I had a photo come up recently in my phone’s memories collection that my husband took of me in the hospital. I’m in a hospital gown, making a goofy face to make fun of the fact that I was going crazy in my third week there. Looking at this photo two years later inspired a profound emotional response: It represents the time just before my life would be changed forever by my systemic mastocytosis (SM) diagnosis journey.
The photo was taken shortly after I nearly died of anaphylactic shock, but that’s not what made me pause. It was taken just before I was left out to sea by our medical system, forcing my own evolution to survive and fight to have my diagnosis seen. This photo compels me to see myself for the last time as that person; I trusted that doctors would just figure everything out and I would be taken care of. This was also a time when I minimized how unwell I felt, because I didn’t really know what feeling well felt like.
Before the photo
The day that I almost died in my living room, I went to work and nothing felt out of the usual, except I was feeling like I was fighting off a virus. You never would have thought that I, a fit 38-year-old woman, would have ended up in the ICU a few hours later; you never would have thought that even within a minute before I called 911. I went from letting my dog out for a pee to receiving IV epinephrine within a 20-minute time frame.
I woke up in the ICU that night with no memory of even dialing 911. I found out days later that paramedics came to find me pulseless, breathless and nonresponsive. They administered Narcan because I had no visible indication of an anaphylaxis, a common peculiarity of SM. I had gone into shock from my blood pressure dropping so fast. 55/32 is the reading that they got after administering epinephrine.
That day, I had never heard of mastocytosis. Nor had my doctors, because my tryptase was never checked to rule out anaphylaxis. Instead I was sent for MRIs of my brain and heart, CT scans of my entire body and even an invasive electrophysiology study to find an arrhythmia that was not there.
Months fighting for answers
After being told by a doctor “You would be dead if you hadn’t dialed 911 before passing out,” I was told by that same doctor three weeks later “We can’t figure out why you almost died, don’t drive, take a bath, ride a bike etc.” Nobody could figure it out because I had a very rare disease that affects every system of the body, meaning multiple symptoms treated by multiple specialists that don’t coincide with the other. I saw a specialist for each system malfunction; not one of them could figure it out. Looking back, it’s extremely frustrating that all they had to do was a tryptase blood test to avoid months of medical trauma.
I was stuck in a place in which I had to either sit in my house in fear of what almost killed me or figure out what the doctors were unable to.
I’ve been a nurse for over 15 years, and therefore had some medical background to help me keep up with my research. I spent three months analyzing my EMS report, my ICU admit and my labs. With that information I put everything together like pieces of a puzzle. I figured out that I had almost died from going into anaphylactic shock by googling every word of my EMS report. But I didn’t have any allergies and I hadn’t consumed anything out of the ordinary, so how did this happen?
I found the answer by googling just that — and that was the first time I saw the words systemic mastocytosis. I knew what had almost killed me, and I knew why I had been struggling for years with severe flushing, GI upset, heart palpitations and even episodes of syncope.
The inception of my health advocacy didn’t stem from the near-death experience, nor the endless hours of research: It was fueled by having to fight and work for a diagnosis that I had literally laid out for doctors — but not one would hear me, because they didn’t understand it. ER visit after ER visit I would ask “Please check my tryptase, I think I might be having allergic reactions.” ER doctor after ER doctor said, “No, you just have a stomach bug” or “No, it’s your heart, your cardiologist will figure out.”
So I went to my cardiologist. He tested me for a rare condition that is a differential diagnosis to mastocytosis, a pheochromacytoma. He did that because it was what he knew with those cardiac symptoms; he was unaware that mast cells could cause them, too. Because my cardiologist ruled out the pheochromocytoma, I had a device implanted in my chest to catch a heart problem that wasn’t there. It’s still in there to this day, and I have not heard from the cardiologist since my official SM diagnosis.
I went to the ER eight times in three months, each time thinking it was the end. Finally, one incredible doctor listened to my theory and had some knowledge of mast cells. Just like that, I got the blood test that would prove me right — eight ER visits later. That test fast tracked me to my oncologist and a bone marrow biopsy confirmed the biggest “I told you so” of my life.
Finding treatment
Antihistamines did not improve my condition; I continued to have severe reactions. More ER visits later, I advocated for a spot in a drug trial for a tyrosine kinase inhibitor, and a week later I took a one-hour flight to my neighboring province to start the process. I could write an entire chapter about how terrifying taking that flight was. I am here two years later and I feel as though I could take a 10-hour flight with very little fear of a reaction. The fear within the first year of diagnosis is so challenging, especially when you’ve had a severe anaphylaxis without an obvious trigger. I am living proof that the fear can be conquered with knowledge, self advocacy and self confidence.
I could also write an entire chapter on how validating and comforting it is to have a care team that knows your disease and helps you understand it, rather than being forced into becoming your own doctor in survival mode.
Starting a tyrosine kinase inhibitor has changed my life. I didn’t know that feeling good felt this good. Do I feel amazing everyday? No. It unfortunately is not a cure. However the effects of the TKI are illuminating how much I minimized my severe symptoms for the years when my SM was not being managed. I have a new calmness within my body. I attribute this calmness to the reduced mast cell burden as well as the knowledge and confidence I have built over the last two years advocating and adjusting for my life with SM.
That photo taken in the hospital now reminds me that not only did I live a more simple life before SM, but that I also had a meaningful journey ahead of me that would shape a new purpose in my life.
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