When I first was diagnosed with mast cell disease and learned my mast cells were overactive, I decided, “Okay, I’ll just be really nice to them. I’ll give them everything they want and they’ll settle down.”
Turns out, nobody, including the world’s leading mast cell activation syndrome specialist, knew what MY mast cells wanted.
Instead of receiving a set list of treatments, my doctor introduced the trial-and-error process, a methodical approach to medications and supplements, slowly and one at a time.
Struggling to eat, move, and sleep, I wanted to say, “Excuse me, sir. But I believe I am dying. I don’t have time to waste. I have bills to pay and dogs to feed. Please fix my mast cells now.”
Luckily, I heeded his guidance. At first, I improved with each incremental change: my joints ached less, my intestines were happier, and I had more energy. Each time he added a new prescription, my specialist warned me MCAS patients often react to inactive ingredients (e.g. fillers, dyes, binders, and preservatives) in medications, and to be mindful of any new symptoms.
“Which ingredients?” I asked.
“We can’t predict that,” he said.
For me, it started with FD&C Blue No. 1 Aluminum Lake. My mast cells didn’t care that my new medication was expensive, or that it was supposed to work. My mast cells only cared that there was a minuscule amount of blue dye in one pill and proceeded to sabotage my entire body. My vision faded, my heart raced, and I vomited like the girl in The Exorcist. One ER visit, three IV medications later, and two sick days later, I was still recovering.
My specialist urged me to try it without the blue dye.
“So, then it’ll work?” I asked.
“We can’t predict that,” he said.
There’s a saying in skating: “If you aren’t falling, you aren’t learning.”
In November, seven months into MCAS remission, I decided to pursue my childhood dream of ice skating. I envisioned myself gliding effortlessly on the ice, wearing a sparkly dress. However, when I stepped out onto the ice in my new blades and boots, I realized, “Wow. This ice is hard and slippery.”
I tried to avoid falling as long as possible, but inevitably my toe pick betrayed me, and I belly flopped onto the frozen pool. I moved just enough to indicate to others I was “okay,” whatever that means, and then laid motionless and contemplated my existence until the ice numbed my freshly bruised limbs and soul.
Not only are some of my skating falls pretty brutal, but if I want to get better, I have to get up and try again like it didn’t happen. If I hold on to my anxiety and anticipate a fall, I’m more likely to fail again, possibly worse. Same goes for mast cell disease. Mast cells are notoriously triggered by anxiety. If you are worrying about trying a new medication, your mast cells can become more reactive and therefore less likely to tolerate the new medication.
I often remind myself learning to figure skating has been much easier than learning to how to treat mast cell disease. It certainly has required less emergency medical care. Unlike skating with its tried-and-true techniques, every MCAS patient embarks on their own difficult journey to find their optimal treatment. There are many treatment options, but at this time, we have no way of predicting treatments or triggers of individual patients.
When I was diagnosed in 2015, my mast cell specialist said it usually takes 4-5 years of medication and supplement trials for an MCAS patient to discover their optimal treatment.
I promptly ignored him. No patient actively getting their ass kicked by their mast cells wants to hear this. However, it was absolutely accurate for me. I reacted to more MCAS treatments than I tolerated. It took me 5 years of trialing medications and supplements to find my optimal daily combination to reach MCAS remission. There was no magic pill, just a lot of trial, error, and renewing of hope.
In skating, although falling still hurts, you usually do get better at it. Your brain learns to track your movement more quickly and your body learns to fall more safely, protecting delicate bones. Although the MCAS reactions don’t get easier, you learn how to identify triggers and stop reactions more quickly.
Here are some tips and encouragement for MCAS trial and error, learned through my own experience both as a patient and skater:
Celebrate your courage and hard work regardless of the outcome.
Don’t quit, but do take breaks. Take time to recover physically and emotionally.
A knowledgeable specialist may save you time and money in the long run. Most doctors are unfamiliar with MCAS treatment. MCAS specialists know how to methodically work through the treatment options and understand each patient is unique.
Build relationships with other patients. They can help you avoid common pitfalls and cheer you on. Don’t forget to cheer them on too!
Keep calm and put on your crash pants when you’re trying something new. For me, this is taking time off work, having rescue medications ready to go, and planning check-ins with friends.
Track your all medication and supplement ingredients, doses, and symptoms. Sometimes reactions can be subtle and build over time or with increased dose. Organized notes can help identify trends and triggers.
Compounding medications can help reduce inactive ingredients and risk of reacting.
Last month, I participated my first figure skating competition. Despite preparing my best, I knew falling was a real possibility and I was absolutely terrified. I showed up, not because I knew I would perform well, but simply because I don’t want my life to be dictated by fear. I want to try to get better.
This week marks one year of me telling strangers, “I’m not supposed to be here.”
As they try to guess where this unsolicited conversation is going, I clarify, “Oh I don’t mean I should be dead. But I should still be in solitary confinement.”
I beam with the satisfaction of knowing I’ve already won the day, as they consider if I’m a public safety threat.
Mast cell activation syndrome {MCAS) has never been easy to explain, so describing the freedom and joy of remission is basically impossible.
I try to relate in simple terms, “I couldn’t do stairs for 5 years. I could only eat 15 foods without any spices, not even pepper. I had to spend Christmas and birthdays alone.”
MCAS remission has affected every aspect of my life. My gratitude is endless. For example, I can buy food without reading the ingredients labels, I can meet new people and remember their names, and I can wear new clothes without worrying about losing my skin.
It’s okay if people don’t understand the details, but I desperately want the world to understand three things:
MCAS treatment can be life changing
MCAS is likely prevalent
Most people don’t have access to MCAS testing and treatment
I want people to get access to help sooner than I was able to, so they can avoid unnecessary suffering and damage to their bodies.
Thank you for your kind messages in response to my video on social media. I’m glad to hear my work is making a positive impact and inspiring hope. It encourages me to continue to share my story and create resources.
“What did you do?” ask the ER nurse, rolling me into an exam room.
“Exist,” I wanted to say.
How could I explain my foot had randomly subluxated while I was making dinner? One minute it was functional, the next a joint had slipped in the middle of my foot. When I stepped down, the uncomfortable protrusion inside my foot radiated pain. After 5 years of walking to the ER with kidney stones, a CSF leak, and mast cell reactions, I had been forced to ask my neighbor for a ride.
“What did you do?” asked the ER doctor, examining my right foot.
Okay, I knew what “I did.” The day before I decided to dust off my recumbent bike and begin training for the AKC National Agility Championship. Unsurprisingly, two minutes into moderate pedaling, my mast cells threatened to suffocate me. My foot joint had become unstable from pedaling the night before.
“Why do you think it’s dislocated?” the ER doctor asked.
“Subluxated,” I corrected him. “Because I have EDS.”
It’s embarrassing, as a writer, to be unable to effectively describe a subluxation. It just feels wrong, followed by an intrinsic desire to push on it until it feels right again. Before surrendering myself to the ER, I desperately bounced on my foot, hoping for a gratifying pop. Instead, I collapsed on the floor in tears. A bruised egg had formed on the top of my foot.
“We’ll get an X-ray,” the ER doctor said predictably.
I knew there was nothing the emergency room could do for me unless I had a fully dislocated joint, and quite frankly, I wouldn’t want a random ER doctor manipulating my bone back into the socket. However, I also couldn’t walk anymore or take care of myself alone in my condo. Friends and family wouldn’t understand if I didn’t try to seek help. Like so many times before, I prayed my problem would be caught by imaging.
Not only did my subluxation not show on the X-rays, but the technician asked how I should position my foot for the imaging. God forbid, I’ve spent the last six years so focused on MCAS, I neglected to learn how to image joints. I knew if there was any chance at catching this subluxation, I would need to be standing, but I didn’t have the energy to suggest this.
“The x-ray didn’t show a dislocation. It’s most likely sprained,” concluded the ER doctor.
“What about a subluxation?” I asked. Sprains are also common with hEDS, but they sear, throb, and shoot pain up and down limbs. This mostly felt a foreign object lodged in the middle of my foot.
“Well the x-ray didn’t show a dislocation, so that’s unlikely,” he said.
“Even for an hEDS patient?” I asked. I have asked this question before.
“Sprains are the most common foot injuries,” he said, backing towards the door and ignoring my question just like the ER doctor I saw when I had a CSF leak. For four months, I shook with pain, vomited and blacked out, while doctors argued CSF leaks were rare. They are not rare with hEDS.
Two fears consumed me as I waited for my ride home. First, I was at the mercy of this joint. What if it never popped back on its own? How would I get around? Who could I trust to try to pop it back in and how much would it hurt?
The second fear had been building for a while. My subluxations and hEDS complications were getting more severe and frequent every month. Thanks to my CSF leak, I was afraid of sneezing and pooping. Now, I had to worry my leg might pop off like that of a Whole Foods’ rotisserie chicken.
Before I was diagnosed with MCAS, the inflammation in my hips became so bad I lost my ability to walk for several weeks. After diagnosis, I developed costochondritis, inflammation of the cartilage in the rib cage. In addition to feeling like I was constantly having a heart attack, my ribs began subluxing so easily I couldn’t wear a bra or sleep on my side. Fortunately, an increase in dose of basic MCAS medication resolved both problems.
Now, five years into my MCAS journey, my right hip was subluxing twice a week on short walks with my dogs. It seemed every time my mast cells seemed stable enough for me to move, I sprained a ligament or strained a tendon. Not only did MCAS limit the foods I could eat, but my joints limited what I could buy and carry into my condo, even with disability parking. I gave up on stairs completely after several dangerous falls. My Invitae test for genes associated with Ehlers-Danlos syndrome was negative. I didn’t quite meet the Beighton criteria, but my CSF leak and other joints earned me an hEDS diagnosis. (Until remission, I repressed many memories of my childhood injuries.)
I wasn’t a ballerina or a circus performer, but I did stand on street corner of the hospital and shoot spinal fluid into my ear.
I scheduled an appointment with an orthopedist for my foot subluxation without any hope that they could help me. I hopped on one foot out of my pajama pants and into my joggers. As I bent to pull up the waistband, I lost my balance and slammed my injured foot onto the bathroom tile.
POP.
It felt so good and sounded so terrible. For once in my life, I was truly thankful for dysautonomia.
Five days after I realized I was in remission, Sancho and I boarded on a plane to Florida. I was intoxicated by freedom and ready to chase adventure.
“Don’t fuck this up,” warned my conscience.
A lot of hope was riding on my recovery and I still didn’t understand what made my joints unstable. I assumed my connective tissue had not changed overnight. Just six months before, I needed my best friend to help me through the airport and I still almost passed out. I couldn’t carry my luggage without subluxing my arms or hip. I warned myself that I would need to pace myself and slowly build strength to support my joints.
But then, I arrived at the beach, got upgraded to a luxury room, watched a pink moon rise over the ocean, and lost all self control. Sunburned and hungover, I spent the morning walking in the sand and wrestling with waves, as Sancho worried about my new behavior. When the trip was over, I stuffed 10 sundresses, 2 swimsuits, 5 pairs of shoes, several books I did not touch (and would not for many months), and two laptops into my bags, and exited the hotel feeling stronger than ever.
When I got home, I took up pickleball and biking, proudly loading my bike into my car (usually after brandishing it above my head first for good measure). Swimming in endorphins, but free of injury, I proceeded to kayak, paddleboard, take barre classes, and train for a 5K. I should have absolutely should have been injured by now. I had gone from very restricted exercise for 6 years to daily–sometimes twice daily– exercise, including high impact sports, in one week. My worst complaint was blisters from running. It felt like a switch had been flipped and now my joints were stable.
So, naturally I signed up for surf lessons. I figured I’d struggle to carry the board and stand up when I caught a wave. No. The real struggle is the brutal ocean beating you get trying to paddle out to catch a wave. On the beach, the instructor taught me how to grip my board and baby cobra as I hit a wave head on. As a Minnesotan, I didn’t really envision how waves work. Just as soon as the wave launched me into the air, to my horror, it disappeared below me. I free fell for five feet, belly flopping against my board as it hit the ocean.
I took a deep breath and yelled, “I still have ribs!”
Surfing was one the best and most exhilarating experiences of my life. I certainly was sore and had a few bruises, but no subluxations or other injuries.
In October, when I told the figure skate salespeople I planned on skating 2-3 times per week, I was purely lying. I wasn’t sure my feet would even tolerate skates, but I had to justify paying hundreds of dollars for a sport I didn’t even know if I could do.
As a child, I dreamed of competing in figure skating, but I kept getting injured. No one understood my injuries, so they questioned if I was faking them. X-rays didn’t show any broken bones, so they diagnosed me with sprains. Instructors told me skaters are supposed to be uncomfortable, so I thought numbness was normal. At age 13, the pain forced me to quit skating. I believed it was entirely my fault.
Not only can I skate now, but I’m absolutely in love with it. I flail my body across ice 4 times a week without injuries. Despite a lifetime of poor proprioception, I now jump and spin on knives. My body is the strongest it’s ever been. I still have hypermobile joints, but they do not sublux or scream with pain. Thanks to supportive boots and custom insoles, my feet are painless. On the ice, I’m surrounded by other people with hypermobility that help me find ways to support my individual bodies’ needs.
If you hunt around Twitter, you’ll find researchers and patients theorizing about mast cells causing connective tissue disorder. Dr. Theoharides often describes tryptase, one of many mast cell mediators, as a “meat tenderizer“ within the body. In a 2021 article, Dr. Afrin states, “Although hEDS is seen in only a minority of MCAS cases, limited studies have identified an association between hEDS and MCAS, fueling speculation that certain variants of MCAS may drive hEDS.”
In Italy, researchers are studying doxycycline’s effects on hEDS, but I know there’s so much more to investigate than fibroblasts. When combined with basic MCAS medications, doxycycline eliminated my chemical sensitivity, dysautonomia, AND hEDS symptoms. While I understand these conditions are extremely complicated to study, the siloed approach to MCAS, EDS, and dysautonomia is not serving patients.
I highly recommend patients with hEDS learn about MCAS. One of my pet peeves is when patients blame common MCAS symptoms solely on hEDS. We have a lot to learn about both conditions, but we do know many of the adverse effects of excessive mast cell chemicals, for example, prostaglandins can cause inflammation and severe fatigue, while heparin can cause easy bruising and bleeding. There are many treatment options to inhibit these chemicals.
It’s also hard for me to believe it’s just a coincidence that both MCAS and hEDS symptoms often get worse at the onset of menstruation. Natural hormone changes are known to activate mast cells, even in healthy people. Again, MCAS treatment can help decrease symptoms and complications triggered by hormones.
I am living proof we need research on the link between MCAS and hEDS. I truly believe my CSF leak, subluxations, and other damage to my body could have been avoided if I had adequate MCAS treatment sooner.
Meanwhile, as we wait for research to explain my new reality, I couldn’t be more excited to shift my focus from MCAS, hEDS, and POTS to loop, lutz, and axel.
P.S. An October 2021 Russian study of figure skaters concluded that successful figure skaters were associated with the AMPD1, COL1A1, COL5A1 genes. Past research has confirmed COL5A1 and COL5A2 “are the major, if not the only, genes involved in classic Ehlers-Danlos syndrome (cEDS).” Luckily, as an hEDS patient, I have no aspirations of becoming a Russian Olympian.
“I really wish this course had been written years ago. I believe the approach explained here would have saved me a lot of tears at doctor appointments trying to explain my whole range of symptoms effectively. This course is essential for anyone living with a chronic illness who wants to communicate with their medical provider in a way that is really heard, professional and understood. Hugely beneficial and very much needed – thank you.” – Jeannie Di Bon, MA PMA-CPT, Movement Therapist and Hypermobility Specialist
