I was a happy 24-year-old suddenly stricken by
paranoia & seizures. Was I going crazy?
Teratoma: One Patient One Disease?
Hospital Medicine: Pride & World Class Costs!
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By
SUSANNAH CAHALAN
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Last
Updated: 6:01
AM, October 4, 2009
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Posted: 3:49 AM, October 4, 2009
IT was a cold March day as I walked to work
from my Hell's Kitchen studio. The weather was clear, people were out in their
coats and scarves, but something did not feel right. The sky was so blue, it
hurt my eyes. The billboards in Times Square
assaulted me with violent reds, yellows and purples. It was like the world had
become brighter, louder, more painful.
When I got to the Midtown newsroom of the New
York Post, where I was a year into my first full-time reporting job, I asked a
friend, "Have you ever not felt like yourself? Have you ever felt
completely off?"
"Sure," she laughed. "Of
course. I hardly ever feel like myself."
I laughed, too, but inside I worried. Lately,
there had been more and more days like this. I had started to feel numb on the
left side of my body. My arm and leg on that side tingled. Until recently, I
had been a healthy 24-year-old, happily starting a career in the Big Apple with
a new boyfriend. Now I spent sleepless hours wondering what was happening to my
body and my mind.
Then, one Friday at my desk, I cracked.
I had just finished an interview, was starting
to write, and for no reason I began crying hysterically. Co-workers gathered,
wondering what was wrong. Moments later, I brushed it all off, and skipped down
an office hall, giddy with excitement.
Later, at home, I panicked. Was I going mad?
Was I mentally ill?
I contacted a neurologist. He sent me to a
litany of tests, including two MRIs. Nothing.
"Maybe it's mono," he suggested.
"Or a virus."
He promised to follow up with more tests. In
the meantime, I curled up with my boyfriend to watch the PBS show "Spain -- On the
Road Again." I remember thinking how much I disliked Gwyneth Paltrow.
Then, he would tell me later, I started grinding my teeth, moaning and biting
my tongue, until I finally passed out.
It was my first seizure. And the last thing I
would remember for a month.
THROUGH interviews with family, my boyfriend
and friends, I'm able to cobble together the darkest moments of my life. My
father kept notes in a spiral notebook, details of what was happening that he
could pass along to my mom as they took shifts caring for me.
From my boyfriend, I know that I awoke after
the seizure at St. Luke's Hospital next to a puking man.
Doctors ordered a CAT scan that came up clean
-- but my behavior was becoming increasingly bizarre.
"I'm dying of melanoma," I told my
boyfriend. (I had been diagnosed with the skin cancer five years ago and was
convinced that was the cause of my seizure.) I started ranting about suing the
doctors and believed that they were out to get me.
But even after the seizure and the paranoid
delusions, the neurologist didn't seem worried.
"It's probably alcohol withdrawal,"
he told my stunned mother and me. He was convinced I was an alcoholic; I barely
drank.
My family packed up my clothes and took me to
my mother's home in New Jersey,
where I continued to deteriorate.
I stopped sleeping. Refused to eat. Paced the
halls. Couldn't control my rambling thoughts, and ranted to my mother. I convinced
myself that I was bipolar and that I was having a nervous breakdown.
Then I had a second seizure. Blood and foam
spurted out of my mouth onto the Oriental rug in my family room.
Overwhelmed by my erratic behavior, my mom
dropped me off at my dad's house in Brooklyn
Heights for one night
before my doctor's appointment. My paranoid delusions got worse. I refused to sleep
and started banging on the locked door, screaming, "Get me out of
here!" I imagined that my father had murdered his wife.
The next day, my mother, stepfather and
boyfriend took me back to the neurologist for an EEG, which records the
electrical activity of the brain. On the ride there, I opened up the car door
and tried to jump out. My boyfriend held me down and my stepfather child-locked
the car. I screamed hysterically until we got to the doctor.
Horrified by my increasing paranoia, and
despite my neurologist's hesitations, my mom and stepfather took me immediately
to the hospital. They demanded that I not be placed in a psych ward.
There I had my third grand mal seizure while
waiting for a hospital bed, and was whisked up to the epilepsy floor of New
York University's Medical Center.
For the first three days, I shared a room with
three other people, mostly suffering from epileptic seizures.
Technicians glued EEG wires to my head, which
snaked into a child's pink backpack on the side of my bed. I was monitored 24/7
by two video cameras mounted on the ceiling.
Two times, I successfully ripped the
electrodes off my head, tugged at my IV as blood erupted from my veins, and ran
shrieking down the halls, trying to find an exit. Nurses tackled me and stuck
me with a sedative. An imposing Jamaican nurse even sat watch with me for 24
hours so that I couldn't escape again.
I had to wear an orange wristband that said
"flight risk."
"The physical discomfort of not being
able to shower or wash her hair coupled with the fact that she was tethered
electronically to a monitor started to take its toll," my dad wrote in his
notebook. "She told me she was dying a little bit every day. I told her to
trust me and I would get her out, but we had to find out what was wrong with
her. She said, no -- get me out now."
It's hard for me to hear about the things that
I believed during my madness. When I turned on the TV, I imagined I was on the
news and that satellite trucks from all the major networks were camped out
outside of my hospital room waiting to interview me.
I imagined the woman beside me was
tape-recording my conversations and talking about me in Spanish to her family.
I was convinced that I could speak Spanish and interpret her words.
Multiple times a day, residents, doctors and
nurses would ask me: "What day is it? Where are you? What's your
name?" Though it would take me over a minute to answer, I did so
correctly, albeit a little pissed off.
One night a nurse came by to do my vitals and
ask the inane questions in the middle of the night. I had finally fallen asleep
and was less than pleased. As she bent down to take my blood pressure -- which
was spiking due to the illness -- I wound up and slapped her across her chest.
She gasped in disbelief and fear.
TWO weeks pass. Every day a new doctor comes
to visit. There are internists, infec tious-disease doctors, immune-system
specialists, psychiatrists, psychopharmacologists. They send me to MRIs,
sonograms, X-rays, CAT scans, PET scans, multitudes of blood tests. All come
back negative. No one could give my parents an answer.
My dad came in the mornings, sat with me and
fed me breakfast. My mom would come in the afternoons, and my boyfriend came in
the evenings and stayed with me until I fell asleep. They never missed a day.
Two comforts for me were apples -- which I
inexplicably craved, eating three a day -- and my boyfriend's iPod, on which he
made a mix of mostly Ryan Adams tunes, which helped me sleep through the night.
My father prayed. "I would walk across
town on 33rd Street
to catch the subway on Park Avenue
South. There is a chapel between 1st and 2nd Avenue that is
dedicated to Jesus and Mary. Each time I walked by I begged God to help
Susannah," he wrote. "I even tried to make a deal. I asked God to
take me right there on the spot or do anything he wanted with me if only
Susannah could be helped."
The paranoid delusions started to wane as the
medications, anti-anxiety drugs Geodon and Ativan, increased. But I was losing
the battle. I could no longer read. My tongue curled, making it hard to speak.
I had difficulty swallowing. It took me a whole minute to answer the simplest
questions.
Day after day passed with no answer. I became
more withdrawn. I found it hard to walk with my father and mother around the
hospital floor. Close friends were alarmed at my passive and incoherent state.
Doctors secretly feared that they were losing me.
The head doctors started speaking of a
mythical miracle man named Dr. Souhel Najjar. "He would find out what it
is," they would say. They even nicknamed him "Dr. House."
My mom requested that we meet this Najjar in
person. And after two spinal taps came back with high white blood cell counts
-- an abnormal occurrence that signifies brain swelling -- the case was
officially passed on to Najjar, a Syrian-born neurologist, neuro-pathologist
and epileptologist at NYU Medical Center.
When he came to speak with us, my family was
buoyed by his confidence.
He grasped my hands and said, "I'm going
to find out what this is and fix it."
Then he handed me a pencil and a piece of
paper.
"Draw a clock," he said.
I grasped the pencil and made a circle.
Feeling a little confused and put on the spot, I drew in the clock face.
My mother and father gasped.
All the numbers were written on the right side
of the clock face, and no numbers were on the left side.
NAJJAR now had five clues as to what was
overtaking me: the seizures, the catatonia, the high blood pressure, the high
white blood cell count in my spinal taps and the bizarre clock. It was
preliminary proof that the right side of my brain (which controls the left side
of the body) was inflamed. He believed it was some sort of autoimmune
encephalitis, or the swelling of the brain caused by an attack by rogue
antibodies.
He decided to send my blood and spinal fluid
to a well-respected neuro-oncologist from the University of Pennsylvania
named Dr. Josep Dalmau to test to see if rare antibodies were present.
But we had to wait two weeks for the results.
In the meantime, a brain biopsy would be
necessary, Najjar said. They would need to cut out a piece of my temporal lobe,
my horrified parents learned.
"I can remember being alone in her room
when she began to cry," my dad writes in his diary. "I went over to
her on the bed and hugged her when I began to cry. Next thing I knew, Susannah
was laughing. I asked why. She told me it was the first time she ever saw me
cry and I must have looked pathetic."
The next day, I was taken to ICU for surgery.
I remember opening my eyes as they sliced into my brain and saying something
like, "I'm awake."
The anesthesiologist looked at me and said,
"Oh, s- - -."
My mom thinks it was just another
hallucination. It seemed so real to me, but I couldn't trust my own mind.
After the surgery, the blood work and spinal
fluid came back positive for rare antibodies called anti-N-methyl-D-aspartic
acid receptor, or anti-NMDAR encephalitis. The name signifies that the
receptors in the frontal lobe, responsible for cognitive reasoning, and the
limbic system, or the emotional center of the brain, are under assault by the
immune system. My body was attacking my brain.
Penn's Dr. Dalmau had discovered these
antibodies in 2003. Until then, people suffering from my madness were
misdiagnosed, likely ending up in mental hospitals, if not dead. Experts aren't
sure what causes it, though they believe it's genetic, not environmental.
According to his studies, the median age for
the disease is 20. The youngest is 21 months. About 75 percent of those
affected by it are women. All show forms of psychotic behavior, some show signs
of catatonia. About 80 percent of patients have seizures and 70 percent of
patients see psychiatrists before any other doctors, according to Najjar.
Najjar estimates that nearly 90 percent of
those suffering from autoimmune encephalitis go undiagnosed.
"It's a death sentence when you're still
alive," Najjar told me. "Many are wasting away in a psych ward or a
nursing home."
I was the first person in NYU Medical
Center's history to be
diagnosed with NMDAR encephalitis.
Najjar wasted no time. He immediately hooked
me up to an IV and started the treatments.
First came the intravenous immune globulin,
which reduces inflammation of the brain. Then came high levels of steroids,
which also reduce inflammation. And finally he hooked me up to a plasmapheresis
machine, which flushes out the harmful antibodies from your system, through an IV
in your neck.
The initial treatment took about a week, until
I was well enough to be released from the hospital. Six months later, I'm still
taking steroids, but I'm back at work, back at home, seeing colors clearly and
not breaking down at my desk.
At a medical conference last week at NYU,
Najjar presented my case and the wide spectrum of autoimmune encephalitis
disorders, saying that I was back to "normal." A friend and co-worker
laughed. "You're better, but you're not normal," she said.
My father reflected on my time in the
hospital. "Najjar told me she could have easily ended up in a nursing home
for the rest of her life. Najjar thought she was extremely lucky. He was
saying, with time, she could get back 90 percent of her cognitive abilities,"
he wrote.
I'm happy to say that today I'm at 100
percent, and marveling at the lost month of my life, paging through my father's
diary like I'm reading about a stranger.
"All I knew was that she was alive, and
her spirit was intact," he wrote at the end, words that bring me to tears.
"We had more hospital stays for treatments, doctor visits, and lots of
medications to deal with, but my baby was on the way home."
Scientific America: https://www.scientificamerican.com/article/brain-on-fire-my-month-of/
