Like most systemic mastocytosis (SM) patients, I have tried pretty much every recommendation for managing this disease.
Besides identifying triggers, after I was diagnosed I began taking antihistamines and mast cell stabilizers, which are known as the best supportive care medications for SM. But I was still debilitated by daily symptoms, so a biologic intended for severe asthma was introduced to my management plan. This off-label medication had been proven to improve SM symptoms in some patients. I seemed to have an allergy to the sun, and I did notice improvement to my skin reactions with the biologic; however, I continued to struggle with chronic flushing, tachycardia, GI distress, bone pain and fatigue.
At the time that I had started the biologic, I also began an investigational tyrosine kinase inhibitor as it was the only available drug that slowed the proliferation of the mast cells, as opposed to sticking a band-aid on them.
You may be surprised to learn that out of all the medications that I have taken to improve my quality of life, a birth control pill was the most impactful on my symptom management. That birth control pill is how I learned that estrogen was a substantial driver behind my symptom severity.
Monthly symptoms without answers
Since my early 30s, I had always suspected that I had some sort of gynecologic syndrome due to experiencing unrelenting malaise and severe mood swings before my period. I remember researching my symptoms and finding PMDD — premenstrual dysphoric disorder. My symptoms corresponded to the diagnosis perfectly. I asked for a referral to a gynecologist, but because my complaints were viewed as minor, that appointment never materialized. This is unfortunately a common occurrence in the over burdened Canadian medical system.
I carried on with the monthly suffering, taking the same birth control pill that had been prescribed to me 10 years earlier.
Persevering with my undiagnosed SM symptoms was a normal part of my life from around age 25 on. In those years I was prescribed beta blockers for tachycardia, benzodiazepines for anxiety, proton pump inhibitors for acid reflux and hypnotics for sleep. There was no thorough medical investigation into the cause of all my symptoms; I assumed that they were chalked up to anxiety. The unfortunate part of that is that it allowed me to become compliant with feeling unwell.
Unwell with both my symptoms and side effects from the medications meant to address them, I convinced myself that I just needed to change my mindset. I had even researched a controversial surgery called a thoracic sympathectomy that involved severing a nerve to stop the flushing that I know now was a symptom of my SM.
Fast forward to my anaphylactic shock at 38 years old. The next day in hospital, the nurse informed me that I had a catheter inserted and that I had started my period. I didn’t know it then, that this was a huge piece of the puzzle of my severe anaphylactic symptoms.
I was discharged from the hospital three weeks later. Three days after my discharge — and nearly a month after my first anaphylactic shock — I had to dial 911 due to the same anaphylactic symptoms. One month after that ER visit, 911 again for the same symptoms. I’ll never forget the brilliant paramedic that noticed the monthly pattern and said, “I wonder if your hormones are triggering something.”
At that time, I still had no comprehension of the pernicious effect estrogen has on mast cells. It was that paramedic commenting on the trend of my 911 calls that inspired me to research the relationship between hormones and allergic reactions. Like I said, it was another piece to the puzzle.
After my diagnosis of SM, I rehearsed how to tell my respected hematologist and immunologist team that I believed my hormone fluctuations were the cause of my anaphylactic shock and reoccurring anaphylactic symptoms. After presenting my case, they didn’t take my theory as seriously as I’d hoped.
I think it may have been because the hormone part of it was not in their wheelhouse. The most arduous aspect of this disease is its heterogeneity. Each body system can not only be affected by this disease itself, but also affect the way this disease acts; therefore, having a specialist for each body system is ideal.
The estrogen dominance loop in mast cell activation
I finally got my gynecologist appointment. The gynecologist did her research on mast cells within her domain, acquiring an understanding of the relationship between mast cells and estrogen dominance. The research indicates that estrogen stimulates mast cells to release histamine, creating a feedback loop in which increased histamine can further stimulate estrogen production. This cycle leads to increased symptoms associated with both estrogen dominance and mast cell activation.
With that science behind her decision, she discontinued the birth control that I had taken for over 15 years because it contained a high amount of synthetic estrogen. She then prescribed me a progesterone-only birth control with the rationale of breaking this estrogen dominance/mast cell activation feedback loop.
I’m happy to say that it worked. Just like that, after years of miserable monthly symptoms that escalated into anaphylactic reactions, I felt relief flood through my body when that week of misery didn’t occur. It was like a spell was lifted.
My SM symptoms will remain unpredictable, and I have other triggers, but removing that trigger felt like slaying a beast. It took a lot of research and persistent advocacy, but the end result brought me one leap closer to managing this disease.
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