Transfusion Dependency Continues.

Todd was tired and ready for a transfusion on our way to Cleveland Clinic August 17, 2016. The had a full day scheduled for him so we needed to start the day early which meant going up the night before. We usually stay at the Hope Lodge in situations like this but now that I'm working full time it is difficult to get there by 7 pm, the latest check in time. I would have had to take off work early on Tuesday in addition to taking off that Wednesday all day. Instead, Todd made hotel arrangements through the Priceline website. If you have never used it, you bid on a room for a certain price. But you have no choice of what hotel you end up with and there are no refunds.

We ended up with the Hilton Garden Inn Downtown Cleveland near the ball stadium. Sounded good. Unfortunately, we got a late start and didn't arrive until almost 11:00 after the long drive. Todd was exhausted. We went to our room and realized it reeked of cigarette smoke. We went back to the desk and tried to explain that Todd was a cancer patient, already didn't feel good and had a cough and we couldn't stay in that room. They informed us that it was a smoking room and that they couldn't move us because the entire hotel was full (which I find hard to believe on a Tuesday night).  They only offered to spray the room with a scent or put in an ozone filter machine, but that it would take hours!  I told them that neither option would rid the room of the smell plus he was exhausted and needed to lay down now. They refused to give us a refund saying their hands were tied because we booked through Priceline. I appealed to their moral obligation to do the right thing but once again said there was no other room. We also challenged the hotel for having smoking rooms in the first place when Ohio has been smoke free for over 15 years!  They said that since they were renting out a private space they could get around the laws and that they plan on getting rid of the smoking rooms when they remodel in the future. 

I don't mean to offend smokers. I have loved many people who were smokers,  most of them having died from the side effects. However, this is the reason why the laws are in place in Ohio: to protect non-smokers who have no choice in the matter. Especially sick people and children. Most smokers I know are conscientious and would gladly smoke outside. But this business wanted to make money on those smoking rooms they were having trouble selling. 

We said that Priceline listed the room as a non-smoking room, but the hotel insisted that they tell Priceline it may be a smoking room and it is Priceline's responsibility to tell consumers. I tried calling another hotel we had stayed at before. The entire hotel was non-smoking and they had a room at the Cleveland Clinic rate available but Todd was too exhausted to go. So we had to endure a night of smoke smell and no curtains. I called the GM but got voicemail. He called me back the next day while we were at the Clinic to offer us a free room but I refused.  I didn't want a free room then. He said it that every single room in the hotel that night was booked. I congratulated him and told him they apparently didn't need our patronage if they are that busy on a Tuesday night. I told him I don't want to stay anywhere that is not smoke free and I said with one in three people getting cancer it was likely he or the men working that night may have a sick family member and may be in our shoes one day. We showered, dressed, and tried to get out of there as fast as possible. I didn't want to walk around smelling like smoke all day!

When we arrived at the Clinic, Todd had to start his day of appointments with a few tests on his lungs, including a pulmonary function test.  We saw the pulmonary physician.  He asked several questions and reviewed the results of his prior lung CT in addition to the morning’s tests and said nothing looked suspicious.  He had no idea what was causing Todd’s cough.  He prescribed him some cough medication, but that was it. 

Next, was lab work.  They were going to give him at least one unit of blood even if his hemoglobin wasn’t below 8.0.  It had been hovering around 8.1 -8.3; not enough to get a transfusion but still not enough to give him energy.   Originally, the trial nurse didn’t have a treatment appointment scheduled for a transfusion, but on the Monday before, I knew he wasn’t feeling good and would likely need it. So, she was able to add it to the schedule.  It was a good thing, because he did need it.  His hemoglobin had dropped to 7.5 and they were going to give him 2 units.  We were waiting for the type and screen and results to come back when Sam the trial nurse and Dr. Hamilton came in to see him.  We were disappointed that all his counts had once again dropped even more:

Whites had dropped to 1.46; ANCs to .95, and platelets to 31,000. 

She thought it was likely that the trial drug AG-221 was losing its effectiveness.  There was just no other explanation. I was confounded when Dr. Hamilton started talking about other options: Revlimid (for patients with Chromosomal Deletion 5q), and even harsh chemo and a second transplant!  I didn’t understand why we were discussing this now; neither one of these last two options would have a high success rate at this point.  I thought we should have at least Revlimid to try and/or going back on Vidaza again before bringing those options up. She was also concerned that he was starting to lose a little weight.

While getting his transfusion, the respiratory therapist came in and gave him his Pentamidine Breathing Treatment and another trial nurse came in for his EKG.  He had to miss two other appointments, because of course, everyone was running behind and we couldn’t get to either.  One was to receive more immunizations.  I wasn’t upset about missing this one.  I didn’t think it was a good idea anyway; to be getting more immunizations with his counts so low. 

We drove through thunderstorms and finally got home around 9 p.m.  I was hoping he would start feeling better right away, but the next day, he felt faint and couldn’t drive home from a haircut.  Luckily he was near his mother’s house and stopped there to take a rest and then drove home a couple of hours later.  That day and the next day he still felt puny and didn’t get out of bed much. 

This concerned me, so on Thursday, I put a call into the doctor and she called me back on Friday morning, August 12.  I told her about Todd’s lack of energy and nausea.  I also asked why she brought up chemo and a second transplant.  She said she just wanted to discuss all of his options.  She said that if Todd started needing transfusions more often, that she would consider taking him off the trial drug and starting the Revlimid, but not until then. 

By Friday afternoon, I was concerned and decided to call Dr. Eytan Stein at Memorial Sloan Kettering in New York City. He saw Todd before he started on the AG221 and I knew he had a lot of trial experience with the drug.  I wanted to pick his brain about other options.  I left a message and by that evening, he called me on my cell phone at home.  We discussed Todd’s case and current condition, the great response he had with AG221, and then the steady decline of his blood counts.  He was surprised at Todd’s great results with the drug, but then said that they had noticed that some patients who stopped responding to AG221 who had the IDH-2 genetic mutation, often developed an IDH-1 mutation in addition.  For these patients, there was a new trial drug AG881.  He asked me to have Todd’s bone marrow biopsy, doctor’s notes, and latest genetic panel sent to his office for him to review.  

I sent an email to Sam, his Trial Nurse at Cleveland Clinic to request they send the information to Dr. Stein.  Unfortunately, they hadn’t done a genetic mutation panel since May 2015, so they would have to wait until his next scheduled bone marrow biopsy to get this, but she sent what they had.  We were charged $45 for this request, but I will pay it happily.  I decided last year that I would do whatever it takes, including seeing the best doctors, traveling to any hospital to help him. 

I talked to Sam on August 17, the following Wednesday and told that I didn’t want to step on Dr. Hamilton’s toes, but that I really wanted to hear what Dr. Stein had seen in his trials and practice that could be of any help to Todd.  I also asked her to call in blood work orders, as Todd was still feeling poorly and I feared he needed another transfusion.  She called them in for the next day, Thiursday, so if he needed a transfusion, he could get it on Friday before the weekend.  Stubborn Todd however, refused to go then.  He wanted to wait until Monday, August 22, 2016 to go have his blood work done that way they could use it for his trial draw and wouldn’t have to repeat blood work scheduled on Wednesday August 24, for his trial draw for Cycle 18, Day 15.  Well, that was a mistake, one he admitted later.  He felt horrible all weekend. He didn’t drive, leave the house, or get out of bed.  The Olympics were on TV, so that kept him entertained in bed between naps.  By early Sunday night, his cheeks looked red so I grabbed the thermometer!  He was running a low-grade fever or 100.3; enough to go the emergency room.  He refused to go. I conceded as we both thought it was just a neutropenic fever.  We kept an eye on it and it was down to 99.6 before bed.

In the morning, he took a shower and his temperature was normal.  I took him to Soin Medical Center for a nurse’s visit in the Cancer Center there on the 4^th floor at the appointed time of 8:30 a.m.  His nurse also felt that he had waited too long to come in and thought he looked especially jaundiced since he was so pale (low hemoglobin).  She said that she wasn’t going to let that happen again and scheduled him for another blood draw to check counts for next week, August 31, 2016. I was glad for that.  After waiting almost 2 hours since we arrived, we finally got his counts back:

Hemoglobin was a low 7.0.  Whites 1.0.  Platelets 22,000 (transfusion of platelets needed at 15,000) and ANCs at .7 (neutropenic at .5).  

She gave him all the necessary warnings about his care: Careful with hot showers because of getting petechia spots, bleeding while shaving, etc.  When she went to schedule the transfusion, she came back and said she set it up for the next day, thinking that was what he would want.  I was upset at this!  I told her that he needed the transfusion TODAY! And that we were not leaving without one; he couldn’t wait.  Todd was so sick, he didn’t feel like arguing with her; so I did!  She told us that it would take 3 hours for the type and screen, longer to get the blood ready, do the transfusions, and that we wouldn’t be done til 8:00 p.m. that evening.  I told her I didn’t care and questioned why she didn’t do the type and screen when she drew blood.  He had already told her that he knew he was going to need a transfusion.  We had already been there for 2 hours!  She said she can’t type and screen for blood type until the initial blood results come back and she gets orders for the transfusion.  To me, this could have been done at the same time; this is what they do at the Cleveland Clinic and I told her so.  Long story short, I was so glad that I took him and told my boss I would be late.  If not, I know he would have went home and waited the next day.  Then, he would have had another day of feeling  bad and getting up early again. 

It didn’t end up being as bad as she thought.  I wheeled him done to the Universal Care Area and they checked him in right away and got him a bed.  I made sure he ordered lunch, then I went into work for a few hours.  They had the first unit to him by 1:24 p.m.  I went back to the hospital about 4:20 and he was finishing his second unit.  We were out of there by 5:00 p.m.!

This is why the caregiver needs to be present to help with the decisions that the sick patient don’t feel like dealing with.  He didn’t want to argue with the nurse; he didn’t feel like it!  But I did!  He told me later that he was glad that he didn’t wait until the next day.  And unknown to the nurse, we were out 3 hours earlier than her prediction! 

That afternoon, I talked to Sam, his trial nurse at the Cleveland Clinic and let her know his numbers and that he received 2 units of blood.  She said that she was feeling pretty sure that he was losing his response to the AG221 and would talk to Dr. Hamilton.  She was also concerned with his low white and ANC counts and would also discuss putting him back on his Cipro antibiotic as a preventative measure.  After talking to Dr. Hamilton, Todd was ordered to take his Cipro 2x a day and we were told that Dr. Hamilton was going to talk to Dr. Stein at Memorial Sloan Kettering to discuss Todd’s case and the option of taking him off of the trial drug AG221 and putting him on Revlimid. 

The transfusion seemed to help.  He was able to get around yesterday and even drive.  I am feeling a sense of urgency to get his medication changed and was greatly relieved that the two doctors are going to talk.  

The trial for AG881 is only available at 5 US locations: New York, Chicago, Boston, Aurora Colorado, and Texas. We won’t be able to find out if he eligible until he has the genetic panel processed on his next bone marrow biopsy at the Cleveland Clinic at his next appointment on September 7, 2016.  Then, it may take a few weeks to get the results back.  I’m hoping in the meantime that they can start him on Revlimid.

Did I mention that Revlimid (Lenalidomide) is on the market already and that it is very expensive?  According to Wikipedia, the cost for one year’s use was about $163,381.00 in 2012. (Wikipedia, 2016). Drugs.com quotes a 10 mg tablet, the starting dose for MDS patients at $16,457.14 for one month’s dose of 28 tablets!  (Drugs.com. 2016). I’m praying we will not have to pay all of that between insurance and a request to the drug company, Celgene, to buy it at a reduced price.  Of course, there is no generic available.

I'll post after blood work next week. Until I continue with my mantra:  Whatever it takes!

For More Information: 

Alsumidaie, M. January 2, 2015.  “The Cost of Saving a Cancer Patient’s Life” Applied Clinical Trials. Web. Retrieved from: http://www.appliedclinicaltrialsonline.com/cost-saving-cancer-patients-life-analysis-celgenes-revlimid

Celgene.com. May 2015. “Celgene Patient Support for Revlimid” Web.  Retrieved from: http://www.celgenepatientsupport.com/revlimid-patient/

ClinicalTrials.gov. August 23, 2016.  “Study of Orally Administered AG-881 in Patients With Advanced Hematologic Malignancies With an IDH1 and/or IDH2 Mutation.” Web.  Retrieved from: https://clinicaltrials.gov/ct2/show/NCT02492737

Drugs.com “Revlimid Prices, Coupons and Patient Assistance Programs.” August 8, 2016.   Web. Retrieved from:  https://www.drugs.com/price-guide/revlimid

Wikipedia. Lenalidomide (Revlimid).  August 24, 2016. Footnote 2, 2012.  Web.  Retrieved from: https://en.wikipedia.org/wiki/Lenalidomide

MORE TRANSFUSIONS MAY BE NEEDED AS COUNTS REMAIN LOW

My last post left off with Todd going into the local oncologist/lab to get his routine labs done for his trial drug AG221 Day 15, Cycle 17 on July 27, 2016. We were anxious to see if the transfusion he received the following Thursday had helped to boost his blood counts.

Unfortunately, they did not. The results showed his Hemoglobin at 8.3, whites 

at 1.4, ANCs at 1.1, and platelets at 46,000.  This means he is once again close to needing another transfusion (hemoglobin < 8.0). This is also the lowest his immune system (reflected by the white and ANC counts) has been since he was in the hospital last October 2015. What does all of this mean for everyday living?  Taking extra precautions with activities like shaving with a razor (low platelets), living with severe fatigue, watching for a fever, and being cautious with what he eats (washing fruits and vegetables well, avoiding raw or under-cooked fish or meats, etc).  But worst of all, being severely anemic means  transfusions. The transfusions themselves aren't necessarily bad for him, even though they can result in a build up of iron, but they are inconvenient and often a multi-day process. First, getting labs to see if he needs a transfusion, then getting typed and screened, then setting up the transfusion at the hospital, and then the almost day-long process of getting the 2 units of blood. The depressing cycle  of fatigue and knowing your counts are dropping and hoping the transfusion will provide the much needed energy can be draining. 

I talked to Todd's bone marrow transplant Doctor this morning to get an idea of the game plan and where we go from here. She informed me that they ran a complete viral panel with his blood work and everything came back negative. If he had something bacterial he would have a fever. And the last bone marrow biopsy showed no increase in blast cells. 

This was her opinion:  Schedule appointment with Pulmonary Specialist to look at lungs, since he is still struggling with the cough. Next, deal with the anemia with transfusions until we know what's going on. I agreed with her that it may take some time to see if his complications and anemia is the result of "the disease evolving." While the AG221 is helping to keep blast counts low, it may not be keeping the disease from causing the anemia and low blood counts. My daughter Abby had brought up a good suggestion, which I put to her: "Would it help/be an option to increase the dose of his AG221?"  It was something she hadn't considered, but said it could be an option, but, if he is already suffering from the side effects of nausea, diarrhea, and high liver enzymes, that increasing the dose would likely make all of these side effects worse.  Not to mention, she would have to approach the drug company and ask it was even an option.

She had just received his cyto-genetics report back from the most recent bone marrow biopsy, and while there was no real major changes or new chromosomal abnormalities, she had noticed an increase in the number of 5q abnormalities, which could be a reason for the lower counts.  She said that about 15/20 chromosomes observed had 5q deletions. She said she may consider the idea of switching Todd's medication from AG221 to a medication used to treat MDS patients with the 5q deletion called Revlimid.  Since the transplant failure, we knew he had this option in our back pocket if we needed it.  It might be time to pull it out and use it.  Unfortunately, it is very unlikely that he could continue on the AG221 and just in the Revlimid, because it would be against the conditions of the trial study.  But, if Todd would become transfusion dependent, and the AG221 was deemed no  longer effective, then this is another good option for him.  We all have been so bullish on AG221 because it has worked so well for him, so we aren't going to let it go until we know for sure that it is not helping him.  I've even considered calling Dr. Eytan Stein at Memorial Sloan-Kettering in New York again if we can't get some answers, to see what he thinks.  Whatever it takes; that is what we will do!

I'm trying to make him rest and take it easy.  It has been so blistering hot in Dayton the past week, that being outside is more  difficult for him. I'm  getting good with the Zero-Turn Lawnmower!  We are planning on taking it easy this weekend, plus, Hannah Lewis is getting wisdom teeth taken out on Saturday!  So, I'll be nurse Kimberley to the invalids this weekend! (just joking). 

Todd will go get his blood counts checked at the beginning of next week, if he thinks he needs a transfusion.  I have to say I was impressed with his nurses at Soin Medical Center.  They sent him a personally signed card wishing him to get better!

The next trial appointment at the Cleveland Clinic will be August 10, 2016.  They have a full day of tests already scheduled.  Maybe we will find out something more by then.  We wanted to try to take a family vacation before the kids go back to college and school, but its not looking good since we just don't know how he is going to feel and if will continue to need transfusions.

Thanks for your support.

MORE INFORMATION:
About revlimid: https://www.drugs.com/revlimid.html

COUNTS DROP FOR UNKNOWN REASONS; TRANFUSION NEEDED JULY 21, 2016

I hate that I tend to blog only when there is a major change in Todd’s condition, but when he is feeling well, we are busy trying to enjoy life!  Unfortunately, it has been since May since I have provided and update, and once again the news warrants the update.

Todd’s condition on the trial drug AG-221 (Agios/Celgene) has been stable.  He hit end of the one year anniversary being on the medication on March 23, 2016.  He had a bit of allergy symptoms in early spring April/May 2016, with a runny nose, sneezing, cough, etc.  We attributed this to the fact that the grass was being mowed often and the spring was wet. 

He felt well enough to visit his brother Ted in St. Petersburg in mid-June, but he was still suffering from allergy symptoms.  He saw his Bone Marrow Transplant Doctor at Cleveland Clinic every month on Day 1 of his drug cycle, and continued to get blood work drawn locally on his Day 15.  The doctor gave him the “thumbs-up” on his June 13 visit to fly to Florida and back for the short visit. He came back still feeling symptomatic, mostly still suffering from a lingering cough. 

He was feeling somewhat tired, but was able to get around most of the day just fine, but with a nap (it isn’t unusual for him to need a nap). We had missed a family reunion in Tennessee in June and wanted to go down to visit the weekend of July 4.  I haven't had a vacation in over two years! He had blood work drawn before we went on Day 15 of Cycle 16, which was June 29, 2016.  Since it was drawn at a local lab, he didn’t get the results until we were driving down to Tennessee on July 1.  All of his counts had dropped.  This was the first time in many months that there had been a significant, noticeable drop in counts.  His Hemoglobin was down to 9.0, white counts to 3.2, ANCs at 2.5, and platelets at 65,000.  Just to give you some perspective, his Hemoglobin had been running in the 10s and 11s, Whites and ANCs in the 4s, and platelets as high as 75,000. 

This was a shock to us; this meant he was only 1 point  away (<8.0) from needing a blood transfusion!  He hasn’t needed a transfusion since he was sick with the pneumonia and cold last October 2015.  Even though his counts had starting dropping slightly before this, we were not concerned, because we all understand that there is often some slight variation from visit to visit and from one lab (Cleveland Clinic) to another (Local Compunet). So, we attributed the slight differences in the differences in labs.

We tried to make the most of our trip to Tennessee, however, we quickly noticed that Todd was getting tired very easy.  When we returned, he didn’t seem well rested and still had that nagging cough.  So I ended up emailing his trial nurse at Cleveland (so did he!) the next morning to let her know.  Todd’s trial appointment was already set for the upcoming Wednesday, July 13, 2016 for Day 1 of Cycle 17.  So, they set-up at Chest CT in addition to his bi-monthly bone marrow biopsy, Pentamidine breathing treatment, EKG, and Echo cardiogram.  

The doctor had asked extensively about his cough and some tightness he was having in his foot/ankle before she gave us the news about the low blood counts.  We both felt something was wrong, and knowing her well, felt she didn’t want to give us the bad news. Then she let the bomb drop; the blood work for that day was back and his hemoglobin had dropped even more:  Hemoglobin down to 8.1, Whites #, ANCs #, and Platelets.   Basically, the initial view of the CT scan looked fine, and she didn’t have any idea why the counts were dropping.  We discussed having Todd tested for CMV or Cytomeglavirus which can affect post-transplant and immune-compromised patients, but she was hesitant to this.  I knew why.  She wanted to wait to see what the results of his bone marrow biopsy were.  If the blast counts, were up, we would have our answer.  This scared us; the trial drug has been working so well!  She said that it was possible that he was sick with a virus, and that the only time his counts had dropped like this (on the trial drug) was last October when he had the cold and pneumonia.  So, the possibility that he was sick, was a good one.  We left a little deflated. 

They don’t transfuse Todd until his hemoglobin drops below 8 and it was already late when we got done with all his appointments.  There was no treatment appointment made to give him a transfusion while he was there.  We would have to follow-up with his local oncologist. We didn’t end up getting home from Cleveland that night until after 11:00 p.m.

So, the next day, Thursday, he was still very tired.  His trial nurse called in blood work orders to his local oncologist and he went in on Friday, July 15, 2016.  We also asked if they would go ahead and have the blood drawn for the CMV test.

Before I go on, I have to tell you about our ObamaCare health insurance nightmare.  We received a letter from our Healthcare provider, In Health Mutual, an Ohio PPO created after the Affordable Care Act was passed in June saying that they were going bankrupt and that if we didn’t want to lose our tax credit/subsidy we had to select a new health care provider by July 1, 2016!  So, Todd had to go back onto the Government Healthcare Marketplace website and try to find insurance that the Cleveland Clinic and his doctor accepted. He found one: An Anthem BC/BS Silver PPO program, which he selected.  We paid for the new premium for the Anthem and had the information for the new insurance before Todd’s appointment at Cleveland Clinic on July 13, 2016, only to find out that IN Health Mutual withdrew almost $1,000 from our checking for the July premium-even though we switched healthcare companies!  We spent hours trying to get it corrected and to get our money back.  The input operator at the Marketplace also accidentally cancelled our Dental Insurance, so, we had to cancel Todd and Abby’s dentist appointments for that week and try to get our Dental re-instated!

Here’s the kicker, we realized when Todd went into see the local oncologist’s PA to have more blood work on July 15, that they didn’t accept our new insurance!  Basically, we would have to pay that bill out-of-network!  This is already in addition starting over on our yearly deductibles half-way through the year, when we had already met our deductibles on the prior In Health Insurance!  

Now, back to Todd.  The blood work came out about the same, Hemoglobin was still at 8.1 and the platelets had gone up slightly, but the PA decided that Todd could wait to have a transfusion and sent him home.  I called the trial nurse that day and asked if the preliminary bone marrow results were back; that it would ease our minds to know the results.  She sent him the results and Thank God, the blasts were still only at 1%!  This was great news!  The cancer had not been ratcheting up, but something unknown then was causing the drop in counts. 

By Tuesday, July 19, 2016, Todd was still very tired and his cough still persisted.  The trial nurse sent orders to the lab, but no one there was certified to take blood from his port, only from his arm and by this time, his arm was getting bruised.  I knew we needed to have his blood drawn again, and something needed to be done about getting him a new hematologist/oncologist locally.  So, I started calling.  First, to the new insurance company, Anthem.  They gave me 2 or 3 names, but only two were actually hematologists.  I also asked about what hospitals in the area were covered because he had always gone to Miami Valley Hospital in Dayton for all of his transfusions.  I found out that Miami Valley was no longer on our list!  Only the Kettering Medical Network of hospitals.  I was upset at first, not sure if any other hospital had their own blood bank nearby.  I also asked Anthem if Cleveland Clinic and Dr. Hamilton was covered and she said no!  I wanted to throw-up in my trash can I was so upset! So, I called the Government Healthcare Marketplace back and they said both were listed as providers! 

I called Dr. Amanda Laubenthal of Cancer Specialist of Greater Dayton.  They were very nice and I began to relax. They had an office in Beavercreek near Soin Medical Center, just minutes from our house.  The doctor was willing to see him right away, but they needed his medical records (at least his recent reports).  So, I called his trial nurse and Cleveland and she was able to send them electronically before we got off the phone!  She also told me that the CMV test had come back negative.  So, one more thing we could rule out!  Dr. Laubenthal’s office contacted Todd and he had an appointment to see the doctor and get blood work for Wednesday afternoon, July 20, 2016.  I was so relieved!  I didn’t get to go with him to the appointment that day because I had to work, but he liked the doctor and the blood work showed that his hemoglobin had dropped to 7.5, whites to 2.2, ANCs to 1.7, and platelets to 66,000.

At first he told me over the phone that he wasn’t going to get a transfusion even though his hemoglobin was under 8.0.  He was just going to “monitor himself” and get blood work next Wednesday!  I was livid!  I tried to convince him that he would likely feel worse and that come the weekend, he wouldn’t be able to get a transfusion and would have to wait until the following Monday to get in.  I didn’t understand why he was so opposed to getting transfused.  He told me he was going to go to SAMS Club to pick up some things we needed.  In about half an hour later, he texted me and said he was going to go back to the Dr. Office/hospital to get “type and screened” for a transfusion.  They had just closed the office, but lucky for him they let him come in and by 5:00 p.m. they had set up an appointment for a transfusion of 2 units of blood at Soin Medical Center for the next morning, July 21, 2016. I’m not entirely sure what made him change his mind so quickly, but if I had to guess, I would say that once he got to SAMS Club and had to walk so much, he realized how exhausted he was and that he had better go get the transfusion!  I knew he was stubborn, but… Anyway, I was just grateful that he changed his mind.

I had to work the next day, but I took a long lunch to go check on him during the transfusion.  I told Todd to make sure the blood they gave him for the transfusion was “Irradiated and Leucocyte-reduced, and CMV negative.”  He asked, and they assured him the first unit they were giving him was.  When I got there, they were just finishing the first unit of blood and getting ready for the second unit.  While they were getting it ready, I asked if it was Irradiated, Leukocyte-reduced, and CMV Negative, but they couldn’t find it labeled anywhere on this unit that it was CMV Negative.  So, I asked them to wait and ask someone.  This is a bit technical, but basically, people who have caught CMV (the virus that can cause Herpes) produce antibodies to fight against it.  Once you have been exposed to CMV, it can stay in your system forever, even if it stays dormant.  So, if the antibodies are present in the blood, then they know the donor has been exposed to the CMV.  We had just confirmed that Todd didn’t have CMV, nor did we want him to get it, so I wanted to be careful.  Most of the time, if the blood is irradiated it kills most germs or viruses.  In addition, if it is leukocyte-reduced, it means that they have tried to take out most of the white blood cells, where viruses like CMV reside.  This is likely good enough, but I knew that since they were able to screen for it now, and since the first unit was CMV negative, why shouldn’t the second unit of blood be?

So, they halted the transfusion and waited from word from the blood bank and the local oncologist.  Meanwhile, I called Dr. Hamilton, his Bone marrow transplant Doctor in Cleveland and his trial nurse to ask.  I got patched through to Dr. Hamilton and she said it would likely be ok, however, Dr. Laubenthal said No, just order new unit of blood that was marked CMV negative.  So, luckily Soin had more blood on stand-by and Todd only had to wait another 30 minutes for them to get the second unit of blood and start the transfusion back up.

I was glad though that I had a chance to talk to Dr. Hamilton, who said she was at a loss for what was causing the drop in counts since the bone marrow blast counts were low and the CMV test came back negative.  They were trying to come up with other possibilities and decided that he should be tested for RSV.  So, they contacted Dr. Laubenthal's office and had the nurses collect a sample (through the nose) while he was there.  The transfusion was long as usual, but helpful.  

I know medicine is a process of elimination, but it can be frustrating not knowing what is causing the drop in counts, even though all the tests for various viruses having been coming back negative, which is good.  The alternative of it not being an illness or virus of some sort, is too upsetting to contemplate at this point.  

He is scheduled to have blood work done for his Day 15, Cycle 17 done on Wednesday, July 27, 2016.  So, we are going to see how much this transfusion helped, get the results back from the RSV swab, and go from there.

We appreciate your prayers and support as always.


WANT MORE INFORMATION:
Cytomeglaviru (CMV)s: http://www.mayoclinic.org/diseases-conditions/cmv/basics/definition/con-20029514

RSV:
https://www.cdc.gov/rsv/
http://www.mayoclinic.org/diseases-conditions/cmv/basics/definition/con-20029514

MDS Foundation News Magazine Spring/Summer 2016 Issue

Here is the link to the latest MDS Foundation News Magazine.  It is truly a great resource for caregivers, patients, friends, and family members of those with MDS.  There are articles about the latest research and survivor stories, which are truly inspirational. 


Check it Out!
http://mds-foundation.org/wp-content/uploads/manual/2016springnewsletter.pdf


Kimberley

One Year on AG-221 Trial Drug

As of March 23, 2016, Todd has been taking the trial drug AG-221 for one year!  It's hard to believe how precarious Todd's health was this time last year: getting blood transfusions every 6-12 days; having no immune system (no ANCs and no White Blood Cells); and having no energy-barely able to get out of bed.  Today, one year later his counts are good (for him)!  The disease, while still present, has not proliferated, and his cancerous blast counts are low!  Besides being in remission, we couldn't ask for anything more!

To give you an idea, here is a comparison of the differences in his counts from last year to now:

Year:                2015                          2016

Whites:                 .2                            3.99
Hemoglobin:       7.3                          11.8
ANCS:                   0                           3.15
Platelets:       70,000                       57,000


As you can see, he has had a remarkable increase in his White count, Hemoglobin, and ANCs.  His platelets vary now between 55,000-75,000, which is about the same as they were a year ago.  His platelets have not responded as well. In trial terminology this is considered as a hematological response without platelet recovery.  That being said, his platelets are still high enough to prevent any serious bruising or the need for transfusions (usually when platelets fall below 10,000). 

After not hearing back from the drug company whether Todd could only go once a month for his trial visits as promised, I finally sent a message to his bone marrow transplant doctor telling her that we were unhappy with how the administration of the trial has been going.  We haven't received any financial reimbursements for months, none of our appointments have been made ahead of time like they should be, and when they have been scheduled, they have been for inconvenient times  (8:30 a.m. when they know we have almost a 4 hour drive to get there).  I "offered" to go straight to the Principal Investigator at Cleveland Clinic or the Drug Company itself, but the doctor finally got confirmation that Todd no longer needs to go to the Cleveland Clinic for his Day 15 appointments just to get blood work done.  They have arranged for Todd to have his Day 15 blood work at our local Compunet Lab.  This means he will only  need to go to the Cleveland Clinic on his Day 1 appointments, only once per month!  What a relief!  No more driving up to Cleveland every two weeks!  I'm so grateful! As to the appointments and reimbursements: they are short-staffed and trying to hire more help.

In other news, we just received his preliminary bone marrow results from his latest biopsy from March 23, 2016:

Results: "Persistent Myelodysplastic Syndrome.  Hypercellular Marrow (90%) with Trilineage Hematopoiesis, Erythroid Hyperplasia, Multilineage Dysplasia and 2% Blasts.  Increased Iron Stores and Increased Ring Sideroblasts. Mild Microcytic Anemia, Thrombocytopenia and Absolute Lymphopenia."

This is a long way to say that he has 2% cancer blasts in the marrow, which is Low! And he is still mildly anemic and all three of his blood counts: white, red, and platelets are low.  We haven't received the latest cytogenics back yet, but the prior biopsies have not shown any major changes.  They still show he has complex chromosomal mutations, but no new additional mutations.

With all this good news we should be on an emotional high-right?  Unfortunately, we are not.  There are still other complications in our lives right now, that we are not able to fully enjoy these blessings to the fullest.  First, Todd has been suffering from persistent diarrhea.  He can't seem to eat anything, without immediately having diarrhea (no fun).  He isn't dehydrated, but it is starting to wear on him.  We  have discussed the issue with the doctor at the last visit, and they have called in lab orders for Todd to bring in a stool sample (which he isn't real motivated to do-who would?). 

Second, I still have not been able to find a full-time job.  It hasn't been for the lack of trying. I put in about 5 job applications per week, which is very time consuming, because everything has to be done online and some places require mandatory personality testing, etc.   My friends have been diligent in helping me find postings, but I don't have anyone who can help me get my foot in the door anywhere!  I've been substitute teaching about 2-3 days a week, and spending some time volunteering at Wright State University Libraries in their Digital Services Department, where I am working on a project.  Never have I experienced such a depressing cycle: applying, following-up, and receiving some type of rejection notice.  Not only is it wearing on me, but it is wearing on Todd, who also feels helpless that he can't work, and that I have to go through this.  (He has always been the bread-winner and has faithfully provided for our family; now, he is so limited and cannot work because of the cancer).  After I fill out applications, I feel so encouraged and confident that I can do the job!  But, weeks later, disappoint sets in when I learn I didn't get the job.  I just don't know what it takes...

Third, we are considering selling the house again.  We have half-heartedly put the for sale by owner sign back out in the yard, but nothing else.  It is not enough to get us the attention we need, so we need to consider getting back online for sale, or hiring a realtor to list it.  The process of having the house ready to show all the time and the thought of moving is so stressful, that this really needs to be a last resort option once again.

Lastly, we both feel like a lot of circumstances are out of our hands.  I am doing all I can, he is doing all he can, but its not enough to get us where we need to be.  This has led to moments of despair for both of us, at least temporarily.  Then, we both come around to putting things back into perspective!  His health is better than it has been in a long time, I am healthy, the kids are healthy, we have a roof over our head, and food on the table!  God is good! 

Prayer requests and needs:

Please pray that I can find a full-time job soon.  I have one prospective meeting next week, and one job interview at Target, but I fear that they will not be hiring full-time nor above minimum wage.  I have about two more months in which I can substitute teach, then school will be out for the summer.  Abby and Lewis have begun looking for summer jobs too, as college classes will let out in a few months.

Please pray we can get answers to Todd's persistent diarrhea and that it will subside.
Pray for encouragement!  And as always, with me working and volunteering as much as possible, Todd is home alone.  He sleeps pretty late each morning, but I'm sure he would welcome a lunch invitation or just having some company. 

Praises and shout-outs:  Thanks to Chris and Jessica White for raising our spirits by taking us out to eat and then hanging out with us!  To Gary Johnson, for helping Todd get the snow throwing attachment off and installing the mower blade onto our Zero-turn lawn mower.  Now we can mow our own grass instead of paying someone to do it (which will save us money).  Todd has already started training Ellie on how to mow the grass!  To Vicki Kennedy and Dana Johns who are forever looking for job postings and sending them to me!  You both are so helpful!  To my friend Darsie Wohler for getting me out of the house one day for lunch and some girl talk!  And to Pastor Chuck Moore, who faithfully stays in touch with us so he can pray for our needs.

Bilirubin numbers too high; No AG221 right now

Todd didn't get to go to his routine trial drug appointment last Wednesday, February 10, 2016. He was up the night before with abdominal pain. He thought it was indigestion or something he ate. It finally passed after several hours but we didn't get much sleep. 

On Friday, he asked if his eyes and skin looked more yellow or jaundiced than normal and I said yes!  I had been asking him to contact his doctor at Cleveland Clinic to check in and get his biopsy results. He decided not to take his trial drug AG221 that day, because we know that it elevates his bilirubin at least 2-3x higher than normal, even though this increase is within the acceptable limits of the drug trial protocol which allows his bilirubin levels to go up to a maximum of 5x the normal level. 

Normal bilirubin about 1.5

Maximum bilirubin allowed on the trial 7.5. But even then they don't like it that high, because it can cause liver injury. 

Todds level on the drug usually stays around 2.5-3.5. 

I decided that it we should call the doctor. Not only I had noticed that he looked more yellow, but had asked him if his urine looked like it was darker than normal. Almost brownish red. He said he didn't think so, but after putting the stomach ache, dark urine, and greater yellowing/jaundice, I thought we wedded to ask. 

I called his doctor, but she was out of town at an ASH conference, but his nurse said she would run it by his trial nurse Sam and the doctor taking over for his doctor. Sam called me back quickly, and it was decided that they need some blood work. She worked with the doctor and faxed in orders to our local lab and have it processed STAT. 

Within 3 hours he had the blood work drawn, the results back, and we got a call back from Sam. All of our concerns were justified. His Total Bilirubin was 7.7 and his direct bilirubin was 4.4. His Alk phos was off the chart at 400!  (Normal  45-115).

So, Todd was told to go off his trial drug immediately. They scheduled an appointment for him at Cleveland Clinic on Tuesday where they will do some extensive blood work and see the doctor. 

Sam also looked up Todd's last bone marrow biopsy results, which sowed another 1% increase in blast cells, bringing them up to 4%. I still don't think this alarming, but I don't like that in the last 4-5 months his blasts have gone up slightly each time. 

 

Please pray that they will find out why his bilirubin went up so much and can recommend appropriate action. I think it is just the accumulation of the trial drug over time causing the escalation. Especially since he already suffered some liver injury when he was in the hospital last October where he suffered similar elevated numbers due to the addition of numerous medications to get his fever down and treat the pneumonia. The highest his bilirubin was then was 8.7 which was crazy high. Even then though his alk phos numbers were about 219. 

I'll post an update of what we learn after Tuesday's appointment. Thanks for your love, prayers, and support. 

January 2016 Begins Month 11 of Trial Drug AG221

It is hard to believe that as of this Wednesday, Todd will begin his 11th month on trial drug AG221. I was reading a news update on the drug (link here:  http://www.nasdaq.com/press-release/agios-announces-data-from-ongoing-phase-12-trial-of-ag221-showing-durable-responses-in-patients-20151206-00039 ).  It looks like they are continuing with additional trials this year for MDS patients in addition to AML patients.  It looks like Todd is not the only one seeing some improvement. When you read the article Todd is one of the MDS patients having a response to the drug. 

As we read the article, we were able to reflect on where he was a year ago! He was so weak and transfusion dependent. I was frustrated with the current rounds of chemotherapy (Vidaza). It is amazing that we know so much more about AG221 now than we did last year!  

I'm so grateful to Dr Etyan Stein for his work on getting this trial information in the news and Agios for putting his presentation online where I was able to watch it and encourage Todd to go on it and fly to New York to see Dr. Stein at Sloan-Memorial Hospital in New York City. 

Todd's last appointment in December went well. His blood counts and his MDS are holding steady. For those of you who are interested in CBC counts for comparison: White: 4.02; Hemoglobin: 10.9; ANCS: 3.42; and Platelets: 51,000.  His Bilirubin remains higher than normal, but still within the study limits. We did get his last bone marrow biopsy results back and they showed a 1% increase, resulting in a 3% total blast count. We aren't upset about this because this is essentially not enough of an increase to make a difference. They always have to allow for a small percentage of error or for slight variations as counts and bone marrow samples differ. The good news was that his cytogenetic report from that biopsy did not show any additional chromosomal abnormalities. 

Early in December, I did have a candid conversation with his Bone Marrow Transplant Doctor about the new research about the TP53 genetic mutation.  This mutation is acquired and is usually what causes the cancer or disease.  There has been some studies trying to determine whether patients with this TP53 mutation, don't respond well to transplants or are more resistant to therapy.  She discussed how the TET2 mutation also is being studied in relation to patients who end up with a "poor prognosis" or at higher risk.  I had learned about the TP53 mutation after reading a friend's blog about her son with Leukemia, who had relapsed after a bone marrow transplant, who is now relying on an alternative trial treatment that using T-cells.  (Craun, November 2015). And, when reading the last issue of the MDS newsletter, there was an article about TP53 and its connection to a patient's prognosis who has this mutation. (MDS News, Fall 2015). I asked Todd's doctor if Todd had this mutation, and she knew the answer immediately: Yes, he did.  If this research proves true, it would answer the nagging question of why Todd's bone marrow transplant failed.  It would also be helpful in the decision to have a second transplant someday; if the evidence shows this mutation inhibits a successful transplant, we would likely eliminate that option in the future.

I'm so thankful that he has not suffered any of the severe adverse events or reactions to the drug. Even though he has suffered terrible nausea and high liver function counts, it has been tolerable. 

I'm a bit disappointed that he has to have another bone marrow biopsy AGAIN this visit. The other tests are less invasive and they don't bother him as much. Hopefully, after he has been on the trial drug for 12 months, they will lessen the frequency of biopsies, although I have no assurance of this.  We were assured though that at after 12 months, his trial study appointments at the Cleveland Clinic would change in frequency from every two weeks to only once a month!  This will be great!

We said goodbye to Ashley, his primary trial nurse, who is leaving the program to take a nursing job back on the Leukemia floor.  We will miss her, as she was there for us from the beginning and whenever we needed answers.  We are confident that we will be in good hands with her replacement, Sam, who has been on the trial study team from the beginning too. 

So, Todd will have a full run of tests this Wednesday, as he plugs along on AG221.  We are curious to see how our new insurance, through the Affordable Care Act, works for us this year. The main focus now is to get him through Winter without getting sick.  We haven't done much socially, and fear vacation travel at this point, but it would be nice to get away for a little bit over spring break. 

 A few concerns and prayer requests:

First, like I stated in the last post in December, we received paperwork from the Social Security Disability Board asking for detailed information in their review of his continued approval for benefits, which is beyond comprehension to me!  I filled out about 10 pages of appointments, treatments, hospitalizations, transfusions, medications, etc. only to receive a second request form (8 pages) last week asking us to describe his daily functions in detail!  I can't imagine how anyone could look at these papers and not determine his continued need.  His health is so precarious, dependent on stable blood counts and staying completely healthy.  We learned how quickly he health could change in October 2015 when he was hospitalized for a cold and pneumonia for 13 days!  As this is our only source of income right now, we are desperate that it continues.  I don't understand how he was a productive citizen and paid the maximum into the system for years, and then we he needs it, they have to challenge it! 

Second, speaking of income, I still haven't found a full time job.  I graduated with my MLIS in December 2015, only to be let down by what little value these credentials are in obtaining a position.  I apply weekly for jobs, and try to do something productive everyday in the job search.  I have several applications pending at UD right now, but everything else I have applied for has not produced any offers.  I am also actively seeking volunteer experience and trying to schedule to attend several professional organizational meetings, but these have also been in limbo.  If you know of anyone who is hiring, even outside the field, please let me know!  I have applied for positions outside of field, and one of those applications is still pending.  It is not always what you know but rather who you know that helps land that job!

We took the house off the market for the holidays, as we needed a break from showing it and our listing had been on the market too long for serious offers.  We will likely put it back on the market in early March. 

We appreciate all those who have sent Christmas cards, money, and gift cards, especially Beau Townsend Ford's Employee Christmas Club Fund and that of our friends and neighbors. 

Right now, we are just trying to appreciate Todd's stability and giving God the praise for the blessings we have daily! 


References:

Craun, Gina. November 24, 2015). Windshield Wipers. Cainan Craun. Caring Bridge. Blog.  Retrieved from: http://www.caringbridge.org/visit/cainancraun/journal/view/id/56549676a589b45a417219e8

Prognostic Impact of TP53 Mutations. MDS News, Fall 2015,Vol. 21, Issue 2. page 11.  Retrieved from: http://mds-foundation.org/wp-content/uploads/manual/2015fallnewsletter.pdf