Myasthenia gravis (MG), effecting two in ten thousand people, is not among the diseases you’d want to be genetically predisposed to if you had to choose (yes, Lizz, if an evil emperor took over the world and forced you to make a choice). It gets classified as an autoimmune disorder, but let me take a side step to explain what that means.
In the States, we all have a basic understanding of the immune system – the army of good guys that fight the bad guys, which prevent us from becoming ill. As it turns out, it is actually a bit more complex than that. Although this is an obscene over-simplification, allow me to further breakdown the army into two parts – antibodies and macrophages. During human embryological development, by random trial and error, our bodies mass produce billions of unique combinations of antibodies, which latch on to complementary amino acid sequences and flag other parts of the immune system for help – namely macrophages. Macrophages kill bad guys by eating them, but they need antibodies to tip them off that something foreign ought to be digested. In a perfect world, antibodies would be specific only to non-self items, such as bacteria, parasites, etc. But it’s not a perfect world. Although during the developmental process these billions of newbie antibodies are tested against pieces of ourselves and are eliminated if reactive, it is impossible to test every new antibody against all forms of self-proteins that are floating around inside of us. And so occasionally one makes it through the screening process undetected, then later in life finds its perfect match, who unfortunately is not a foreign intruder but a natural part of the same body. You can imagine the epic battle that begins – antibodies get mass-produced and latch on to an endless supply of proteins, which are then eaten up by macrophages and friends. It is akin to suicide on a cellular and sub-cellular level. That is autoimmune disease in a nutshell.Now imagine if the troublesome antibody was specific to receptors for the neurotransmitter Acetyl Choline – the end point of neuromuscular joints. So what? Well, the brain sends signals directed to muscles through nerves, and nerves pass on that message to muscles through Acetyl Choline. So then, if you attack half of the receptors of Acetyl Choline, the muscles receive a watered down signal and are slow, weak and quickly fatiguing. The bad news is that the diaphragm is a muscle, and if it doesn’t move, you don’t breathe. This is the extreme form of myasthenia gravis, called myasthenic crisis. It describes Esperanza, a 27-year old, mother of two who rolled into the ER earlier this week.
“Can you talk?”
Slowly she shakes her head no.
“Can you write?”
She does not move.
Safadi puts a pen between her fingers and holds up a piece of paper. She starts moving her hand around in a practically illegible fashion: I have m-y-a-s
“MYASTHENIA GRAVIS! Do you have myasthenia gravis?”
She nods her head yes.
The puzzle pieces start coming together. This describes the huge scar she has down the midline of her chest, the aftermath of the removal of her thymus, an organ that pumps out components of our immune systems (a controversial strategy with the thought being that in select cases it is worth making a patient immunocompromised in order to decrease the likelihood that their body will produce Thymus cells essential for activation of B-cells that, in autoimmune patients, produce self-reactive antibodies).
Within an hour of arrival she was too weak to breath on her own and needed to be intubated, putting her at severity class 5 of an equal number of classes. Lacking mechanical ventilators, however, meant that Esperanza would be on the ambu around the clock for two days. The family was finally convinced that she would not get better on her own and came up with money they did not have in order to transfer her to a hospital with life-saving services, namely plasmaphoresis – a blood filtering process that removes autoimmune antibodies from the circulatory system. It is a short-term solution needed to buy time in emergency situations, as had become ours.
She needed a doctor for the trip, but we don’t have extra doctors. “Esperanza, my name is Benjamin. I am from the United States. I am here to help you. We are going to Quito together in an ambulance to get you more help. Is that okay?” She did not say anything, but reached her sloth-like hand out toward her mother who took it and assured her she’d be okay. After scurrying around for an hour, grabbing a stockpile of meds, putting in a secure IV and double checking what to do under plausible changes in her condition, I loaded her into the back of the Ambulance. As the door slid shut, Safadi shouted “Neostigmine every 2, Atropine every 3. Control her pupils and good luck. I’ve got my phone on me all night if you need anything.” Right, I thought, Neostigmine every 2, Atropine every 3 – unless her heart rate drops below sixty and she starts drooling at the mouth, then up the Atropine. If her pupils dilate open and become unresponsive to light, she’s oxygen deficient so she’d need more O2 out of the tank, and quicker, deeper respirations from the ambu.The only flashlight I’ve got for checking her pupils is the one my mom handed me off her keychain on my way out the door 2 months ago back in is-this-heaven Iowa.
“Here, take this,” she said, “you might need it.”
“Mom – I’m not a kid anymore. I know what I need and what I don’t.”
“Just take it”
Thanks Mom.
Turns out I did need it, because their aren’t flashlights in the hospital and there certainly aren’t flashlights in the Ambulance – a 15-passenger van with a gurney, an oxygen tank, and a rack of lights and sirens up top. No cabinets, no seatbelts, no drugs, gloves or monitors. No monitors means the only way to detect her oxygen saturation is by physical examination – shining my mom’s keychain flashlight in her eyes and watching the response.
And then suddenly we were warp speed to Quito. Her mother and sister sat up front with the driver, a John Candy figure called The Mafia who consumed the wheel and punished the roads. I was in back with my buddy Brendan, an inner-city high school English teacher, attending to the patient. As The Mafia took his chances swerving through narrow traffic gaps and peeling out around cutback corners on our way into the Andes, the two of us were five minutes on, five minutes off the ambu. We kept getting slammed against the side of the ambulance, or pulled the other way across Esperanza’s cart. I breathed for her with one hand on the bag – the other hand free to catch my fall, wipe my brow, or brace myself against vomiting. Every few off-turns I checked her vitals, peaked in her eyes, and prayed against a crisis within an emergency, then shut myself down and tried to rest up for the next go around.Her limp arm rolled off the cart and waddled around helplessly with each bump. I picked it up and put it in mine. “Espy, squeeze my hand.” I wanted to know if she could still hear me. Inside my warm grip, her cold fingers gave a little pinch. “Espy, if you need anything, squeeze my hand and I will try to figure out what it is you want”. An hour and a half later she started squeezing, but I could not make out what she needed. We did yes and no questions for a while - squeeze my hand if this is what you want – but her muscles soon became too fatigued to give consistent, reliable responses. So I put a blanket over her, hoping she was feeling cold and wanting warmth.
Before leaving Esmeraldas, she received her dose of the immunosuppresant Prednisone, but every two hours I snapped open a vial Neostigmine, drew out its contents and inserted the drug into her veins. Neostigmine blocks the action of Acetyl Cholinesterase (ACh’ase). In medicine, the suffix –ase denotes a degrading compound. In this case, ACh’ase degrades Acetyl Choline so that after a signal is transmitted from nerve to muscle, lingering Acetyl Choline particles do not latently fire off unintended muscle twitches. But if receptors for Acetyl Choline are blocked, then it makes sense to remove the degrading substance in order to keep more Acetyl Choline around longer in close proximity with potentially non-blocked receptors. Thus, pushing Neostigmine allowed her to maintain the ability for small muscle movements, which I depended on to tell me she was still okay.We made the 8-hour trip in about 4 and a half, and finally pulled into Espejo – the largest, newest, nicest public hospital in all of Ecuador. It was quite shocking, actually, after coming from our little three-story trashbin in Esmeraldas to look up and see a fancy mountain-sized shoebox of healthcare eating a large portion of the sky and thinking this would be where my patient would heal. It was floored with the same square tiles the US puts in its shopping malls, which I followed through the 100-bed ER back to a lookout point where the evening’s head doctor was working. “Here are the transfer papers from Esmeraldas for a patient with Myasthenia Gravis,” I said, delighted to have finally arrived at this moment. He took them without saying a word. Brendan was still bagging Esperanza in the back of the ambulance because we did not have a portable O2 tank to bring her in with, but he’d soon be relieved.
“We cannot take her.”
“What?”
“We cannot take her. She has Myesthenia Gravis; we don’t have the treatment here for her illness. You should have called ahead of time,” he said smugly.
“But sir, you have to take her. You and I both know, with or without treatment, she is better off here than she is in Esmeraldas. We don’t have anything back there, you know that. If you don’t take her, she’ll die,” I pleaded.
“Look, you don’t understand. This patient is gravely ill. She needs a lot – she’ll have to be intubated…”
“She’s already intubated,” I interjected. “She’s got an endotracheal tube in place and we’ve been five hours on ambu getting her here. I am tired. I cannot physically get her back. Please take her.”
He tried another route: “Listen, kid, we only have a certain number of beds in the intensive care unit; we don’t have room for her -”
I was pissed and quickly retorted, “Tell me straight, you don’t have the treatment or you don’t have the space, because you’re changing your story. If you don’t have the room, then at least let the family put her body on the floor; I’ll teach them how to use the ambu and they can bag her until space opens up. It won’t take any of your resources, just a corner in a room somewhere.”
“Get out of here!” he barked, “You hardly speak Spanish. Quit wasting my time”
You fucking piece of shit, I thought. You scum of the Earth son of a bitch. At that I paused and stood there for a couple of seconds, letting the air between us chill - then, in a low, steady voice, asked “How do you sleep at night?”
Silence.
“You have not even looked at her, and she’s Ecuadorian – she’s your sister, and yet you’re letting her go. How do you sleep at night?”
Still the uncomfortable coldness of two people not hearing each other remained.
“Sir, at least lend me a few vials of Neostigmine to get her to Guayaquil.”
He looked dismissively at me and shot back in a condescending voice, “You’re really asking for Neostigmine? You’re not in America anymore with your fancy hospitals and nice things. We don’t have Neostigmine – not in all of Ecuador.”
Unzipping the front pouch of my fanny pack, I held up my last vial of the drug in question. “If we have Neostigmine in Esmeraldas, you have it here.”
The family was blank-faced when I relayed the news. By the distraught, disgusted look on my face, whatever I said clearly wasn’t good. But they did not seem to know what to do with it, so I outlined the options. We can either go to the other public hospitals in Quito or take our chances and go to Guayaquil.
They looked at me blankly again. “So what are we doing?” they wanted to know.
“That’s your choice. We got her to Quito but the healthcare system here is not as we thought. From here forward I can give her care but I cannot make the choices. This is something the family needs to decide.”
We tried the other two public hospitals and got the same story – no treatment, no room. The other ones, at least, lent us the meds we needed to continue our journey on to Guayquil – an even longer stretch, with the front-end beginning at 4:30 AM on a sleepless night. But as I told the head physician at Espejo, there was no physical way for me to continue on – so my now blistered thumbs took the bag one last time, showed the family how fast and how deep to breath, then set my watch alarm to get me up in time to push her next set of meds.
After a string of unfulfilling sessions of unconsciousness, I roused myself and started in on a conversation with Esperanza’s mother. According to the literature, 5% of patients with MG have family members who are also affected.
“Does anyone else in the family have MG?”
“No,” came the simple reply, “just her older sister, but she already passed away.”
There was a sobering gap between that answer’s expected emotional content and that which she used. While thinking on the meaning of this family’s acceptance, another card was dealt into their hands – from the back popped the news:
“Doctor, it’s not making the noise anymore”.
“What’s not making a noise?”
“The oxygen tank – it used to make noise and now it’s not making noise. Is that okay?”
Sure enough we had run out of oxygen, but looking worried wasn’t going to help the situation, and since there were no alternatives I decided not to tell the family the seriousness of this new twist.
“Doctor, how is she?” they asked, rephrasing the question.
“Clearly it would be better to have oxygen, but under these circumstances – with MG and without O2, she is doing remarkably well.”
Upon arriving to Louis Espernasa Hospital, we rushed her out the back of the rig and into what could have been Chicago’s Union Station – organized chaos with people walking every which way. Calling for oxygen and pushing our way into deeper and deeper chambers, we were finally taken seriously and could not have been happier. Care was handed over with respect - and as quickly as it began, so this crazy journey ended. It was hot on the equator, so on the way home I cracked open the windows, stripped to my boxers and tethered myself into the stretcher - 34 hours and ready for bed.***
Intermission
***
Brendan is a great writer, and took the time to jot down his take on the same story for anyone to read:Expensive music always has a monster baseline, “Boom, Boom, Boom, Boom, Boom” that jumps your bones up and down. Pulsing just above the baseline is the clap line, “Clap, Clap…Clap, Clap, Clap…Clap…Clap, Clap, Clap…Clap, Clap…Clap…” off beat and chaotic in a way that frenzies up the juices of the soul and gets them jiving toward the heart. Finally, in this three-seated coaster of sound, the front seat is reserved for the voice line “Gracias por Dios, vive Jesus Cristo”, delivered poorly in tone but with the voice of a lioness rich in hope. Amplify this concoction of lines to the fifth power, and let your ears be punished for the sake of a musical cleansing.
Listening to this beautiful ear-beating did not cost me a dime, but nevertheless, it was costly. It was costly because of where it was being published; not at an outdoor stage or at one of Ecuador’s many Discotecas, but rather, from the inside of a rickety ambulance, tearing at 140mph through the western half of the country to its elevated/mountain dwelling capital city. And it was costly because it was sounding through the air behind the most expensive thing possible; a human life.
What was wrong with the girl? I couldn’t tell you. Its name was 20 letters long and not easy on the tongue. But I can tell you that it was a vampire, and its teeth put her into a critical state at 9pm on a humid Saturday night. And the hospital balked, put its hands up, and shrugged. Operating in a world of uneven vampire remedies, it did not have a shot, pill, drug, or machine that could cure the bite. So they did the next ‘logical’ thing. They wheeled her into an unfortunate mans hearse and sirened her off to Quito. To stroke the conscience, and comfort the family, they threw two doctors into the back of the van. Ben and me…
Let me explain what an Ambu is to a medical ignorant like myself. Basely, it is a piece of stretchy rubber that, when inflated, is about the size and shape of a rugby football (more round than an American football). On either side of the Ambu rests a small hole for a tube to be attached to. Hooked up to an oxygen tank it doubles as an angel and a demon. An angel, because when squeezed with force, fills the lungs with fresh, clean air, sustaining life. A demon, because when squeezed with force, fills the fingers with fresh callouses, the hand with clean muscle cramps, and the mind with shots of frustration.
And it was in this juxtaposition, between angels and demons, between hope and despair, sleep and alertness, that Ben and I found ourselves pumping prayers from our hearts to our forearms, through our fingers, and into the failing body of a mother of two…
More than once, as my body jolted back and forth, and my stomach ran in the gerbal cage, I looked at Ben with the knowledge of necessity. Ben needed to check her eyes, to check her pulse and feed her shots, to whisper confidence into her ear and hold her hand. I needed to squeeze the Ambu, to catch her saliva on my leg, to stroke her hair, to pray to God. Her mother needed her lioness voice, an offbeat clap, and soft eyes for which to see her daughter. And finally, our bus driver, in some backwards way needed to remind me of John Candy’s “Uncle Buck” and needed to drive like a drunk Ricky Bobby in “Talledega Nights”.
None of us, the five of us, had the luxury of choice. We, all of us, needed to perform, all the way to Quito…
So the Lord God said to the serpent,
“Because you have done this, cursed are you above all livestock
and all the wild animals! You will crawl on your belly
and you will eat dust all the days of your life.” -Genesis 3:14
And there were snakes in Quito. Born to operate with coiled grips and sharp lickers, they dealt out injustice, one hospital at a time.
“No you may not bring her here” hissed the first.
“We just don’t have the right treatment” slipped the second.
“Can’t sleep here” gumped the third.
And from the king of the pack:
“Sorry, no treatment.”
“But this is the best hospital in Quito.”
“Well, we just don’t have room.”
“So which is it, you don’t have treatment or you don’t have room?”
“You don’t speak good Spanish, you need to go.”
And the venom stings worse, when applied to the veins of a crying mother. Worse, when pumped into a fading oxygen tank. Worse, when tapped into the skin of a dying patient 10 stretcher lengths from help and a renewed life. And then the music in our van stopped, and silence entered with a deep breath, a bitter sigh, and the realization of what must be done next…
Guayaquil. 6 hours. 6 hours!? 6 hours. Another drive, this time an oxymoron. Cutting into the shaft of daylight, the beauty of the creviced Andes stood next to the ugliness of the previous hours, the light of our world against the darkness inside the ambulance. Hope pushed up against despair. A decision from where I now sat, in the front seat, cheek pressed up against the dashboard. Remain in darkness, ugliness, and despair. Or reach for beauty, light, and hope?
For the young girl’s family the decision was easy. From the backseat, rising above the familiar sound of the Ambu, came a hearty laugh, and then another. And then another. And then a constant. Somehow, a dying girl was funny. But maybe not. Maybe in that moment, the girl wasn’t dying at all. Perhaps for them, dying wasn’t even a consideration, never even a possibility. Maybe this was what holiness is; catching the giggles in the midst of the absurd…
How many times can one stare imminent disaster in the face, and avoid it by the margin of a paperclip? This was the question yet again as we hit the heavily trafficked streets of Guayaquil, this time with a driver holding bloodshot eyes and fidgety hands. He was a man possessed by the wheel, driven by the adrenaline of maximum speed. But if there ever was a need for such addiction, it was now. Three hours earlier, the oxygen tank touched empty, and the only air our girl was receiving was the dry, dirty air from the inside of the van. Each breath was a crossed finger, a minor miracle.
And so it was with great charm and little tact that we slammed the brakes and busted her through the doors of Louis Espernaza Hospital in Guayaquil. And it was with great conviction and little argument that the doctors received her. And it was with great speed and anxiety that we pulled out of the parking lot before they changed their minds. And it was with great relief that we looked back and saw her fading from our reach…
With finality in our hearts at last, we moved back up the coast, toward our final destination. Stretched out in the previously occupied gurney, behind the sounds of chatter from the front, I began to scribble a soft poem. I found no luck in this poem, partly because of the endless bumps and swerves that were still fancying our aforementioned madman. Rather than continue to labor, I instead closed my parchment and began to hum a new tune. My mind found the right pitch, and I let it play me through the night…
“T’was grace that brought me safe thus far, and grace will lead me home.”
