope your sister has a fantastic response.

Best,

Jason

20

(40 replies, posted in Clinical Trials)

Hi Melinda,

That is fantastic news.  Thank you for letting us know how it is going.  I can't tell you how helpful it is (to me anyway) hearing stories like yours. 

If I am reading you right, when you next go back to NIH, you will have been 12 months without treatment?  Amazing.  Congrats!

One question if you have a moment:  Do you know what the NIH is looking for when they look at the genetic sequencing?  If it is something exotic, people can try and make sure it is covered when they get a sequencing done.

All the best,

Jason

21

(14 replies, posted in Members' Cafe)

Today feels weird with no World Cup!

I guess I can go work on the patient registry smile

Jason

Thanks all for the ideas and the encouragement.  We are not at all thinking about giving up, so not to worry on that front.

The basic plan is to do chemo and then either radiation or ablation on the (hopefully) shrunken tumors.  It sounds like they would lean towards ablation given the previous radiation and the potential for overlap (they are checking on this).

They thought there was evidence that Xeloda kept the tumors in check since Jan, so the plan is to start there and monitor CA 19-9 for a 6 weeks or so.  If it keeps rising, we will have an early scan and change strategy (tougher chemo or local therapy).

Jason

I can't believe how long it has been (7 months!) since I officially updated this thread.

As I said in November, we had this plan:

"We have decided to continue on Gem/Cis at Stanford until January, and then switch to UCSF and do IMRT + Xeloda."

and that is pretty much what has happened. 

Andrea had her last Gem/Cis treatment Dec. 18.  In total, she was one treatment short of twelve cycles.  Overall, she handled the chemo really well.  A little tired, and a little hair thinning, but pretty manageable all things considered.  Towards the end of the treatment though her blood counts were starting to get low and not rebounding quite as quickly as earlier. 

The net result of chemo: 
-Main tumor 1/3 original size and PET inactive
-Two satellite "medium" tumors significantly small, necrotic looking and PET negative
-"Constellation" of tiny tumors no longer PET active or visible

Pretty good from where we started.

In late January (2014), Andrea started IMRT radiation therapy.  The plan was to irradiate the area around the main tumor.  The logic given was that this tumor would be the one most likely to cause problems in the future, and that all the shrinkage gave an opportunity to treat it within a manageable IMRT field.  The treatment regime consisted of 28 days of radiation (5 days a week for 5.5 weeks).

Radiation, it turned out, was much harder than chemo, both mentally and physically.  About halfway through treatment, Andrea was having difficulty keeping down any food or drink.  This lasted throughout the remaining weeks of treatment and didn't really start improving until 3 weeks post treatment.  During those weeks, Andrea lost 15-20 pounds and was very weak.  Mentally, the challenge was to get up every day and go to another treatment when you knew that was what was causing you to feel so lousy. 

Andrea had her last radiation treatment March 5.  I guess you never know how you will respond.  During chemo, the side-effects for Andrea seemed easier than advertised, but just the opposite was true for radiation.

Andrea also took Xeloda (aka capacitabine aka 5FU) during radiation, since that supposedly helps the effectiveness of the radiation treatment.  Since radiation was only treating the main tumor, the Xeloda also acted as systemic therapy for the other tumor areas.

Andrea got her first post radiation scan in late March.  Good news.  The treated areas looked as expected, and the untreated areas looked dormant (looking at the untreated areas was actually the purpose of the scan since they said it was too early to evaluate the radiation region).

At this point, the oncologist suggested a treatment break until June.  The idea was to let Andrea recover from the radiation, and then see what happens to the tumors.  If all remained quiet, the likely path would be localized treatment to each "medium" tumor.  If there was a recurrence, then that would dictate the treatment path.

By April 1 (Andrea calls it her "Canniversary"), Andrea was mostly recovered from radiation, and quickly getting her strength back.  When not knocked on her butt from radiation, Andrea likes to be active and involved.  Her new passion is fund raising for CCF...you will all be hearing more on that later!  smile

It is now mid-June, and the last two months without treatment have been fantastic.  Andrea feels great, and things seem almost normal.  We even got to spend a week in Paris.  Neither one of us had ever been to Paris, and it was fantastic.  Since the diagnosis last April, I think this was the best two months that we have had.

However, everyone says that this disease is a roller coaster, and I can see that more and more.  We just got the results from Andrea's June PET/CT scan, and the news was not good.  Two new tumors in the liver.  One is 1cm and one is 2.5 cm, both PET active. 

We don't have much in the way of details yet (we see the oncologist next Monday), but that seems like an awful lot of growth if nothing was showing up in March.  My theory is that since the March scan was not a PET scan, but only a CT scan, they missed the beginnings of these tumors.  I will find out more in a couple of days. 

I guess the one positive is that the tumors are still contained to the liver.  The report also indicated that no lymph nodes showed signs of involvement.  Still a major bummer.

So that is the latest.  Hopefully, Andrea can get back into the "good news / what's working" column soon.

Jason

24

(68 replies, posted in Suggestions)

Trying to register for the discussion board would take you to dead links.

It looks like the dead link issue has been fixed, but that has uncovered another problem.  Previously, a human would have to approve your registration request.  That step no longer seems to be happening, and now we are hip deep in bots!

Jason

Googled cholangiocarcinoma as well.