Category: Adrenal insufficiency

MCAS, AI, and the signed emergency protocol

Stethoscope, pen and paper

I didn’t immediately go to an endocrinologist, because I thought I could manage secondary adrenal insufficiency on my own. After all, I’d conquered living with mast cell activation syndrome (MCAS), arguably one of the most complex health conditions in existence.

I assumed that because secondary adrenal insufficiency has been studied since the 1950s and could be treated with one daily medication, it would be easier to manage than MCAS. I knew low cortisol could be life-threatening, but surely, it was better than drowning in hundreds of mast cell chemicals, right?

In December 2021, I passed out five hours after a skin cancer biopsy. Retrospectively, I believe the epinephrine in the lidocaine shots tanked my cortisol in addition to the bleeding and stress. I felt nauseous, confused, and overwhelmingly sad. My head also hurt, but I had no rashes or flushing, so I knew my mast cells were likely not to blame. (MCAS and AI symptoms are SO similar!) I took prednisone and waited an hour for it to kick in, but I was still getting worse. When I began vomiting, I knew I needed medical help.

I told the emergency room nurse at the check-in desk that I had adrenal insufficiency and my cortisol was low. I assumed I would be taken to a room immediately for IV steroids. Instead, she told me to take a seat as if I was just constipated. 

As the room faded in and out, I wobbled back to the desk and begged for an IV. “My cortisol is really low,” I said. “I’m going to pass out.”

*crickets*

“I need steroids.”

*crickets*

“I am going to go into adrenal crisis if I don’t get medication soon.”

The nurse finally looked at me, “Oh, you have Addison’s? Get her a room.”

Was this a medical facility or a speakeasy? Apparently, “Addison’s” was the passcode to treatment, even though it’s a more rare form of adrenal insufficiency that I don’t have. Before MCAS gained awareness, I often had to refer to mastocytosis, a rare type of mast cell disease that I also didn’t have, for doctors to feel more comfortable treating me.

Same shit, different health condition.

“I need a signed emergency protocol,” I told my endocrinologist, who wasn’t the least surprised by the emergency room’s ignorance. 

At first, my endocrinologist handed me an unsigned note. I explained it would be dismissed without a signature. Most emergency room doctors care more about protecting themselves from liability than following peer-reviewed treatment protocols or notes in medical charts.

In 2018, I was hospitalized when a doctor refused my mast cell treatment because I presented her with a MCAS protocol that wasn’t signed. Instead, she attempted to administer morphine, which my protocol clearly indicated I was deathly allergic to. I reported the trauma to patient relations and the Board of Medical Practice, and of course, I got my protocol signed. Now every time a medical professional opens my chart, a threatening pop-up that requires them to read and acknowledge my signed MCAS protocol. 

… which is awesome… until you go into MCAS remission and suddenly your life depends on a different piece of paper.

I thought MCAS remission would entail healing from trauma, not experiencing new horrors. For me, adrenal insufficiency is scarier than MCAS because crises are harder to recognize and can escalate more quickly. At least with MCAS, I always had an EpiPen as backup. There is no auto-injector for adrenal insufficiency! On top of that, epinephrine works fast, but steroids take time to kick in. Adrenal crisis can accelerate into a full body shutdown if the steroids are too little, too late. 

In the end, I got my new permission slip to receive medical treatment signed and added to my medical chart, but I sure as hell wasn’t going to rely on the ER. I vowed to myself that I would do everything to avoid an adrenal crisis. And so began my Boundary Era.

Tips for emergency protocols

  • Customize your protocol to fit your body’s needs. If your doctor signs off on a protocol that your body doesn’t tolerate, your protocol could make your situation worse.
  • Carry physical copies of your protocol, especially when traveling. Doctors outside of the patients’ health system often ask to keep a copy.
  • Save a photo or copy of the signed protocol on your phone.
  • Keep your protocol up to date. Ideally, have your doctor sign a new copy annually (even if nothing has changed) to demonstrate your protocol is current.

Update: In June 2024, I successfully tapered off prednisone and regained function of my adrenal glands and HPA axis. However, I still must monitor for symptoms of low cortisol. 

Related posts

My adrenals didn’t RSVP to my MCAS remission party

Interested in re-reading my remission journey? Read the posts.

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My adrenals didn’t RSVP to my MCAS remission party

Keeya and others in a Zumba class on the pier

When my MCAS symptoms went into remission in April 2021, everyone wanted to know how long it’d last. After years of trialing medications, suddenly fragrances no longer bothered me, I could eat anything I wanted, my joints stopped subluxating, and I could stand without fear of passing out. What if remission was temporary and I turned back into a pumpkin without warning? There was no research or fairy godmother to guide me. 

In June 2021, I wrote, “Several people have asked if I worry about remission ending. I don’t. The end of remission would probably be soul-crushing. No one knows how or when anyone’s MCAS baseline will change or if treatments will stop working. I am still susceptible to trauma, infections, stress, and so many other immune system triggers. Everyone is. But I know I can handle it.”

So, I set out on a 10 day road trip from Seattle to Los Angeles. Within 24 hours of landing in Seattle, I walked 22,937 steps through three different parks. Exhilarated by my body’s stamina, I wanted more, so I joined a group of strangers for a Zumba class on the pier in the 90F sunshine. I had zero interest in Zumba, and didn’t even know what it was, but because my mast cells would have despised it, I was determined to give it a try. I flailed and sweated, trying to keep up with high energy dancers. Drunk on exercise endorphins, I felt invincible.

Back in the hotel, my body revolted. As first, I assumed it was dehydration or heatstroke, so I chugged some water. Then I vomited and the bathroom began to spin. Within minutes, I went from “I absolutely deserve this” to “is this the end?!” 

I looked in the mirror. My face and neck were not red at all. My throat felt fine despite the light fragrance in the room. Maybe I caught COVID? I vomited again. A wave of doom reminded me this had happened before. Shortly after my MCAS diagnosis, I thought I could handle travel, but I ended up fighting for my life in a Florida hospital and wishing I’d never left home. Was this also a big mistake? 

I tried not to worry about the implications. I just needed to regain control of my body. I dug into my medication bag and retrieved my bottle of prednisone… which I had just stopped taking.

I started using prednisone in February 2015, months before my MCAS diagnosis. Like many MCAS patients, my MCAS symptoms had been ever-present since childhood, but since the diagnosis didn’t exist back then, I accepted my symptoms as background noise in my daily life. 

Mast cells don’t like to be ignored. 

At age 28, my mast cells ramped up to a new level of aggression, inflaming my muscles, joints, and tendons. Although the MRIs came back normal and I received a disability parking permit, I cried limping into work each morning. My hip sockets felt as if they contained shards of glass and my quadriceps felt like overstretched rubber ripping with every step. As a last ditch effort, my rheumatologist prescribed prednisone, and my pain subsided. My rheumatologist quickly warned me prednisone was not a long-term solution because of its risks like adrenal insufficiency. I begged for refills, so I didn’t lose my job, health insurance, and mental health while fighting for a diagnosis. 

Then on May 29, my throat swelled for the first time. We weren’t sure why, but my doctors threw prednisone at it without any hesitation. Throat swelling scares rheumatologists.

For the next six years, I took low dose prednisone daily in order to eat, move, sleep, breathe, and work in combination with other MCAS medications and disability accommodations. Prednisone was also part of my premedication and rescue medication protocols, helping me to survive severe reactions, surgeries, and procedures. (But not that one brand I’m allergic to!) For me, the risks of MCAS (e.g. GI bleeds, hydronephrosis, anaphylaxis) outweighed the risks of prednisone (e.g. diabetes, osteoporosis, adrenal insufficiency).

Within an hour of taking prednisone at the Seattle hotel, I started to feel better. I had stopped taking prednisone, because I no longer needed it for MCAS. Although I resumed my low dose for the remainder of the trip, it took me weeks to realized I had almost Zumba-ed my way into adrenal crisis. Both exercise and heat burn cortisol quickly. If you don’t have adequate cortisol in your body, you die. I would have never knowingly spent all my cortisol on Zumba, FFS. 

I thought I had tapered slowly enough to avoid adrenal insufficiency, but my assumptions were way off. (Spoiler: After 6 years of use, it took an additional 3 years to taper off. And I’m considered lucky.) I realized although I was in remission, my body was still healing from years of damage from MCAS. 

In 2022, I was diagnosed with secondary adrenal insufficiency by an endocrinologist after realizing I couldn’t navigate tapering alone. No test was necessary for diagnosis considering I was still dependent on steroids and had used them for many years. Prednisone and other glucocorticoids suppress the pituitary gland from creating ACTH which simulates the adrenal glands to make cortisol. (So, cortisol and ACTH blood tests aren’t usually helpful if you’re on glucocorticoids. I wish I had gotten baseline tests before starting prednisone.) If your adrenal glands are suppressed for too long, they can permanently stop making cortisol. 

Primary adrenal insufficiency is when there is damage to the adrenal glands affecting the production of cortisol and aldosterone. I did blood tests to rule out primary adrenal insufficiency. 

In our 2022 MCAS Q & A, Dr. Afrin and I discussed adrenal insufficiency.

This blog post is one of a three part series on my experience with secondary adrenal insufficiency. In June 2024, I successfully tapered off prednisone and my adrenal glands began making a normal amount of cortisol again. However, I still must monitor for symptoms of low cortisol.

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