What is Mast Cell Activation Syndrome (MCAS)? I like the definition given by the National Institute Health Rare Disease, MCAS “causes a person to have repeated severe allergy [ anaphylaxis] symptoms affecting several body systems. In MCAS, mast cells mistakenly release too many chemical agents, resulting in symptoms in the skin, gastrointestinal tract, heart, respiratory, and neurologic systems.”
What Is the Difference Between A Typical Allergic Reaction & MCAS?
Mast cells are also responsible for typical allergies we are familiar with. In those instances, the mast cell releases a “mediator” called IgE antibody which causes mild to severe symptoms of an allergic reaction. Some symptoms of an allergic reaction are chest tightness hives, itching, redness, runny nose, shortness of breath, sneezing, swelling, and wheezing. These symptoms are normal if a person with allergies to a specific substance is exposed to that allergen.
On the other hand, in MCAS the mast cells release mediators inappropriately. The mast cells are not triggered by an allergen. They are triggered by and internal or external stimuli such as changes in temperature & barometric pressure, chemicals such as fragrances, emotions, food, household cleaning products, hormonal changes, illness, & physical pressure. When the mast cells are triggered in MCAS, they release a flood of mediators called Cytokines, Histamine, Heparin, Leukotriene, & Prostaglandin. In most cases, the symptoms are not mild; they are called anaphylactoid symptoms. Which means anaphylactic-like symptoms. The fact is, there is no difference between anaphylactoid reactions and anaphylactic reactions, they both produce the same symptoms. Anaphylaxis is a severe, potentially life-threatening allergic reaction. The only 1st line treatment for anaphylaxis is an EpiPen. It is important to recognize the symptoms of anaphylaxis and treat them before a person goes into Anaphylactic Shock.
3 Types of MCAS
There are 3 types of MCAS: primary, secondary and Idiopathic. Primary MCAS is caused by a mutation of the mast cell gene. This mutation produces an overproduction of identical mast cells which spontaneously release mediators. Secondary MCAS is cause by an indirect result of another condition or disease. Idiopathic MCAS occurs when the cause can not be found. According to the NIH, it is possible to have combinations of all 3 types.
Please click the reference links below for details about testing and further insight into MCAS.
What Is MCAS To Me?
All of the above and more! My current diagnosis is Secondary MCAS. I have several conditions that Triggers my mast cells to degranulate and cause anaphylactic reactions. The ones that we know of are Adenomyosis, Ehlers-Danlos Syndrome, Cold Induced Urticaria, Premenstrual Dysphoric Disorder and Progesterone. We are still working on diagnosing an underlying autoimmune muscular disease. I also tested positive for Anti-Mitochondrial Antibody which is positive in 95% of the cases of Primary Biliary Cholangitis ( formerly know as
Primary Biliary Cirrhosis). When this antibody was discovered 2 years ago, there was no reason to start treatment because my liver enzymes remained normal. I have liver blood work every 6 months to monitor my liver enzymes, primarily Alkaline Phosphatase. I will elaborate more on my other diagnosis in another post.
Years of Unexplained Illnesses, Coincidence or Related?
I believe I that I have had mild symptoms of MCAS for approximately 10 years before being diagnosed. My symptoms progressively worsened after pregnancy, a viral illness or stress from life changing events. Some of the symptoms I will discuss from here on are not singular to MCAS however, they did have an impact on how MCAS manifested itself secondary to years of misdiagnosis and not receiving proper treatment for other conditions.
Unexplained Respiratory Symptoms
In the year 2010, I was diagnosed with exertional asthma. My main symptom was shortness of breath with minimal activity such as walking up a hill or up a staircase. I thought that was odd because I was able to run 5-10 miles with no issues. I was prescribed an Albuterol inhaler but I rarely used it.
Every year, I was guaranteed to have at least 4 sinus infections. It’s was what we knew & we were ready with the decongestant, Ibuprofen, Emergency-C, heating pad and Vicks to drain my sinuses. Some time during the year 2013, I had what we thought was a really bad sinus infection that wouldn’t go away after our usual ritual. I went in to see my PCP Dr. Gwozdz. He was a kind gentle man. He had been an engineer prior to becoming a doctor. He agreed that I needed antibiotics. I finished the course he prescribed yet not only did I not improve, I had a disturbing cough. I was more tired than usual and couldn’t build up the momentum to start any activity. As it would turn out, I had walking pneumonia. Dr. Gwozdz strongly counseled me to remain home & return to see him after I completed a second course of antibiotics he prescribed. I conceded, and took rest.
Although, the pneumonia cleared I didn’t feel quite the same. Breaths were harder to take, fatigue lingered, and I’d wake up in pain from head to toe. A burning pain raced through my body each day and my stressful job further burdened my fragile body. My bones would ache and they cursed me whenever I pushed myself too hard. I woke up in the middle of the nights with panic attacks. This would spill into the day time with me quickly seeking coverage at home because the down pour of emotions would overwhelmed. I’d seek refuge under my husband’s faithful umbrella of comfort. He would console me as tears made waves down my face. This was too much, I knew something was terribly wrong.
Then I started waking up in the middle of the night with hysterical coughing fits. My husband suggested we use my inhaler, I refused for a while telling him I did not have asthma. Well, I did one night, and voila! The coughing fit would stop. Then I started needing my inhaler more often. Dr. Gwozdz prescribed Atrovent in addition to my Albuterol inhaler. I did not use the Atrovent daily as prescribed, only during coughing fits or shortness of breath.
Heat Intolerance, When Hot Became Too Hot
A turning point for me was the year 2013, I became intolerant of the heat. Summer was my favorite time of the year for the longest time. I loved the heat, I’d lay on the porch soaking up every bit of sun in the sweltering heat with no repercussions. Just the reward of a sun kissed glowing bronze skin. All that ended the year 2013. It quickly became apparent that I could not longer tolerate the heat. Any exposure to the sun or heat left my face swollen, my body would quickly become overheated, then nausea, fatigue and headaches would set in. I’d get in the car, turn the AC to the coolest degree, swooning away unable to carry a conversation. By the time we’d arrive home, my body would be unable to hold itself up, I’d crawl into bed and my husband would take my shoes off.
Gastrointestinal Symptoms
My abdominal problems didn’t really take off until the pregnancy of my son 8 years ago. I had episodes of pancreatitis with elevated liver enzymes, hyperemesis gravidum & crippling abdominal pain. After his birth, my symptoms continued. I had a colonoscopy & endoscopy which lead to the diagnosis of delayed gastric emptying & Irritable Bowel Syndrome- Constipation. I was always skeptical of the diagnosis of IBS because my symptoms persisted after a bowel movement. I didn’t always abdominal pain every time I ate. My symptoms seemed to appear randomly without warning. I’d have severe heartburn, the pain would wrapped around me underneath my breast. Every breath worsened the pain. I’d feel my diaphragm stiffen then set off on a frightening course of uncoordinated movement that reminder me of a 3 year old learning to dance for the first time. The right quadrant of my stomach would become swollen with a visible blushful red color on my skin. I’d use every laxative know to man but the symptoms would persist for 1- 2 weeks. In addition to those symptoms, I started experiencing generalized & localized muscle weakness with my the onset of the above mentioned abdominal symptoms. Soon after, when I ate I’d have a sharp shooting pain straight to my left shoulder. My left arm and shoulder would become partially paralyzed. I did notice these episodes followed a stressful event, consumption of wine, hard cider, fermented, cured or smoked foods. Eating roughage also triggered unbearable pain. Running & weight lifting also triggered this bizarre cascade of events.
Over the course 10 years, I have eliminated almost every food on the food pyramid. The first food group was seafood. Initially shellfish would make me vomit violently along with diarrhea ( sometimes both at the same time), abdominal cramps, sweating, shakiness & dizziness. The exception was shrimp, some fish & squid, my reactions were inconsistent & mostly mild with facial swelling here & there. I remained this way for about 7 years.
I tried every diet on the planet, FODMAP, Keto diet ( I did lose lot of weight), Raw Food diet (that lasted less than half a day), vegetarianism and veganism. I of my biggest regret is following the weight loss trend of daily consumption of apple cider vinegar & honey. That was a terrible, terrible mistake. I slowly decreased my intake of wine & hard cider. I noticed when I drank as little as 1/2 a cup of either beverage, I’d wake up the next day with a burning heavy feeling in my stomach, bloating, nausea, sluggishness, shaky and weakness. So, I decided to switch to Brandy, Vodka & Rum for a while, but there was noimprovements with my symptoms. Eventually, I stopped drinking alcohol.
An Usual Dental Reaction
Early in the year 2017, I had root canal done. My reaction to the procedure was something I never felt before. The dentist had to inject a copious amount of lidocaine in my gums to numb the area and I still felt the pain. Afterwards, I had pain in my gums that I still don’t have words for. The nerves where the root canal was performed felt like I had burning coals on them.The area of my mouth that the work was done on kept spasming. I couldn’t speak for days, the dentist felt so bad for me, he personally drove to my house in the evening to drop off a prescription of Vicodin for me. He looked so worried, even I felt bad for him. He said I had developed a condition he had only seen a couple of times in his career but mine was the worst he had ever seen. I don’t remenber the name of it. Poor thing, he looked so worried. Even started to feel sorry for him. He referred me to an oral surgeon, they knocked me out & took care of the rest of it, whatever “it” was.
From Bad to Worse
After the birth of my daughter, more and more foods started coming off the list. I also had a difficult pregnancy with her. My blood pressure plummeted to 80/40 often, I would almost pass out, vomited the entire pregnancy, had severe abdominal pain, degenerating fibroid, back pain, joint pain, my left hip would pop out of place, unstable gait, separated pubis symphysis & headaches. After her birth, I had postpartum preeclampsia, mastitis twice & worsening reactions to foods.
December 2019 I had a flare up of spams of my esophagus, difficulty swallowing, even water. The spasms would worsen with eating. I even had difficulty swallowing my saliva. This led to another flare of epigastric pain, diaphragmatic spasms, muscle weakness, exercise intolerance and paralysis of my left shoulder.
One Last Try For Help
I reached the nadir of my suffering January 2020. I was in pain from head to toe, eberjoint in my body ached including those of my hands, my feet wpuld get so cold they couldnt touch each other. At nighy my ankles burned with pain, it felt as if my ankles wrebeing ripped apart while on a roasting spikect. Getting dressed was exhausting, a simple touch from my kids felt like a thousand blows. Whenever I would braid my hair,my entire shoulder on both sides would swell. I looked like the hunch back of Noterdame.
A friend of mine who also dealt with multiple health issues told me about a Nurse Practitioner she had seen for pain and he discovered she had an autoimmune condition. Honestly, by this time I was fed up with doctors. And it’s not that I wasn’t taken seriously by them, when they ordered labs and it came neck normal my medical problems were attributed to stress, depression, IBS & Fibromyalgia. Which felt like a catch all for the unexplainable. In exception to a rheumatologist I saw in 2013, not over ordered any autoimmune panel, genetic tests or inquired about my history. I was sure my symptoms were connected but most physician dismissed any assumption that was out of their specialty. I concluded that since no one could find what was wrong with me, there was no point in seeking further help. I felt like a fake ashamed, and embarrassed because I couldn’t explain to family and friends why I didn’t feel well.
Hesitantly, I booked an appointment with the Tyler Terry NP. And that appointment changed my life..
To Be Continued…
https://www.aaaai.org/conditions-treatments/related-conditions/mcas
https://rarediseases.info.nih.gov/diseases/12981/mast-cell-activation-syndrome
https://www.nhs.uk/conditions/anaphylaxis/
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3224511/
https://tmsforacure.org/overview/mast-cell-activation-syndrome-variants/