In late April 2016 I went to see my hematologist for a follow up appointment. I had been seeing a hematologist regularly for the past 18 years, ever since I had been diagnosed with a rare disease of the bone marrow called essential thrombocythemia. The primary manifestation of that disease is elevated blood platelets. In my case that had been kept under control, and I had been otherwise asymptomatic, except for anemia that had developed over the previous 8 or so years.
I’ve lived in a few different countries, pursuing work as a professor of Philosophy; and I’ve seen my share of hematologist/oncologist offices with waiting rooms often populated with visibly sick people, sometimes ashen and weak and occasionally wearing protective surgical masks. I never much enjoyed visiting the doctor, being confronted by the serious illness of others, and being reminded of my own disease. Although I taught Philosophy, I did not have what might be called a philosophical attitude towards my own mortality. But after a hundred or so visits to these offices, I had become more or less accustomed to it.
This appointment was different. It followed up on one that I had a couple weeks earlier during which my doctor, a squat man whom I often saw trotting from one examination room to the next, had administered a bone marrow biopsy on me in order to have a looksee at the state of the underlying disease. That biopsy had been the third that I had had in 18 years.
It would be an understatement to call bone marrow biopsies unpleasant. A doctor uses a needle to pierce the tissue on the back side of the hip in order to gain access to the pelvic bone. The needle is twisted one way and then the other as it is pushed toward the bone. Although the tissue can be numbed, the bone cannot. Apparently there is no way to get the biopsy needle into the bone and to the marrow other than simply driving very hard, again with the twisting motion, until the biopsy needle pierces the bone. To speak of it as a “needle” might leave the wrong impression. It is hollow to allow for the collection of the marrow. But the stainless steel shaft has to be strong enough to pierce bone, and robust enough not to buckle under the pressure applied to jam it into the bone. The needle has a handle at a right angle to the shaft made to be grabbed by the fingers and palm. The handle facilitates the required twisting motion. The original JamshidiTM needle boasts a knob at the top, a protuberance of blue, industrial strength polymer. Once the needle is forced in about an inch and a half, an inner core of the needle is removed and there is some 360° twisting. People sometimes talk of undergoing surgery as going under the knife. I like to think of having a bone marrow biopsy as going under the corkscrew.
Fortunately, there is little that can go wrong. So, other than anticipating the unanesthetized bone pain, the immense discomfort of the pressure needed to drive the needle into the bone, and the weirdness of the twisting, I had very little to worry about on that day. As the doctor pushed and twisted the needle into the bone, he and the nurses chatted away about a soccer game the night before. “Don’t forget about the needle sticking out of my hip,” I wanted to say. But I refrained, and braced myself for more pressure. Once the needle had been rammed to the marrow, the inner core of the needle was removed. More twisting. The marrow filled into the hollow cylinder. More soccer chat. As the needle was finally removed part of my soul seemed to be sucked away. With the game fully analyzed, they sat me up, ensured that I was steady on my feet, and dispatched me only slightly daze homeward.
When my doctor ordered the bone marrow biopsy, I assumed that he was not driven purely by curiosity. I had been informed early in the treatment of essential thrombocythemia that one risk was that it might transform into secondary myelofibrosis, which involves bone marrow scarring that inhibits the production of blood cells. If you are accustomed to thinking of your longevity in terms of decades, with myelofibrosis you have to reorient to thinking in terms of years. The process can be slowed a bit by medication, but the only cure is a stem cell transplant. Given that anemia can be an indication for myelofibrosis, and that fibrosis can be seen from a marrow sample, I had a reasonably good hunch what my doctor was looking for.
That day in late April was sunny, the sort that lightens my spirits. The sun doesn’t shine nearly often enough for me up here north of the 50th parallel north. Too many years spent in the subtropics have left me constitutionally ill-prepared for the days and days of cloudiness. I enjoyed the sunshine as I walked to my doctor’s office for the follow up appointment. Once there I had my blood drawn as usual, and waited to be called into his office. In his office we did the routine survey of the results of the bloodwork. Nothing remarkable there. The platelet counts were under control and the other counts were out of whack in my usual ways. I was as anemic as ever, no more and no less than in recent history. He was advising me on when we should meet again, when I reminded him of what he seemed to forget, but I could not, the bone marrow biopsy. Duly prompted he sought the results and informed me without surprise that the indication was myelofibrosis. Calmly I asked him about the prognosis; he was non-committal. It seemed to depend on how quickly my counts fell, and all we could do was monitor that. In the end the scarring of the marrow that is myelofibrosis inhibits all blood production. The question is how long that would take. He couldn’t say, really. And he gave no indication that there should be a therapeutic change. Then he made his way rapidly down the hall, a flash of a white lab coat disappearing into another office. I made an appointment for my next regular visit and left. On my walk home the April sunshine was hard to notice for the fog of my confusion. What did I just learn?
An established principle of bioethics is that there should be no unnecessary invasive procedures. My doctor had conscientiously sought more information, doing so required more than the usual amount of discomfort, but then he did nothing with the information. Is the diagnosis not as bad I had imagined? Why the biopsy if the results would not mandate some sort of change of care? When I got home, the internet offered confirmation that the diagnosis was as bad as I had thought and although a stem cell transplant might offer the hope of a once and for all cure, it was risky, and not an option for many patients. How long did I have? I could not even hazard a guess. More than a year, I reckoned, but perhaps not long enough to see my son graduate from high school or from college, and probably not long enough to reach retirement.
It did me no good to wonder without some better answers. So, I decided to seek a second opinion. I e-mailed the head of Hematology at the medical school of my university. He answered promptly, re-assured me, and was happy to conduct an examination and offer a second opinion. Thus began my experience of being a stem cell transplant patient.