Aloha to all. I live in Honolulu, HI and was diagnosed with IgG Kappa 17p deletion Multiple Myeloma in January of 2013. Like probably most of you out there, prior to my diagnosis I had never heard of Myeloma. Like some of you, I only very rarely went to a doctor. As of September of 2012 however, I had developed severe hip pain.
Pain is a powerful motivator, even for someone as obstinate as myself. Unfortunately, neither I nor my Dr. recognized my CBC blood panel for what it was. In retrospect, even without any other blood tests, the low white blood cell count, red blood cell count, hemoglobin, hematocrit, platelets and neutrophil counts should have been a clear warning, but I ignored them. I wasn't really being obstinate at that point, my pain levels were too severe. I just didn't realize what these numbers should have been suggesting to me, and my doctor at the time didn't either.
Fast forward through my massive nose bleeds and constant infections and on December 23rd my wife, Sandy, found me on the bathroom floor curled up around the toilet. I was in extreme pain, horribly nauseous, and shaking uncontrollably from chills as I simultaneously sweat buckets. Somehow she managed to get me into the car and off to the Kaiser emergency room.
I only have snippets of memory about the rest of that night. Sandy had very literally saved my life, but, it seemed, only for the moment. None of the doctors that saw me expected me to survive the night. But I did. I was admitted Christmas Eve day to an Acute Care room where, once again, I was not expected to leave the hospital alive, and for the next week, their doubts seemed well founded.
I went into the ER weighing 165 pounds and left 12 days later weighing 128. I'm 6' 1" tall and thin to begin with so you can imagine how I looked. I spent the next three weeks in bed in excruciating pain being waited on hand and foot by Sandy. She cooked for me. She emptied my urine bottle and suffered through my pain with me. For weeks, neither of us could work and I have never gone back to work since that first night in the ER, but our economic woes are a story for another time.
Despite my dire condition and the fact that I really was not expected to survive, I did. My oncologist put me on Revlimid, Velcade and dexamethasone (RVD) and I responded seemingly miraculously. In May of 2013 I went to City of Hope, a Kaiser co-facility in Duarte, CA for an Autologous Stem Cell Transplant (ASCT). As many of you know, ASCTs are very unpleasant, but I responded well achieving a VGPR (Very Good Partial Response).
Following a few months of recovery, I began Velcade injections as my maintenance therapy. After about 6 months all treatment was discontinued and I did well for about another 10 months or so. It was then that my series of relapses began. I tried a succession of drug protocols including Kyprolis, Ninlaro, Darzalex, Empliciti , etc. I had some successes but, despite my high hopes for several of these, all failed within short periods of time.
In March 2017 a full body MRI revealed new lesions in my hips, legs, ribs, sternum, etc. I had an unscheduled appointment with my doctor the morning of March 30th that lasted more than 1 hour. It seemed that my already high-risk myeloma had become even more aggressive. We opted to change to a considerably more aggressive protocol which included another round of radiation--something I had previously claimed I would never do again. I don't actually remember exactly what I was on, but I know it included Kyprolis twice a week. This allowed us to stave off the Myeloma progression, or at least slow it down, but only for a time.
Come sometime in late June I was called in for another emergency office visit with my oncologist. We had reached the end of my protocol options, all that remained were the Bendamustine or Cytoxin protocols. Neither of these were real options in my mind due to my previous experiences with Cytoxin. I was told it was time to get my affairs in order.
My CAR-T Journey Begins
I was admittedly in some degree of shock at my doctor's prognosis. Following several days of talking to and commiserating with my wife and later my family and friends, I awoke one morning with the firm conviction that I was going to find a way to get into one of the CAR-T trials. Mind you this was not a decision I had reached lightly. We could not afford the airfare, much less all the other expenses associated with getting to one of the centers that were offering these trials. Then too, there was the fact that all the Myeloma blogs were clear that there were no openings for CAR-T trials at any of the centers.
Over the course of the last 5+ years, I have experienced over and over again the truth of the adage that if you set your mind to doing something, no matter the obstacles, you will succeed. Well, I'm still far from convinced of the absolute truth of this statement - what if your intention is at odds with your heart? That being said, once again a series of miracles seemed to unfold.
On the morning of July 12th, I awoke with the conviction that I was going to call UPenn to explore getting into their CAR-T trial. Before I called, I private messaged Dana Holmes--Dana is the gatekeeper for several FaceBook Myeloma blog groups and a fount of knowledge and information. She almost immediately messaged me back and asked why UPenn; why not the NIH? Well, I didn't have an answer. I hadn't chosen UPenn for any particular reason I could recall.
I considered Dana's question briefly and then called the head research nurse for the NIH CAR-T trials. Much to my amazement, she actually answered the phone. From everything I had read, this was an extreme rarity. At best one might expect to be able to leave a message to which a response may or may not ever be received. NIH was in the initial phases of starting a new CAR-T trial that I should qualify for.
Two weeks of constant phone calls, emails, texts and frustration, finally ended with all my Kaiser medical records being submitted to the NIH. Additionally, my oncologist at Kaiser, Dr. Ghelani, had called to recommend me as an ideal candidate for their trial. This despite the fact that my current health didn't really reflect that as true. Fortunately, Dr Ghelani had seen me rally time and again from the doors of death.
Dr. Ghelani also emphasized the fact that I would work harder than any patient they'd ever had. This was probably true, at least as far as it went. What he had left out was the fact that I would also question every decision they made and just generally be a pain in their collective asses. Dr. Ghelani rather gleefully admitted to this "minor" omission....
Between my initial contact on July 12th and my flight to Bethesda on July 28th, I had solicited help from every source I knew. In addition to everything else, I needed a caretaker to go with me. Sandy could not go for two reasons: first, we desperately needed her income, and second: we had just gotten a totally unexpected notice that we had to move. My brother, Craig, who had recently retired stepped up in a big way and said that not only would he act as my caretaker but he would also pay for the airfare. Between Craig, Sandy, Don and Darlene (my parents), and a brigade of friends, I had compiled enough money to make the trip.
As if all of that was not enough, I then discovered that I had relatives who lived in the area! Susan and Ray, Darlene's daughter and son-in-law insisted that we stay with them. So I met Craig and his wife Cyndi in Seattle and early the next morning set out for the Baltimore airport followed by the long car ride to the Bethesda area.
Over the course of the next week, I submitted to a barrage of medical tests, scans, biopsies etc. The good news, I qualified for the trial. The bad news, they had no openings. The head of the CAR-T trials at NIH, Dr. Kochenderfer, kept suggesting that I consider the Bluebird CAR-T trial. The Bluebird trial was their initial trial and I had not considered it because I knew there were no openings. Oddly, at least in my mind, Dr. K was insistent.
To be clear, I had no resistance to the Bluebird trial, in fact, I would much have preferred it. It was further along and all the dosing tests were completed. I had just so firmly implanted that I couldn't get in that I was only confused by Dr. K's suggestion. Still, I made it clear that I would gladly participate in the Bluebird trial. Another round of tests and I was cleared as a potential candidate for the Bluebird trial.
The problem, again, no openings and a list of equally, or even more highly qualified candidates ahead of me. Dr. K and the team suggested that Craig and I return home as there was no point in waiting around there. Craig, Sandy, and I talked it over and all of us decided that Craig and I would not return home. We would stay with the relatives (who again graciously kept their home open to us). We so informed Dr. K and the trial team.
On the morning of August 11th we got a call from the Judith, the NIH head research nurse stating that while there were 3 qualified candidates ahead of us, since we were still in the area, we might just as well come in for the final tests for trial compliance. On our way to the NIH Center we got a call stating that 2 of the 3 candidates had "washed out" of the trial. We were warned not to get too excited because the one remaining candidate was perfect in every way and would undoubtedly get the slot. Equal parts excitement and disappointment ensued.
By the time we reached the clinical appointment center, both Craig and I sensed that something had changed. Craig came into the waiting room and said he was pretty sure he had overheard them saying that I was in. I was so stunned by Craig's words that I had difficulty processing them. Could this be true? Could all the support, effort, time, money, and frustration really have paid off in this way?
Dr. K and the team walked into the conference room, sat down, and Dr. K said I won't waste any time, the person ahead of you just "washed out" and you're in. I was stunned and excited in equal measure. I didn't quite know how to react. Admittedly, part of me felt guilty about the three myeloma patients who no longer qualified, and we were never told why they didn't qualify, but guilt aside, I was in the trial!!!
The CAR-T Process
On August 17th I spent the day at the Apheresis Center to have my T-Cells extracted. For those of you who have had an transplant, this process is similar except that it is accomplished in less than a day, and, so long as your veins are healthy, which mine were, it's done with a conventional I.V. line--technically referred to as "leukapheresis". For those of you unfamiliar with T-Cells, they are our body's most potent cancer fighters. They are big. They are powerful. They are deadly. They are also rather dumb and can easily be fooled by the much more cagey cancer cells, especially Myeloma cancer cells.
Once extracted, the T-Cells are sent off to a lab for "programming". I'm not completely clear on the entire process, though I do know that the HIV virus is used as a Vector to supercharge the T-Cells, a 30 day process. Once completed, these newly programmed cells can easily recognize our myeloma cancer cells coated with the BCMA (B-Cell Maturation Antigen) marker, a marker that the vast majority of my myeloma cells displayed. That's it. I was now free to return home for 30 days while my new T-Cells matured and propagated.
I was more than a little concerned that I would not survive the remaining 4 weeks before my return. The only treatment that was allowed was 4 consecutive days of 40mg/day of Dexy. Oddly, I was so weak and in so much pain at this point that the Dexy did not affect me nearly as severely as it had over the prior 5 years. More importantly, it did keep me alive. We returned to NIH sometime after the first week of Sept. and again I was poked, prodded, scanned, irradiated, and had a PICC line placed in my left arm.
I got my Chemo treatments (technically referred to as: "lymphodepletion conditioning chemotherapy regimen" on Wed-Frid, the 13th -15th, with cyclophosphamide and fludarabine. Unlike the massive doses of cyclophosphamide (and others?) we receive prior to a transplant, the intent here is not to "kill" the immune system, but rather to weaken it. The next day, Saturday, I felt like death was a real possibility. Interestingly, primarily because they had told me that adverse reactions to those low-dose chemos were very, very rare, I was thinking this was something other than a chemo reaction, but I really didn't know what.
I was admitted to the NIH SCT wing on Sunday afternoon. I won't tell you I felt well, but not nearly as bad as the day before. Come Monday, Sept 18th, I received my new and improved T-Cells. Exciting, but nothing noticeable occurred. Then Tuesday arrived. Oh holly crap! I can count on my fingers the number of times I've felt worse in my lifetime, but I'd have fingers left over! Uncontrollable chills and shakes. Horrific nausea, and the few times I could get to sleep, I would awaken in pools of sweat. The team had never seen this reaction before, certainly not the day after the T-Cell infusion. The head research doctor and I conjectured that this was actually a bad side effect to the chemo--despite the fact that they'd never seen anything like it before.
After much discussion, they decided to give me Phenergran (promethazine), apparently a much stronger anti-nausea med than either Ondansetron or lorazepam, plus an H2 antihistamine. I have minimal memories from that point other than that I slept. I continued to wake up in pools of sweat, but the nausea was much improved, the pain levels were better and, so long as I kept well covered, the chills and shakes almost ended. Most importantly, the nausea was gone. Mind you, I still wasn't interested in eating anything, but I did feel better.
During the next 3-4 days, I managed to briefly respond to some texts but not much else. Thankfully, Craig was posting reports on FaceBook and keeping Sandy informed. Between the two of them, I think most of our friends and relatives were also kept informed.
It was Sept 22nd before I was able/allowed to sit in a chair. This may not seem like much, but for me it was a godsend to be allowed out of that hospital bed and to sit upright. It also prevented the constant infusion pump alarm sounding. This was a result of the fact that every time I would bend my elbow, the PICC line would crimp.
I feel like this is an appropriate point in my story to remind everyone that you MUST check, and then double check everything the doctors, nurses, and other staff members tell you. Ask questions ceaselessly. If you are not totally clear, use what has become my tagline, I'm really sorry but I've had multiple myeloma for years, I've been on chemo, pain drugs, and who knows what other mind distorting pharmaceuticals. Could you please go through this again? I found that my confusion often served in revealing a mistake, or a mistake in the making!
Unlike the norm, I never did experience the Cytokine Release Syndrome (CRS), sometimes referred to as a "storm", that is so common for CAR-T recipients. The "storm" is a by-product of the new T-Cells killing off more cells than the body can readily eliminate. It's basically fever, nausea, chills etc. This information was a little disconcerting to me because pretty much everything I had read, or been told, indicated that the "storm" also indicated that the new T-Cells were doing their job.
Did this mean that my new T-Cells were not effective? I was told that the odds were almost overwhelming that the lack of a "storm" did not indicate a lack of effectiveness. Apparently, once the optimum dosage for the Bluebird trial had been determined, minimal, or almost non-existent symptoms had become increasingly common. Then too, there was another indication that my new T-Cells were doing their job, my CRP (C-reactive protein) levels.
Some of you may be familiar with c-reactive protein (CRP) as it is a protein marker that indicates inflammation. So if you have arthritis or some other inflammatory disease you have probably had your CRP checked. If so, you were informed that high CRP was a negative indicator. But if you've just received your newly invigorated CAR-T cells, an increase in CRP is a positive indicator. It demonstrates that the new T-cells are doing their job! I was put on an extremely low-dose form of a steroid to compensate until my CRP levels increased.
I was released from the hospital the first week of October. I don't know what it is about hospitals that make me feel like a caged-rabid dog willing to chew off his foot to escape. Let's just say that I was really ecstatic to be out of there. Before my doctor gave me the final clearance and pages of instructions, she told me that the lesions riddling my rib cage had diminished by 50% or more. In what I interpreted as a backhanded apology, or at least an admission of agreement, sort of, she did state that my IgG levels have also decreased by over 1000 points.
IgG levels (or whatever immunoglobulin Myeloma type one has) had been poo-pooed by the NIH team as not being a reliable marker for Myeloma activity. I had initially argued the point that, in my case, it had proven time and again to be an effective and prescient indicator anytime my Myeloma had reactivated.
Since my IgG had done nothing but increase for the previous 3-4 months, and it had now diminished fairly substantially within a few weeks, they were kind of forced to admit that perhaps (just in my case:), it is a better marker than they had been willing to admit.
Almost by way of punishment, they gave me another Bone Marrow Biopsy (BMB), and then forced me to go lie on an MRI table for over 1/2 hour, followed by another 1/2 hour on the x-ray table. I had George for the BMB (this name will mean nothing to many of you, and everything to many others). Let's just say that in the BMB world, he is renowned. He even did a " House call", he came to my hospital room!
Following that last BMB, I was released from the hospital and I returned to the nearby hotel where Craig had been staying. No more hospital food. No more constant interruptions to test my blood pressure, or some other stat.
I was free to do whatever I wanted, whenever I wanted to. I also didn't have to page someone to follow me into the bathroom!!! Due to the BMB, I was more than a little careful about where and how I sat or lied.
My energy levels were low. They say that will last for several months. But I was free for the next 6 days. I wouldn't have to chew my leg off after all.
So it was just after mid-October when I returned to Honolulu. A flight that long under the best of circumstances would be taxing, but given my current situation, it was very depleting. I'm sure the six weeks in a time zone with a 6 hour time difference also didn't help. Just before I left the NIH I was informed that in addition to the fact that my M-Spike had improved and my IgG levels, which had been in excess of 3500, were improved by almost 50%. Additionally, since one of the main sources of pain had been the lesions in my ribs, combined with the large plasmacytoma inside my left ribcage, the fact that they too had diminished considerably afforded me a great deal of relief.
While I'd been gone, Sandy had been forced to deal with moving from our residence of the last 10 years. I felt so guilty for subjecting her to that responsibility without my help. She did hire movers for part of the process, and we had help from friends and relatives, but it was still a monumental undertaking. So I was returning to a new home with a new body, at least a body no longer on the brink of death.
So I continued to feel just a bit better as every day went by--being home with Sandy was wonderful beyond measure! Eventually, I hoped to be able to sleep past 4:00 a.m. I'm told that I looked dramatically better than I did when I had left for Bethesda, especially the first time back in late July. Admittedly, I had been told to get my affairs in order at that time, so I would certainly hope that I looked better. For the next 3 weeks, it was back to the usual frustrations of life.
While I had been gone, Kaiser had replaced a large number of the personnel that answer the phones. Between the fact that they were new and had either been mis-trained or, at best, poorly trained, I was not able to get anyone in oncology on the line to make my needed appointments. As you've no doubt deduced by now, I'm pretty persistent. So, like many other hurdles I'd encountered over this 5+ year ordeal, I managed to overcome this hurdle, with some help from relationships I'd formed with the higher-ups I'd developed at Kaiser over the years.
Although my recovery was so much slower than I'd anticipated, this outlook had only garnered momentum following my initial dramatic improvements. But, as they kept telling me, my healing would not be a straight line, there would be, and certainly have been, numerous setbacks. Clearly, the monthly return trips to NIH each month surrounded, as I was on the planes, by sick people and at the hospital, subjected to 5 or 6 different time zones, all combined with hectic rounds of tests, didn't speed up my process. Still, my numbers continued to improve.
Currently, my IgG is actually below the bottom of the normal range. I won't get my next M-spike results for another week or two, but, as I've mentioned, my IgG levels have always proven brilliant prognosticators of what my M-spike will be. My last M-spike was .3 g/dL. I have no reason to believe that it won't again improve on this next go round.
So, as of today, January 18, 2018, I'm feeling decent. I no longer have any fears that death lurks beyond the next tick of the clock. In fact, while I am clear that CAR-T is probably not a cure, I see no reason not to believe that I haven't added at least another 2 years, very possibly more, to my lifespan. Two more years to spend with my wife is reward enough for all that I've endured. Add to that all my friends and relatives, and the price I've paid has been small indeed.
Here's wishing the best to all my fellow MM'rs and their caregivers and supporters,
Love and Aloha
about the author
Myeloma survivor, patient advocate, wife, mom of 6. Believer that patients can help accelerate a cure by weighing in and participating in clinical research. Founder of Myeloma Crowd by HealthTree and the HealthTree Foundation.