The Story of The Mutant and The Myeloma Beast!

7

BY DON BEHLER

Long before any memory could imprint itself into my consciousness, my infant life began as a basic fight for survival. Many years would pass before my transformation into the world of mutants in an attempt to grant myself the strength to compete round after round in a terminal fight to the death with my arch enemy, the Myeloma Beast. 

Maybe we should start all the way back at the beginning. That first fight for life was basically just a sparring match when compared to the battle to come, yet it turned out to be an experience already imparting a will to survive that would serve me well in that future fight!  As an infant, severe digestive issues and stomach spasms led most people and several doctors to conclude that my life span, or my first fight, would be a very short one. Apparently, they had underestimated my perpetual drive to live and internal resolve! 

 I guess that it is beyond unfortunate that some need to learn to fight for their lives at such an early age. My conclusion, however, is that there may be nothing like an encounter with the angel of death as an infant to begin to strengthen the basic will to survive.  Long before I ever crossed paths with The Myeloma Beast, unwittingly, I was already beginning to arm myself in preparation for the real battle set to happen years in the future.

As a teen, having been someone with a feeling of invincibility running through my veins, a few minor brushes with death just did not seem to slow me down. Like an adrenaline junky on a quest toward whatever the next adventure might happen to be, life just continued on!

Becoming a very good athlete at the mere weight of 140 pounds, I was somehow able to set several high school football rushing and yardage records and also earn MVP honors at my area’s high school all-star classic football game. Fear of challenging any competition larger and stronger than myself never entered my mind. 

Having amassed a broken nose, broken rib, two cracked vertebrae, and 11 concussions, the realization that I was probably too small to continue this particular adrenaline-driven activity into my college years became fairly evident. However, the time spent healing and rehabbing from all of those injuries, while continuing to compete through the level of pain, would again be a great training tool for the upcoming “Battle with the Beast”!

Looking back on it now, my first glimpse of my mortal enemy, the Myeloma Beast, occurred in my mid-twenties. The time spent constantly working 55-65 hours per week as vice president and salesman for my small family-owned mold and tooling shop, while also performing several nights a week in a local rock band, led me to believe that the back and rib pains that mysteriously began to appear were merely fatigue and that those old football injuries were finally catching up with me. The chronic sinus infection that soon followed became a vocalist’s nightmare and a full-time music career-ending malady. The Beast had quietly invaded my life without warning, and although I was completely unaware, our battle had just begun!

As several years passed, the Beast kept boring deeper and deeper into my body and began to weaken my immune system. Not knowing, I just simply went about my daily life and ignored the signs along the way. Continuing the same work schedule, and now coaching both Little League Baseball and Junior High Football, the back and rib pain continuously progressed, and the sinus infections became relentless. Every cold and flu seemed to find their way directly to me. Still, my life went on missing the warnings! 

Then, in my later forties, while lifting weights, the 20 years of minor back and rib pain morphed into several broken vertebrae. Each time the vertebrae would seem to heal, practically any physical activity would seem to break them again. The level of fatigue, which had never been an issue for me in the past, continued to increase day by day.

Having ignored all of these symptoms for years, the time had finally come to seek some medical advice. 

After several misdiagnoses and six months of treatment for osteoporosis, a biopsy of a tumor found near my spine via MRI, along with some off-the-charts blood work, and finally, a bone marrow biopsy confirmed that the Myeloma Beast had been discovered deep in my bones. Hearing the diagnosis of Multiple Myeloma (“MM”), a previously unheard of incurable cancer, and also learning that I might only have three years to live hit me like a ton of bricks!  At least, finally becoming aware of my lethal opponent and learning about his brutal reputation triggered the realization that the time to fight back was at hand!

In 2007, the standard medical procedure to begin the battle with this dread disease was several months of high-dose pulse Dexamethasone (a steroid), followed by a stem cell transplant. So, now that the fight was on, that particular procedure was to be the strategy for Round 1, and it went well! 

The Beast was hit hard and went down, but not out! Despite the fact that I was overmatched and new to this fight, luckily, my MM specialist Dr. Dan Vogl from U. Penn Hospital had been contracted to be my ringside doctor, cut man, and trainer for the duration of this fight. Little did I know, the undefeated and seemingly invincible reigning champ, “The MM Beast,” also had allies in his corner. I was about to encounter one of the worst! 

Three months after that first knockdown in Round 1, a severe case of shingles broke out and covered the majority of my upper left arm. The Beast had unleashed a dirty, behind-the-back sneak attack! The bell for Round 2 had sounded; and the Beast and his new ally were up off the canvas and taking the fight to me.

The damage to my skin and nerves from that shingles outbreak took nearly seven years to completely heal. Several doctors commented that they had never seen anything like it. The name that they termed for the shingles attack was “Mega Shingles”!  At first, a few of the doctors thought that I might even lose my entire arm! The pain that feels much like I had been hit by a baseball bat on that arm sporadically continues to this day. There is no doubt that the MM Beast fights dirty, and he definitely plays for keeps!

Round 2 decisively went to my opponent!! The scars and residual pain from the shingles attack are still there and serve as a daily reminder of just how brutal this fight would be!!

Just after completion of my first stem cell transplant in 2007, Dr. Vogl suggested adding a regimen of Revlimid (a daily chemo pill) as a maintenance drug, or weapon, to keep the Beast under control for a longer period of time. Unfortunately, my insurance company would not approve the usage of Revlimid without first taking Thalamid (a daily chemo pill).

While still reeling from the shingles attack, the addition of Thalamid as a weapon signaled the beginning of Round 3. Using Thalamid as a maintenance drug had extended the time already won by the successful stem cell transplant and turned the momentum of the fight back into my favor. This medical weapon kept the Beast on the ropes for nearly 18 months. Round 3 was mine!

As the Thalamid began to cause some nasty side effects, and also seemed to no longer be keeping the Beast completely out of the fight, my insurance company finally agreed to allow the switch to Revlimid. At this time, my M-spike and light-chains (measurements of the MM disease in the blood) had begun to rise slightly, just enough to make it evident that the Beast was back in the middle of the ring and ready to start Round 4. He was in for a surprise!

The new Revlimid and Dexamethasone (steroid) combination suggested by Dr. Vogl completely neutralized my opponent for another 30 months. Actually, the Revlimid medication took my M-spike and light-chain measurements to an even lower level than the stem cell transplant. This was the closest to remission that I would ever experience throughout the fight. Round 4 had definitely gone in my favor, and the overall fight in general was going almost entirely my way!

As the Revlimid strategy began to fail, my ring doctor suggested trying Velcade (an infusion) as a new weapon, and that move kept the Beast at arms-length for another 18 months. Another round in the books and one more easily going my way!

As the Velcade began to fail, the next weapon of choice was Pomalyst (a daily chemo pill). This would be the last of the highly successful single treatments with the ability to keep the Beast down, if not out of the fight!  Another good 12 months all went in my favor, and another round goes my way! 

One major problem for each of us battling this disease is that Myeloma constantly seems to morph and has an ability to almost alter its own genetics to grant itself the power to become practically immune to each medication that is used against it. My opponent, the Beast, had begun to ramp up this process. Apparently, he had lost too many rounds and was now preparing to overpower any of the new weapons that we might have in store to throw his way. 

Carfilzomib (an infusion) was my next choice of available approved medical weapons. That choice was followed by Darzalex (an infusion).  At that juncture, our strategy moved to a host of combinations of most of the previously successful medications, the theory being that combining two or more of any of these medications actually creates one entirely new medication and a weapon that, hopefully, Myeloma may not recognize. This new combination of medicines should then form a new unrecognized weapon, forcing the MM Beast to take some additional time learning how to morph to eventually render this new attack harmless. 

Some of those medications helped in the fight for a short period of time.  Others, unfortunately, were basically ineffective. The Beast was on the attack and more rapidly learning how to counter each of my assaults. 

The fight which had begun in 2007 had now extended into 2014.  After seven years, the scorecards in general showed most of the rounds going in my favor, but the Beast is the undefeated and (of course) highly-despised champion, and he now was finding his second wind! 

In 2015, the trajectory of the fight was about to change and not at all in my favor!

Those of us in any way connected to this disease all know that the Beast does not stay down for long. Halfway through 2015, nothing we tried seemed work. My M-spike and light-chains again began to rise. That is always a bad sign! 

Our strategy switched to a few different clinical trials without much success and/or with such violent side effects that those options simply became intolerable. Then, as most of the remaining approved therapies also began to fail, the Beast began to win several rounds back to back to back. I was suddenly losing not only the physical fight but, at the same time, also beginning to lose the psychological fight. I was in trouble! 

In April 2016, I entered a true danger zone in the ring. The Beast knocked me down for the first time. My M-spike went through the roof to a 5.1 reading, and my light chains climbed to a very dangerous 7560. In a matter of weeks, my kidneys began to fail. The MM Beast had gone completely aggressive, and Dr. Vogl immediately sent me to U. Penn Hospital for an emergency stem cell transplant. 

In my favor, there were still enough previously collected frozen stem cells from my original 2007 transplant procedure to accomplish this life-saving operation and in a hurry. So, maybe I was down, but I was not out!

This second stem cell transplant saved my life and extended the fight! My M-spike quickly fell back to 1.8 and the light-chains dropped to 1890. My kidneys began to heal, although some permanent damage still remains. So, another round for me, but as fate would have it, this one was short-lived! 

The Beast was not finished with me yet. Despite the successful transplant, a few weeks later, another ally of the Beast (a myeloma tumor) developed in my sinus cavity.  Within a 24-hour period, this tumor damaged the C6 nerve, which controls the muscles that guide the movement of my right eye. Like I have said, “The Beast plays dirty, and he plays for keeps.”

Due to the nerve damage, my right eye had completely buried itself into the inner corner of my eye socket. This caused a double-vision situation, if not to mention a rather creepy overall appearance! 

It took close to seven months for the vision problem to begin to alleviate itself! That round certainly went to the Beast!  Much like Rocky in the movie, there were many times I just wanted to cry out, “Cut me, Mick!” so that I could see again!

The radiation treatments that Dr. Vogl suggested at U. Penn Hospital did eventually solve my vision problem.

Now that most of the approved medications seemingly no longer had much effect on my incurable, invincible, so very deeply despised opponent, my trainer suggested a brand new clinical trial that was being conducted at U. Penn Hospital as our next weapon of choice. The theory and strategy behind this trial takes a totally new scientific angle in battling the Myeloma Beast. This treatment would now battle the Beast right down at the cellular level, using my own immune system with a little twist of help from science! No chemo involved!

 Similar trials, which involve collecting a patient’s white cells and then adding a new gene to those white cells in a lab before re-infusing the now mutated white cells back into the patient, had been done in the past to attempt to battle other blood cancers, such as leukemia and lymphoma. These cells were termed CAR T Cells CD 19. The CAR T cells of this type had worked very well in many cases to successfully battle those other blood-related cancers. In early trials versus Myeloma, unfortunately, the CAR T Cells targeting CD19 in general did not have the same type success against the MM Beast (because CD 19 is not typically found on myeloma cells). 

The science involved goes a bit beyond me, but as I understand it, the gene that was added to the white cells to create CAR T 19 was not able to recognize a particular protein marker that is located on the exterior of the Myeloma cells and identifies those cells as cancerous. The added gene is programmed to recognize those particular protein markers on a cancer cell and then signals the immune system to begin destruction of that cancer cell.

The MM specialists had decided to try CAR T Cell CD19 versus the Myeloma Beast, in an effort to prove its effectiveness and/or lack thereof, as a starting point for this new scientific approach to the battle in general.  My understanding is that the very first MM patient to try the CAR T Cell CD19 trial went into full remission and has remained there for several years. Unfortunately, for the rest of us, similar results were never able to be duplicated. 

So, for my next round, a similar but adjusted clinical trial was just getting under way. This trial consisted of collecting my white cells and adding a different gene at a different location within those white cells. The new gene was programmed to recognize a different protein that the MM specialists felt was located on the exterior of all MM Cells.  This trial was termed CAR T Cell BCMA. The danger behind this particular experiment was that only several people in the world had begun testing with these cells, and as with any new scientific procedure, the knowledge of exactly what in the body these ramped-up, killer, mutated white cells would attack was still somewhat a mystery. I was originally in line to be patient #8 in the BCMA trial, that is, until the Beast diverted the plan by throwing the sinus tumor into the middle of our new strategy! 

In August 2016, when the C6 nerve damage occurred, I was immediately admitted to U. Penn Hospital, and my opportunity to become CAR T Cell BCMA mutant #8 had been derailed by the MM tumor.  The Beast has incredible timing! My BCMA infusion had been scheduled for the following week. I could swear that I could hear him laughing across the ring in his corner!

Five days of radiation followed in an attempt to destroy the MM tumor that the Beast had thrown my way. The radiation treatments were followed by a four-day, round the clock, three different chemotherapies plus Dexamethasone (“Dex”) battery.  The protocol for the BCMA trial does not allow any chemo or radiation within one month of the BCMA infusion, so Dr. Vogl added high doses of Dex for the next month to keep the Beast under control until the time limit passed, when I could again be back on schedule for the BCMA infusion.

Have I mentioned that I absolutely hate the effects of DEX?! This double vision round went completely to The Beast.

Now, the four-day chemo treatment plan along with the steroids worked just as expected, and while the Beast was temporarily neutralized, my position in the BCMA trial was moved to mutant #16.  It seems that by creating the tumor, the Beast placed some doubt in the minds of my team of BCMA specialists in regard to my candidacy for the trial itself.  They feared that if the Myeloma had found its way into my central nervous system, this situation would disqualify me from participating in the trial. The Beast was on his game, creating as many obstacles as he possibly could!

In some cases of this trial, the acute reaction that accompanies the assault of the mutated killer white cells attacking the Myeloma cells has been known to cause brain inflammation and even coma.  It is a shame that patient #15 passed away due to complications that arose from this type of coma. There are some dangerous possible side effects in this regimen.

So, while admitted to U. Penn Hospital for radiation and the four-day chemo treatment, I also submitted to a battery of tests, including a spinal tap, to determine my viability to continue as part of the BCMA trial. If the Beast had been suspected of having entered my nervous system, my chance to participate and use this trial as another valuable weapon in the fight would have been eliminated. It felt very much as if the referee had called time out, mid-swing?, to decide if the fight would stop due to a technical knockout. Luckily, though, all of the tests came back negative –  and the fight was back on! I was back in the BCMA trial!

My mutated, ramped-up, killer white BCMA cells were infused on September 27, 2016. Most Myeloma patients consider the date on which they are infused for a stem cell transplant as a new birthday. That is because a stem cell transplant is preceded by a heavy dose of melphalan (chemo) that destroys all of the patient’s bone marrow and existing immune system. The infused stem cells find their way into the bones and regenerate a new set of bone marrow and immune system: a new life! Since I already had two new stem cell birthdays under my belt, the BCMA infusion on September 27 has become not only my third new birthday, but more importantly, the day I entered the world of mutants! Hey, anything to continue the fight against the MM Beast! 

As I mentioned, most patients who receive the BCMA cells develop a high fever and experience a fairly aggressive attack on all of the myeloma cells throughout their body. For some reason, my BCMA cells reacted much differently.

It is possible that my case may be different because of the stem cell transplant I received just six months prior to the BCMA infusion, and also the four-day chemo treatment I had received just one month prior to the BCMA infusion. Then again, every myeloma patient is different, and each reacts differently to practically every treatment that exists. My system may just have been different than most of the others! All I can say is, for some reason, there was no high fever for me.  There was no violent attack.  My mutated killer BCMA white cells went to work slowly over a five-month period and continuously battled the Beast at the cellular level. 

By the end of November, my M-spike dropped from 1.3 to 0.8, and my light-chains dropped from 1730 to 1094. This round was going my way. Go, you mutated BCMA killer white cells, go! Take out the Beast!

By February 2017, my numbers again began to rise, but very slowly this time. It seems my BCMA killer white cells had gone dormant. The Beast got up again and was gaining strength.

I can say that, other than some fatigue and chills, the mutated BCMA cells had very few side effects for me personally. My four- to five-month Beast-beating ride with the BCMA cells as the weapon of choice was the second longest ride compared to the prior 15 patients! Some patients received a greater M-spike and light-chain reduction, but their BCMA cells had gone dormant more quickly.

Tests showed that several tumors throughout my body either had been completely eliminated or had shrunk by about 30%. All in all, fantastic results! The Beast was no longer laughing!

Despite the mutated killer white BCMA cells going dormant, they are scientifically engineered to continuously duplicate and remain in the patient’s system on a permanent basis. With the onset of this dormancy, Dr. Vogl devised a new plan of attack.

Before the Beast could again become aggressive, Dr. Vogl comprised an idea to use some approved antibody medications in an attempt to “wake up” and re-fire the killer BCMA cells. The theory behind the antibody medications (Keytruda and Empliciti, both infusions) was that they are designed to remove the natural braking system from all of the white cells throughout a patient’s body. The removal of this natural barrier in the cells should allow the white cells to begin to recognize and destroy cancer cells that had been ignored in the past.

Again, as with the mutated BCMA cells, the danger was that the non-braked. ramped-up, killer white cells could also decide to attack other parts of the patient’s body. Those possible side effects can be a bit concerning, but then again, doing nothing in this brutal fight to the end could be even more daunting!

The Keytruda and Empliciti treatments, along with Revlimid and Dex, began in late February 2017. My M-spike and light-chains have consistently dropped, week by week, over each of the past six weekly treatments. Keytruda is infused on a three-week cycle. The Empliciti is infused on a weekly cycle. Revlimid is taken on a two-week-on and one-week-off cycle. The Dex, of course, is weekly…have I mentioned that I hate Dex?! 

My M-spike has dropped throughout the six week period from a 1.6 to a 1.2, and the light-chains have plummeted, incredibly, from 2060 to 216. In general, the M-spike lags a little behind the light-chains in regard to showing movement of the level of the disease.  Dr. Vogl expects my M-spike to soon follow the light-chains and drop more drastically in the near future. This new round, at least for the moment, is easily going my way!

Having asked Dr Vogl how my progress compares to others on this same protocol, to my surprise the answer was:

“Don, there are only a handful of people, maybe 16 to 18 in the world, that have both Multiple Myeloma and also have received the BCMA mutated cells. Of that group, there are only two or three people receiving Keytruda and Empliciti as a follow up treatment. Since you are also taking Revlimid and Dex, it is very possible that you are the only person on the planet, for now, following this exact protocol!”

So, not only have I entered the world of mutants in my quest to finally be the first to knock out the Beast, I now seem to have also possibly stepped into territory “where no man has gone before!” 

Did I mention that I am an adrenaline junkie? 

Bring it on, Beast, I am not ready to give up on this fight just yet!

We have more rounds to go! Stay tuned on the Myeloma Crowd and my facebook page for continuing updates in regard to the ongoing Battle with the Beast and results of upcoming treatments! 

PS: So much thanks to my beautiful wife, Darlene, who has been my rock throughout this long, hard battle. I don’t believe I could continue the fight without her amazing strength and support!!  All my love!

Also thanks to my very supportive children and close friends, Sal and Michelle M., for everything you have done to make this journey a little easier!

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About Author

Don Behler

I have always been an overachiever. One who is constantly questioning and seeking answers from outside of the box. I have recently found a absolute love to create. Desiring to leave behind a little bit of a legacy, while I still have time...

7 Comments

  1. Wow, your story is amazing! Keep up the fight, you’re winning and there is no doubt your strong enough to conquer this awful disease! Thank you for sharing and God Bless.

  2. Alexis Walmsley on

    What incredible strength and courage. Awesome (as you Americans say 😀) And heartening. My husband has been battling the Beast for almost 10 years and it lay quiescent (felled by Revlimid) for 5 years! Such an easy time too – popping a few pills and seeing the doc every 5 weeks. But John’s Beast returned last year. We got Darzalex (first patient in our area in U.K. to have it thanks to private insurance ) plus velcade in August 2016. Treatment has changed to Darzalex with pomalidomide so we hold our breath to see if it’s worked on the rising light chains and paraprotein.

    Very best of luck in your fight – my husband is fighting on the UK front too.

  3. Ron Bartizek on

    I’m on a very similar path, now at the Dara stage. Also see a doc at Penn. Thanks for this frank inside look at the long-term battle against MM. I’m saving this for future reference.

  4. Wonderful story of your battle with MM, I hope they will find something soon that will make everyone’s journey for a long remission or possible cure easier and quicker! I am in the same battle being a 5 year survivor and hoping for more years ahead.

  5. Angela Walker on

    Thank you for sharing your story. My son was diagnosed at the age if 17 with innumerable lesions throughout his body. His pediatric oncologist told me he probably had two years to live but he just celebrated his 25th birthday April 14th and recently went back on infusion treatment. Blessings to you…

  6. Don Behler

    I wish him much luck ! The treatments that are now available give him so much more chance of living quite a long life! Good attitude and the will to fight are also top priorities!

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