Brain on Fire

  Dr. Najjar handed me a blank sheet of paper that he had ripped out of his notebook and said, “Would you draw a clock for me and fill in all the numbers, 1 through 12?” I looked up at him with confusion. “As you remember it, Susannah. It does not have to be perfect.”

  I looked at the doctor and then back down at the paper. I held the pen loosely in my right hand, as if it were a foreign object. I first drew a circle, but it was lopsided and the lines were too squiggly. I asked for another sheet. He tore another out for me, and I tried again. This time a circle took shape. Because circle drawing is a type of procedural memory (one that was also still present in the famous amnesiac patient H.M.), that is, an overlearned practice, like tying shoes, patients have done it so many times before that they rarely get it wrong, so it didn’t surprise him that I drew it with relative ease the second time. I outlined the circle once, twice, and then three times, an act called perseverative dysgraphia, a disorder in which a patient draws and redraws lines or letters. Dr. Najjar waited expectantly for the numbers.

  “Now draw numbers on the clock.”

  I hesitated. He could see me straining to remember what a clock face looked like. I hunched over the paper and began to write. Methodically I wrote the numbers. Often I would get stuck on a number and draw it several times: more perseverative dysgraphia.

  After a moment, Dr. Najjar looked down at the page and nearly applauded. I had squished all the numbers, 1 through 12, onto the right-hand side of the circle; it was a perfect specimen, with the twelve o’clock landing almost exactly where the six o’clock should have been.

  Re-creation of my clock drawing.

  Dr. Najjar, beaming, grabbed the paper, showed it to my parents, and explained what this meant. They gasped with a combination of terror and hope. This was finally the clue that everyone was searching for. It didn’t involve fancy machinery or invasive tests; it required only paper and pen. It had given Dr. Najjar concrete evidence that the right hemisphere of my brain was inflamed.

  The healthy brain enables vision through a complex process involving both hemispheres.34 First, certain receptors are activated in the retina, and information passes through the eye and visual pathways until it reaches the primary visual cortex, located at the back of the brain, where it becomes one single perception, which the parietal and temporal lobes then process. The parietal lobes provide the person with the “where and when” of the image, situating us in time and space. The temporal lobe supplies the “who, what, and why,” governing our ability to recognize names, feelings, and memories. But in a broken brain, where one hemisphere isn’t working properly and the flow of information is obstructed, the visual world becomes lopsided.

  Because the brain works contralaterally, meaning that the right hemisphere is responsible for the left field of vision and the left hemisphere is responsible for the right field of vision, my clock drawing, which had numbers drawn on only the right side, showed that the right hemisphere—responsible for seeing the left side of that clock—was compromised, to say the least. Visual neglect, however, is not blindness. The retinas are still active and still sending information to the visual cortex; it’s just that the information is not being processed accurately in a way that enables us to “see” an image. A more accurate term for this, some doctors say, is visual indifference:35 the brain simply does not care about what’s going on in the left side of its universe.

  The clock test also helped explain another aspect of my illness that had largely been ignored: the numbness on the left side of my body that had since become a long-lost nonissue. The parietal lobe is also involved in sensation, and malfunction there could result in a feeling of numbness.

  This single clock-drawing test answered so much: in addition to the numbness on the left side, it explained the paranoia, the seizures, and the hallucinations. It might even account for my imaginary bedbugs, since my “bites” occurred on my left arm. Ruling out schizoaffective disorder, postictal psychosis, and viral encephalitis and taking into account the high white blood cells in the lumbar puncture, Dr. Najjar had an epiphany: the inflammation was almost certainly the result of an autoimmune reaction, caused by my own body. But what type of autoimmune disease? There had been an autoimmune panel, which tests for only a small fraction of the hundred or so known autoimmune diseases, that had come back negative, so it couldn’t be one of those. Dr. Najjar then recalled a series of cases in the recent medical literature about a rare autoimmune disease that affects mostly young women that had come out of the University of Pennsylvania. Could that be it?

  There were other questions: How much inflammation? Was my brain salvageable? The only way to answer these questions was to do a brain biopsy, and he wasn’t sure my parents would agree to one. No one liked the sound of a brain biopsy, which involved cutting out a small piece of my brain to study, but without quick intervention, my condition might not improve. The longer the problem continued without proper intervention, the worse my chances for returning to my former self became. As he mulled this over, he pulled at his mustache absentmindedly and paced around the room.

  Finally, he sat down on the bed near me. He turned to my parents and said, “Her brain is on fire.” He took my small hands into his large ones and leaned down to my eye level. “I’m going to do everything I can for you. I promise I will always be there for you.”

  For a moment, I seemed to come alive, he would later recall to me. I’ll always regret that I remember nothing of this pivotal scene, one of the most important moments of my life.

  Dr. Najjar saw tears gather at the corners of my eyes. I sat up and threw my arms around him. For him, it was another crucial moment in my case: he could sense that I was still in there, somewhere. But it was just a blip. After that outpouring, I lay back down and dozed off, exhausted by the brief display of emotion. But he knew I was there, and he would not give up on me. He motioned for my parents to follow him outside the room.

  “Her brain is on fire,” he repeated. They nodded, eyes wide. “Her brain is under attack by her own body.”

  CHAPTER 27

  BRAIN BIOPSY

  Dr. Najjar’s news didn’t end there. “The next course of treatment, I feel, should be steroids, but we need to confirm the inflammation before moving on,” he said.

  “How?” my mother asked.

  “There is a doctor at the University of Pennsylvania who specializes in autoimmune disease, and I believe he will have the answers we are looking for. Meanwhile,” he paused, knowing my parents would not be pleased by what he was about to say, “there are several ways we can go with this. There are the steroids. There is plasmapheresis. There is IVIG.”

  My parents nodded again, in unison, entirely taken by this force of a man.

  “But I think the best thing to do,” he said, lowering his voice, “is to do a brain biopsy.”

  “What does that mean?” my mother asked quietly.

  “We would need to look at her brain and take a small”—he put two fingers up and separated them by a centimeter—“piece of her brain.”

  My father bristled. “I don’t know about that.”

  “I promise you that if it she were my child, I would do a brain biopsy. The risks of not doing it far outweigh the risks of doing it. The worst thing that can happen is that we end up in the same place we are now.”

  They didn’t say a word.

  “I want to do this Monday, Tuesday at the latest,” he said. “But it is up to you. In the meantime, I will talk it over with the team and the surgeon. Let me think about it. And I will let you know.”

  As Dr. Najjar walked away, my mother whispered, “He’s a real-life Dr. House.”

  Later that afternoon, Dr. Russo arrived to confirm to my parents that the team’s decision was to move forward with a brain biopsy. My mom tried to remain calm, but she felt helpless. She motioned for Dr. Russo to join her outside the room in the hallway. She had so many questions for her, but all she could grasp on to were those two simple, bone-chilling words: brain bio
psy. After weeks of maintaining a facade of composure, she had finally hit her breaking point and started to sob. Dr. Russo stood with her hands crossed over her chest and then reached out and ever so slightly touched my mother’s arm.

  “It’s going to be fine,” Dr. Russo said.

  My mom wiped her tears and took a deep breath. “I better go back in.”

  When she returned, my dad threw her an accusatory glance. “We heard you,” he said.

  Despite his gruffness, as he would later write in his journal, he felt the same concerns as my mother: “The very sound of a brain biopsy scared me. I could hear my mother’s voice telling me not to do it. I could hear her tell me to never let anyone mess with the brain. She had seen a lot of bad things happen as an RN and she didn’t trust brain surgeons. I had to remind myself how long ago that was.”

  Depleted by the morning’s events, the clock drawing, and the brain biopsy news, my dad walked across town from NYU to Thirty-Third Street, to catch the subway on Park Avenue South. Between First and Second Avenues, he spotted the Chapel of the Sacred Hearts of Jesus and Mary. Impulsively, he walked into the chapel, admiring the stained glass windows and the vibrant painting of an angel wrapping her arms around a broken man. He got on his knees and prayed.

  That same afternoon, downtown in the Manhattan district attorney’s office, my mom was doing something similar. She held hands with her secretary, Elsie, and her coworker Regina, who was also a Baptist minister. All three closed their eyes and held hands in a circle as Regina’s voice rose high above them: “God, cure this young lady. God, hear us, hear our prayers. We pray for you to cure this girl and make her better. Hear our prayers. Please hear our prayers.” My mom, a no-nonsense agnostic Jewish girl from the Bronx, now swears she felt the presence of God.

  I was blissfully unaware of my parents’ anguish. I texted my college friend Lindsey, who lived in St. Louis: “I’m getting a briannopsy!”

  “What? What do you mean?” Lindsey wrote back, confused by the misspelling.

  “They’re taking a peece of my brain!”

  My friend Zach, who was splitting cat-sitting duties with another coworker and friend, Ginger, called that day as well. I shared the news in the same manner that I would have told him what I had for lunch.

  “I’m having a brain opsy,” I said.

  “Hold on, Susannah. They’re doing surgery on your brain?” he asked, clearly concerned. It was the first time that someone had expressed to me directly how distressing the surgery sounded. I began to cry tears of fear and confusion and finally hung up, too upset to continue with the conversation.

  It was Easter weekend. On Saturday, the surgeon’s head nurse arrived to describe the preparation for the brain surgery. She seemed upbeat and managed to make the biopsy sound routine. This did not quell my father’s fears. As she described where they were going to shave my head—the front part above my right forehead about four inches in toward the crown—I listened impassively, and my dad was impressed by my dignity. It was only later that night that I began to break down. Seeing me upset made my dad cry too. Then he heard me laughing.

  “You look funny when you cry,” I giggled. Suddenly we were both laughing and crying. Through his tears, he reminded me of our motto.

  “What’s the slope of the line?”

  “Um.” I couldn’t remember the answer.

  “Positive. And what does that mean?”

  “Um.” I moved my arm upward, indicating progress.

  “Right. Getting better every day.”

  The next day was Easter morning, and my dad brought me an Easter basket, the same basket he had given to me since I was a child, filled with chocolates and jellybeans. He was delighted to see me looking like a kid again, eyes wide, ready to pounce on the candy.

  My parents arrived extra early on Monday morning, filled with both dread and excitement. For my part, I seemed unnaturally calm. Eventually an orderly, whom they thought looked like a Hells Angel, put me on a stretcher and wheeled me down into surgery. My parents hung back for a few seconds. Setting aside years of betrayal, emotional estrangement, and petty fights, they briefly exchanged a hug and a few quiet tears.

  The OR was the embodiment of medicine on an industrial scale, a sterile place with doors snaking into dozens of operating theaters. Gone were the landscape paintings and soothing music; this was where the serious surgeries happened. We waited in the area immediately in front of the elevators, held back by large, clear vertical blinds. Everyone on the other side of the blinds wore surgical scrubs.

  The neurosurgery resident came by to shave my head. The doctor shaved a good five-inch diameter section from my head, but, although I appeared fully conscious, I did not scream, call out, or cry. My dad again admired this strength, though it’s possible I simply had no idea what was going on. I sat on the bed unfazed with my head wrapped in a towel, as if I had just received a spa treatment.

  Fighting back tears, my dad knelt beside me.

  “Remember what I told you. What is the strategy?”

  “One step at a time.”

  “What is the slope of the line?”

  “Positive.”

  Neurosurgeon Dr. Werner Doyle donned his surgical gown and prepped for surgery. He entered the operating room flanked by a scrub nurse, a circulating nurse, and an anesthesiologist. Despite the relative safety of this procedure, any number of things could still go wrong: they could have chosen the wrong location to dissect, and there is always a risk of infection or mistake with any type of surgery, especially the kinds involving the brain. But still, brain biopsies were simple compared to the more complex epilepsy surgeries that he had grown accustomed to performing over the years.

  A new MRI had been loaded into his office computer and workstation, and it guided the surgeon through a process called frameless stereotactic neurosurgery, which involves the visual mapping of the brain in both three and two dimensions so that the surgeon can swiftly and accurately target one specific area of the brain—in this case, the right frontal cortex. He had already chosen an area, one without any large draining veins, farthest away from the parts of the brain responsible for motor functions. My gurney was rolled over to the operating table, my scalp shaved and cleaned. Then they put me under general anesthesia.

  “Count down from 100,” the anesthesiologist instructed.

  “100 . . . 99 . . .”

  As my eyes closed, they secured the head holder onto my temples to keep me still. With a scalpel, Dr. Doyle made an S-shape incision, 4 centimeters from the midline of the scalp over the right frontal region. The arm of the S extended just behind my hairline. He parted the skin with a sharp blade and gripped each side with retractors. Grasping a high-speed drill in his hands like a skillful carpenter, he pressed it down on the skull, making a “burr hole,” or a 1-centimeter-diameter aperture through the bone of the skull. He then went over the burr hole with a craniotome, a bigger drill, grinding the bone into dust. He removed a 3-centimeter piece of the bone plate, exposing the dura, or the outermost, leathery, protective layer of the brain, and saved a section to send for testing along with the brain tissue.

  With a fine #11 blade and a dissector, he cut several cubes of tissue, equaling about 1 cubic centimeter in volume, which included white matter (strands of nerve fibers) and gray matter (the cell bodies of neurons). He set aside specimens for future studies and an extra sample to be frozen in case other tests were needed. He swabbed the brain matter and stopped the blood flow using cottonoids, highly absorbent synthetic fibers.

  He then very carefully stitched a dura graft onto the outer layer of the brain, suturing it together, and then reattached the bone plate. He pushed the plate to one side, butting it up against the existing bone so that it would fuse, and then secured the plate using screws and a small metal plate. He ended the procedure by returning the outer layer of skin to its original position, and closing the scalp with metal staples. The whole procedure took four hours.

  “Count down from 100,??
? a disembodied voice says.

  “100 . . . 99 . . . 98 . . .”

  Darkness.

  Blink. Blink. Blink. “I’m still awake.”

  Darkness.

  A crowded recovery room. I’m alone. There’s a family to the right of me, surrounding another patient. Where are my parents?

  Then I see them. Mom and Dad. I can’t move.

  Then Stephen and Allen. I try to raise my arm slightly to wave; it feels as heavy as a fifty-pound weight.

  Darkness.

  “Thirsty.” My voice is hoarse. “Thirsty.”

  “Here,” a brusque nurse says, shoving a water-soaked sponge into my mouth. The texture is unpleasant but the water is a godsend. I suck and suck. “Thirsty.” She shoves another one in my mouth. I hear the parents beside me feeding their kid ice chips. I raise my arm. I want some. A male nurse approaches. “Ice.” He brings me a few ice chips and places them on my tongue. I can hear the female nurse telling him not to give me water. “She can’t have any water. Just ignore her.”

  “Water, water,” I moan.

  She approaches. “I’m sorry but you can’t have any more.”

  “I’m going to tell everyone how you treated me. I’m going to tell everyone when I get out of here.”

  “What did you say?” Her tone scares me.

  “Nothing.”

  Darkness.

  I’m in a claustrophobic one-person room. I have to pee. I have to pee. I push. My catheter comes undone and the urine sprays all over the bed. I call out. A nurse comes in.