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FBE University 2020 (D)

Mothballs, Crowbars, and Blue People in Kentucky

In 1960, Madison Cawein III was a freshly graduated Hematologist. He was displaced from the hallways and coffee shops of Lexington into the rural hills of moonshine country. He was no longer a doctor in a lab or clinic, he was an investigator chasing a legend. Through small talk amongst his colleagues over meals or in casual get togethers he had heard rumors of a unique family living in Eastern Kentucky. This family dwelling in the hills were your average rural family with one definitive exception; they all had dark blue skin. 


I was caught off guard to say the least. What little fluid drained into the bag at the end of the catheter was a dark and rusty brown color.

What had struck me immediately as I first encountered my patient was how cyanotic she was. Her nose, lips, ears and hands were tinged deep purple. Her work of breathing was increased, her shoulders heaving and the muscles around her clavicles tugged with every struggling breath. The smell of vomit lingered in the air and a small quantity of green bile lined the porcelain in the toilet. It had started as a headache, then nausea, then vomiting and abdominal pain. Though she had not noticed, her urine output plummeted. Dehydration, and possibly something more sinister was slowing her kidney function. She called 911 when she couldn’t breath. Now, around 24 hours after her first headache, she was lying on a hospital bed in a rural emergency department. 

A ventilator hissed in the corner and an IV pump with two bags of fluid or medication dripped regularly into a green capped catheter in her left arm. The nurse had placed a foley and I had watched the dark fluid run slowly into a foley bag. Labs had been drawn and sent to the lab. No other crews were out on calls so we spent some time in the ED watching and assisting in her care. My curiosity was there and I wanted to know what had driven a healthy 30 year old woman to a ventilator in 24 hours. She had no medical history. Her husband, quiet but visibly scared gave an uneventful history of her health that aside from some minor dental surgery was completely empty. 

The doctor walked in moments later. He scratched his forehead and looked at the sedated patient.

“She has methemoglobinemia”. He said looking back down at his pen and paper. 

“Oh okay cool”, I responded. ”whats methemoglobinemia?”

Truthfully, I knew that methemoglobinemia was a disease. I had heard a few rare stories regarding pediatric cases and knew it was related to teething medicine. And…well yeah that’s it. So coffee in hand and computer open I ventured forward…and called Tyler to ask him what methemoglobinemia is. 

Methemoglobinemia is the condition of having an abnormal percentage of methemoglobin in your total hemoglobin. Why does this matter? Hemoglobin carries oxygen, methemoglobin does not. 

Picture yourself standing in line at a lazy river. As people exit the tubes upstream, the line files into the river as empty float tubes pass by and are snatched up by the people in line. Oxygen binds in a similar fashion to hemoglobin. The opposite charges of the oxygen molecule and the iron molecule attract each other and bind together. Now you have oxyhemoglobin. Your oxygen is on its way to the bodies tissues. 

Illustration by Austin Quillet @QuilletAustin

But now imagine the lifeguard steps into the river and begins placing fence posts into the seats of some of tubes. Now no one can sit in those tubes. So they float unoccupied for another cycle. Meanwhile, people who already have tubes are scared to give there tube up in case they can’t get another one the next go around.  Methemoglobin, its iron heme blocked by two hydrogen ions, cannot host an oxygen molecule, reducing its carrying capacity and causing the remaining 3 hemes to aggressively protect their oxygen binding. 

Methemoglobin occurs naturally in the blood stream. Between 1-2% of hemoglobin at any time. Thats not a big deal! You’re still working with over 90% of your hemoglobin at full carrying capacity. But what happens when more and more hemoglobin begins to take on hydrogen ions and convert to methemoglobin? Your oxygen carrying capacity begins to fall…

When Dr. Cawein arrived in Hazard, Kentucky his saving grace was Ruth Pendergrass. She not only had heard the rumors, but had confirmed them herself. She was a nurse at a small clinic and had treated a woman with blue skin.  What stood out to Ruth was that despite her profoundly cyanotic appearance she had no symptoms of illness or oxygen desaturation. She had only come to the clinic for a routine check. Dr. Cawein had a suspicion that these individuals were suffering from methemoglobinemia. But without bloodwork it couldn’t be confirmed. He needed to find a patient. 

Why did my patient have methemoglobinemia? She was a healthy woman in her thirties who took no medication and seemed to live an active lifestyle. 

I don’t know. Writing this article today, I still don’t. I tried to follow up with the hospital system. I know she lived but I don’t know why she got sick in the first place. But there are a few reasons why people develop this rare disease. 

Medication reaction is the most common. Pain medications utilizing topical anesthetic (benzocaine, lidocaine etc) are a risk factor and are the most common cause in the pediatric population. Certain antibiotics, nitrate containing medications and even Reglan have caused it in adult populations. Naphtalene poisoning from mothball ingestion has been a cause multiple times in suicide attempts and accidental ingestion.  The exact pathophysiology behind this is complex. The point being that these medications and causes although rare have caused incidences of methemoglobinemia. 

The second cause…genetics. 

Madison and Ruth had no luck in locating an individual willing to talk about their blue skin. They made treks into the local community but were either ignored or denied a conversation, no less a full assessment. Stunningly though, a husband and wife presented to the clinic for a routine check up. They were blue, very blue. This middle aged couple ended up being the key. They were open to assessment and research and honest when answering questions although skeptical that a medication called methylene blue would make them turn pink. Although not dangerously inbred, there was some distant relations in that valley between the families that inhabited it. Both sides carried a recessive gene that predisposed this family to methemoglobinemia. Due to the chronic nature of this, they were naturally polycythemic and compensated for the higher levels of the altered hemoglobin. Over time, with occasional treatment with methylene blue, the blue people of Kentucky became a thing of the past. 

I wrote this because I found this entire history fascinating. But I was also highly intrigued by my patient and the disease process. Sometimes there is no initial reason why they develop it…that we can ascertain. These patients need oxygen support. Its basic, but unless you have methylene blue on your ambulance that is your best place to start. Get them to a facility that has access to methylene blue. If the cause is environmental remove the environmental and toxic cause. Simple stuff, and rarely used…maybe it will save a patient’s life. 




That one time I got kicked out of my own ambulance...

My boots tread softly on the hardwood floor as I weave myself, monitor in hand through the home - first responders scramble to move furniture and other objects blocking the path. Assuming they are expediting this process due to the condition of the patient, I press forward with a little more urgency. I laugh internally, reflecting that it seems the sickest patients are always the furthest away from the doorway.

Only after this call would I learn of an expression from another crewmember on my shift. Paramedics should be like ducks. Cool, calm and above the water, but paddling like hell underneath. Did I do that? I’ll let you be the judge of that by the end of this.

Knowing when to treat on scene, and when to facilitate rapid transport has been the subject of dogmatic education for years within EMS. Throughout my time as a student I anecdotally reflect on a basic principle deeply instilled. Load and go? Stay and play? Patients were assigned to one of two categories based on presentation. The sicker the patient the faster transport should be expedited. Definitive care would be at the hospital, your job was to stabilize en-route.

I rounded the corner and saw an elderly female, lying in bed flat on her back. That position is almost never beneficial. I could see her breathing hard and she was sweaty and pale. An aerosol inhaler laid on the nightstand and a nasal cannula hissed from a D tank propped on the floor. A pulse oximeter showed a 92% saturation with a heart rate of 120.

“Hey ma’am, I’m Jay and this is my partner, Andrew, what’s wrong today?”
She tugs a deep breath and exhales her words rapidly

“I’m...Rosa...I can’t,'' she inhales sharply, “breath”. Exhaling out of a pursed mouth.
I sit her up. The first corrective action I can take for someone experiencing breathing difficulty.

“Has this happened before?” I ask as I take a stethoscope from across my shoulders and prepare to listen.
“Yes she has COPD, she doesn't take anything for it” Says her husband out of the corner of the room.

She nods her head. “I felt dizzy earlier in the afternoon and weak, it just got worse and worse,” she inhales aggressively, collecting air she lost during the sentence.
I press a stethoscope to her chest. I think I hear rales.
I ask Andrew to take a listen. He hears wheezing.

I do a 12 lead. Nothing significant stands out.
“I'm going to get the truck around and get the stair chair out so we can get to the truck” says Andrew walking out of the room.
I compare the vitals I am assessing to the vitals taken by first responders.
She's breathing fast, and she's breathing hard.

What are some problems you might face in a patient who is quickly headed to respiratory arrest? Picture yourself on the front seat of a roller coaster with a major drop ahead. The car carefully slides over the edge of the drop and you peer down to ground and the track below. As the full weight of the car slides over the edge, momentum and gravity carry the car with increasing velocity to the bottom.

These patients are essentially on a roller coaster, from the first sign of acute distress (peering over the edge at the ground), to respiratory arrest (the bottom of the hill, this is the fastest the car will reach). Throughout the drop the car will increase in speed towards the bottom. The closer they get, the faster they get. Why do I illustrate it like this? Because these patients will be harder to treat the closer they get to respiratory arrest, that's when the momentum is greatest.

“I’ll get you a blanket the minute we get in the truck,'' I tell my patient consolingly as she is lamenting being immensely cold. It is brisk and windy outside, but I am almost always too warm and I am looking forward to shedding my job shirt.

A brief history; I feel that my partner and I communicate well for how long we have worked together. We haven’t had any issues yet and have been working very well. But, I can’t recall us having had what I would consider a critical patient together...yet.

In the truck I throw a blanket over her and listen again to her lung sounds. I was wrong the first time and I audibly confirm wheezing as I reach for a nebulizer mask. My partner opens the IV kit and explores her left arm until the patient jerks her arm away. She manages over the distressed respirations to explain she had a left sided mastectomy and cannot have an IV on that side.

“This is albuterol and another medicine to help you breath just wear this mask for me ok?” I explain, putting on the neb mask as my partner tosses me the IV kit. I see a vein. I try. I blow it.
I go to reattach the tourniquet higher up and assess for an AC access point when I see my partner stand up.

“I’m going to go ahead and start heading down the mountain, speak up if you need anything”. The side door shuts, and I am alone with my patient. She has 4 leads, a pulse oximeter, and a roaring nebulizer mask on her face. I sit her up as the truck pushes into drive and bumps down the mountain.

Did I mention mountain? Running emergency traffic we clocked in at a 32 minute response time from our assigned base. Because of this, we have aggressive protocols and, for the most part, competent and confident providers. You have to be, you’ll be there for the long haul.

There were a few more actions I had wanted to take before I was by myself. Nasal capnography and definitive IV access being the primary. I had her positioned, I had her on a neb and I was able to monitor a baseline set of vitals. But I wanted more.

“What interventions do I have left?” I think quietly inside my head. I glance at her pulse oximeter. 90%.

My patient is white knuckling the stretcher rails and leaning forward and I hear audible wheezes.
So what interventions did I have left?

COPD can involve some complex management. These patients' airways are fiercely inflamed and the already narrowed openings are clogged with a thick mucous. Nitrogen molecules, big and sluggish, cramp closer and closer as they pass lower and lower into the alveoli. Nitrogens size and tension is responsible for keeping the alveoli open and low pressure which allows oxygen to diffuse into the bloodstream. As nitrogen becomes trapped in the narrowed upper airways those alveoli lose tension and slam shut. You’ve just experienced atelectasis, if you want these alveoli back, you have to recruit them.

Albuterol was my first pharmacological intervention. We know what it does. It's a bronchodilator. Those nebulized molecules hopefully pass into the airways and reduce the swelling. Allowing oxygen and nitrogen to pass through to the alveoli.

I also initiated ipratropium bromide, an anticholinergic. It’s there to reduce the mucus in the airway, again increasing the diameter of empty space and reducing resistance in the lower airway so that oxygen and nitrogen can pass through.

I haven’t initiated a steroid, I’ve got magnesium sulfate, I don’t have an IV, I should work on that.
I have CPAP and, if push comes to shove, I have a host of sedatives, paralytics and a laryngoscope.

My patient sits up suddenly.

“I can’t breath you’ve got to help I can’t breath” I glance at her pulse oximeter. 94%.

One of your biggest factors in managing a COPD patient is how stubborn they are.
We all tend to see these patients halfway down the roller coaster. They’re already closing in on terminal velocity. Why? They wait. They stay at home, they tough it out. Until they start getting tired...and hypoxic.

While you were sitting at your station or running a host of other calls. They were at home, beginning the first stages of respiratory distress. They start to trap air. COPD in all its shape and forms is often as much if not more of an expiratory issue as an inspiratory one. They can’t offload carbon dioxide, it becomes trapped in

the lower airway, blocked by the stalled nitrogen. It’s like getting off a train into a crowded station. The stairwell is packed and you can’t leave. As carbon dioxide begins to trap in the lower airway, the equilibrium of oxygen and carbon dioxide becomes offset. There is less oxygen to pull from the alveoli, and there are too many carbon dioxide molecules for the body to justify offloading them at the capillary bed. So instead, they stay in the blood plasma. They saw how crowded the station was, so they remained on the train.

As they begin to notice the ever more present carbon dioxide molecules and reduction in oxygen uptake. They will start to modify their breathing. Unfortunately, it's not always an effective modification and can increase the dead space they have to overcome. How?
The tachypnea you see is not effective in reaching and recruiting alveoli. They trade tidal volume for respiratory rate, at the sake of adequate alveolar ventilation.

As they breath in rapidly and exhale poorly, this leaves more and more nitrogen in the middle, carbon dioxide in the alveoli, oxygen is nowhere to be found. The station is becoming more and more crowded as an anxious public ups the pace to the station before it shuts down entirely.

I’m sweating. I had turned up the vents and lowered the fan moments ago to increase the comfort of my patient. But I doubt she is paying any attention at this moment. She still has the rails locked in her fingers. The only time they unclasp is to fidget with the CPAP mask. I escalated my treatment. Initially, she tolerated the mask, but this was short lived and now she is fighting it.

I sit down, I focus on calming my voice and I do my best to look at her in a way that exudes confidence and care. “Ma’am I know it's uncomfortable but I need you to keep this on, it isn't suffocating you and you are doing well with it. It’s helping you breath.”

She nods affirmatively, and I cross my fingers that she will follow my direction. I return to my original goal. Retrofitting a T handle nebulizer onto a CPAP. I squeeze a vial of albuterol into the basin as I hear the hiss of air and the slam of plastic on plastic.

“You gotta help me breath you gotta help”

The mask of the CPAP screams against the stryker as I return to the code seat.
In my head I am screaming at her to put the mask back on. I calmly examine the PEEP. It’s as low as it will go. 

“Would it help if you held the mask to your own face?”

I place the hissing face mask in her hand and guide it up slowly. I allow her to press and show her how to make a full seal. Slam! She throws it down and begins unbuckling the belt of the stretcher.

Often, as these patients progress, your biggest war will be with their anxiety. With lungs packed with nitrogen and carbon dioxide. And a steadily decreasing oxygen saturation. They will feel as if they are being smothered. The alveoli, dry and under immense pressure collapse without surfactant and nitrogen.

Their brain goes into survival mode.

If you have ever truly choked. You know the fear of knowing your airway is blocked. The fear is obvious. Their brain is screaming at them to get oxygen but they can’t. As hypoxia increases this only further reduces their mental faculties and increases their adrenaline. Fight or flight is in play.

Versed anybody?

I glanced at my phone, but I don't have any signal. If I could get orders to administer Versed, or maybe Ketamine, I could reduce this patient's anxiety and help increase their tolerance of CPAP. I need orders, but I can’t get them.

Well... I could have called; a VIPER radio to the hospital hung on the cargo webbing of the seat. But it never occured to me. I too, was beginning to experience significant anxiety. I don’t have access. I could do IM, but that would take longer than I thought the patient had. IN was an option, but I wasn’t a fan of the efficacy.

I needed access, and I needed to get her oxygen. Her blood pressure was still up and holding. But her oxygen saturation was, ever so slowly, going down. She was nearing the bottom of the drop. We had two options and I disliked them both.

“Andrew, pull over and hop back here for a second”.

Now, at this point my partner stepped into the back of the truck and saw a few things.
Namely, a disconnected CPAP on the bench, a broken down T handle nebulizer, an empty neb mask, and a patient on a non-rebreather tugging away at fourty short and shallow breaths a minute. I saw them step up into the back and observe the scene in front of them.

I looked at them and simply said “would you get me an IV?”

“Just drive me to the hospital”

I looked up as they sat down into the captain seat, looking at the ipad, empty of patient information.

“You’re kicking me out of the truck?”

“Just drive me to the hospital, we need to be there now.”

“She needs an IV and medication we need to stabilize her”

My partner flashed me a look that fully declared the conversation over. I was in no short terms fuming. I was angry at my partner for taking over in such a blunt way. But I was also hurt. Stung by the implications that my partner should handle this trip. That it had grown too far out of my confidence range. I hopped in the driver's seat, got my bearings and sped off to the hospital.

What did I do wrong? Well, alot. My treatment plan was there. In my head, it was perfect. What my partner saw and heard was much different. My execution sucked. They saw a critical patient, an ambulance strewn with CPAP and nebulizer equipment. They heard me ask for an IV without any context as to why. They saw a flustered and incompetent provider mumbling around a crashing patient with no obvious treatment plan. They made an executive decision to take over the call and attempt to mitigate disaster.

How would I have mitigated this and maintained control had I been able to go back in time? Communication is key.

When my partner got in the drivers seat, I was left outside of where I wanted to be. In an ideal world, I would have communicated that.

“Would you mind helping me get her on nasal cap while I get this IV?”

Low impact, easy, quick. I would have started that call with the footing I wanted.
Had I forsaken that I could have still redeemed myself when they stepped into the back of the ambulance.

“Hey, she is immensely anxious. I can’t get her to keep the CPAP on or sit still long enough to get an IV. I would like to sedate her slightly. Would you mind trying for an IV while I draw up medication. I am worried she is going to arrest before we get to the hospital.”
This conveys confidence and clarity. I explained what had been going on, where we were now, and why I wanted what I wanted.

Be confident and clear when you communicate with your partner. Have a game plan when you need them and clearly express your treatment path. We had not been together long enough for the measure of trust and respect that allows for silent calls to occur yet. I needed to express myself confident and clear and show that I had a game plan. What if my clear, concise language and communication still did not convey enough confidence for them to follow along? The cpap on the floor and the ever sickening patient was still plenty of evidence that I was out of my element to them.

No one can tell you how to handle that kind of situation. But there are a few factors to consider. Am I prioritizing pride, or patient care?

If you see a medic about to administer the wrong medication or dose. To not speak up would leave you accountable for what follows. But would my standing down and allowing my partner full control change the outcome?

When we arrived at the hospital, shortly after we rolled into the ED room. She went into respiratory arrest. She was placed on a ventilator and stayed there for around a week before discharge from the ICU. No harm no foul?

Would my plan to sedate and continue CPAP have changed the course of the call?
I have no idea. My partner was able to convince her to try the mask on again.
She was able to tolerate the CPAP a little longer this time and experienced some improvement before ultimately removing it. During that time of relative calm my partner achieved an IV.

She was on a non-rebreather and her blood pressure was dropping when we entered the ED.
Had she been given a sedative and tolerated CPAP it's highly possible intubation could have been avoided. We could have utilized ketamine and paralytics for intubation had that failed. Hopefully avoiding a long term hypoxic event like respiratory arrest and improving a then unknown outcome. What is the point in all this writing?

I’d like to think the person who reads this will be able to foresee a similar situation should they be in my shoes and execute a plan far better than I did. Communicate what you want from your partner. Do it kindly, calmly and confident.

If you are uncomfortable, speak up. If you have a game plan, explain it concisely and assign clear roles. If your partner isn’t making an obvious deadly mistake. Use your judgement in determining what is more important and what you are trying to protect. Your pride? Or your patient?

Get aggressive with these patients early. By the time you arrive they’re halfway down the drop. You have to put a lot on the table and quickly if you don’t want them tiring and arresting. CPAP quickly before they become anxious and hypoxic. Don’t be afraid to utilize sedation to manage hypoxic anxiety. And have a back up plan.

Have a plan that prioritizes your patient, and communicate that plan early and clear. Speak up!
And execute your plan better than I executed mine.
Thanks for reading.


(Patient and partner information have been changed to maintain discretion and HIPAA compliance.)



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