In a sense, my cancer journey began before I was born. How this came about was the death to cancer of one of my aunts on my father's side. I never knew her and learned about her only at reunions. Hearing her story meant little to me until another aunt on the same side of the family developed leukemia in 1974. It was apparently an acute leukemia, as her fight did not last long. What did not help was that leukemia treatment in the 1970s was nowhere near as developed as it is now. Five years after that, cancer really hit home. My father, who had smoked for 40 years (but quit when he experienced a mild cardiac event), developed tumors in his throat and lung - apparently metastatic lung cancer. He fought it for some months, going as far as to seek alternative therapy in a foreign country. In the end, I think that he simply resigned himself to the inevitable and stopped fighting.
Taking all of this into consideration, I became convinced from then onward that, at some point in my life, I would also probably hear a diagnosis of cancer. The only connection between all of us appears to be a genetic inclination toward weak immune systems. Fast forward to late April, 2008. One day, as I rested my head in my left hand, one of my fingers detected a lump behind my left ear. Would this have happened if I was right handed? In any event, due to the long-standing suspicion that cancer was in my future, I remembered my first thought being, "So, this is it." I had no symptoms, and something just told me that this was the cancer that I had been expecting all those years. That mental preparation also meant that I was not panicked or devastated to suspect cancer.
In a very un-manly move, I called doctor soon after for an appointment. I have become accustomed to difficulties and roadblocks in my life, and this was to prove no different. My internal physician of the past 25 years was retiring that month, so I was scheduled to see a junior replacement, who was relatively inexperienced. I explained the family history and my suspicions, but felt that I was not taken seriously. I received the usual antibiotics which are routinely given in cases of enlarged lymph nodes, and blood was drawn for testing. The series of "waits" began.
However, I soon developed fevers, drenching night sweats, fatigue, and protruding lymph nodes in my neck, which were visible from 50 feet away! The symptoms were almost exactly those of the mononucleosis I had 35 years before. The first blood test showed that I had "mono" before - but I already knew this - I was never asked about it. I inquired if those tests showed that I had active mononucleosis again. I was told that the blood tests did not check for that. At my request, further blood was drawn, further waiting ensued, and I was told that I once again had active mononucleosis. I was not worried about the mononucleosis, as there was a larger concern.
This relapse told me (if not doctor) that my immune system had failed, as mononucleosis is normally a one-time occurrence, with the immune system keeping the virus in a dormant state thereafter. Since I was still thinking "cancer", and the doctor was understandably thinking infection and anxiety, I was given anti-anxiety medication. Except that I was not anxious, but convinced. Dissatisfied with those results, I made an appointment with my Ear/Nose/Throat doctor, as he had a working knowledge of immune systems. Another wait. He examined the nodes and said, "We have to be concerned about lymphoma." His next suggestion was that one of the rapidly growing nodes had to come out for biopsy. However, his surgical schedule was filled for the next two weeks - a little too long for comfort, as fast as it was progressing.
My wife called a major hospital seeking a earlier biopsy date, and was referred to an independent surgeon not far away. After waiting for that dat to arrive, we met with him and amazingly, he was able to perform an excisional biopsy of a cervical lymph node the next day. He had worked with Fred Hutchinson Cancer Research Center and had a good basic knowledge of cancer. He described lymphoma and provided other very useful information. Regarding oncologists, he said, "Nothing against the older doctors, but as they near retirement, they may not be at the top of their game." He went on to recommend a mid-career oncologist to whom he would go if he thought he had a cancer. That was good enough for us. As an aside during our conversation, he gave me the advice, "If you ever hear the word 'wait' from a doctor, run for your life." That advice would soon save my life.
We immediately called for an oncology appointment but, of course, the recommended doctor had just gone on vacation! We were referred to his partner, which suited us, as we were becoming a bit nervous about the worsening signs and symptoms. When we met that doctor, we immediately noted that he was near retirement age. Hmmm. He scheduled a bone marrow biopsy and later reviewed the pathology report on the excised lymph node. The report stated that the pathologist had found "abnormal B and T cells" but was unable to identify any malignancy. We mentally compared this to my rapidly deteriorating condition. Then, doctor told us that he wanted to "wait" and see what developed. "Wait"? There's that word! We ran for my life.
Even though I as yet had no diagnosis - actually a misdiagnosis - my wife (who has been my angel throughout this) had already called the Fred Hutchinson Cancer Research Center in Seattle and had made an appointment for an initial consultation. She was referred to the Seattle Cancer Care Alliance (SCCA), which is the treatment arm of Fred Hutchinson. Fortunately, the center was only about an hour away from our home. Or first consultation there was with a doctor named Andrei Shustov, who displayed an amazing level of knowledge regarding cancer and treatment. Examining me and reading the pathology report, he ordered more sophisticated testing of the biopsy sample. The results were expected, but perplexing to me: "Peripheral T-Cell Lymphoma, Not Otherwise Specified." This meant almost nothing to me at the time, other than I had been correct in my impression that the original enlarged node was some form of cancer.
I do not believe that such a thing can be a coincidence, but Dr. Shustov just happened to be SCCA's specialist in the rare T-Cell Lymphomas - a perfect matchup! The bone marrow biopsy done prior ultimately showed cancer in my marrow. Scans revealed that I had "innumerable" tumors, and the pathologist had stopped counting at 50. All of this within two months of finding that first lump. Peripheral T-Cell Lymphoma - Not Otherwise Specified, is an unknown sub-type of a rare and aggressive lymphoma. To place this in context, I specifically remember hearing in the 1970s of a man who had "lymphatic cancer" (lymphoma). At the time, my thought was that he had no chance. Now, I had that same disease! As a practical matter, I began mental preparation for the end of my life.
Well, naive me was still thinking of being treated locally, not wanting to commute to Seattle and back. As I expressed this thought, my wife suddenly blurted out to Doctor Shustov, "Will you treat him?" His answer was a simple "Yes." In retrospect, I know that doctors probably do not like to receive late stage patients, but Dr. Shustov did not hesitate. Rather, he rose to meet the challenge. Treatment would have to be guessed at, based on then-current research, since so little was known about T-Cell cancers. The recommended first line therapy was "clinical trial" but, what if there is no applicable trial? Well...
Dr. Shustov went to work and devised a treatment plan. "I treat it aggressively" he told us. Having no experience at that time with chemotherapy, I thought to myself, "great!" Later, I would experience somewhat different thoughts! I was to receive a double regimen involving eight different drugs, with the thinking being to attack the lymphoma essentially from one direction, then suddenly reverse tactics and attack it from the other. The first regimen was called CHOEP-14, which consists of drugs named Cyclofosfamide, Hydroxydaunorubicin, Vincristine, Etoposide and the steroid Prednisone, to be infused on a 14-day cycle. After four cycles, this was to be followed immediately by four cycles of GVD (also called GND), a different regimen which contained the drugs Gemcitabine, Navelbine and Pegylated Liposomal Doxorubicin.
Knowing no better, I worked 10-hour night shifts through the first four cycles, but it became increasingly obvious as time went on that I could not maintain that level of activity. I suspected that I would need to stay home at some point. What tipped the balance was the fact that my supervisors began to ask me more than once during each shift, "Are you sure you're OK?" If it was that obvious to them, then I simply had to consider my responsibilities to those around me. Now, a "response" to chemotherapy is considered to be one of the following: slowing the cancer's growth, stopping it, obtaining a partial remission, or (rarely) a complete remission. Against all odds, mid-way scans showed a complete response. Additional scans at the end of treatment showed that the response was still complete - very satisfying. In retrospect, perhaps a little too satisfying.
Dr. Shustov told me to go home and recover, and that we would re-scan in two months. Which we did, but Dr. Shustov was examining the results on the computer screen too closely for comfort. The PET scan showed four tumors which were not present at the end of treatment - an immediate relapse. I will not forget the feeling I had at that point. Doctor told us that a relapse within six months of initial therapy reduced the prognosis further from the initial "poor" ...to...what? I began again to prepare for the end. However, Dr. Shustov, still glaring at the screen, spoke ten words that have stuck with me, "I want to get this one while it's a baby." Such determination was very inspiring to me. Still, there was little reason to hope for much, as we began this journey in very marginal condition.
After eight drugs, there was precious little left to fight it with. I was scheduled for the unfortunately named "salvage therapy" of "ICE" and possible palliation at home. Best wishes. This did not require further explanation. However, wouldn't you know it, doctor soon called with information about a clinical trial of a new drug called Romidepsin, aimed at the exact relapsed T-Cell Lymphoma that I had. I do not believe in coincidence. However, as many others do, I had viewed clinical trials as reserved for those who were desperate. Then, Bam! It hit me: I was desperate! Entering that trial was a very easy choice to make. I was concerned about the long-term effects of the drug on the body. When I asked Dr. Shustov what they were, he smiled and said, "You'll tell us!" Of course, it was experimental - how could they know? Talk about uncharted territory.
Sometime during all of this, I had decided to learn about this cancer. The learning curve was steep, out of necessity. By this time, I knew and trusted Dr. Shustov explicitly, but I wanted to know - I had to know, what was occurring. All of the terms, medications, cell surface proteins, inhibitor class drugs - it took time to appreciate the complexity of it all, never mind attempting to comprehend it. I learned that I had a form of T-Cell Lymphoma, which is simply when one T-Lymphocyte (a type of white blood cell) mutates and begins an uncontrolled cloning of itself. It was an unknown sub-type that was similar to the initially unpronounceable AngioImmunoblastic T-Cell Lymphoma. That which was online told me that the median survival amounted to about two years, a 50-50 chance of living that long. What's worse, I had a 30% chance of living 30 months!
Amazingly, I received a full response once again to the trial drug and this continued for so long that the clinical trial closed. At that time, I was asked to remain in the long-term study of the drug. Due to the very satisfactory response, infusion frequency was reduced from three infusions every four weeks, to two, then a single infusion every four weeks. I was in full response for about four and one half years (2009-2013), but then a number of odd symptoms suddenly appeared: skin rash, joint pain, swelling in arms and legs, an incessant dry cough and others. Oh, and there were some enlarged nodes to go along with all of this. Naturally, they were too deep to biopsy, so we proceeded on the reasonable assumption that I had relapsed, and treatment was increased to the original three infusions per four week period.
However, this time, we received a mixed response. Some nodes were smaller, some had grown slightly, but there were new nodes, which is never a good sign. Not wanting to fight cancer again, I searched for an autoimmune disease that would produce those symptoms, and identified one, but this was only wishful thinking. No longer responding to Romidepsin as before, treatment was stopped in early 2014, and we began a stare-down with the cancer. The active surveillance technique of "watch and wait" is commonly used in cases of slow-growing cancers, but this one was aggressive. It was an almost surreal time and I felt like a soldier when the war ends - what to do now? My wife, reading a foundation article which Dr. Shustov had written about Angioimmunoblastic T-Cell Lymphoma said, "Read this. These are your symptoms exactly." And, yes they were.
Strangely, the symptoms essentially resolved on their own. But not for long. As it rebounded, there was finally a lymph node that was in a reasonable location to biopsy. There was much discussion over the results, as they were not clear at all. But, the final result was that the lymphoma had apparently mutated into AngioImmunoblastic T-Cell Lymphoma, a slightly different, but classifiable form of T-Cell Lymphoma. With my rather macabre sense of humor, I liked the sound of the name: Angioimmunoblastic. It rolled off the tongue much better than "Not Otherwise Specified." During the years that had passed, two new drugs and another clinical trial had become available. Doctor and I had remained aware of them, as relapses are so common. We tried one drug but quickly stopped it so that I could enter a clinical trial. I failed the trial drug, receiving the same mixed response as before. Back to the other drug. Same reaction. On to a third drug. No change. I now had three consecutive PET scans stating the one-word conclusion: "progression." After all the years and treatments, I was at peace with this.
Once again, Dr. Shustov produced what ended up being a miracle drug regimen - a new combination of existing drugs that had been developed by a colleague at Fred Hutchinson. At the time, I had intermittent sharp pain in my abdomen and it was subsequently found to have been lymphoma which had broken out of my lymphatic system. That lymphoma was accompanied by about two dozen tumors in my lymphatic system, as well. To combat all of this, the new regimen called "TREC" was administered. After two cycles, I was scanned once again. Dr. Shustov called me one evening and asked how I thought the scans went. Being accustomed to bad news at the time, I told him that I thought they would show residual disease, or perhaps even more progression. He countered with, "Now, don't get pessimistic on me!" to which I explained that if I expected bad results, I could not be disappointed.
Well, he was elated to advise me that every trace of cancer was gone - all two dozen tumors and the small intestine involvement in the CT scan. No enlarged nodes and no unusual uptake on the PET. The great news soon gave way to thoughts of where to go from there. We could wait to see if it relapsed, but it had done so several times, and it was now a smarter cancer than we started with. It was a matter of time. My life then was fully dependent upon some new drug appearing, and one had appeared before, but again? We had more serious discussions, as there would be essentially nothing to use against it. We had thrown fourteen drugs at it. Oh, we could try some non-lymphoma drugs "off-label", but there was no guarantee of any effectiveness.
So, we made the decision to prepare for a stem cell transplant. Finding no acceptable donors in either the nine-plus million US donors, or even in the world registry, we had my two children tested. Both were an acceptable match - another "coincidence." WE chose my son's stem cells, as there are less complications if the transplant genders are the same. One thing we knew going in was that I would very likely develop Graft versus Host Disease, in which the new immune system would attack parts of my body. Well, we were not disappointed, as it certainly did arrive. The flip side is that the new immune system is much more likely to destroy any remaining or new lymphoma cells that might not have been eradicated in the intense conditioning chemotherapy for the transplant.
One other factor is that when you receive someone else's marrow (blood stem cells) in a transplant, you not only develop their blood type, but also their allergies. My son has had a lifetime of numerous and serious food allergies, and now I share them with him. So, life has changed and is changing, but we are dealing with it on a day-to-day basis. At this point, the transplant has been successful, but the associated complications point to the fact that this is not the ultimate solution to the problem of blood cancers. Clearly, further research and participation in clinical trials will expand the frontiers of fighting these rare and aggressive T-Cell Lymphomas. Dr. Shustov's T-Cell Leukemia Lymphoma Foundation was created to ensure that this happens. If you have a similar diagnosis, you really need to consult with a hematologist, but even more so, a T-Cell specialist - and an aggressive one - at a major cancer research center. Consider clinical trials, for your own sake as well as those who follow you. Travel if you must, as survival is at stake here.
To close this novel, entering my eighth year in this journey, I realize that I really have nothing to complain about. This has been as difficult for the family as for me, but it has strengthened us and drawn us closer together. Our marriage is stronger. And as odd as it sounds, if I had the chance to go back eight years and make the choice, I would choose the cancer journey again. It has intensified the experience of living. I must add that my Catholic faith has both prepared me for this journey as well as sustaining me through it - especially since I have received the Sacrament of Anointing ("Last Rites") so many times that I have lost count. I no longer believe in coincidence, as there were and are simply too many providential aspects along the way.
The bottom line is, to quote the legendary New York Yankees catcher Yogi Berra, "It ain't over 'til it's over!"
James Ferrell, T-Cell Lymphoma Fighter & TCLLF Gladiator, Enumclaw, Washington