Lessons from Living, Dying and Living Again
Chapter 2
My Memorial Service
It’s hard to know what doctors hate more, Google, where patients and patients’ families and friends can turn for thousands of poor, incomplete, misleading, wrong-headed or possibly even accurate takes on important medical subjects, or patient portals, where test results and other data are posted with remarkable speed and are thus open to misinterpretation before any professional input can be considered. The correct answer is probably Google, where I was quick to turn once I felt my diagnosis had been well and truly confirmed (there were no such things as patient portals back in 2009 anyway).
What I found under “myelofibrosis” then and in the years since has been riveting, even the many parts I haven’t understood. The pages on “Causes,” for example, I thought would be about things like smoking and working in asbestos factories. Instead, they talked about “an acquired mutation of JAK2, CALR or MPL in a hematopoietic stem/progenitor cell in the bone marrow.” I kept reading because they were talking about what was going on inside of me, but, honestly, how deep into the capital letters was I expected to go? There was a reason I didn’t apply to medical school, even with doctors in my family background. And if you told me that the names of some of the chemotherapy drugs – Ojjaara, Inrebic, Vonjo – were international soccer stars, I would have believed you. Occasionally an interesting, more relatable tidbit turned up. Exposure to benzene, for example, is widely cited as increasing the risk of developing myelo. Benzene is described as a colorless, flammable liquid with a sweet odor. It is naturally produced by volcanoes and forest fires, found in emissions from burning coal and oil, gas stations and car exhaust. It can also be found in detergents, drugs, dyes and pesticides. I went down the list. Volcanoes? Certainly not. Forest fires? No. Burning coal? No. In fact, I couldn’t claim more than normal exposure to any of the benzene carriers. As anyone would, I wanted to blame my myelo on something specific, like the pack of Larks I smoked back in 1974. But most of the time, apparently, according to all that’s known today, it just happens.
Sometimes a Google search yields a tremendous trove, however. It’s where I found something called “Overview of MPNs: History, Pathogenesis, Diagnostic Criteria and Complications,” the author of which is my own Dr. Ronald Hoffman at Mount Sinai and two of his colleagues. It’s a very detailed, meticulous summary of what was known at that point about myelofibrosis and other MPNs (myeloproliferative neoplasms), with the promise of more detailed papers by other experts to come. I didn’t read every word, of course, not even close, but I did learn some things:
1. Myelofibrosis was first reported in 1879 by German surgeon Gustav Heuck, who’d identified two of his patients with massive splenomegaly, leukoerythroblastosis and bone marrow fibrosis. Eponyms for the disease are Heuck-Assmann Disease or Assmann’s Disease, for Herbert Assmann, who published a description under the term “osteosclerosis” in 1907. It wasn’t until 1951 that Dr. William Dameshek connected the three conditions which he termed myeloproliferative disorders – polycythemia vera, essential thrombocythemia and primary myelofibrosis. He found them to be biologically related but otherwise distinct.
2. Myelofibrosis is a rare disease, affecting 0.3 in every 100,000 persons, meaning one person in New Orleans might have it. It tends to attack older folks, the median age being 60, but those younger, even including children, can get it, too. It’s more common in males.
3. There is something in our bodies called the “splanchnic bed,” which I’d never heard of before. It really has nothing specifically to do with myelo, but I just wanted to let you know about its fantastic name.
4. There are lots of different leukemias, which can be fellow travelers with myelo. The one that late-stage myelo can morph into, with nearly always fatal consequences, is acute myeloid leukemia (AML). Much, much effort is made to avoid AML at any and all costs. My doctors and I would end up going to great lengths to avoid it, too.
5. The dawn of hematology occurred in the mid-1650s when a Dutch biologist, using a new device called a microscope, detected the red blood cells of a frog.
6. The survival rates of the myeloproliferative disorders go like this: essential thrombocythemia – 20 years is the median from time of detection; polycythemia vera – 14.1 years; “overt” myelofibrosis – 3.1 to 5.8 years.
Seeing such grim numbers may explain why I, for the most part, eventually decided to stay away from Google and the internet. There are those among us who haunt the online myelo pages in search of the latest news from the research front, reports from experts in the field and stories from fellow patients. I didn’t want to do that. I felt that the “latest news,” if it was actually a breakthrough of real merit, would come find me. And my interest in the stories of others, and the keep-your-chin-up dispatches from the myelo support groups, was minimal. In fact, after an initial flurry, my interest in my disease itself eased off as the months and then years went by and my condition remained stable. To some degree, absurdly, I felt that if I didn’t think about it, it wasn’t there. Or maybe, like a loudly humming refrigerator, it would somehow fix itself. Even so, things would occasionally intrude to remind me of the truth: a clock somewhere out there was ticking.
The first of these came on March 8, 2010, my 60th birthday. Normally, a “0” birthday calls for a celebration beyond the norm, maybe even a big party. Mine, however, was like the classic memorial service for someone who’s still alive. Marcia had set things up, a catered evening affair at a local country club, and invited dozens of family and friends. Everyone knew about my recent bad news, and so the undercurrent in the room was hard to miss: “We’re losing Charley. Let’s not spoil things by saying anything overt, but let’s give him a good send-off!” Consequently, the tributes flowed freely. My early years were recalled with affection, songs were sung, my children, even middle child Matt, beaming in from South Korea, recalled their own early years and my loving and occasionally (to them) hilarious place in the middle of it all. As for me, I started out strong but eventually was reduced to tears. I couldn’t be coolly philosophical about it. I thought I was dying, too, and all of this being put before me, these many faces that I loved so much, were exactly what I wanted to hold onto. It was proof that I wasn’t ready to leave. Not at 60. There were too many more stories to be told, songs to be sung and toasts to be drunk.
Sometimes the reminder of my own condition came more starkly and unexpectedly. One morning in June 2010 I chanced to read in my local newspaper that a man named Jonathan Wolken, age 60, had died at Mount Sinai Hospital in New York, with the cause reported as myelofibrosis and complications from a stem-cell transplant. Wolken had been a year ahead of me at Dartmouth and, with a couple of college mates, had started the remarkably innovative dance troupe Pilobolus, based not far from us in Washington, Connecticut. We’d seen them perform several times. I’d felt a special affection for them because of our college connection, but I loved and admired their spellbinding takes on dance and movement, too. Now I was startled to see that one of the founders had died of something I also had. Same age, same college, same disease, same hospital – it was like he was the unlucky version of me, or I was the lucky version of him. I probably didn’t even really take in the news story’s reference to stem-cell replacement. I still was unaware of it as something that might be for me. It was seeing the word “myelofibrosis” on the printed page that made me sit up straight. You just never saw it in black and white like that, as if it were a weapon found at a murder scene. I tried to bravely spoon up my Wheaties that morning, but in truth I’d once again felt the hard summoning tap on my shoulder.
I couldn’t afford to dwell on it, though. I had too much to do. I’d soon finish up my 24-year tenure as Editor of Connecticut Magazine and begin some types of writing I’d never tried before. The magazine had been a wonderful, sustaining job for a long time. We got many good things done there, even winning some national journalism awards, but its purchase by the now defunct Journal Register Company and its arrogant, inept and personally offensive leadership marked the beginning of a protracted decline. In retrospect, I shouldn’t have stayed as long as I did, but I guess I felt an attachment to the staff and loyalty to the magazine itself. I may have been worried about my medical insurance coverage, too, although Marcia’s benefits at her work were far more generous. Anyway, I hung around until they started the salary-dumping phase of their hideous regime, just before they sold the magazine off to a rapacious hedge fund. They came for my head, and salary, in mid-2013. We said our sad goodbyes and I lit out for my desk at home. I found some good freelance work and started on my boy’s dream of writing fiction.
Maybe my myelo was pushing me to try new things before it was too late, or maybe it was my recent birthday, but the decade of my sixties proved fruitful. First came writing the book and lyrics for a musical, “Mad Bomber,” and seeing it staged in 2011 at Seven Angels, our regional theater in Waterbury. It was based on the story of local guy George Metesky, who in the 1940s and ‘50s set off a string of pipe bombs in Manhattan and became known in the city tabloids as the Mad Bomber. I had no background in the stage so I don’t know what made me want it to be a musical, but I did and that’s what it became. We never did quite figure out a good final number for the show, but it was exciting for me to sit up in the top row of the theater and listen to my words and rhymes coming out of the actors’ mouths.
Three novels came next in the busy decade. The first was “Carrie Welton,” published in 2014, a historical fiction based on another actual Waterbury figure, this one from the mid-19th century, a young woman who was in some ways ahead of her time but prone to personal mishaps and misadventures. It was fun for me to research Waterbury, New York, Saratoga Springs and Boston as her story took her from place to place, and I loved going along for the ride, giving her and her contemporaries simple, smart and outrageous things to do and say. The second novel, 2018, was a mystery/thriller “The Easter Confession,” a Waterbury story set in 1955 involving art theft, murder and a Catholic priest who, tossed by circumstances, must solve it all basically by himself. The third novel, “The Darlings,” also written during this period, is still looking for a publisher, but no publisher seems to be looking for it.
Anyway, not bad production from an anemic cancer patient. Also not nothing were our Sunday group hikes of three to five miles through the Connecticut woods, or my participation in a weekly golf league, or spending a part or all of March in Florida and all or part of July on the Rhode Island shore in the little beach community of Quonochontaug, commonly called Quonnie, where Marcia had gone in summer for her entire life and where we, as a family, felt happiest and most at ease.
During this period, people very rarely asked me how I was feeling. Maybe I looked healthy enough. Maybe they were afraid I’d give them an answer they didn’t want to hear. I continued to regularly visit Leever for blood draws and an occasional consult. I became very familiar with the blood routine. Everyone seemed most concerned with my white blood cells, hemoglobin, platelets and red blood cells. My first counts, taken for my initial visit with Dr. Bowen in January 2010, were 6.7 for white blood (in the normal range), 11.6 for hemoglobin (a little below the 12.0-18.0 norm), 3.82 for red blood (4.20-6.10) and 534 platelets (higher than the 150-400 norm). I would have blood drawn literally hundreds of times over the next 14 years and each of these blood components would demand its troubling, dangerous time in the spotlight. For that first decade, though, the numbers stayed generally the same: normal range for white cells and platelets, low red cells and hemoglobin (thus my persistent anemia).
But a peace this weak and wobbly couldn’t last. Too many things conspired against it. Everyone knew, right from the start, that the bad days would eventually arrive. As it turned out, 2019 would be the year they’d begin, slowly at first but then, like an old steam engine leaving the station, picking up speed.
