Ferrari's

    Today as I walked to church the sun was shining, a gentle breeze was blowing and the smell of blossoms in the air was sweet.   It was a great balm for my soul.   Spring is increasingly becoming my favorite season because of all of the reminders that life can be beautiful even after a difficult, cold harsh winter.  I also couldn't help but notice that while some items are in full bloom, and others have already begun to sprout leaves, others were barren still hibernating from winter.   It's was about six months ago that we found out about the multiple myeloma.   Spring hasn't quite yet come to our journey, but the warmth of the sun and the gentle breeze I'm hopefully it's very close on the horizon.

     We met with Dr Sborov again Friday to figure out what the next step is.  Chris was originally supposed to have a break from chemo until May but just prior to collection we found that his Lamda Light Chain numbers were high.   In October his Lambda light chains were 323 (Normal is .57-2.63).   Starting chemo they dropped fairly quickly and were down to 4.67 by December and dropped regularly each subsequent test.   They were normal for the first time in February at 2.03.   Just prior to collection they were 12.6, and when tested again this week they are at 18.4.  So all the plans to start treatment were moved up.   Dr Sborov would like to start again as soon as possible, he would have started this week if he could have.

      Trying to describe the status of myeloma is tricky.   There's approximately 724 trillion blood cells in the average human body, so there's a lot of places for myeloma cells to hide.  There's a lot of debate about using the term "remission"" for myeloma.   Most myeloma patients, even those who do transplant, are on maintenance chemo to keep the myeloma at bay.  Compared to most other cancer patients when in "remission" are not currently being treated.  One term is "Complete Response" which I interpret to mean that the drugs are doing their job.   I've learned that when they decided to do collection Chris was in SCR (stringent complete response) meaning that according to his blood tests and bone marrow biopsies things were in normal range.   The meyloma was behaving so they used the opportunity to collect.  Because his numbers are climbing he has "relapsed".   Since he hasn't been doing chemo it's to be expected but they had hoped it wouldn't come back quick so quick.   

     When Chris was originally diagnosed, because of his kidney failure their options for treatment were limited.   Now that his kidney's have improved he's eligible for the "Ferrari" of treatments.  While Dr Sborov meant it in terms of the quality, it's hard not to be staggered at the cost two of his medications are each both likely 6 digit figures in terms of cost per year. 

     Years ago I remember standing in line picking up a prescription in St. George.   The person in front of me was picking up one that was about $250 and trying to figure out how to pay for it.   I was shocked and wondered what the costly medication could possibly be.  I remember then picking up mine and being grateful that it was only about $10. 

     Over the years we've been shocked at the prices of medication.   $600, $900, $1000, $1100, $3000...Several times it was common to hear the timid pharmacists carefully ask "You know this is expensive right?".  We are very fortunate to have insurance that covers a large portion of the cost.  Especially since his chemo usually has a 5 digit price each treatment.   However one bit of knowledge that I can highly recommend to anyone is that if you get a prescription that's very expensive is to see if the company has an assistance program that can help bring your co-pay costs down to a reasonable amount.   Not sure what would be covered by insurance and what we would have to pay, I looked into this as soon as I got home to get signed up for anything available.

     Next week he'll have his first treatment of DRd

daratumumab (Darzalex®)

revlimid (lenalidomide)

dexamethasone (Decadron®)

     His previous treatment was all done once a week on the same day (an infusion, a shot and pills).  DRd will be different.   It is a weekly infusion (Daratummab), Revlimid (daily pills) and dex (weekly pills).

    Daratumamab is a monoclonal antibody.  It targets the CD38 protein that is highly expressed on myeloma cells and attacks and kills them or allows the body to kill them.  The way I picture it is like a military operation and we are sending in reinforcements.   The body doesn't know that's what they are, so for the first couple infusions most people experience a reaction and in varying severity.   When a reaction happens, they will stop the infusion, treat the reaction, and then when safe to do so start the infusion again at a slower rate.   Because of this the infusion can take a long time, usually about 10 hours.  After the first couple infusions, the body seems to realize that we're sending reinforcements and instead of causing a reaction they become allies and fight the myeloma.    He will do these infusions weekly for a while, eventually going to bi-weekly and then eventually monthly.

    Revlimid is an oral medication that will likely be taken for 21 days followed by a week off.   Revlimid is an IMiD  (Immunomodulatory drugs).   This Immunotherapy treats the disease by inducing, enhancing, or suppressing an immune response.  It can cause low blood counts, fatigue and blood clots.   They will monitor his blood counts carefully and because of a family history of blood clots he will also be on a blood thinner.  It's a powerful drug which required several pages of consent, and regular phone calls with the company to make sure he's taking it correctly.   

     Dexamethasone is a corticosteroid that is very common and included in most myeloma treatments.  It is used at almost all stages and sometimes even alone when nothing else works.     It can help with inflammation, help decrease nausea, and at high enough doses can even kill myeloma.   It can also help make other myeloma drugs work better.  

    Anyways, enough of the technical and informative.   The next couple weeks are likely to be tough, but we've been through many tough weeks and survived.   This new treatment will hopefully provide relief from some of the difficult side effects and improve the quality of life for both of us.   The first treatment CyBorD served it's purpose and was able to get the myeloma under control and save his kidney's.   That is no small achievement!   

       After the first couple weeks things will gradually get better and getting down to only one infusion a month instead of one a week will be amazing.   The past six months we've had little time or energy to do anything else.  It will take time, and these new medications will surely have it's own side effects to deal with but I'm hopeful there's a chance to to reclaim a part of ourselves and have time to make some happy memories.  Treatment, in one form or another, are likely a permanent part of our life but with a few windows of opportunity opening it hopefully won't be all encompassing.  

Milestones

     The past week and a half of collection were exhausting and we are both so glad to have that milestone marked off the list.   We haven't had time to celebrate yet and instead have just been trying to recover.   However this is a huge and potentially life preserving milestone!  With so many new treatments on the near horizon we hope that one of them can bring the cure for myeloma and that it will come during his lifetime.   However transplant is the best treatment to give him the longest life expectancy to wait for this cure, so collection was a very critical step.  It was very long days for me, however I'm so glad I could be there with him to support him because as exhausting it was, it was so much more difficult for Chris.

     The process was very interesting and even though we tried to learn what to expect ahead of time, there were side effects we didn't know to expect.   We were prepared for 1-2 shots per day, however in total he received 32 shots, not a fun surprise.  As well, Tylenol and Claritin only took the edge off the pain.   We also did not know that it would cause fluid shifts and retention.   We've been trying so hard to get the extra fluid off, but are back again at square one. 


     Dr Sborov's goal for collection was 10 million, and Chris exceeded the goal and was able to collect 11.5 million.   Yay for reaching this goal!   The minimum that they need for a transplant is 2, but closer to 5 is ideal.   So depending on what they choose, he has at least for 2 transplants.   Multiple transplants are pretty common, and sometimes they even do them one right after the other (called Tandem). 

      During collection, we had three different tech's overseeing the process and running the machine.   These techs are the ones who also work in the lab processing, counting and prepping the cells for their time in the freezer.   They are also the ones who are responsible for thawing them and helping with transplant so perhaps we will see them again.  They were great at answering questions and we learned a lot, or at least had a lot explained to us that we tried to absorb.  Much of the details go above our head, but they explained that they use the marker CD38 to count the stem cells.   I had researched about the new medication they want to start him on that had mentioned CD38.   Daratumamab (Darzalex), is a immunotheraphy drug.   It helps the body's immune system to find and destroy the cancer cells by binding to CD38. 

     If you haven't gathered it already, myeloma is complicated! 

     Because of collection, the meeting that we've been anxiously awaiting with the cardiologist, Dr Ryan, that was scheduled for the week of collection was moved to the week before.  His father has heart disease and has been in and out of the hospital for the past six months so hearing that there may be problems with Chris's heart has had a very worried for the past couple months.   The appointment was a bit anti-climatic but we came away with some assurances and clarification.

• Pulmonary Hypertension is high blood pressure in the arteries to his lungs.  All of the respiratory issues we have been dealing with (the tracheal stenosis and trach) have been difficult for his heart.  However, the condition will likely not have any impact on his life expectancy.   It is both good and safe for him to exercise. 
•  Dr Ryan does not feel that it is a barrier to receiving a stem cell transplant. 
•  The swelling in his legs that we have been battling is a symptom/side effect. 

     One aspect of our marriage that I've always loved is how much we enjoy talking to each other.   He's someone who I can talk to about anything and we never run out of things to talk about. 
     Before his most recent challenges, he would often take me to and pick me up from work.   I work only a few miles away, but it was nice to have this time in the morning and at night to look forward to.  He hasn't been yet been able to do resume doing this, but with all of his appointments we have a lot of time to talk while driving and waiting.   I'm so glad he proved the doctors wrong when they said he likely wouldn't speak again!   Among other things, we've talked quite a bit about a bucket list.   Even before the cancer diagnosis we would toss out ideas, but with a bucket that developed holes and is leaking out precious moments we are trying to figure out how to find the balance to accomplish some of the things that we've always wanted to. 

     We'd love to hear some inspiration, 

what's on your bucket list?

Collection

     Today started very early, driving to Huntsman while the moon was still up and the sky was still dark.   Often while making the drive my thoughts wander and I try to focus on being grateful instead on on some of the other things.   Today in the dark with Chris silently sleeping beside me I turned to where we were headed.  I'm amazed at how lucky we are to have a top notch cancer center, that just so happens to specialize in his rare cancer nearby.  Being treated by a myeloma specialist is important in his key to his survival.   While the drive is often filled with traffic and usually takes an hour, it's a blessing that most don't have.  Today we learned that the older man in the bay next to us had to come all the way from Oregon.   With all of our appointments so far this year, we've driven about 1,000 miles, I can't fathom the logistics of trying to coordinate everything even if we still lived in St. George.  Being able to sleep at home, and just visit the doctors in the hospital is a blessing.  Collection was delayed from the original plan of February but we grateful that he's being able to do the process as it's an important one in his treatment.

      Today marks the first potential day of actually collecting.  We've driven here several days already to receive Nupogen shots to tell his body to over produce stem cells so that we can collect them.  We weren't sure how quickly the shots would kick in, but shortly after getting them the bone pain kicked in.   Strangely Claritin can help, so he's been taking that and Tylenol to try and keep the edge away.   They only take the edge off, I'm so grateful that I'm able to be with him as sometimes the simple act of human touch is the greatest for pain relief.  The pain tends to come in waves sometimes in his hips, sometimes his shoulders or his legs or arms.   Often in multiple places at once.  He's described the pain a few ways.... like his bones trying to get out of his body, like someone is taking a chisel and hammer to his bones and also like a vice grip on his bones.   The pain ebbs and flows in each area and he's been a champion and handled it pretty well.   I'm touched with his thoughtfulness as each day he's thanked me for being with him and for being patient with him.   He's going through a lot, yet he still things daily of how it is for me.

      Yesterday in addition to the Nupogen shots, he also had a line placed for the collection.   This one is very similar to the line he had for dialysis but with an additional catheter.  It usually sticks up out of the patients neck but we were able to request that they tunneled it down through his skin instead. 
     
     Yesterday was an unusual day at Huntsman.   While in the waiting room waiting to be taken back to IR we were both surprised to see two young little toddlers.   Being treated at a cancer treatment is strange because unlike a regular hospital, you know that everyone there is either being treated for or is there to support someone with cancer.   We usually feel so out of place with so many around us the age of our parent's or grandparents.   Having a toddler walk by and say "bye-bye" and wave sweetly brought a smile to both our faces. 

      As well, after they wheeled Chris back and I was sitting waiting for him to come back they wheeled another patient into the curtain next door.   His voice sounded very young and I could tell he was alone, it made my heart ache and I nearly peaked around the curtain to introduce myself.   About that time a visitor stopped by and I learned that his indeed was very young, likely about 19.   He had just until recently been serving an LDS mission in California.   He, like us, was grateful to be in good hands with his diagnosis. 

      Most people don't collect on their first available collection day, but the preliminary numbers testing for the number of cells in his blood stream looked good so instead of having to wait 3 1/2 hours for the blood tests to come back, they were able to get him started a little bit early.   Chris has been very optimistic that this will go smoothly so being able to collect some today was a win in our book

     They usually process 15 liters of blood in a session of apheresis and the speed that they do so can vary the time that it takes.  Today's session was about 3 hours.  They collected about 175mL of stem cells and 200 mL of plasma. 

      As well, there is approx 250 mL of blood in the tubing, but at the end of the process they put most of it back in, usually returning about 230mL.

     To prevent his blood from clotting in the machine and tubing, they add an anti-coagulant as part of the process.   This anti-coagulant binds to calcium which can cause his calcium levels to drop which can cause tingling in your lips and extremities.  Because his calcium was low before even starting this morning they started a calcium drip shortly after they got him all hooked up.   Because it can mess with your other electrolytes (potassium, sodium, etc) they run additional labs to make sure he's OK to go home. More waiting for those to come back, but they all turned out good and he was given the OK to leave.   About 9 hours after we left this morning we arrived back home and both took a nap. 


      The tech today said it's really hard to estimate but that his best guess would be about 1.2 million.  We're now getting ready to head back up to the hospital for a new nightly shot.    This shot, called mozobil, tells the stem cells to "get out" of the bone marrow and into the blood where they can collect it.    This also is pretty common and something we expected.  He will continue to get the Nupogen shots in the morning as well. 

    While hanging out in a hospital hooked to a machine isn't fun, and brings back memories of doing dialysis, we know this is a good thing and a step in the right direction.  We also are touched by the support and thoughtfulness of others.   Our friend Rachel stopped by for a visit, wearing her Team Chris shirt.   We're getting to know all of the nurses, techs and support staff but it's always nice to see a friendly face and reminisce about her recent trip to Hawaii.   Also, we returned home to a delivery of groceries on our porch from Smith's.   Our good friends from St. George had promised Chris a Dr Pepper and had Smith's deliver a case along with a whole bunch of other goodies and essentials.   I had started a small list of what I knew we needed and planned to try and go to the grocery store sometime this week but they saved us. While I'm sure there are several who would have picked up groceries for us if we had just asked, we weren't quite sure what this week would be like so having it just show up when it was needed to me was a tender mercy and someone following a prompting.   So lucky to have such good friends! 

      Off we head to Huntsman again, but at least we get to return home again tonight to sleep in our own bed!