Our hope was that with the tumor removed, the ventricles would return to their normal size, and the flow of cerebrospinal fluid would return to its normal dynamics. Time would tell.
Two days later, when Nina and I were at the airport, waiting to fly home, I got an email update from Ian.
Janel is coming around slowly, similar to his last wake up. He is awake, saying a few things, following commands with his left side reliably, beginning to move the right side weakly. I suspect he will come around in time.
Anyway, we have to give this young man time to recover.
I really appreciate that you involved me here.
Encouraged by Ian’s message, I was eager to see Janel. After sleeping off our twenty-hour flight home to Boston, I went to the hospital the next morning.
Janel was still in the neuro-ICU. Thick black stitches ran in an ear-to-ear arc along his hairline, resembling a zipper. A thin yellow tube coming from his nose was taped to the side of his face—it was being used to feed him and passed all the way from his nose to his stomach. He stared ahead, wide-eyed, blinking rarely. I said his name, but he didn’t respond to my voice. He occasionally moved his arms and legs a little, but that was it. I thought once when I asked him to move his left arm he did it, but I wasn’t sure if it was in response to me or if he had just moved it coincidentally at the time I had given him the instruction. He looked awful.
Later that day, I ran into Ian and asked him how he thought Janel was doing. He could see I was worried. “He’ll come around,” he said with casual confidence. “Give him some time.”
Morning after morning over that week, I continued to visit Janel’s bedside before I started my workday. The ear-to-ear incision began to look more like an early scar and less like a fresh wound. He appeared awake but not clearly alert. His eyes were always wide open, staring forward, but it was unclear if he was seeing anything.
Each morning I went through the same steps: saying his name a few times (no response), shouting his name a few times (no response), trying to get him to follow some simple directions in Creole (“Stick out your tongue,” “Close your eyes”—no response), poking briskly toward each eye without touching it (sometimes it looked like he blinked in response, but I was never convinced that it wasn’t just a coincidental blink), and finally rubbing his sternum with my knuckles (he moved his arms and legs a little bit but didn’t make any clear effort to stop me). He was unable to interact with me in any meaningful way.
I was growing concerned that Janel could become what I sometimes irreverently described as “neurosurgical success, neurological failure”: the surgery had gone technically well as far as the neurosurgeons were concerned (no more tumor), but the patient was no better off—or even worse off—than before the operation.
Ian and his team tried several times to wean Janel from the drain by clamping it off to see if his brain could tolerate this. Each time, the intracranial pressure on the monitor rose dramatically and clear cerebrospinal fluid oozed from around the site where the drain entered the scalp. Janel would need a shunt. The shunt surgery would be minor compared to the first two, but it would still require another round of anesthesia, and he still hadn’t woken up from the second surgery.
And so ten days after his second surgery, twenty days after his first, having never left the neuro-ICU, Janel went for his third surgery to have the shunt implanted.
* * *
The pathologists puzzled over the appearance of Janel’s tumor under the microscope. After performing a number of specialized tests on the tissue and analyzing its genetic makeup, they finally reached a diagnosis: a tumor of the pineal gland called a pineal parenchymal tumor of intermediate differentiation, or PPTID. I had never heard of it. The oncologist and radiation oncologist who specialized in brain tumors and who would be treating Janel had heard of it but had never seen a case.
The pineal gland is a small seed-size structure deep in the center of the brain. Its name comes from its resemblance to a miniature pine cone. Seventeenth-century philosopher René Descartes thought the pineal gland was the seat of the soul. Some spiritual traditions believe it is the anatomical correlate of the mystical third eye. Neurologists don’t give it much thought. It calcifies with age, and this calcium provides a bright anatomical landmark on CT scans. It occasionally develops benign cysts that rarely cause clinical effects. It produces melatonin, a chemical involved in the sleep–wake cycle, but tumors of the gland (or surgery to treat them) do not appear to have any significant effect on sleep. Pineal tumors are rare, accounting for less than one percent of all brain tumors.
Brain tumors are graded on a scale of 1 to 4 based on how aggressive they appear under the microscope: 1 is the most benign, 4 is the most malignant. Grade 1 pineal tumors are called pineocytomas, and grade 4 tumors are called pineoblastomas. Intermediate tumors considered grade 2 or grade 3 are so uncommon that they didn’t earn a succinct name, so they are classified as PPTIDs.
For the relatively benign pineocytomas, surgery is often enough to fully treat the tumor, with radiation added if the tumor cannot be fully removed. For the malignant pineoblastomas, surgery, radiation, and chemotherapy are needed to reduce the risk of recurrence. As for PPTIDs, they are so rare that there is hardly any scientific literature on how to treat them.
The pathologists classified Janel’s tumor as grade 3. This suggested it was highly aggressive. But with so few reported cases, it was unclear how aggressively to treat it. The oncologist and radiation oncologist at Brigham who would be treating Janel wrote to other brain tumor experts around the country to discuss his case. These experts recommended that we err on the side of being maximally aggressive, treating Janel’s tumor as we would treat a pineoblastoma, the most malignant pineal tumor. This would require eight months of intensive treatment: two months of daily brain radiation and chemotherapy, followed by three days of chemotherapy per month for the next six months.
Janel was going to need to spend nearly a year in Boston. Nothing seemed to be going as planned.
* * *
It was the end of January, one week after the shunt operation, two weeks after the second surgery, and almost a month since the first surgery. I continued to check on Janel every morning before work. He had finally made it out of the neuro-ICU and back to the tenth floor neurology ward where he had first arrived, but he still didn’t seem to be able to do anything except stare and occasionally move his arms and legs. I was increasingly concerned that we’d failed on one of medicine’s guiding principles: do no harm.
I trusted Ian, and I hadn’t taken care of any patients after such massive neurosurgeries to know how long it took them to wake up. But Janel looked worse than before all of his surgeries, and I was troubled that there was no explanation for why he remained in this state—at least not one that our high-tech medical tests could pick up. Janel’s scans showed he hadn’t had a stroke or brain hemorrhage, and EEGs (recordings of his brain waves) showed he wasn’t having seizures. Still, I worried. Maybe he had suffered some microscopic damage to his thalamus during the surgery that was too small to detect on the MRI. Or maybe he had developed a condition called posterior fossa syndrome, in which patients become mute after large surgeries involving the posterior aspect of the brain, where Janel’s first surgery had been. This syndrome is seen mostly after pediatric brain surgery, but I found some reports of it occurring in adults.