“Ken,” I answered. This, again, wasn’t too unusual for someone at my level of impairment. They call it phonemic paraphasia, where you substitute one word for another that sounds similar.
When she asked me to write my name, I painstakingly drew out an “S,” tracing the letter multiple times before moving on to the “U,” where I did the same. It took several minutes for me to write my name. “Okay, would you write this sentence out for me: ‘Today is a nice day.’”
I drew out the letters, retracing each of them several times and misspelling some words. My handwriting was so poor that Gendal could hardly make out the sentence.
She wrote her impression in the chart: “It is difficult to determine at just two days post-operation to what extent communication deficits are language-based versus medication or cognitive. Clearly communication function is dramatically reduced from pre-morbid level when this patient was working as a successful journalist for a local paper.” In other words, there seemed to be a dramatic change between the person I had been and the person I was now, but it was difficult at the time to distinguish my problems understanding from my inability to communicate—and whether these would persist in the long or short term.
Later the next morning, neuropsychologist Chris Morrison arrived with her auburn hair piled high on her head and green-flecked hazel eyes flashing. She was there to test me on something called the Wechsler Abbreviated Scale of Intelligence, as well as other tests, that are used to diagnose a number of things, from attention deficit disorder to traumatic brain injuries. But when she entered the room, I was so unresponsive she wasn’t even sure that I could see her.
“What is your name?” Dr. Morrison began brightly, walking me through the basic orienting questions that by now I had been conditioned to answer correctly. Her next stage of questions assessed attention, processing speed, and working memory, which she compared to a computer’s random access memory (RAM), as in, “How many programs you can have open all at once—how many things you can keep in your head at once and spit back out.”
Dr. Morrison provided me with a random assortment of single-digit numbers, 1 through 9, and asked me to repeat them to her. Once we got up to five digits, I had to stop, though seven is the normal limit for people of my age and intelligence.
Next, she tested the word-retrieval process, to see how well I was able to access my “memory bank.” “I’d like you to name as many fruits and vegetables as possible,” she said, starting a sixty-second timer.
“Apples,” I began. Apples are a common fruit to start with, and of course they had been on my mind a lot lately.
“Carrots.”
“Pears.”
“Bananas.”
Pause.
“Rhubarb.”
Dr. Morrison chuckled inwardly at this one. The minute was over. I had come up with five fruits and vegetables; a healthy individual could name over twenty. Dr. Morrison believed that I knew plenty more examples; the problem seemed to be retrieving them.
She then showed me a series of cards with everyday objects on them. I could name only five of the ten, missing examples like kite and pliers, though I struggled as if the words were on the tip of my tongue.
Dr. Morrison then tested my ability to view and process the external world. There are many different things that must come together for a person to accurately perceive an object. To see a desk, for example, first we see lines that come together at angles, then color, then contrast, then depth; all of that information goes into the memory bank, which labels it with a word and, depending on the object, an emotion (to a journalist, a desk might elicit guilty feelings about missed deadlines, for example). To track this set of skills, she had me compare the size and shape of various angles. I scored on the low end of average on these, well enough for Dr. Morrison to move on to more difficult tasks. She introduced a set of red and white blocks and placed them on the foldout tray in front of me. She then showed me a picture of how the blocks should be arranged and asked me to re-create the picture with a timer running.
I stared at the pictures and then back at the blocks, moved them into a pattern that had nothing to do with the picture, and looked back at the picture for reference. I fiddled with the blocks some more, getting nowhere, but refused to give up. Morrison wrote down “tenacious in her attempts.” I seemed to realize I wasn’t getting it right, which frustrated me deeply. It was clear that, for all my other impairments, I knew that I was not functioning at the level I was used to.
The next step was for me to copy down complex geometric designs on graph paper, but my abilities here were so weak that Dr. Morrison decided to stop altogether. I was flustered, and she worried that moving forward would only make me feel worse. Dr. Morrison was convinced that I was very much aware, despite the cognitive issues, of what I could no longer do. In her review later that day, she marked cognitive therapy as “highly recommended.”
CHAPTER 31
THE BIG REVEAL
Later that afternoon, my dad had been trying to interest me in a game of gin rummy when Dr. Russo and the team arrived.
“Mr. Cahalan,” she said. “We have some positive test results.”
He dropped the playing cards on the floor and grabbed his notebook. Dr. Russo explained that they’d heard back from Dr. Dalmau with a confirmation of the diagnosis. Her words flew at him like shrapnel—bang, bang, bang: NMDA, antibody, tumor, chemotherapy. He fought to pay close attention, but there was one key part of the explanation that he could hold on to: my immune system had gone haywire and had begun attacking my brain.
“I’m sorry,” he interrupted the barrage. “What is the name again?”
He wrote the letters “NMDA” in his block lettering:
Anti-NMDA-receptor encephalitis, Dr. Russo explained, is a multistage disease that varies wildly in its presentation as it progresses. For 70 percent of patients, the disorder begins innocuously, with normal flulike symptoms: headaches, fever, nausea, and vomiting, though it’s unclear if patients initially contract a virus related to the disease or if these symptoms are a result of the disease itself.42 Typically, about two weeks after the initial flulike symptoms, psychiatric issues, which include anxiety, insomnia, fear, grandiose delusions, hyperreligiosity, mania, and paranoia, take hold. Because the symptoms are psychiatric, most patients seek out mental health professionals first. Seizures crop up in 75 percent of patients, which is fortunate if only because they get the patient out of the psychologist’s chair and into a neurologist’s office. From there, language and memory deficits arise, but they are often overshadowed by the more dramatic psychiatric symptoms.
My father sighed with relief. He felt comforted by a name, any name, to explain what had happened to me, even if he didn’t quite understand what it all meant. Everything she said was matching up perfectly to my case, including abnormal facial tics, lip smacking, and tongue jabbing, along with synchronized and rigid body movements. Patients also often develop autonomic symptoms, she continued: blood pressure and heart rate that vacillate between too high and too low—again, just like my case. She hardly needed to point out that I had now entered the catatonic stage, which marks the height of the disease but also precedes breathing failure, coma, and sometimes death. The doctors seemed to have caught it just in time.
When Dr. Russo began to explain that there are treatments that have been proven to reverse the course of the disease, my father nearly sank to his knees and thanked God right there in the hospital room. Still, Dr. Russo cautioned, even once you have a diagnosis, there are still substantial question marks. Though 75 percent of patients recover fully or maintain only mild side effects, over 20 percent remain permanently disabled and 4 percent die anyway, even despite a swift diagnosis.43 And those aforementioned “mild” side effects might mean the difference between the old me and a new Susannah, one who might not have the humor, vitality, or drive that I did before. Mild is a vague and undefined term.
“About 50 percent of the time, the disease is instigated by an
ovarian tumor, called a teratoma, but in the other 50 percent of cases, the cause is never discovered,” Dr. Russo continued.
My dad looked at her quizzically. What the hell is a teratoma?
It was probably best that he didn’t know. When this type of tumor was identified in the late 1800s, a German doctor christened it “teratoma” from the Greek teraton, which means monster.44 These twisted cysts were a source of fascination even when there was no name for them: the first description dates back to a Babylonian text from 600 B.C. These masses of tissue range in size from microscopic to fist sized (or even bigger) and contain hair, teeth, bone, and sometimes even eyes, limbs, and brain tissue. They are often located in the reproductive organs, brain, skull, tongue, and neck and resemble pus-soaked hairballs. They are like those hairy, toothy creatures in the 1980s horror film franchise Critters. The only good news is that they are usually—but not always—benign.
“We will need to do a transvaginal exam to see if there are any signs of tumors,” said Dr. Russo. “We’ll also check her over to see if there’s any link with her history of melanoma. If so, we’ll have to move on with chemotherapy.”
“Chemotherapy.” My father repeated the word in the hope that she had gotten it wrong. But she hadn’t.
My dad looked over at me. I had been staring off to the side, disassociated from the exchange, not seeming to gauge the magnitude of the moment. Suddenly, though, at the word chemotherapy, my chest began heaving, and I let out a deep sigh. Tears streamed down my face. My dad ran from his chair and threw his arms around me. I continued to sob without saying a word, as Dr. Russo waited quietly while he rocked me. He couldn’t tell if I understood what was going on or if I was just attuned to the amplified electricity in the room.
“This is killing me,” I said, my voice high yet unemotional, despite the sobs. “I’m dying in here.”
“I know, I know,” he said. With my head in his arms, he could smell the glue on my hair. “We’re going to get you out of here.”
After a few moments, my sobs stopped, and I lay back on the bed, my head against the pillow, staring straight ahead. Quietly, Dr. Russo continued. “Overall, this is good news, Mr. Cahalan. Dr. Najjar believes that there is a possibility that Susannah could get back as much as 90 percent of her former self.”
“We could get her back?”
“There seems to be a strong possibility.”
“I want to go home,” I said.
“We’re working on doing just that,” Dr. Russo replied with a smile.
Over the weeks, I had gone from being a notoriously difficult patient to a favorite, the ward’s “interesting consult” for a host of attending doctors, interns, and residents hoping to catch a glimpse of the girl with the unknown disease. Now that we had a diagnosis that had never before been seen at NYU, young MDs, hardly a day older than me, stared at me as if I were a caged animal in a zoo and made muffled assessments, pointing at me and craning their heads as more experienced doctors gave a rundown of the syndrome. The next morning, as my father fed me oatmeal and chopped-up bananas, a group of residents and medical students arrived. The young man leading the group of nascent MDs introduced my case as if I weren’t in the room.
“This is a very interesting one,” he said, leading a gang of about six others into the room. “She has what is called anti-NMDA-receptor autoimmune encephalitis.”
The group ogled me and a few even let out a few quiet “ooohs” and “ahhhs.” My father gritted his teeth and tried to ignore them.
“In about 50 percent of the cases, there is a teratoma in the ovaries. If this is the case, this patient may have her ovaries removed as a precaution.”
As the spectators nodded their heads, I caught this somehow, and began to cry.
My father bolted from his seat. This was the first time he had heard anything about my ovaries being removed, and he certainly didn’t want either of us hearing it from this kid. A born fighter and a strong man for his age (or for any other age), my dad bum-rushed the scrawny young physician and pointed a finger in his face.
“You get the fuck out of here right now!” His voice bounced around the hospital room. “Never come back. Get the fuck out of the room.”
The young doctor’s confidence deflated. Instead of apologizing, he waved his hand, urging the other interns to follow him toward the door, and made his escape.
“Forget you heard that, Susannah,” my dad said. “They have no idea what the hell they’re talking about.”
CHAPTER 32
90 PERCENT
That same day, a dermatologist arrived and conducted a full-body skin exam to check for melanoma, which took about thirty minutes because my body is covered in moles. But after a thorough search, the dermatologist concluded that, happily, there was no sign of melanoma. That evening, they wheeled me down, yet again, to the second-floor radiology department, where they would conduct an ultrasound of my pelvis in search of a teratoma.
I am awakened, even though I hadn’t been asleep. I had imagined this moment: the time when I would find out the gender of my child. Momentarily, I think, “I hope it’s a boy.” But the feeling passes. I would be happy with either a girl or a boy. I can feel the cool metal of the transducer against my belly. My chest wall leaps up into my throat in reaction to the cold. It was almost exactly how I imagined it to be. But then again not at all.
Distraught by the first ultrasound, I refused a transvaginal one, the more invasive pelvic examination. Still, even from the imperfect first test, there was good news: no sign of a teratoma. The bad news was that, ironically, teratomas were good news, because those with them tend to improve faster than people without them, for reasons researchers still don’t understand.
Dr. Najjar arrived the next morning alone and greeted my parents as if they were old friends. Now that they had identified the disease, and knew that there was no teratoma, it was time to figure out what treatment could save me. If he miscalculated, I might never recover. He had spent the night deliberating about what to do, waking up in sweats and rambling to his wife. He had finally decided to act with abandon. He didn’t want to wait for things to worsen; I was already too close to the edge. He delivered the plan of action while tugging at the corners of his mustache, deep in thought.
“We’re going to put her on an aggressive treatment of steroids, IVIG treatment, and plasmapheresis,” he said. Although he had a terrific bedside manner, sometimes he expected his patients to follow him as if they were trained neurologists.
“What will these all do?” my mother asked.
“It’s a three-pronged attack, no stoned turned,” Dr. Najjar said, missing the English idiom. “We’re going to reduce the body’s inflammation with steroids. Then flush the body of the antibodies with plasmapheresis, and further reduce and neutralize the antibodies with IVIG. It leaves no room for error.”
“When will she be able to go home?” my dad asked.
“As far as I’m concerned, she could leave tomorrow,” Dr. Najjar replied. “The steroids could be taken orally. She could return to the hospital for plasma exchange, and the IVIG treatment, if the insurance company approved it, could be done with a nurse at home. With all these treatments I believe that it’s likely Susannah will get back to 90 percent.”
Though I don’t remember the diagnosis, my parents tell me that when I heard this my demeanor changed, and I seemed bolstered by the news that I would be returning home soon. Dr. Russo noted in my chart that I appeared “brighter,” my speech “improved.”
Home. I was going home.
The next morning, Saturday, April 18, I was finally discharged. I had been in the hospital for twenty-eight days. Many of the nurses—some of whom had washed me, others who had injected me with sedatives, and a few of whom had fed me when I could not feed myself—came to say their good-byes. Nurses seldom find out how a patient fares after she leaves the hospital, and I was still in a particularly bad place. A small, hunched-over man entered the room holding papers. He had secured
an at-home nurse to tend to me and had recommended a clinic where I could receive full-time rehab. My mother took the papers, but only absentmindedly flipped through them; she would address these later. For now, we were going home, and that was all that mattered.
My mom, my dad, Allen, Stephen, and my college friend Lindsey, who had flown in from St. Louis the day before, all grabbed my possessions—stuffed animals, DVDs, clothes, books, and toiletries—and crammed them in clear plastic NYU “Patient’s Belongings” bags; they left behind the flowers and magazines. A transport staff worker helped me into a wheelchair as my mom placed slip-on flats on my feet. It was the first time I had worn shoes in a month.
The night before, my dad had made a sign thanking the nurses for their support. He posted it near the elevators:
THANK YOU
On behalf of our daughter Susannah Cahalan, we would like very much to thank the entire staff of the epilepsy floor at NYU Medical Center. We came to you with a difficult and desperate situation, and you responded with skill and compassion. Susannah is a wonderful young woman who deserved your hard work. Her mother and I will forever be in your debt. I cannot think of more meaningful work than what you do every day.
Rhona Nack
Tom Cahalan
My prognosis was still unclear—the projection was only “fair”—and no one could say with any certainty if I would ever get to that optimistic “90 percent,” or if I would ever regain any semblance of my former self. But they had a plan. First, I would continue to see Dr. Najjar every other Wednesday. Second, I would get a full-body positron emission tomography scan (PET scan) that creates a three-dimensional image of the body, which is different from MRIs and CT scans because it shows the body in the process of functioning. Third, I would be enrolled in cognitive and speech rehabilitation, and they would arrange for a twenty-four-hour nurse to care for me. Fourth, I would take oral steroids, receive plasma-exchange treatment, and get several more infusions of IVIG. But the doctors were aware that even months after the disease has run its course and immunosuppressants have been worked into the system, antibodies can still persist, making recovery a painful march of two steps forward, one step back.
Brain on Fire Page 15