This week, I am celebrating TWO YEARS of mast cell activation syndrome (MCAS) remission. Most of my fellow skaters have no idea how terrible and scary my MCAS symptoms were from 2015-2021. They don’t understand how impossible skating was for me before remission. I look “normal” aside from my steady bursts of humor and wiggles.
Note: Competitive skaters with less severe mast cell activation (MCAS) symptoms do exist. I’ve met several of them! MCAS symptoms can range from discomfort to disability. My remission is incredible is because of the severity of my illness despite years of proper treatment. Many doctors gave up on me. I was told I would likely never be able to use stairs, let alone compete in athletics.
I never want to take my remission for granted. I never want to forget how without treatment, MCAS complicated every aspect of my life: eating, moving, sleeping, socializing, and breathing. Some people keep a gratitude journal; others do daily meditations.
I practice gratitude by counting the ways my pre-remission mast cells would have tried to kill me every time I walk into a skating competition.
First, assuming I could tolerate the usual fragrance (perfume, shampoo, detergent, deodorant, etc.) of a public event, one whiff of hairspray would swell my throat before I could set my skate bag down.
For me, this usually wouldn’t require a stab in the leg, a ride in a fancy taxi, and an expensive hospital bill with a side of medical gaslighting. (Like many MCAS patients, I haven’t had to use an EpiPen, but I definitely carry one.)
Hopefully, I would be able to force pills down my throat and play the game: should I stay or should I go?!
Obviously, fleeing toxic air is always a good idea.
Why would I stay? To overcome my disability and skate anyway? Absolutely not.
I would stay because of the bathroom, projectile vomit, and uncontrollable pooping. I don’t like squirming out of my skating dress on a good day, so I can’t imagine doing so in a full body rash. Best case scenario: I empty my guts and someone drives me home where I recover for three days.
Okay, so let’s pretend I got a disability accommodation to prohibit hairspray inside the arena and nobody smells like MCAS death.
My mast cells don’t like the cold. Once inside the arena, my mast cells would begin pumping chemicals into my blood–dilating and permeating my vessels. This can trigger poor circulation, dizziness, and weakness–a recipe for skating disaster. My numb, tremoring hands would likely need help tying my skating.
So why not just warm up? Well, my mast cells don’t like that either. Just a few off-ice waltz jumps would be enough to the ignite the inflammatory process–pushing my lungs, heart, and brain into overdrive and destabilizing my hips and feet.
As my body would swell, every rhinestone on my dress would be at risk of spontaneous ejection. Other skaters would attribute in my urgent and incessant need to pee to performance anxiety, when really histamine would be inflaming my bladder and literally making it smaller. In bathroom mirror, bright red rashes would outline my dress as my skin reacted to the synthetic fabric–definitely not the pretty lines Griffies aspires to.
No matter the temperature, I would be spinning before I got on the ice.
This where my gratitude practice ends.
Don’t worry, Coach Debbie–when I check in with the rink attendant, I stop envisioning my demise.
When step on the ice, I don’t think about my mast cells at all. I take a deep breath and my body swells… with gratitude.
On December 22, I celebrated 7 years of MCAS diagnosis. Yes, I say celebrated, because getting a diagnosis in 2015 was almost unheard of and without it, I wouldn’t have been able to advocate for myself and get treatment.
This is how I describe the past 7 years:
2016: Total destruction and despair
2017: Renovating my life
2018: Building a support system
2019: Survival and self-advocacy
2020: Dreaming again
2021: Adventure and feeling
2022: Finding my balance
Please note: the majority of MCAS journeys are not this extreme. Many people with MCAS are undiagnosed because their symptoms are not severe.
My 2022 holiday card
Remission has been interesting to navigate. Sometimes I catch myself subconsciously living like I am still controlled by MCAS. For example, for 5 years, I didn’t have enough vacation time to even consider taking the time off between Christmas and New Year’s; all my paid time was spent on MCAS reactions. Last week, after receiving my 9th auto response from a coworker, I seriously had to reassess my choices. I no longer need to fear going to losing my job because I want to spend two days relaxing and eating cheese.
Last year, I had an unsatiable desire for adventure–not knowing what I liked or how long my remission would last. This year, the urgency subsided (but not my gratitude!). Instead of chasing adventure, I took pleasure in building habits. Until remission, my mast cells never permitted routine.
I did literally work on finding my balance: scratch, camel, and sit spins. However, the hardest work was learning how to balance adrenal insufficiency (a life-threatening complication of MCAS) and my newfound love of athletics. Although I’ve met plenty of competitive skaters with MCAS, I have yet to meet another competitive skater with adrenal insufficiency, because exercise requires cortisol. A few ruthless adrenal crises reminded me: even though my body CAN go longer, faster, or higher, it doesn’t mean I should. I need to really WANT to do the thing. And then stress dose accordingly.
Speaking of stress dosing, isolation is terrible, but this year, I re-learned that human interaction is really complicated. It’s easier to set boundaries when you’re deathly allergic to people. Other people’s expectations are especially rampant in the figure skating world. You can’t go a day without hearing “axel” and “Olympics.” Meanwhile, I’m sliding around in a pumpkin costume, most interested in “joy.”
Two weeks ago, I found myself skiing 7” of wet, heavy snow, being chased by a professional race coach yelling, “Right, left, right.” My trembling muscles warned me this would not end well, but I told myself to be grateful for this unexpected private instruction. I did not feel grateful a few days later hugging my toilet in adrenal crisis and vomiting so hard brain juice shot out my old CSF leak.
Remission or not, life is messy. I’m not making any New Year’s resolutions because I’m not pretending to be in control. Joy is my compass, gratitude is my motivator, and humor is my Band-Aid for when I inevitably get my ass kicked.
In case you missed it, on October 30, I had the pleasure of reconnecting with Dr. Afrin, my MCAS specialist from 2015-2017, in a live MCAS Q & A hosted by Mast Cell Research. Watch the recording.
“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
