A tumor stole every memory I had
Wednesday, September 30, 2015
The following is an excerpt of an essay by Demetri Kofinas that appeared in Quartz. Read the full article here.
My brain tumor introduced itself to me on a grainy MRI, in the summer of 2009, when I was 28 years old.
It had been with me from the day I was born. Tumors like mine develop in utero. They are usually discovered in children, but no one could tell me why mine had only been found once I was an adult. It wasn’t cancerous. Perhaps this tumor at the base of my brain, near the pituitary gland, had reached maturity long ago and would never grow further. That would mean I had nothing to worry about. Or, maybe it had just woken up, and was growing for the first time. In three months, I could be blind. There was no way to know.
Certainty only came with surgery, whose outcomes were presented to me in the form of averages and standard deviations. Thirty percent risk of blindness. Forty percent risk of morbid obesity caused by hypothalamic damage. One hundred percent risk of complete pituitary loss, and hormone replacement therapy for life. And the risk of doing nothing? Undefined.
What eventually did happen was something none of the experts ever suggested would be possible. Assessing my type of tumor—which is known as a craniopharyngioma—the surgeons each gave me their different best guesses as to what the risk of inaction was. Those guesses varied wildly. There was no way I could rely on their predictions to make a decision.
What eventually did happen was something none of the experts ever suggested would be possible. Over time I would lose my memory—almost completely—of things that happened just moments before, and become unable to recall events that happened days and years earlier. To the rest of the world, I would become a young man behaving bizarrely, perhaps drunkenly, and I lacked the ability to acknowledge it, or fully explain what was going on. As it grew, eventually to the size of a small egg, the tumor dug a hole in my consciousness, of ever-increasing depth. I became incapable of living the life I had made for myself.
I’ve now been given my health back. Through persistence, luck, and maybe something more, an incredible medical procedure returned my mind and memories to me almost all at once. I became the man who remembered events I had never experienced, due to my amnesia. The man who forgot which member of his family had died while he was sick, only to have that memory, like hundreds of others, come flooding back. The memories came back out of order, with flashbacks mystically presenting themselves in ways that left me both excited and frightened.With my health back, I was able to live a life again, but it’s not the same life as it was before. The tumor changed me forever. And I am grateful for it.
The best-case scenarios presented to me by doctors at these well-regarded academic hospitals in Manhattan carried with them the same slew of “side effects” that were as unacceptable to me now as they were back then. The most horrific of the two standard options available involved sawing off the top of my head, lifting my brain out with forceps, and dissecting my tumor. The most horrific of the two standard options available involved sawing off the top of my head, lifting my brain out with forceps, and dissecting my tumor while hopefully avoiding damage to my optic nerve and hypothalamus. My pituitary would still need to be “sacrificed,” but the doctors seemed to think that replacing the beautiful symphony of hormonal function coordinated by my brain with a synthetic orchestra of daily injections was a small price to pay. Their procedures seemed barbaric, and their disregard for my beautiful life felt just as cruel. In the end, everything came back down to those same numbers — 30%, 40%, 100%. They rang in my ears like death knells. They rang so loudly that I almost didn’t hear the words of one doctor, a neurosurgeon working at Weill Cornell Medical College and Memorial Sloan Kettering, who promised me a scenario with only one outcome: a cure.
I met Dr. Jeffrey Greenfield on a sunny Tuesday morning, in a small examination room at Memorial Sloan Kettering’s pediatric day hospital. It was June 4, 2013, exactly two weeks since my sobering consultations with other New York neurosurgeons. Their prognoses, though not shocking, were nonetheless unacceptable. My father, also a doctor, would spend the intervening days sorting through thousands of available studies dealing with pediatric tumors, determined to find someone, somewhere, who could offer us something better, something whose side-effects I could live with.
Out of the hundreds of studies dealing with craniopharyngiomas, only 22 were published after my 2009 diagnosis. Some of these studies offered alternative treatments that we had not heard of before. A study out of Brazil used interferons (signaling proteins meant to attack tumor cells) and chemotherapeutic agents to shrink craniopharyngiomas, both with mixed results. Japanese researchers deployed CyberKnife radiation in order to shrink the tumors, though long-term outcomes were less than certain.
In the end, it was a combination of luck and persistence that was responsible for my introduction to Greenfield. An offhand recommendation from a pediatric oncologist at the Dana Farber Institute at Harvard University led my father through a series of introductions that culminated in my appointment with Greenfield on Manhattan’s Upper East Side.
“Mr. Kofinas?” There it was. My father and I both stood up. “Follow me please,” an orderly said. We were taken to a small examination room and told that we would see the doctor shortly. My vitals were taken, and I was asked the same, basic questions, to which I gave the same, perfunctory answers: “Have you been experiencing headaches?”
“Have you had any problem with your balance?”
“Have you experienced any loss of vision?”
No one ever asked me about my memory. I was relieved when the questioning was over.
It wasn’t long before the door to my examination room opened again, but this time, the visitor was a gray-haired doctor who looked to be in his early forties, forty-five at most. “Hello, I’m Dr. Greenfield. You must be Demetri.” I shook his hand. “Could you tell me a little bit about your symptoms?”
I told him that I wasn’t sure if they were “symptoms,” but that I had been a little depressed and forgetful. My father interjected, adding more details where I was reluctant or unable to do so.
Greenfield listened as my father explained my concerns about the side effects of surgery and the risks we had been presented with up until that point. Greenfield then turned to me again and asked me more directly to articulate my concerns. I explained how I had chosen to live with this tumor for the past four years, specifically because the risks of surgery were so unacceptable to me. He listened until I was finished. Then he said, “For a disease like a craniopharyngioma that has lots of treatment options, the outcomes, which are tied to the surgery, can be life altering. These types of tumors are located in such a sensitive epicenter of the brain that we have gained an appreciation for the less is more philosophy in dealing with them. Hearing your concerns, I feel the best option for you is a minimally invasive surgery.”
Dr. Greenfield’s proposal involved drilling a hole, roughly half the size of a dime, in the top of my skull, from which the entire surgery would be conducted. What did that even mean? I was hesitant to ask, but as usual, my father was not. “You mean endonasal?” he said.
“No,” Dr. Greenfield said. “In your son’s case, my recommendation would be an intracranial approach.”
I don’t remember exactly how Greenfield described the surgery to me at the time—and he and my father have helped me reconstruct our conversation as you read it today. But our subsequent discussions have made the details of the procedure difficult to forget. His proposal involved drilling a hole, roughly half the size of a dime, in the top of my skull, from which the entire surgery would be conducted. A catheter would be inserted, followed by an endoscope, all passed through two and a half inches of my brain, straight into the cystic, fluid filled portion of my craniopharyngioma, which had grown so large it was protruding from my brain’s third ventricle. This sounded horrifying at the time. It still does.
“What are the side effects?” I asked, nervously.
“We don’t have any complications with this procedure,” he said. “Some bleeding is possible, and you may get an infection on the skin that we would treat with antibiotics; I’ve never had that.” I didn’t understand what he was suggesting.
My father followed up, “Now, what do you mean there are no complications? What about his hormones? Is there any risk to the hypothalamic-pituitary axis?”
“No,” responded Greenfield.
“What about his hypothalamus? Is there any risk to the optic nerve?” my father asked.
“We map out our route on the computer using neural navigation software,” Greenfield began, “and we figure an entry point and an exit point, making sure that we are not going to hit any major blood vessels or critical regions of the brain along the way. Once the procedure is underway, we use real-time MRI in order to follow the catheter, which we insert first in order to clear the path of entry for the endoscope. The cystic component of the craniopharyngioma appears to be applying pressure up into the hypothalamic and cognitive centers of your brain, Demetri. We can use suction through the endoscope to puncture and drain the cyst.”
He turned to look at my father, then looked back at me and said, humbly, “I feel confident that I can alleviate your symptoms without any side-effects.” I must have looked baffled, because I proceeded to repeat my question about side effects a few more times.
Despite Greenfield’s confidence that he could cure me without causing any further damage, I remained unconvinced. And I remained a mess. The dementia continued to take its toll. I was no longer just forgetful, unfocused, and depressed; I was living moment to moment with no grounding in the past, no understanding of the future, and no temporal awareness whatsoever.
By the time we left Memorial Sloan Kettering, I had already forgotten most of what Greenfield had told me. Instead, I clung, again, to those old numbers: 30%, 40%, 100%. They remained seared in my mind from 2009, displacing all new information. The depression played its usual role, prejudicing bad news in place of good. The risk that the tumor had already invaded the hypothalamic walls of my brain was one of the few things I managed to retain from this otherwise hopeful introduction. I also think that it was hard for me to be hopeful, because I was so scared of having my heart broken. I loved my life. I didn’t want to let it go.
Lauren, my girlfriend of two years, emailed me later that morning, asking how my appointment went. I responded, “really well.”
That’s it. My subsequent 21 emails to her in the span of four hours were a compilation of confusion and nonsense. I tried to meet her at work but got lost looking for her office. I returned home, alone, only to find that I had lost my keys. We eventually met up later that afternoon. She had gotten a couple of baseball tickets from a friend. The Yankees were playing the Cleveland Indians. Neither of us particularly cared. We left in the seventh inning. She had work early and I had had a long day. We were a young couple for whom life had become very old.
Over the next few days, a process of acceptance started taking hold. I was given a date for my operation: June 17, 2013, just 13 days after my meeting with Greenfield. I remember wishing that it could be sooner. I was afraid, but I also wanted it to be over with. Some of my emails to friends and family during this wait conveyed confusion, some conveyed hope, and others, despair. I was dealing with what felt like an impossible situation.
Three days before my surgery, in the early morning hours, before daybreak, I awoke from a dream. I described it to a friend in an email shortly afterward. My fear was gone and I felt life flooding through my veins for the first time in so long. I had forgotten what it was like to have a future, and tonight, I felt normal again, and God, if it was not the sweetest feeling in so, so long. “My fear was gone,” I said to him, “and I felt life flooding through my veins for the first time in so long. I had forgotten what it was like to have a future, and tonight, I felt normal again, and God, if it was not the sweetest feeling in so, so long.” Though I no longer remember the substance of that dream, I remember the feeling very well. It was grace. It was grace that came over me, and it carried me through that weekend, and into the Monday morning of surgery.
I remember everything from that day. I had given up trying to sleep, and made a call around five o’clock in the morning to one of my cousins in Greece who had comforted me with words of encouragement and love in the weeks prior. I told him I was scared, and I think he was too, but he did his best not to show it. I told him I loved him, and then we hung up. I came back to bed. Lauren had been sleeping. I gently woke her, and we began to gather our things. The surgery was scheduled to take place at Weill Cornell, and at six in the morning, it was a short cab ride to the hospital. We met my parents in the lobby of the Greenberg Pavilion, where my sister arrived shortly thereafter. She was sick with a cold, and it was advised that she not stay long. She gave me a card that I opened after she left. Reading it still makes me cry.
The grace that visited me in that early morning dream remained with me during the final hours before my surgery. My heart was open. I was afraid, but I was not regretful. I had done the most with the time I was given, and I intended to spend what time I had left sharing my love with my family, and with Lauren. My concern for her only grew as the time of surgery approached. I wanted to know that she would be ok. She was the most beautiful thing that life had gifted me, and all I wanted was to keep her safe.
The last memory I have is of shivering and needing to urinate. “Mr. Kofinas, surgery is ready for you now.” There it was. I was wheeled down on a gurney, put through one last MRI, and fixed with the brain markers needed for guiding the catheter. Everyone watched, as I lay silently. I was trying to understand where it all went wrong. What it was all for, what it all meant, if my life had meant anything at all. Maybe I still held onto the hope that I could reason my way to God. I didn’t want to die. Death felt like the loss of everything, and this felt like death.
The last memory I have is of shivering and needing to urinate. I felt a connection to death, and it was utterly terrifying. It was somewhere amid this intensity that I was put under anesthesia.
My first memory post-surgery was of being positioned in my room by nurses in the intensive-care unit, where my family and a few close friends waited to greet me. I could feel pain in the top of my head. This turned out to be the first evidence of the procedure’s success. Had the surgery failed, I would not have been able to draw a connection between the events leading up to the operation and my awakening in the ICU.
With the others gathered around, my father shared the good news. He explained what Greenfield had conveyed to him—that the surgery had completely collapsed the cyst, which fell away like a deflated balloon from the hypothalamic walls, exposing inflammation, and an immediate pulsation of new blood rushing into the healthy tissue in my brain.
“Wow,” I said. “He actually saw the hypothalamus pulsating?” My father stared silently at me for a moment, and then his eyes began to fill with tears. He hadn’t seen that kind of understanding in me for a long time. I was able to reason. I understood what the pulsation meant, and more importantly, I understood what it could mean.
Deductive and inductive reasoning are skills we use constantly in our daily lives in order to make sense of our world. They enable us to act successfully on our surroundings. These are basic, evolutionary processes that we all take for granted. Without them, we cannot function in society. I had lost them. Now I had them back.
It turns out Greenfield’s operation was beyond successful. My tumor had been reduced to a fraction of its original size, making it an easy target for a proposed six-week, daily bout of targeted radiation. There was now hope for not just a cure, but a permanent cure.