Sunday, March 1, 2015

AG-221 Trial Study: The Recommended Treatment


First, allow me to apologize for the delay in getting this post written.  At first, we needed time to take everything in.  Second, I began to suffer a severe migraine and ear ache for several days now, and was out for the count.  I couldn't focus on a computer, phone, or any reading the first few days.  I broke down and went to the doctor and got a message on my head, neck, and shoulders.  I am being treated for possibly TMJ (from clenching my jaw in stress) and a possible sinus infection, although the doctor thinks that my symptoms are mainly the result of stress.  (You think!) 

We received a call from Todd's BMT doctor Wednesday evening about 5:30 pm, February 25, 2015.  She called our home phone, which is unusual, and I walked in while Todd was on the phone with her.  He put the call on speaker so I could hear. 

She told me that she had conferred with her colleagues, the other bone marrow transplant doctors, and they came to the consensus that they were NOT in favor of doing a second stem cell transplant at this time.  We were a little shocked, knowing this had been her intent since December, 2014, once Todd's relapse was discovered.  Even now, she did not necessarily "agree" with this choice, but felt she needed to defer to the majority who would rather Todd pursue the trial study for the drug AG-221, because they felt like that would provide the best outcome for Todd, considering the aggressiveness of his disease. 

I knew from my own research, that the success rate for second transplants was not good, but I never wanted to discourage Todd, and after talking to his doctor back in December, we both agreed to be positive and hope for the best; that Todd was NOT a statistic, and that his case has not been textbook MDS from the beginning.  However, this ugly fact had to be considered now when determining his treatment options.

The doctors were not that impressed that his blast counts had been reduced from 7% at the time of relapse to his last biopsy which showed 3% blasts.  Because Todd relapsed within those critical 100 days post-transplant, and that after three rounds of Vidaza,  his disease was still present, they didn't think a second transplant would be successful.  This was devastating news all the same.  For the past three months, we had been pinning our hopes on this second transplant, and now they didn't think it had a good enough chance to work.  I asked why the change in that thinking.  She told us the success rates for second transplants with an aggressive disease was in the "teens."  I somewhat knew this, but had hoped Todd's case would be different.  He is younger and healthier than most of the patients who are in published studies; most are about 60 years old.  These odds are not very good, and they didn't want to set the transplant up for failure.  She said that with his chimerism at 75% of recipient cells (Todd's original bone marrow) and only 25% of his brother's bone marrow left, and with still have disease present (3% blast cells), they would have to do some type of intensive chemotherapy prior to the second transplant to suppress Todd's diseased bone marrow, for there to be any hope of success.  Then, he could get a bad case of Graft versus Host disease on top of the transplant not working, making his overall health condition even worse. 

She said that the only reason they do second transplants, even though the odds of success are so low, is that up until now it has been the only main option, apart from the Donor Lymphocyte Infusion (DLI), which Todd wouldn't be considered for, since his disease is still present (not an aggressive enough treatment).  But, now they have another option: The drug AG-221 in phase 1 trial studies.  The doctors at Cleveland collectively agreed that this drug would be Todd's best chance of controlling his disease.  It would NOT be the "curative" treatment, like the second stem cell transplant, but they have  had success with it controlling the disease and achieving "complete or partial responses."  This is the reason that his doctor was not in favor of the drug versus the transplant, because she said that knowing us so intimately, she wanted to go for the curative treatment. 

Todd asked her if she had been "overruled" by the other doctors in the department, and she said she didn't want to use that word, but that the other doctors were in a position to be much more objective than her.  They all felt it was a better option, to get the most bang for the buck, and help further research in the field, which made us feel like the other doctors just wanted to "guinea pig" Todd by enrolling him in the study.   This didn't sit well with us. The lack of confidence that she had in this decision troubled me.

She encouraged us to get a second opinion and that she would refer us to another physician at another research and BMT hospital.  In addition, they would still consider doing a second transplant at some point, if they could continue to "prove" that he can fight the disease, gaining more time between his last transplant and a future transplant: translation, that he can stay alive long enough and his condition improve enough to justify the second transplant.  Which, they feel is a good possibility if he could start taking the trail study drug: AG-221.  A good number of patients, most 60 years or older, who have not been candidates for a bone marrow transplant have sustained complete or partial responses to the drug since they have been on it.  More about this later.

As to pursuing the study, we would have to wait for the rest of the bone marrow results to come back, which includes cytogenics and the test confirming that Todd still has the gene mutation, IDH2 which is required to be enrolled in the drug study.  After suggesting this study to his doctor a while back, she investigated and found that Todd did have this gene mutation prior to transplant, when he was enrolled in a study with his oncologist at Cleveland Clinic after his diagnosis.  So, the study hospital  needs an official confirmation of this before Todd could be enrolled.  We all agreed that it would do us no good to pursue a second opinion or seek to enroll until we got these results back, which would take another week. 

We also needed to consider where he would enroll in the trial.  The Cleveland Clinic has applied to participate in the study, but it has not yet been approved.  Of course, there a multi-step process that the hospital must go through to obtain this approval.  She said it could take a month, or even longer to get this approval.  In light of this, she suggested that Todd could do another round of Vidaza, to keep his blast counts down, and then revisit the status afterwards.  The only problem with this, they still may not have the approval by then, which would put us in the same situation we are in now.  The closest hospitals that are enrolling patients are in Chicago and Nashville (Vanderbilt), both about six hours away.  We would not be required to stay during the entire trial, but we would have to return for tests at designated points in the study. 

Cleveland would obviously be closer at 3-4 hours away, but I hate to think about delaying treatment for another month or more with Todd's low counts, low energy levels, susceptibility to infections, and requiring blood transfusions every week. 

Concluding the conversation, this was the plan:
1.  Wait until next week, when all the test results were in, including the genetic test confirming the IDH2 gene mutation.  Without this confirmation, Todd would not be eligible for the study at all.  Then would have to go for the second transplant.
2.  If the results show he still has the IDH2 mutation, we will be seeking a second opinion at Vanderbilt Hospital in Nashville.  The BMT doctor will refer us to a doctor there.  We decided if we were going to get a second opinion, that we might as well have it done at a place where they are currently conducting the trial drug study, so that we could get feedback about how well it has been working.  If I had the money to fly often, I would rather go to Memorial Sloan Kettering in New York where the lead doctor and investigator, Dr. Eytan Stein is at.  He has been working with the drug the most.

So, with all this thrown at us in a matter of minutes, we were overwhelmed, sad, and heart broken.

We kept discussing the conversation that evening, questioning their recommendation.  We refuse to allow Todd to be a guinea pig.  I got up the next morning and decided that WE were going to make the final decision about what treatment he would do.  If we wanted to go for the second transplant, we would tell them that was our choice, and if they refused to do it, then we would go somewhere else.  

Still feeling like I needed more information about this decision, I began a further investigation into the drug and the trial study.  I'm so glad I did. 

About the Drug
The drug AG-221, is a pill that is taken over the course of 28 days per cycle.  Tests to check progress would include bone marrow biopsies, blood work, liver tests, etc.  After talking to the doctor that night, I was under the impression that the drug would work similarly to Vidaza, in regards to still producing low blood counts, but after further research, I don't believe that this is the case. 

After going on various websites, I found where Dr. Stein had presented his findings at the most recent ASH convention in December, 2014 on the drug company's website (Agios).  When I heard him say that patients taking the drug were responding in as little as one cycle, and that their blood counts had come up while blast counts had been reduced, I jumped for joy.  He cites one case where a patient's blast counts had gone done from 44% to 12%; Hemoglobin increased from 10.9 to 11.2; Platelets increased from 29,000 to 106,000; and ANCs went from .1 to 1.3!  (Stein, 2014, slide 28).  He even goes on to explain that even partial responses had to have platelets over 100,000 for it to be considered a partial response. This is so much better than what Todd is living with now (Platelets running 10,000-25,000)!    Here is the link if you are interested in this brief presentation.  Start at slide 18 and end at slide 29.  It does take a Shockware plug-in I believe to watch, but it shouldn't be an issue for most.
http://edge.media-server.com/m/p/s4b9a35f/lan/en

Key information: 
  • Patients even on the lowest doses have received some response. Many have had relapse of their disease after a bone marrow transplant. 
  • This Phase of the trial is to help determine the amount of the drug patients can take with minimal toxicity and still receive a response.  So, Todd would receive increased doses over time to "help determine the MTD or maximum tolerated dose and/or the recommended Phase II dose."  (U.S. National Institutes of Health. Purpose. 2015)  Retrieved from: 
    https://www.clinicaltrials.gov/ct2/show/NCT01915498?term=AG221&rank=3
  • We are guaranteed certain rights, like they have to tell us which amount he will be given, he can stop or quit the study at anytime, and if they prove that a certain amount is the most beneficial, they can stop the trial and give him the appropriate amount.  (Memorial Sloan Cancer Center, 2015)  Retrieved from: http://www.mskcc.org/print/cancer-care/clinical-trials/how-decide-whether-clinical-trial-right-you  They have yet to reach a maximum tolerated dose yet. 
  • The drug company runs the study at the designated hospital, under the care of a primary investigator (doctor) and co-investigators (other doctors), which can be a bit scary, but on the positive side, all of the costs of the drug, tests, and hospital expenses are paid by the drug company.   
  • The job of the drug is to block the enzyme that allows the cells to mutate.  Therefore, it allows a good number of the blood cells to mature to healthy functioning cells. Thus, higher blood counts and lower blast counts.  Here is another great article that features information from Dr Stein.  http://www.targetedonc.com/conference/ash-2014/ag-221-sparks-durable-remissions-in-idh2-mutated-aml/2
 Of course, with any study, there are bad results too.  There have been some that have died from disease progression, or other causes and some that have not had any response.  But, a majority have done well.  With every treatment comes risks, but also the hope of remission.

In conclusion:

At this point, the only other step to the above discussed treatment plan, would be another addition:

3.  If the second opinion is the same, to pursue the trial drug study, then I think we will likely enroll soon thereafter at Vanderbilt, instead of waiting for the Cleveland Clinic to get approved.

Of course, all this is subject to change, in light of further information.

Thanks for your patience in waiting for me to get this posted and for the length of the post.  I will give another update when we have further information.  In the meantime, I expect that Todd will need another transfusion before Wednesday of this week.

We covet your prayers and support,
Kimberley

Other information about AG-221:
On YouTube:
https://www.youtube.com/watch?v=Pnkr2gZfr64

http://ashclinicalnews.org/trial-roundup-december-2014/

http://www.agios.com/pipeline-idh.php

http://investor.agios.com/phoenix.zhtml?c=251862&p=irol-newsArticle&ID=1916041&highlight=


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