Brain On Fire: My Month Of Madness Part 13

"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. 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. Rus...o...b..gan 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 G.o.d 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. 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 Mild is a vague and undefined term. 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 h.e.l.l is a teratoma? What the h.e.l.l 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, teraton, which means monster. 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 ma.s.ses 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 hairb.a.l.l.s. They are like those hairy, toothy creatures in the 1980s horror film franchise 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 ma.s.ses 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 hairb.a.l.l.s. They are like those hairy, toothy creatures in the 1980s horror film franchise Critters Critters. The only good news is that they are usually-but not always-benign.

"We will need to do a transv.a.g.i.n.al 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, disa.s.sociated from the exchange, not seeming to gauge the magnitude of the moment. Suddenly, though, at the word chemotherapy, 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. 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 m.u.f.fled a.s.sessments, 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 b.u.m-rushed the scrawny young physician and pointed a finger in his face.

"You get the f.u.c.k out of here right now!" His voice bounced around the hospital room. "Never come back. Get the f.u.c.k 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 h.e.l.l 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 pa.s.ses. 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 transv.a.g.i.n.al 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 good news, because those with them tend to improve faster than people without them, for reasons researchers still don't understand. 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-p.r.o.nged 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, Sat.u.r.day, 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 compa.s.sion. 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 NackTom 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.

They gave my mother a list of the medications I would now be taking: prednisone; Ativan, an antianxiety drug used to treat and prevent signs of catatonia; Geodon for psychosis; Trileptal for seizures; Labetalol for high blood pressure; Nexium to deal with the acid reflux caused by the steroids; and Colace for the constipation caused by the combination of all the drugs. Still, in the back of everyone's mind was that 4 percent mortality figure. Even with all of this, with all the proper intervention, people still died. Sure, they had a name for my illness and actions we could all take, but there was still a long uncertain journey ahead.

Stephen, Lindsey, and I filed into Allen's Subaru. When I had been admitted in early March, it was still winter; now it was springtime in New York. We drove back to Summit in silence. Allen turned on the radio, tuning it to a local lite FM station. Lindsey looked over at me to see if I recognized the song.

"Don't go breaking my heart," a man's voice started.

"I couldn't if I tried," a woman's voice returned.

This had been my go-to karaoke song in college in St. Louis. At this point, Lindsey doubted I would remember it.

I began bopping my head out of rhythm, my arms at rigid right angles. I swung my elbows front to back like I was awkwardly cross-country skiing. Was this one of my weird seizure-like moments, or was I dancing to an old favorite? Lindsey couldn't tell.

CHAPTER 33

HOMECOMING

My mom's house in Summit looked particularly striking that spring day, my homecoming. The front lawn was lush with fresh green gra.s.s, white azaleas, and the blooms of pinkish-purple rhododendrons and yellow daffodils. The sun beamed down on the aged oak trees that shaded the maroon door at the entranceway to the stone-front colonial. It was gorgeous, but no one could tell if I even noticed. I certainly don't remember it. I just stared ahead, making that constant chewing motion with my mouth as Allen swerved into the driveway of the place I had called home most of my young adult life.

The first thing that I wanted to do was take a real shower. There were still clumps of glue in my scalp that looked like pebble-sized pieces of dandruff, and I still had the metal staples from the surgery, so I could not be too vigorous with my was.h.i.+ng. My mother offered to help, but I refused, determined to do this small thing on my own, at last.

After a half hour, Lindsey headed upstairs to check on me. Through the opening in my bedroom door, she could see me sitting on the bed, freshly showered, with my legs flexed rigidly off the side, fidgeting with the zipper on my black hoodie. I was struggling to connect the zipper with the pull. Lindsey watched for a moment, unsure of what to do; she didn't want to embarra.s.s me by knocking on the door and offering aid, because she knew I didn't like to be babied. But when she saw me go limp, drop the zipper, and begin to sob out of frustration, she headed into the room. She sat down beside me and said, "Here, let me help," zipping up my hoodie in one fluid motion.

Later that evening, Stephen cooked a pasta dinner as a quiet celebration for my return. Allen and my mom left the house so that the three of us could have some alone time. My mother was so relieved that they finally had a name for what ailed me that she had truly convinced herself the worst was behind us.

After dinner, we sat outside on the back patio. Lindsey and Stephen made small talk while I stared ahead, as if I didn't hear them. But when they lit cigarettes, I got up without a word and walked inside.

"Is she okay?" Lindsey asked.

"Yes, I think she's just adjusting. We should give her a moment alone."

They are smoking together. Who knows what else they'll do together.

I grab the home phone. For some reason, I can't remember my mother's number, so I look it up in my cell phone. Ring, ring, ring, ring.

"You've reached Rhona Nack. Please leave a message after the beep." BEEP.

"Mom," I whisper. "He's going to leave me for her. Please come home. Please come home and stop them."

I pace around and watch him from the kitchen window that looks out on the patio. He catches my eye and waves. Why does he want to be with a sick girl? What is he doing here with me? I look at him waving, certain that I have lost him forever.

When my mom listened to the voice mail, she panicked: I was becoming psychotic again. Because Dr. Najjar was often difficult to reach, she dialed Dr. Arslan's private number, which he had given her the day before we left the hospital. She was worried that NYU had let me go home too soon.

"She's acting paranoid," she said. "She believes that her boyfriend is going to run off with her best friend."

This concerned Dr. Arslan. "I'm worried that she may be reentering a psychotic state. I would give her an extra dose of Ativan to calm her for the night and then check in with me tomorrow." In my case, though, the return to psychotic behavior was actually a sign of improvement, because the stages of recovery often occur in reverse order: I had pa.s.sed through psychosis before I got to catatonia, and now I had to pa.s.s through it again on my road back to normality.45 Dr. Arslan didn't forewarn us about the progression of the disease, because no one yet knew that people often slid back to psychosis. It would be only two years later, in 2011, when Dr. Dalmau released a paper with a section on that very subject, that the stages of the disease would become widely known. Dr. Arslan didn't forewarn us about the progression of the disease, because no one yet knew that people often slid back to psychosis. It would be only two years later, in 2011, when Dr. Dalmau released a paper with a section on that very subject, that the stages of the disease would become widely known.

Lindsey's weekend with me had come to a close. She and our friend Jeff (my karaoke partner in St. Louis), who happened to be in New York for an unrelated trip, were planning to drive the sixteen hours back to St. Louis together. When she called to give him directions, he said he'd like to see me. She warned him I wouldn't be the same.

Jeff rang the doorbell, and my mom invited him inside. He spotted me hovering beneath the staircase, slowly approaching the doorway. He first noticed my smile, a frozen, vacant, idiotic grin that frightened him. I held my arms out, slightly bent, as if pus.h.i.+ng my body against a door. Nervously, he smiled and asked, "How are you feeling?"

"Goooooood," I said, drawing out the syllables so much that the one word took several seconds. My lips hardly moved, but I maintained piercingly direct eye contact. He wondered if I was trying to communicate through my stare. It reminded him of a zombie movie.

"Are you happy to be home?"

"Yesssssssss," I said, drawing out the "s" like a strained hiss.

Jeff didn't know what to do next, so he leaned forward and embraced me, whispering in my ear, "Susannah, I want you to know that we're all here for you and thinking of you." I couldn't bend my arms to return the hug.

Lindsey, who stood behind us watching the scene, readied herself for the good-bye. She was not p.r.o.ne to histrionics and hardly ever cried. She had been so stoic throughout the visit, never once letting on how agonizing the stay had been for her, but she couldn't contain herself anymore.

She dropped her luggage on the floor and embraced me. Suddenly I was crying, too.

Lindsey left that morning not knowing if she would ever get her best friend back.

CHAPTER 34 CALIFORNIA DREAMIN'

On April 29, less than two weeks after leaving the hospital, I returned to New York University Medical Center for another week of plasma-exchange treatment. Because my symptoms were no longer considered epileptic but related to autoimmune encephalitis, I was placed on the seventeenth floor: neurology. Unlike the epilepsy unit, this floor in the old Tisch Hospital had not been redone. There were no flat screen televisions, everything seemed dingier, and the patients here seemed older, frailer, and somehow closer to death. A senile woman in a private room at the end of the hall spent her afternoons screaming "PIZZA!" over and over. When my dad asked why, the nurses explained that she loved Fridays, which were pizza days.