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When Occam’s Razor encounters a zebra: Another incredible survival story

I was awakened by Anne’s voice coming through over our private channel on our HAM radio located at the head of our bed.  Channel 146.455  She asked if I was going to come down to the ER?  I replied with a matter of fact, “nope.” I was more than half asleep.  Besides, who was going to watch our kids if she was already in the ER and it was the middle of the night?

I thought maybe she hadn’t thought through things being it was the middle of the night, and she was tired, but then she went on to tell me what was going on in the ER.  No, it was I who hadn’t thought things through.

She had a 41 year old female who was only satting around 70% on 15 liters of oxygen via a non-rebreather mask (she was not able to breathe well enough to provide oxygen to her body).  She wanted me to come down to help intubate her (place a tube in the trachea and connect her to a machine that would breathe for her).  She also said she would send someone to house-sit in the middle of the night while our children slept.

When I heard this, I came down to the ER promptly (as soon as I saw Dave Fields pulling up on his motorcycle to our house).  There was already a group of physicians and nurses present in the ER when I arrived, a clear indication that a “rapid-response” had already been called on her that I hadn’t heard.  The first question was what was going on?  The reason we had to ask was because we wanted to be convinced that her condition was reversible in our setting, and that by intubating her and connecting her to “life-support,” we would have a reasonable chance to help her, and not harm her.

Anne advised me that she thought the patient must be going into flash pulmonary edema (lungs suddenly fill up with fluid).  Her heart rate was 170 and her blood pressure was 270/170.  Yes, those numbers were double and triple checked, and they were real.  Her physical exam was contradictory to her vitals. She looked the way hypotensive people look: clammy, with very cold extremities, and the pulse ox wasn’t reading well on her finger. But, her blood pressure nevertheless was very high. Combined with an oxygen sat of 70% on 15 liters of oxygen and a respiratory rate of at least 40, she was an extremely unstable patient and on the verge of coding at any moment.

Her initial EKG

Did she have a pulmonary embolism (a large clot in the lungs)?  Did she have a stroke?  A heart attack?  Was she “just” in unstable supraventricular tachycardia, SVT, (a heart arrythmia where basically the heart is beating so fast it is not able to function correctly)?  If it was flash pulmonary edema, why?  What was causing all this?
It was the middle of the night, and outside of the EKG shown above, those vitals, and a physical exam, there was no further lab or x ray information available initially to make rapid critical decisions.

We decided to intubate her and connect her to the ventilator, at least until we could start to piece things together.  We sedated her further with a drip medicine.  I put an ultrasound on her heart and could see that it was beating so fast it appeared to be functioning like a ventricular fibrillation (an emergent rhythm that needs to be shocked).   It still didn’t make sense why her blood pressure was so high, but we went ahead and treated her for unstable supraventricular tachycardia.  We did a trial of adenosine twice.  Then we tried diltiazem.  Finally, we placed paddles on, and shocked her heart (synchronized cardioversion).  Twice.

Nothing happened.  Her heart continued at a rate in the 170’s.

Small pieces to the puzzle were starting to come together as she failed to respond to our treatments.  Her lungs were ‘wet’ and given the fact that her heart was beating so fast and her blood pressure was so high, it made sense that she would go into flash pulmonary edema.

The question was, why was she in this heart rhythm (SVT)?  We started treating the pulmonary edema with Lasix.  We then started to attempt to slow down her heart rate with beta blockers.

Her heart was extremely resistant, almost unaffected by our meds.  Her blood pressure remained unimproved and her pulse only slightly improved.  Only after we placed a nitroglycerin patch did her blood pressure start to drop.

Then as we started searching through her chart, reading through her history, it was noticed that up until about 7-10 years ago, she had had normal blood pressures.  However, over the past few years she had become a severely hypertensive patient.  It seemed odd.

We started talking about secondary causes of severe hypertension, and Anne mentioned “pheochromocytoma,” a rare type of tumor that arises from adrenal gland tissue and secretes hormones like epinephrine into the blood. (the “fight or flight” hormones)

I immediately agreed, and thought that had to be it.  I don’t normally so easily jump on board with diagnosing a rare disease before finding definitive evidence, but I did this time because I couldn’t think of anything that better explained what we were witnessing.  We immediately added terazosin, an alpha blocking blood pressure medicine that helps to offset the extreme vasoconstriction caused by adrenaline and other fight or flight hormones.

Anne at bedside running the vent

If it was a PE (a clot in her lungs) why was she so hypertensive?   If it was a stroke, why was she so tachycardic?  If it was “just” SVT, why was she so hypertensive, and why didn’t it seem to respond at all to any of our interventions? Something seemed to be “driving” both her hypertension and pulse in a way that was unusual, and unusually unresponsive to our typical treatments.  A known potential complication of a pheochromocytoma is a life-threatening cardiomyopathy-induced pulmonary edema.

The next morning, we received laboratory results.  They challenged our diagnosis.  Her amylase was 4,348.  This was significantly elevated above the normal upper limits of our lab (which is 200).  This level of elevation is very frequently associated with pancreatitis.

 

That didn’t make sense to me as she did not present like a typical pancreatitis patient.  Yet it was possible, as pancreatitis can cause massive fluid shifts within the body and lead to severe pulmonary edema.  Still, it didn’t seem to fit what we were seeing completely.  Do we trust our gut, or do we make decisions based on numbers?  We were at a crossroads in our decision-making with this new information.  Treatment of pancreatitis actually involves giving liters upon liters of IV fluids.  In pancreatitis, the body loses its ability to keep fluids within the blood vessels, and fluids leak outside, filling up the abdomen and even sometimes the lungs with water.  The water goes outside the vessels, but ironically this then puts the kidneys and even the heart at risk for severe dehydration.  In order to offset these losses, fluids are given in large quantities.

The problem with the treatment in this case, was that if we treated pancreatitis, our patient would die.  Her lungs were already full of fluids, if we gave her more, she would only fill up with fluids to the point we could not longer give her enough oxygen, and she would die.

It was a critical decision, should we be taking fluids out of her body with Lasix, or should we be giving her more fluids to treat pancreatitis?  They were opposite directions.  We had to choose one.

I put an ultrasound on her heart, near the liver in order to try and estimate if her intravascular volume was low or high (was she dehydrated or did she have too much fluids).  I was looking for more information to help make our decision.  I was observing the inferior vena cava near its entrance into the heart to see if it was collapsing between heartbeats, an indication that the CVP (Central venous pressure) was low and that the patient was dehydrated.  It was not.

An ultrasound picture similar to what I saw

But as I was looking with the ultrasound probe in the epigastrium (over the stomach), I immediately  noticed a large mass. Was it a pheochromocytoma or was it a pancreatic mass (like pancreatic cancer, which could elevate an amylase, and even cause pancreatitis)?  In one view I could see both the mass and the upper pole of the left kidney, which could indicate it was arising from the adrenal gland like a pheochromocytoma.  It was difficult to determine with my own inexperience as an ultrasonographer.

I wanted to ignore the amylase.  And in fact, we did.  We kept diuresing her in the face of the pancreatitis.  It was really our only choice, she would die if we did anything else.

It was such a tough call to make, but I wanted to know why I didn’t “believe” the amylase.  So I started researching other causes of elevated amylase.  (lists shown below just to demonstrate length)

Page 1 of potential causes of  elevated amylase

 

Page 2 of potential causes of elevated amylase


I read through the lists and when I got to the end, under “miscellaneous causes,” I found:

there it was at the bottom of the list on page 2 under misc causes

Of course, when I read this, it became reassuring that we were on the right course.  In fact, as we continued to treat her for a pheochromocytoma with cardiomyopathy induced pulmonary edema, (the opposite direction of pancreatitis treatment) she improved.  I continued my online research, and I started finding case reports.  You see, when you read about pheochromocytoma, there is no warning about “watch out for pancreatitis”.  Nor is it listed in our common medical sources as a known complication. But as I searched, I found case reports from other doctors who had seen the same (rare) complication with their own patients.

With this information in mind, we felt more confident that we were taking the right treatment course.

After 48 hours on the ventilator, we were able to take our patient off the ventilator and life-support.  We gradually weaned up her meds to 200mg of metoprolol three times daily and 20mg of terazosin daily.  (Max dose of both meds).   Her vitals finally stabilized with her pulse in the range of 100-110.

It wasn’t even another 48 hours and she was walking around our hospital, begging to be discharged.

She went home with orders to get a CT of the abdomen and a urine metanephrines in La Ceiba (1.5 hours by private vehicle from our hospital).  Here is her image:

CT demonstrating a retroperitoneal mass consistent with a pheochromocytoma

I actually wrote this blog about a month or so ago, but have not posted it until this moment as I have been waiting to get confirmation of our diagnosis prior to posting.  Its looking like we wont have 100% confidence in the diagnosis until the tumor is removed, with the surgery date being planned for the next couple of weeks.
What follows (which I wrote a month ago) has a very similar message to what was just written in a Cornerstone newsletter.  In it there is discussion of what is or qualifies as a miracle?  Thomas Aquinas argues for 3 types of miracles.  The author of the newsletter argues for a 4th type of miracle, one where odds alone cannot explain what we see in nature.

Was it a miracle that she survived?

I think what we see can be explained by natural means (not a miracle by Thomas Aquinas first three categories) but falls nicely into what the author of the newsletter labeled a fourth kind, a kind we are commonly seeing at Loma de Luz.  The chances of the patient’s survival were so low, that the fact that she did survive points to more than just natural occurrence, or something that natural odds can possibly explain.

A couple years ago I read a book, Not a chance: God, science, and the revolt against reasonby RC Sproul.  In it, Sproul talks about how people often talk about things that happen “by chance”.  He points out, as many other philosophers have as well (he gives an overview of their views), that chance itself is not a force that can cause something to happen.  Statistics cannot move a chair.  The point I would make by adding this is that whether you add this type of “miracle” to the list given by Thomas Aquinas or not, this extremely improbable occurrence begs the question , “What caused this?”  Even if we concluded this was not a miracle, one cannot escape the challenge that the event poses on the reasoning mind.

Chance alone does not explain her survival. Reason (not just faith) leads me to believe it was the work of the Lord as I personally find there is no better explanation.  Thus I would say, miracle or no miracle, we are still left with the same conclusion.  Dios obra aqui.  Who then did save her?  It was He (there is none other), the ruler and maker of the heavens and the earth.

“I, at any rate, am convinced that He is not playing at dice…”
-Albert Einstein

 

This is my Father’s world.
O let me ne’er forget
that though the wrong seems oft so strong,
God is the ruler yet.
This is my Father’s world:
why should my heart be sad?
The Lord is King; let the heavens ring!
God reigns; let the earth be glad!

****Please do keep her in your prayers this month.  Although she has survived incredible odds to return home the past few months, the surgery to remove her tumor will and can be a very challenging surgery.  Entire books are written just on the anesthesia for this specific type of tumor removal.  We continue to put our faith in the fact that He who has brought us this far will take us to the end.  Yet, His purposes are far above our knowledge or understanding. Of course not everything goes as we plan.  Lord, for the sake of Your great name, and Your great mercy’s sake, help us.****

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