John has just signed up for a Phase 2 trial of the Selective Inhibitor of Nuclear Export (KPT-330). The study drug works by trapping the "tumor suppressing proteins" within the cell and so causing the cancer cells to die or stop growing. It is a kind of targeted therapy. The team is aiming for John to start on April 20.

In the meantime, John had his PEG removed yesterday (hooray!) after two and a half years. It was a day that, had I been asked six months ago, I would have say would never come. So far, John has had six dilatations done on his esophagus. He is eating everything by mouth and, with the advice and support from a dietitian, is maintaining his weight. At his swallowing test two months ago, the SLP was very impressed with the fact that John had kept his swallowing muscles working by doing his swallowing exercises daily even though he was tube-dependent. We have been speaking to all the medical people we know about the new procedure and the good work that the interventional radiologist has done. I actually went and told John's RO that he, of all people, really needed to know about the procedure and to encourage patients not to just give up and go away.

I think what bothers me the most in all this is the fact that doctors keep telling me that not very many people lose the ability to swallow that way John has. That has not been what I have heard on this forum and from other patients I have met at the hospital. It appears to me that more often than not, doctors tell patients that nothing can be done and the patients just go away and live with a PEG for life. At the very beginning of our ordeal, at the time when it was still possible to get a wire down, a surgical oncologist flatly refused to even consider giving John a dilatation because he said the esophagus would inevitably close up again, so he wasn't going to spend the time doing multiple dilatations.

Since I spoke to the RO, I have heard that they have referred a couple more patients to the interventional radiologist, so I hope the word is spreading.

I will update when the trial begins and let you know how we are getting on. Thanks for letting me vent a bit.

See that irritates me. The reality is that most of the drs have never had to go through anything like their patients do, as such instead of stepping outside the comfort of their own little knowledge boxes they prefer to quote the standard as opposed to trying something different.

hugs and hoping that he does well on this new trial.

Best wishes with the trial. I checked it out and the phase 1 data looks really good.
Maria

Thanks for the update. I hope the new drug is effective.

Hi Gloria, I am pretty clueless when it comes to esophagus dilation. What kind of symptoms/patients should consider getting this done?

Would someone who is starting to struggle with swallowing (needing to force, making sound effects to swallow at times during every meal)?
Or someone who coughs up bits of food regularly to prevent aspiration?
Stephen's fibrosis is getting progressivly tighter in the past 6 months, he mentions there is a pain aspect that wasn't there before.
I am guessing this proceedure is for more severe/pronounced narrowing of the esophagus.

Thanks for sharing and advocating, Sophie

This is excellent news -- I wish you both the very best on this trial.

I am so glad John had his PEG finally removed -- I know this has been such a trial. It is such an unfair shame that you guys had to go through so much on this but I am glad he is finally free of the tube. I hated mine so much I could not stand it so I can empathize.

Keep us updated on the trial -- best of luck! These targeted therapies are the bees' knees -- I am very hopeful for you guys!!

The Hellion

Hi Sophie, sorry I missed your post and did not respond sooner. John's esophagus was totally blocked after radiation, he could not even swallow his own saliva. However, even at ten weeks post radiation, the doctors were saying to us to not be impatient and that the best help for swallow was to swallow. We finally got him a barium swallowing test which showed that he was not swallowing anything.

Given Stephen's symptoms, I would think the first step is to get a barium swallowing test. The doctors may then decide to perform a dilatation or have him work with a speech and language pathologist on swallowing techniques. The fibrosis can get worse as the internal scarring continues. (Yes, it takes a few months for the scarring to happen.).

In Ontario, it takes ten to twelve weeks to get a barium swallowing test. I strongly recommend that you start the process ASAP. Try to get yourselves on the cancellation list if you can manage to go at the drop of a hat. That way you may get lucky and get in early. If Stephen is coughing up bits of food, he is having difficulty getting them down.

Thank you Gloria, I will get him signed up for a barrium test. Smart to plan ahead, at least this way we will know where we stand.

I am also greatful John has access to what sounds like a cutting edge trial.

Wow and good on you guys for doing the swallowing exercises. Its amazing that John can swallow after almost two and a half years of being on a PEG. This will give so many people hope and determination.

Please keep sharing
ps:Stephen is flying into Toronto tomorrow to be represented by a gallery on Young St.

Hi Gloria,

So sorry I missed this earlier posting of yours -- Hurray for John having his PEG tube removed!!! I know he did a good job with it, but he must feel so good without it. I remember when mine came out (ouch!!) - it was so good to shower without it and not worry about tugging on the tubing.

Best regards,
Nancy

Update after two cycles of trial meds:

So we met with the MO yesterday to go over the results of the first CT scan; unfortunately, the report with tumour measurements was still not in; however, we were told that the largest mass in the lungs looks smaller. I would like to think that this indicates that the trial drug has had some effects. So we press on.

This has been a rather rough two months for John. He had had some postural hypotension before the trial started, but during the trial it became quite significant. My own observations told me that it usually happened on the days when John was off dexamethasone. I made up a spreadsheet showing the twice daily postural BP results and highlighted the days when he wasn't getting dexamethasone. The numbers actually showed that most of the low BP (below 60) occurred during that period. The doctor has now prescribed florinef and how, a month later, the BP seems to have stabilized.

At the same time, after two months, John's stoma still hasn't healed completely. It was leaking and worse, he was getting burned by the stomach fluids. We saw the doctor who performed the magnet procedure yesterday. He will book John in for an endoscopy so that he can go in and put clips to help the opening in the stomach close. A wound nurse who saw John a month ago suggested gluing the stoma but the IR told us yesterday that it was not a viable option as the pressure was coming from the inside, gluing the outside will not help matters at all. I do have to say that even with some leakage, the stoma now measures only 1 to 1 1/2 mm which means it has closed quite well up to a point.

With all the drama, I am happy to report that John still has a positive attitude. He is able to to keep his weight at 182 lbs though he does call me the food police. My therapist told me at my last visit that every moment of happiness makes it worthwhile. I think this is truly insightful as on reflection, it is the many moments of happiness that have helped us cope.

I do hope the tumor news was positive. I am so sorry about the stoma -- hopefully a resolution is in sight. Please keep us posted -- I feel like you guys are a few months ahead of us although I suspect we will wind up taking different trial paths !!

Best wishes and best thoughts to you both!!

The Hellion

Hugs.. and I am so glad his attitude is still good. fingers crossed his tumor is smaller and continues to shrink.

Update:

John had his second CT scan since starting the trial and we met with the MO yesterday. It was somewhat disheartening to hear that even to the naked eye, new growths were apparent. The MO's decision was that John was to be demitted from the trial.

The last two months since the trial started have been a bit of a nightmare for us. The postural hypotension took several weeks to sort out, then he got aspiration pneumonia. A swallowing test yesterday confirmed the MO's suspicion that he has developed silent aspiration -- a rather dangerous situation as the test showed that he had absolutely no idea/response when he aspirated. Now, everything that he drinks has to be thickened.

We are unsure of the way forward. The MO would like John to take a month off to recover from the pneumonia and to rebuild his strength. I did ask about the trial that was previously tossed about as a possibility -- MEDI4736 in combination with another drug. It turned out that the doctor had already made inquiries on our behalf and it is only open to patients who have just come off the MEDI4736 monotherapy. So, that's not a possibility anymore for us. The MO said she would think about what other trials are out there that we could try.

John's take on the situation is that his quality of life was close to zilch the last two months and he's actually a bit glad that the MO made the decision to terminate for him. He feels that he is now on borrowed time, but honestly, it has been borrowed time for us for a long while now. This, however, does not mean we are giving up. We will continue to take it as it comes and I will update on what the doctor manages to come up with.

Despite the termination, we both feel that we have an outstanding team of doctors and nurses at the hospital. We have had nothing but the best care.

Always appreciate reading your updates. I feel that John, together with yourself, are pioneers with these Trials for the rest of us. Your experiences are so valuable for any others that have a need for this path. I sincerely hope that John has a good month now before he again tackles this. Good quality time, for you both.
Time to readjust your sails.
Tammy

Thanks Tammy. I always make a point of taking notes so that I can post what I know on the forum for anyone who might need the info further down the road. The trials may not have worked for us, but who knows, from our experience, the doctors/researchers may have learned something that may enable them to move forward. A trial that did not work is not a failure in my books.

Thank you for sharing this topic of interest even while going through your own struggles. I have been following the immunotherapy drugs myself since it seems to be the way of the future. Good luck moving forward, and hope the break from treatment will helps.

Hugs and blessings and fingers crossed something else comes up...