Mast Cells and Me

An Immune System Malfunction

Over a decade ago I experienced my first mast cell episode. I ate a normal dinner (foods I had always eaten without a reaction) and suddenly felt itchy. I was covered in hives.

The specialist I went to ordered a battery of the medically accepted tests for detecting allergies. When the results came back, I found that I was IgE negative: not a typical allergy. He sent me on my way with a handful of emergency medications.

Recently, I met with a mast cell specialist who said my experience was not unusual as diagnosing mast cell disorders is difficult. This is due to the fact that only a very small percentage of allergists in this country study the mechanics of mast cell disorders: the research is in its infancy and there are frequent updates to the literature. This means that people suffering from a mast cell disorder might be confronted by doctors who are misinformed. For instance, there are still medical professionals who believe that anaphylaxis (throat constriction) occurs only in the presence of hives. This is untrue and belief in this myth, especially by emergency room personnel, can be life-threatening for patients.

To be an advocate for yourself or a family member with a mast cell disorder, it’s important to be up to date with research findings. This is the only way to combat misconceptions and to make sure your loved one or you receive proper care.

What is Mast Cell Activation Syndrome (MCAS)?

In my previous essay I wrote that mast cell disorders, including Mast Cell Activation Syndrome (MCAS) are often comorbidities with hypermobility Ehlers-Danlos Syndrome (hEDS) and Hypermobility Spectrum Disorder (HSD). The Mast Cell Disease Society defines mast cells as “immune system cells that live in the bone marrow and in body tissues, internal and external, such as the gastrointestinal tract, the lining of the airway, and the skin.” People suffering from mast cell disorders often have a wide range of reactions to chemicals, foods, and other environmental triggers in everyday life.

Why is MCAS so difficult to diagnose?

Mast cell research is fairly new even though research groups have been studying the cells’ functions and malfunctions specifically since the 1990s. Even today researchers do not understand exactly why mast cells malfunction and there is no efficient testing for all the biomarkers of the disease. Currently testing for MCAS follows a three-tiered structure wherein the patient must meet the following criteria*:

· Two or more affected organ systems
· A change in symptoms (decrease of frequency, severity, or resolution) when treated with histamine blockers or similar medications
· Elevated biomarker

When I first started experiencing sensitivities and reactions to various foods and chemicals most doctors had never heard of MCAS. Now even though allergists might have heard of MCAS, I have found them unprepared to follow the entire testing process. A single test or two for elevated biomarkers does not always yield results on the first try; a negative result for one test is not enough testing to say a patient does not have MCAS or a mast cell disorder. Diagnosing mast cell disorders is rather like the process for diagnosing hEDS or HSD: the patient is required to undergo multiple tests for confirmation as well as testing to rule out other disorders. Only after this long process can a proper diagnosis and treatment plan be suggested.

The last allergist I visited wrote me off after a single biomarker test. In the world of Western medicine, a one-to-one match of a single symptom is sufficient to decide on a diagnostic label. Yet, with MCAS a three-tiered diagnostic structure must be followed and revisited if symptoms persist. Not only did I need to find a specialist who was willing to test more than once, but also one who had access to a lab designed to test for mast cell disorders. I was shocked to read in Disjointed (2020) that some patients who underwent biopsies received false negatives because the lab did not test the samples correctly. This is heartbreaking. The error was discovered when these patients, still experiencing mast cell disorder symptoms, found more experienced specialists who knew of the potential lab errors that occur in mast cell testing.

Moving Forward

I am at the beginning of my mast cell disorder journey and have not gone through all three tiers of testing. Admittedly I have some doubts. Mast cell research shifts constantly, and I worry that the biomarker they test for today will be different by next year. The one positive outcome of this journey is that I follow research closely. I know that even if the research leads to changes in testing, I will be informed and able to weigh my new options.

*criteria taken from the book Disjointed, 2020

Note: Please remember that research related to these connective tissue disorders is ever-changing; new findings are released often. What you read in these essays might be outdated by the time you read them. Moreover, I am not a doctor. I’m writing about my personal experience and health journey. The facts of the research and its purpose is objective; the opinion of the facts is mine alone. I am not offering medical advice. Always consult your doctor. That said, next I’ll explore the relationship between chronic illness and anxiety. Till next time.

Fiction writer, essayist, and poet. Author of the historical fiction book Babchi: A Love Story.