Hematological Response: End of Cycle 1 of AG-221

Todd reached a major milestone this past week, where he completed 28 days or the First Cycle of the trial drug study for AG-221.  The Research Team kicked-off Day 1, Cycle 2, the following day, Thursday, May 21, 2015 with the standard 10 hours of blood draws, EKG, and another bone marrow biopsy at the Cleveland Clinic's Taussig Cancer Center. 

The results at the end of just one cycle on the trial drug have been amazing!  His blood counts have come up enough to go 4 weeks without needing a blood transfusion and his immune system has come up to allow him to go off antibiotics, get off the neutropenic diet, and live a little less restrictively for fear of getting an infection that could be deadly.  It is also allowed the team to schedule to have his Hickman Central Catheter removed on the next visit.  They have been wanting to take it out for fear of it causing an infection, but his immune system has been too low.  Now, however, they feel it is strong enough to handle having the Hickman removed and having a port put in on the other side of his chest.  His platelets are still under the recommended 50,000 for having this procedure done, but they will give him a unit of platelets during the procedure to help counteract any possible bleeding complications.  If you have never seen someone with a port, it is a button-sized device that goes into a central vein, under the skin.  So, when they need to draw blood, or give him an injection, the needle will go through the skin.  Here is a brief overview of catheters and ports.  He will have a catheter removed and an port put in:  Port information.

Just to give you a comparison of how his counts have improved here is the 4 week difference:
                                4/20      4/30      5/7      5/14     5/21/15
Hemoglobin:              7.6       8.5       8.7       8.6         9.0  (low still: normal range 13.0 min.)
White:                         .47       .83     1.47     2.03      2.92 (low still; normal range 3.7 min.)
ANCs/Neutrophils:     00        .53     1.07     1.66      2.39 (now falls in lower end of normal)
Platelets: (1000s)         34        41     38         36         35   (Low still normal 150,000 min,)
Bilirubin(total):            n/a      3.8     3.9       3.0        2.6  (want to drop; normal 0.0-1.5)


This is amazing!  In just one cycle, he has seen this much improvement! You can see how he is no longer  Neutropenic (when ANCs/neutrophils are .50 or lower) or no longer needs Transfusions (thresholds for Todd was 8.0 or lower). The elevated bilirubin is caused by the drug, however, you can see the numbers are dropping as his body adjusts better to the medication.  The Indirect bilirubin numbers are fine and the research team is not worried about the elevated total bilirubin, as it would take his number to be 5x the normal or at 7.5 before they would adjust the dosage or make a change in his trial protocol.  The only side effect of the higher bilirubin has been some jaundice, especially in his eyes. 

The best news is, that the first bone marrow biopsy from Day 15, cycle 1, showed a normal range of blast cells in the bone marrow itself.  There was still signs of diseased cell (ringed blasts), but the drug is helping to block the mutation to allow his blood cells to mature, leaving less blast cells in the marrow.  There is also still chromosomal abnormalities found in the cytogenics results, which still show he is having the 5q deletion. 

He had a second bone marrow biopsy done on Day 1, Cycle 2, but we have not received any results back from those yet.  I asked the doctor if he would be considered to show a "response" or if they could say he was in remission, but she made it clear that Todd still has a way to go to ever say he would be in remission.  Under the drug protocol, there are established guidelines for what constitutes a "partial response,"  a "complete response," or "remission."  She stated that Todd would always have the diagnosis of MDS, actually relapsed MDS, that it would never go away.  The only "curative" treatment was the transplant, and that failed.  So, he cannot be "cured" of the disease at this time, but who is to say that there won't be a new curative treatment in the future.  Research and technology are moving so fast in this field, that anything can change in a year.  For example, this drug and the trials were not available a year ago! 

I guess it would take not seeing any blast cells, any chromosomal deletions, and for his counts to be at a certain level before they can use the word remission.  While he is not there yet, it is early in the treatment, and I feel confident that he can at least reach the level of a partial response soon. 

The doctor will not categorize the results as anything but they can see that the drug has produced a "hematological response.*"  

I'm not sure if his blood counts will ever be "normal" again, but I think they can be close to it, or maybe on the low side of normal someday.  In the meantime, he will still suffer from fatigue, some weakness, with a limited lifestyle or activity level, but he has already been forced to adjust to this. 

I honestly feel so relieved that I don't doubt that he is getting better!  While most of his counts are still below normal, we have already achieved the two main goals Dr. Stein, from Memorial Sloan Kettering Cancer Center in New York City, had in mind for Todd:  to lessen his transfusions (which were 6-12 days apart) or to NOT BE transfusion dependent, and to NOT BE neutropenic.  The idea that they now hope to remove his Hickman catheter and put in a port is wonderful!  That means I won't have to flush his catheter lumen's every night, change his bandage and line caps once a week!  He will only need to have the port flushed monthly.  I'm not sure if that is something I will have to do, or whether a nurse will do it at his appointments.  He will also be able to shower without covering it, and hopefully swim this summer!

Since he has he not been transfusion dependent, we haven't had to run to the local oncologist's office once or twice a week to have a CBC drawn to check his blood counts.  That's a lot less running! 

The first cycle of the study was a bit demanding on our schedule, having to go to Cleveland Clinic every week, and sometimes stay for 3 days, but that is behind us now.  With the start of the second cycle, we only have to be there on Day 1, which we just did on May 21, and on Day 15 for a short visit!  The same goes for Cycle 3.  Day 1 will be a long 10-12 hour day (which will be mean a 3 day stay) and then he only has a short visit again on Day 15.  After Cycle 3, he only has to go on the first day of each cycle from there on out! These days will be the long 10-12 days again, but we might be able to resume a more "normal" routine when all of these appointments are no longer necessary. All this is assuming that the drug continues to help and Todd responds positively to it, but I feel confident it will! 

Todd began seeing a physical therapist finally for his shoulder stiffness and pain.  It has helped some, but he still limited in using it.  Our chiropractor had seen Todd before the transplant and then after, was in shock seeing how much muscle mass Todd has lost all over, but especially in his arm and shoulder.  He felt that without the lack of muscle to help support the shoulder, it was making it harder for Todd to use it. Please continue to pray he will find some relief from this pain.  It does inhibit his activity quite a bit and he can only take prescription pain medications, instead of any ibuprofen, and he doesn't like to take them and they don't seem to help as much as he would like.  It wouldn't hurt if he could put on some weight or at least stop loosing it.

So, his next visit at the Cleveland Clinic will be June 4, 2015.  

Family News
Wow, a lot has happened here too!  Hannah Lewis graduates this Saturday!  We've had multiple year-end award assemblies, choir concerts, etc. to attend.  I have been cleaning like mad, with the help of my many good friends and family (Pam, Vicki, Darsie, Dana, Lynda, Mom)!  Two of Todd's friends from work came over and painted one of the garages for him (thanks Chris and Pat). 

We were able to put our house on the market for sale this past week.  It is all bittersweet.  We know it has to go, but we will be sad to leave it as it has been a labor of love building it, finishing it, and living in it these past 12 years.  Please pray for a buyer, for financing so we can purchase a smaller home, and for the entire selling/buying/moving process.

Two praises regarding finishing my Master Degree: I was able to find a practicum at the University of Dayton in the Library/Archives department starting this fall. I have to submit a lot of paperwork to Kent State in the new few weeks to get it ready, but hopefully everything will work out so I can begin in the fall.  I start my summer class and workshop next month!  So, I am hoping I will be able to juggle everything once that starts.  Second, I was able to get into the class I needed for the fall semester.  I was originally on a wait list, but Kent State contacted me last week, offering me an opening in the class!  I needed to take this class to graduate this fall, so now I can apply to graduate in December. 


It's been an emotional roller coaster this past year.  I crave a "normal" life whatever that is!  Change has been the only consistent thing in our lives.  It would be nice to be able to settle down into a mundane routine and stay in one place for awhile.  With our family having to be split-up last fall, with Todd and I in Cleveland, Abby in Cincinnati, Hannah Lewis and Ellie here with my mom, and now having to move from our house into somewhere new, it is a bit traumatic for all of us.  I know it does no good to worry about the timing, and wondering who will buy our house and when, or where we will move and when, but I still feel there is a sense of dread or a cloud that hangs over me.  It is hard to convenience myself that it is all somehow punishment for some reason.  If it is not the cancer and Todd's health, or the finances, then it is the uncertainty of "home" that seems to hang over us. For me, it is emotionally fatiguing, this roller coaster ride we find ourselves on.  I just want the ride to stop, so I can get off, sit down, and rest a while.  I'm still suffering from vertigo (literally), and I wonder why?  Life and this world keeps spinning, and I am trying to keep upright, without falling, despite all the obstacles. 

I gave the entire family this little pep talk last year about how God was going to use Dad's cancer, us going to Cleveland, Abby going to college Cincinnati, and Ellie and Hannah Lewis staying here with grandma, to GROW AND STRETCH US in ways we couldn't imagine!  I had forgotten about this until someone reminded me of it recently and gave me my own pep talk in return!  I know that God continues to mold us, refine us, strengthen us, give us insight, compassion, empathy, make us lean, teach us that living with less is more, help us focus on what is really important, and that home, wherever it will be, will be us together as family.  But growing and stretching usually doesn't come without a bit of pain!

Thanks for your love and support.  We appreciate your prayers and generosity, which continues to amaze and bless us!

Reference:
* According to the National CML Society, http://www.nationalcmlsociety.org/living-cml/response
 Hematological Response is a normalization of the blood counts, particularly white blood cell counts. This is the first noticeable indicator that treatment is beginning to work, though not necessarily in the bone marrow. The response can be partial HR (reduction in white cells,but not down to normal range) or complete CHR (white blood count at or below approximately 12,000 white cells/microliter).

Transfusions and CBCs in Dayton too slow; Todd becomes Neutropenic

When Todd went in for his third day of Chemo on Wednesday, January 14, 2015, they checked his blood counts again.  His hemoglobin dropped from 8.0 on Monday to 7.9 on Wednesday.  So, he needed a transfusion. 

But the nurses and staff can't seem to get the ball rolling.  First, they stalled collecting blood to have his type and screen done to order blood, because they say this can't be done at the Kettering location. So, he had to drive to Miami Valley South to have it drawn.  Apparently, only a nurse is allowed to draw blood from his Hickman Catheter Port.  The phlebotomists are not allowed to touch it.  They can only draw from his arm.  So, this means he has had to sit and wait for a nurse, when one is done with their own patient to come draw his blood.  This has taken anywhere from 30 minutes to over an hour.

After waiting, a phlebotomist came up and asked him if he just wanted stuck in the arm and he said yes.  He was tired of waiting.  So, they got the sample and told him it could take up to an hour to get the results.  He was tired and so he told them just to arrange to have the transfusion done the next day.  They made an appointment for him at the Main Campus of Miami Valley Hospital in Dayton for the next afternoon. 

He shows up there the next day and they can't get the results from the type and screen done at their South location (they have different computer systems).  So, he waits an hour for them to get the type and screen.  Then they have to order the blood.  It always takes about 1 1/2 hours per unit of blood and he always gets 2 units.  So, needless to say it was a long day. 

Today, Monday, January 20, 2015, he purposely goes to the Cancer Center location at Miami Valley South, as arranged at his last appointment with his local oncologist, so that he can get his blood drawn there on non-chemo days.  If he needs a transfusion, he can have it done there (supposedly).  When he gets there, there is NO standing order, or orders of any kind, for him to get his blood work done!  So, he has to wait til they call the doctor and get orders.  Then, there is no nurse available to take blood from his Hickman again, so he waits.  After getting frustrated, he allows them to stick him in his arm again, just to get the blood sample.  Then, they say it is going to take another hour to get the results and that is with it labeled "STAT."  Feeling pretty confident he doesn't need a transfusion, he leaves and tells them to call him with the results and if he needs to come back he will.

Hours later, they call.  Instead of giving him his CBC results, they just said he whites were low at .7 and that's all.  So, he calls back asking for the rest of the numbers; of course he can't get anyone to help him.  He has to leave a message for someone to call him back.

Late in the day, someone from CompuNet from Miami Valley South calls the house and says that they finally got a standing order for him to have blood work done there from here on out, but that he will have to fill out all the paperwork over again!  I don't know if he wants to go back there if it takes 1 hour STAT to get blood work results.  At the Kettering location it takes 10 minutes and at the Cleveland Clinic it takes about 15-20 minutes.  So, I ask her why can't they get someone to take it from his Hickman, and she informs me of the policy that only a nurse can do it.  She said, "He has good veins, they can just stick him."  And, trying to keep my cool, say, "No, not really, not when his platelets are low, he doesn't need to be stuck!  It could cause him to bleed and bruise!"  And she says, "Yes, I guess your right.  I hadn't thought of that!"  Ugh...

Between waiting on orders, waiting on nurses to draw blood, waiting on results, waiting to get a type and screen, and waiting to get the type and screen results and getting blood ordered, to actually getting the transfusion, Todd is just tired of waiting.  He doesn't have much energy or patience right now.  Who would? No patient should have to go through this much confusion and waiting!

It looks like I will be calling the local oncologist's office to let the doctor know that not much has been going according to his arrangements.  I think he needs to know that something is wrong with the process, which should obviously function more smoothly than it does.

 He finally got a call back from another nurse with the other results:  His Hemoglobin is a 9.3, well above an 8; no transfusion needed.  His Platelets have dropped to 67,000, but well above 10,000; no transfusion needed.  But, his other counts are problematic: his whites dropped to .7 or 700 and his ANCs to .3 which makes  him Neutropenic (anything below .5), which means new restrictions on diet, foods, environment, getting out, etc.  These very low numbers put him at HIGH RISK for contracting infections and little immunity to fight them.  Under other circumstances, they could give him infusions of Neupogen to help build up his white cells, but unfortunately, they cannot do this when there are cancer cells present, because it could cause the cancer cells to multiply too. 

It is normal for his white counts to drop after finishing a round of Vidaza, and they will usually begin to bounce back after a week or so.  That being said, its not good that they are this low.  We all are going to have to be careful in the meantime.

I'm glad he has an appointment back at the Cleveland Clinic with his BMT doctor on Wednesday, so we can ask if he needs to go back on his antibiotics.  We are not sure what this visit will entail except for the routine CBC and seeing the doctor.  I'm sure they will take blood for a chimerism study and we will discuss whether he will do another round of Vidaza before the stem cell transplant.  At some point, I'm sure his BMT doctor will schedule another bone marrow biopsy to check the progression or regression of the disease.  He needs his blasts cells to be as close to 0-1 as possible before beginning the stem cell transplant. 

I will post more after Wednesday.  Thanks again for caring enough to read this blog and to pray for Todd. 

Day +97: Cleveland Clinic Appointment Results December 22, 2014

Todd's treatment appointment started with the routine vitals and blood work.  His counts were down a bit more today, but this was expected after the week of chemotherapy.  His whites were at 1.54 (1,540); Hemoglobin 9.4; and Platelets down to 20,000.  These counts were strong enough to alleviate the need for any transfusions.

We ran into a friend and former transplant patient and her husband in the doctor's waiting area.  Today was her last appointment!  Her chimerism was struggling a bit, but her counts were much higher than Todd's.  Her bone marrow biopsy came back clean; no signs of disease!  This was such a praise!  I told her how hard to was to see other patients from the transplant floor reaching their 100 day mark and getting to end transplant follow-up treatments, when we are back to where Todd started before chemotherapy and the transplant.  She empathized and told me that although it may seem that the marker to ending treatment seems to keep getting pushed backed for us, we just need to concentrate on getting to the same end result: recovery and remission.  It might just take Todd longer to get there.  I found such wisdom in this!  We don't understand why God is taking Todd down a different path, but I'm sure he has a reason (His ways are higher than ours).

We saw a different doctor today, an associate of his regular BMT doctor, since she is on vacation.  Todd's nurse coordinator also joined us to give us the latest news from his doctor.  He examined Todd as usual looking for any sign of Graft versus Host Disease rash.  I asked if there is still a good chance that he can get GvHD and he said absolutely yes; with the mixed chimerism, there is still a chance.  This would be ideal!  If Todd's body could develop a bit of Graft versus Host Disease it would help attack and kill his diseased bone marrow and give his brother's donated marrow the upper hand.

They took him off his magnesium tablets, which is normal a few weeks after stopping the Tacrolimus.  The Tacrolimus causes low magnesium, therefore the added supplements.  So, without taking the Tacrolimus for several weeks, he shouldn't need it anymore.  He does still need to continue with the rest of his medication until further notice.

Todd's cytogenetic report was back from his bone marrow biopsy.  It did show one chromosomal abnormality: a deletion in chromosome 5 [46,xy,del(5)(q13)(17), xy(3)].  He had this same deletion, along with three others earlier in the year in April and May, but they had gone away with the four rounds of Vidaza before his bone marrow transplant.  So, this one deletion has come back.

I asked what the next step was going to be, and his nurse coordinator jumped in and said that his doctor would like to get a couple rounds of Vidaza in before doing the second stem cell transplant.  So, we know that he will start his next round January 12, 2015.  I'm not sure if there will be a third treatment in February or if they will go on to the stem cell transplant.  They will be looking for a decrease in his blast counts when they do a follow-up bone marrow biopsy.

I also asked if there was a mistake in his chemotherapy, the trial medications, or the immunosuppression regimen during and after the transplant that allowed him to relapse.  I explained that I had heard of other patients having their own marrow suppressed instead of the donor and that his doctor was going to do this for the next stem cell transplant, and he said that this was not standard protocol for an initial BMT. He reassured me that it is standard procedure to always suppress the donor marrow because without immunosuppression  there would most definitely be severe GvHD.  I also asked if having the chromosomal deletions prior to the transplant contributed to the relapse of his MDS, and he said there is no way to know the answer to that question.  He explained that Todd's current doctor is trying to learn the answers to this question with her clinical trial.  In his opinion, nothing done during the first transplant contributed to the relapse.

We don't need to go back to Cleveland for treatment on Friday, December 26, as long as we arrange to have his CBC blood work drawn here in Dayton, and follow-up with any needed transfusions at a local hospital.  Our next Clinic appointments will be for treatment and seeing his regular physician on Wednesday, December 31, 2014, where we will discuss his latest chimerism results, the plans for future treatment, and to schedule additional appointments. 

His 100th day is Christmas Day.  Although we won't be celebrating a cure or remission, we will be celebrating life! 

Day +84: Biopsy scheduled Today Dec. 9, 2014

On Sunday afternoon, December 7, 2014  Todd received a message from his online "My Chart" that the BMT Team finally scheduled his bone marrow biopsy for Tuesday, December 9. That meant we were going to have to be at the Cleveland four days this week!  This was too much since we have been trying to drive home in between appointments on Mondays and Fridays. This week, however they had scheduled his doctor's appointment on a Wednesday instead of a Monday with 3 treatment appointments Monday, Wednesday, and Friday. I think the schedule was changed due to his doctor's schedule. He hasn't needed a Wednesday appointment since the first week he was out of the hospital. 

Todd decided he wasn't going to go up for his Monday treatment appointment, so I called the doctor's office to rearrange this week's schedule. We decided to drive up on Tuesday for the biopsy and spend the night so we could be there early for his Wednesday treatment and doctor's appointment. 

We won't get the results of the bone marrow biopsy for about 4-7 days and then the cytogenetics will take a few more days longer. They are looking for a few specific things. 

First, that there is bone marrow from the engraftment. If there is no significant amount of engrafted marrow this would likely indicate a failure in the BMT. 

Second, they will look at the chimerism of the cells present: how many are from his own original bone marrow and how many are from his brother's donated marrow that has engrafted. 

Third, they will look to see if there is any disease present (MDS). They can determine this by looking for blast cells and/or the type of blast cells, and how many there are. 

It looks like they are going to do a CBC today and/or tomorrow to check his counts. 

We are expecting good news. We have been praying that God would bless us, and Todd's body specifically. We are praising God in advance for his wondrous mercy. Please continue to pray on his behalf. 

Day +73: Mixed Chimerism Still an Issue

Todd with his Great Nephew Jeffrey at Thanksgiving, November 27, 2014

To follow-up to last week's post, we saw the bone marrow transplant doctor on Monday, November 24, 2014, with no real new news or progress.  After reviewing Todd's CBC blood counts, she concluded that they were stable with no significant decreases.  She had ordered another chimerism study to be drawn that day too.

It was difficult for her to hide her concern.  She recommended that Todd drop down another Tacrolimus pill, from 3 per day to 2 per day immediately. I asked her to give us some indication of her level of confidence that reducing the immunosuppressant would be effective.  She really hated to put a number on it, but I got her to say about  50%.  She tried to encourage us that she was really stepping up the process of removing the immunosuppressant (starting Day+62), because normally, they don't start tapering patients off this drug until they have reached Day +100 and even then they taper it slowly for months.

I confirmed that Todd did have a full intensity, myeloblative allogeneic bone marrow transplant and not Reduced intensity chemotherapy (RIC) which is more common with bone marrow transplant failures.  Additionally, she confirmed that even the timing and the type of medications used in the trial regime wouldn't have necessarily had anything to do with the mixed chimerism that Todd is experiencing. 

She also reiterated that she wants to move the date for a bone marrow biopsy.  Normally, this is done around Day +100, but she wants to do it around Day + 80, which would be around December 5.  She said then they could look to see how many cells are actually in the bone marrow, what their chimerism would be, if there was any presence of the disease (meaning his MDS or blast cells), and if so, at what percentage.

I also implored her to talk about what would happen if removing the immunosuppressant didn't work, and she did somewhat reluctantly.  The most likely scenario would be to harvest stem cells from Todd's brother again, but this time it would be done peripherally,which means they would remove the stem cells from his brother's blood instead of from actual marrow in the hip.  This process would require Todd's brother to receive medication injections prior to the harvest to boost the number of stem cells they could obtain.  However, the process would be much easier on his brother Tom than before.  This 45 second video shows the process: https://www.youtube.com/watch?v=2bTCSslVmKs or this 4 minute video: https://www.youtube.com/watch?v=pZgee5RWccs.  She did not say whether or not he would need additional chemotherapy prior to this transplant, but that they would use a different kind of immunosuppression therapy that would work to suppress Todd's bone marrow right away and not the donor's stem cells like  normal. 

I asked if Todd would be required to be admitted and stay again under close monitoring at the hospital for 30 or more days like before, but she just stated that the process wouldn't be quite as "strict."  She then responded that I didn't need to worry about it; that it was her job to worry about it.  I agreed that it was out of my hands and that she was the professional, but that I would like to have an idea of what might in store just in case, especially since I was planning on going back to work, or finding a job.  If all of this happens, I may not be able to work at all for some time. 

After his appointment, we headed home to spend an entire week there!  We were allowed to cancel his Friday appointment (since it would be the day after Thanksgiving) and return on Monday.  Todd did receive a call from the doctor though late Wednesday night, November 26, 2014, asking him to take away another immunosuppressant/Tacrolimus pill starting that day (cutting them down to 1 tablet/day). She was able to review the levels found in his blood from Monday's results and described the amount found in his system, as "therapeutic" and that this was still too high for him. I imagine she will likely take the last pill away sometime next week. 

Todd's chimerism results showed up on his MYCHART app today: 57% of the cells being from Todd's original bone marrow and only 43% of his brother's donated cells.  This was likely what she saw when she called on Wednesday.  Chimerism results from this week may not be in until Saturday or Monday since much of the staff was off for the Thanksgiving Holiday on Thursday.

Despite this discouraging news, we were so Thankful for Life, Love, Friends, and Family this Thanksgiving Holiday.  We spent Tuesday afternoon with our dear friends Chris and Lynda, with my family on Thursday, and we are planning a dinner tomorrow with Todd's mother.

We are still praying that the removal of the immunosuppressant will be effective.  We go back on Monday, December 3, 2014 for: blood work, an appointment with the bone marrow transplant doctor, and a physical therapy appointment for his frozen shoulder late in the afternoon. 

Bone Marrow Biopsy Results returned prior to transplant.

Todd's bone marrow transplant doctor sent him a message yesterday, August 22, with his cytogenic results from the bone marrow biopsy he had on August 7, 2014, as part of his staging leading up to the transplant. 

We were shocked to learn that with four rounds of Vidaza, all four of the chromosomal abnormalities/deletions he had been diagnosed with biopsy earlier this year, had been reversed, and that NO deletions were detected on this biopsy.  While we knew this was possible, we were surprised to learn he had responded so well to the chemo.  According to the transplant doctor, this is great news to receive prior to having the bone marrow transplant. 

Not only did the Vidaza help with the deletions, but his blast counts were still down to about 1%.  These results will give Todd ideal conditions going into the transplant.  We are thrilled, but couldn't help to question, "Why should we still do the transplant then?" 

So, I began trying to pull up research on the reversal of chromosomal abnormalities after receiving Azacytidine or Vidaza.  After reviewing some research, I learned that these results are short lived and are dependent upon repetitive use of the drug.  It seems like with those who did achieve some kind of remission, it was only for four to fifteen months before relapse.  Some also suffered from neutropenia, which is low blood counts that require treatment, and others had some long-term side effects from the toxicity of the drug. (Raj and Mufti, 2006: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1936359/#!po=46.9780)    The actual dysplasia of the blood cells that is found with the initial MDS diagnosis are still present in all cases, meaning there is no change with cause of the disease. 

Once again, we feel reassured that Vidaza was a great short-term option for pre-loading before the transplant, but it is not a great long-term solution for Todd's case of MDS.  We still feel like the transplant with his perfect match donor is the best long-term solution for him at his age of 45 and with the progression of his MDS.

Update: Bone Marrow Biopsy scheduled for Friday, May 23, 2014

We received a call from Todd's bone marrow transplant doctor at The Cleveland Clinic on Tuesday, May 20, 2014.  She was back in town and wanted to review the "game plan" with Todd.  She asked how the first round of Vidaza went and how he was feeling.  She assured us that the low blood counts were indicative of having the Vidaza treatments and that the blood counts would go back up to his typical numbers in time. 

Todd discussed changing our local oncologist and she said that was fine.  She said she wanted to schedule the bone marrow biopsy for next week.  While we were under the impression that she originally wanted to do the biopsy to review the affects, if any, that the Vidaza was having on the progression of Todd's MDS, this isn't the case. Instead, she wants to do a biopsy NOW to make sure that Todd's MDS isn't naturally progressing on its own since the last biopsy.

Todd was discouraged by this.  He hadn't been flooding his body with the soy peptide or nutritional supplements he has been taking the last two years to help keep his body in optimal health. These products, purchased through the company Reliv, have had positive affects on keeping Todd's blast counts down (basically the cancer cells, which are immature and ineffective blood cells that can't function properly). Since he was experiencing nausea and lack of appetite, he had a hard time drinking them. 

His doctor met in conference with the other bone marrow transplant doctors and the consensus was to do another round of Vidaza if his bone marrow results are "stable" which means about the same: 7-9% blast counts and no new chromosomal abnormalities.  If the results show any more progression, there will not be another round of Vidaza.  The bone marrow transplant will continue to be staged and scheduled as soon as possible.

The scheduling office called today and had an opening for this Friday morning for the bone marrow biopsy to be done in Cleveland. So Todd will be going this Friday instead of next week. Results will take several days, with preliminary results by Wednesday, May 28, or Thursday, May 29.

So, we wait.


Notes:

Defining a blast cell:

In a patient with a myelodysplastic syndrome, the blood stem cells (immature cells) do not become healthy red blood cells, white blood cells, or platelets. These immature blood cells, called blasts, do not work the way they should and either die in the bone marrow or soon after they go into the blood. This leaves less room for healthy white blood cells, red blood cells, and platelets to form in the bone marrow. When there are fewer healthy blood cells, infection, anemia, or easy bleeding may occur. (US Gov., web).

For more information about MDS, blood cells, MDS, and to see a diagram of a bone marrow biopsy see:  http://www.cancer.gov/cancertopics/pdq/treatment/myelodysplastic/Patient/page1

In assessing MDS, using the original IPSS scale (International Prognosis Scoring System), the doctors looked at blast cell counts in determining the risk assessment of the progression of the disease.  When the blast cell count reaches 20% or more, it is then considered to be full Acute Myeloid Leukemia.  Since diagnosis, Todd's counts have gone up and down from 2% up to 7% until reaching their highest point of 9% in the first few months of 2014. Now, the MDS community uses the IPSS-R (the Revised International Prognostic Scoring System) which takes more things into consideration, like hemoglobin, absolute neutrophil count, platelets, and cytogenetics and the number of chromosomal abnormalities, in addition to bone marrow blast cell counts.

Todd's doctors were using the 10% blast count as a personal threshold for doing the bone marrow transplant before he started developing chromosomal abnormalities a few months ago.

To learn more about the IPSS and the IPSS-R:

http://www.qxmd.com/calculate-online/hematology/myelodysplastic-syndrome-prognosis-ipss

http://www.mds-foundation.org/ipss-r-calculator/

Todd celebrates his 45th bithday on his 5th day of Vidaza

Good News:  Today we celebrated Todd's 45th Birthday!
Bad News:  He had to get his 5th treatment of Vidaza.

No one wants to spend their birthday getting chemotherapy or being weak, nauseated, and sick.  However, we are praying that this treatment and the treatments to come will give us another 40 or more years of birthdays for Todd!
                                                                                                                            

Todd with daughters Abby, Ellie, & Hannah

Day 4 was not as good as day 3.  He wasn't allowed to have the Aloxi that day, but they wrote him a prescription for a different anti-nausea medication called Ondansetron HCL that he takes orally every six to eight hours as needed.  While it helped, he didn't feel as well as Day 3.

Day 5: He received the Aloxi, but I think depression accompanied the fatigue and sickness today.  He has things he wants to do and it is depressing to spend your birthday being sick.

Todd with wife Kimberley and daughter Abby.

We took him out for lunch at Panera Bread and he ate some soup, bread, and half of a sandwich.  Then he came home and took a long nap.  He got back up this evening as our family went over to his mother's house for dinner, cake, and ice cream.  He ate moderately and enjoyed the taste of the cake, but felt sick after he ate it.

Although he has lost some weight, he still looks great!  Hopefully, he can rest up and bounce back a bit with a respite from the treatment on Saturday and Sunday.  He will begin treatment again on Monday and finish with his 7th treatment on Tuesday.  They will do some blood work to check to blood counts, which have a tendency to decrease with the seven-day treatment of Vidaza.  Round one will be done.

At some point, they will also follow-up with a bone marrow biopsy.  The waiting period for re-treatment is 28 days, but we are not sure he will receive another round or if the bone marrow transplant will be scheduled by then.  It depends on his blood counts, bone marrow results, whether staging for the transplant has been completed, and his bone marrow doctor's re-assessment.

Todd's mother, Anna, after the birthday dinner. 

If your dad were here Todd, he would say, "KLU, Keep Looking Up!"  The rest of us who are here say, "We love you!  Keep the faith! and Hang in there!"  We are happy to celebrate your life today.  Just think it has been two and half years since your diagnosis and we are still fighting!  I know that God will use this to his glory!  He's got so much more in store for you!

Background: Diagnosis October 27, 2011

Todd was first diagnosed with Myelodysplastic Syndrome, or MDS, on October 27, 2011 at the age of 42. 

Todd had been routinely getting blood work done as a precaution while taking a certain medication every 6 months.  After several blood tests, his family doctor noticed that his blood counts were declining with each test.  This prompted his doctor to refer him to a local hematologist for further investigation.

Todd met with a local hematologist, who ordered blood work and a bone marrow biopsy.  Todd's initial blood tests showed all three blood counts, white, red, and platelets, on the low side of normal. 

The hematologist diagnosed Todd with a "De Novo" case of MDS, which means it developed without any known cause.  He also gave the diagnosis of Thrombocytopenia, which means that he had an abnormal low number of platelets in the blood. (Clevelandclinic.org/disorders/myelodysplatic).

Using the IPSS or the International Prognostic Scoring System, he rated Todd as "Low Risk" of progression toward Acute Myeloid Leukemia or AML. (Progression to AML happens to about 30% of those with MDS).  His blast count, or the immature, unhealthy blood cells in his bone marrow biopsy, at this time was in the normal range at  <1%.

The Hematologist said that it could take up to 10 years for the MDS to progress, but that he would guess Todd's life expectancy at the time with  no treatment would be about seven years. 

His recommended treatment was to give  Todd iron infusions via an IV and watch Todd's blood counts and blasts counts in a "Watch and See" approach.  Todd  wasn't supposed to return for another follow-up for 6 months.  If we felt uncomfortable with plan, so he suggested that we go get a second opinion from Cleveland Clinic or Ohio State.

Of course, after doing two rounds of Iron infusions, for some unknown reason, because Todd's iron was not low, we decided to do just that and made an appointment with the Cleveland Clinic for November 11, 2011.