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

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

Pursuing a Second Opinion while tranfusions continue

This past week Todd had two appointments.  One with the local oncologist on Tuesday, March 3, and one solely for blood work on Friday, March 6, 2015. 

The oncologist appointment was uneventful.  We were happy that Todd's counts were high enough not to need any transfusions that day!  The oncologist just wanted us to update him on what the doctors at the Cleveland Clinic had suggested for Todd's next step in treatment.  He was unaware of the trial study drug AG-221, but after describing how it has worked so far, he was optimistic that this was a good choice. 

When Todd was getting his blood drawn by his regular nurse, she was anxious to hear the latest recommendations too.  We told her their decision, to pursue the trial drug study, and she began to tell us about a friend who too was proactive about researching her disease and possible treatments and had great success with a second opinion and treatment at Memorial Sloan Kettering Cancer Center in New York.  She also told us about a free flight program for cancer patients that took her friend to New York for treatment.  We brought this up to the oncologist and he told us that this was true.  The program uses corporate jets who are flying company executives to New York on business by offering cancer patients empty seats on their flight.  I went home and looked this up; actually there are several programs, but the one that his doctor and nurse were referring to is called Corporate Angel Network. 

I found this program along with the others on the Memorial Sloan Kettering Cancer Center's website under: Air Travel Assistance.  I have sent them an email and we are awaiting a response.

On Thursday, Todd had contacted his BMT doctor at the Cleveland Clinic to see if the other bone marrow results were in yet (including the much needed confirmation of the IDH2 gene mutation required for the study).  He received a message back saying that they had NOT come back yet and that the trail study in Nashville was not at Vanderbilt, but some research hospital there.  (I kinda guessed this when I was unable to find any information on Vanderbilt's website; and in Chicago, I could only find the study at Northwestern University).  So, this dashed my hopes of getting an expert second opinion at Vanderbilt. 

Todd had to go back to the Dayton Cancer Center on Friday, to have his blood counts checked again.  It confirmed what we already knew, he needed a transfusion.  Wednesday and Thursday had become increasingly draining for Todd and by Thursday afternoon, Todd didn't get out of bed.  His red count was just below the threshold at 7.9 and his ANCs and white counts were almost non-existent.  His platelets were up though around 56,000.  By this time of the day, almost noon on a Friday, the cancer center was NOT able to get Todd into the Advanced Treatment Area for a transfusion at either hospital: Miami Valley or Miami Valley South.  The only way for him to get his transfusion that day was to be temporarily admitted and put into a regular hospital room on the oncology floor.  Todd resisted this idea, and tried to side-step it once we got to the hospital, but they only sent us to admission and registration anyway. We both knew it was going to be a long day, but I have come to accept this.  I purposely did not plan on accepting any sub jobs for the day, anticipating this. I tried to reassure him that he was just going to have to suck it up and wait.  What was the alternative?  He would have maybe been able to get a type and screen done that day, and come back the next morning, but that would still end up taking at least five hours or more.  Plus, he would have been in bed the rest of the day Friday feeling so weak and tired, with no relief.  At least he could make himself comfortable, order dinner from the hospital menu, and get his transfusion done so he could start feeling better the next day.  After his transfusion, he felt extremely hot to me, and he did have a low grade fever of 99.9, which I was concerned about.  But, the nurse reassured us that this sometimes happens during or after a transfusion, and that he was allowed to take two Tylenol when he got  home.  He was about to get angry with him about fussing about the fever, begging me to be quiet so he wouldn't end up staying in the hospital for two days!  Instead, the nurse released us and told me to monitor the fever.  Oh, the grief the caretaker has to endure just because you are trying to do your job!

While waiting around in the hospital room that day, I decided to call Dr. Eytan Stein at Memorial Sloan Kettering, even though I had been delaying the call until we had received the final results back from Cleveland.  They were able to start a file for Todd, I answered all their questions, they told us which medical records were needed to be faxed, and anxiously informed us that the doctor had appointments as early as next Monday morning.  I told them that we wouldn't be ready to come that soon, but that I would get to work in requesting the necessary records from both sets of doctors here in Dayton and Cleveland. 

It ended up being a really long day (as always)!  We made it home about 9:30 p.m. and found my mom there playing Wii games with our youngest daughter. (Thanks mom for entertaining and feeding her!). 

We slept in late on Saturday morning and Todd felt well enough to have lunch with friends.  They could tell his energy level and spirits were much increased from the last time they had seen him.  I felt good that day too.  I felt optimistic that this study drug would be a good choice for Todd.  I pray it will be able to restore his blood counts enough to function without the need for transfusions, risk of infections, and eliminate his fatigue. 

But, for the meantime, he has been taking naps every day, which helps get him through the day.  I wish he had a better appetite, but I really can't complain.  I keep asking if I can get a current picture for the blog, but he usually responds like, not while I'm laying down! (Vanity still there! ha!).  

I was blessed to be able to work two days last week and I also received two calls for other jobs.  When talking with our friends and family, most have assured me to continue to trust God's timing and not push things to the point of just making things more messy and stressful.  Accepting a job that is not right for our family right now could be detrimental.  I knew just talking to Dr. Stein's staff the other day, that no one knows Todd's medical history, his counts, numbers, conditions, tests, or the research in the field better than me right now.  I'm in the best position to take care of him and its really where I want to be: by his side.   

P.S.  Talked him into it!