The
morning starts the way so many others have.
It’s pitch black and I am awakened to the sound of a phone ringing. “Dr. Doug, we need your help. There’s a baby seizing.”
The hour
is 4AM. The experience is not an
unfamiliar one; however, it has taken an odd flavor. No longer am I speaking to a seasoned
pediatrician with an arsenal of medications immediately at hand. Conspicuously absent is the EEG technician
who can tell me in real time exactly when a child is or is not having a
seizure. Instead, the interaction is
parsed down to its most fundamental elements: a patient and her team of medical
professionals facing the electrophysiologic demons fighting for possession of
her soul.
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| The wazungu (white people) volunteer group at FAME. |
The
mosquito net is thrown aside as I quickly find my way into scrubs. The hospital is a 5 minute brisk walk down a
pitch black dirt path, lit solely by my flashlight under the majestic canopy of
the Milky Way. As I walk, the treatment
algorithms that I had memorized run through my head. None of the medications that I would normally
use at each step would be available. It
was time to rethink the algorithm, to step outside the comfortable world of
child neurology to which I had been accustomed over the past few years.
It is not
long before the enemy is met. It has
taken the form of a 5 year old girl, whose brain, ravaged by fever, responded
by seizing continuously for hours on end.
Her body, 1/6 the size of an
adult, had already received more diazepam than an adult would before stopping
breathing. Despite this intense
sedation, the child was awake, her eyes eerily open but unseeing, searching the
right half of her world but seemingly never finding anything of interest. Every ten seconds, she would call out; not
the harsh scream of one in pain or the plainchant cry for her mother, but an
otherworldly yelp more expected from the mouth of an animal than from a human
child. She kept her left arm flush tight
against her body and her right extended outward, a pattern I had seen before: a
figure-of-four pattern, one commonly seen when one side of the brain is
furiously in the grips of ongoing seizure.
The
internal dialogue began. There was no
way to know for certain if the child was still seizing, but my instincts told
me she was. The mental algorithm at this
point was clear: she needed a dose of a medication intravenously that was
certain to stop her from breathing, but would stop her seizures. Then I would be left with a child, unable to
breathe on her own, and no machine available to breathe for her. Instead, I chose an equally perilous path: to
allow what might have been seizures to continue in order to keep her breathing.
Perhaps
in the real world, this would seem a common sense decision. However, it flies in the face of the
instincts that were developed in the American medical system over the
years. There, I am a brain specialist;
my decisions are devoted purely to what is in the best interest of the nervous
system, potentially at times at conflict of other bodily organs. However, here, I am the child’s doctor: any
potential risk introduced to the child will always be mine to bear, and any
problem I create I must reliably and immediately solve.
The
algorithm broken, I work with the team to make a plan for the night. Every complication is envisioned and an
escape plan diagrammed, even down to choosing the size of the endotracheal tube
should she stop breathing. Medications
are ordered and personally measured and delivered to the bedside, a process
that ordinarily is simplified to the typing of an order. The existential barriers of technology that
came between myself and the diseases I fought came crashing down, and the
experience became far more raw and personal than anything felt in an untainted
academic environment. Gone was the
sterile smell of rubbing alcohol and latex, replaced by the smell of the
evening campfire and urine.
The
fears of the evening staff were assuaged by 6AM, when I wandered back home; it
would be closer to 8 before I had finished assuaging my own. On the walk back to the hospital that
morning, I walked past a dead black mamba, one I must have passed at least once
in the dark just hours earlier. The
metaphorical significance of walking that close to death was lost to me, at
least for the moment.
 |
| This is not the snake I saw that morning. This is a cobra I saw while on safari in Lake Minyara. Alarmingly, the snake in this story is deadlier than this cobra. |
The next
day is quiet; her seizures calm, the fever abates. However, she remains fixed in that same
figure-of-four position, one that with the benefit of time I now recognize as a
sign that one half of her brain is injured, but not actively seizing. More curiously, she continues with those
animal-like sounds as her face irregularly twists and contorts into
lightning-like sardonic smiles.
At this
point, she is too complicated to be managed without the benefit of some aspects
of modern technology. The exam tells me
there’s something wrong with her head, and to answer what’s wrong, she needs a
spinal tap. However, in a cruel
catch-22, I need to know what’s wrong in her head before it’s safe to do a
spinal tap. This situation is easily
remedied in the United States by any type of head imaging. Unfortunately, this is not an available
option, as her periodic need for oxygen makes her impossible to transport to a
place able to image her head.
It is at
this point that this story takes a brief detour for a planned weekend Zanzibar
getaway. As this story is not about
Zanzibar, I will woefully inadequately describe it in one paragraph. Prior to going, it was described to me by a
colleague as, “a magical place where everyone says hakuna matata.” It is exactly that, and more. Predominantly now a mixture of African and
Arab peoples, Zanzibar was its own country for years until it was united with
Tanganika in the 1960’s to form Tanzania.
In its storied history, it was colonized on-and-off by numerous colonial
powers drawn to this tropical paradise: the Arabs, India, England, France,
Portugal, Germany, and even traces of the Far East. As a consequence, the island is a cultural
hodgepodge of many peoples of Europe, Africa, and Asia. Although predominantly Muslim, there are
active Christian, Jewish, Hindu, and Jain communities. The land is supportive of every kind of fruit
and spice, including multiple fruits I had never tasted before (my favorite was
the umbrella mango). As a consequence,
the streets smell sweet and savory. The
food combines all these elements: pineapple beef curry, roasted sesame roti
bread, cinnamon pilau rice, cardamom in mashed potatoes. Each night was spent by the pool, watching
locals play on the beach as the sun set over the Indian Ocean.
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| I also got to have coconut water. Unlike what they serve in the US, this doesn't taste like ass. |
Despite
being in such a wondrous place (and during Eid, the equivalent of the Muslim
Christmas), I was often glued to my phone, texting those still caring for this
little girl. She continued looking very
unhealthy during my brief siesta, and more inexplicably continued to need
oxygen. It made me wonder if the same disease that was causing her seizures was
also causing her to stop breathing.
There is such a beast, known as NMDA receptor encephalitis, most
recently popularized in the book Brain on Fire.
Unfortunately, there would be no way to test for this disease locally.
Still
obsessing over the problem, I remembered that a large number of girls with this
disease have a growth on their ovaries. So
I considered that instead of testing for the disease itself, I could test for a
fairly common cause of it. It was a very
circuitous way to test for the condition, but it was the only option available
to me. I ask a volunteer nurse
practitioner to investigate her ovaries.
The text back: “It’s definitely abnormal.”
The
possibility of this being an autoimmune rather than an infectious disease was
very exciting. An infectious process
would likely cause irreversible long-term damage to her brain. However, NMDA receptor encephalitis has a
large number of people making great recoveries (albeit after a very long
time). All we would have to do is remove
the mass on her ovary.
 |
| This is not that little girl. But I like to think she will grow up one day and be this healthy. |
Rarely
is one excited to return to work after finishing vacation, but such was the
case here. I went to see this little
girl; she continued to look awful, without a drop of tone to her body and
meaningless words spilling from her confused mind. But now there was a new plan: don’t target
the brain, target the ovary.
Over the
next several days, she made a remarkable recovery. She was ravenously eating, and able to walk
to and from the bathroom. Two weeks
after the start of her illness, I was preparing for her discharge. First, her roommate was sent home. As I finished giving her roommate’s discharge
instructions, I could hear her voice over my shoulder: “My turn?” Weeks ago, she was making animal noises,
seemingly possessed. Now, she was back
to a normal 5 year old.
She has
since gone on to a larger facility to remove the ovarian mass. I carry back to the United States with me a
sample of her cerebrospinal fluid: the key to proving her disease. In a few weeks’ time, we shall have a much
better idea of her future. But for right
now, she is an extremely satisfying success story.
