Please stop calling MCAS rare

Maybe it’s the pain and prednisone, but I’ve been really peeved by the eagerness to raise awareness for mast cell activation syndrome (MCAS) on Rare Disease Day.

First of all, why wasn’t the MCAS community half as engaged on Mast Cell Disease Awareness Day?

Second, there is growing evidence that MCAS is not rare. Some researchers believe more than 5% of people have MCAS! Yes, some organizations currently categorize MCAS as rare, but only because that is the default categorization for newly-recognized diseases. For example, AIDS used to be categorized as a rare disease. Furthermore, there is growing evidence that conditions like fibromyalgia, which affects 3-6% of people, are symptoms of MCAS.

Calling MCAS rare is not only unfounded, but it hurts diagnosed and undiagnosed MCAS patients. It signals to doctors that they do not need to learn about the disease. It signals to drug companies that there is not enough of a demand for new, lower cost treatments, like ketotifen in the U.S. It delays the development of accessible diagnostics. It signals to policy makers, who often vote based on numbers, that they do not need to listen to us.

Worst of all, calling MCAS rare discourages undiagnosed patients from getting tested and learning about MCAS, because they believe it is improbable.

That being said, please support Rare Disease Day. At this time, MCAS patients experience many of the same struggles associated with rare disease. Furthermore, mastocytosis continues to be classified as a rare disease through evidence-based research and we definitely should support fellow mast cell disease patients. After all, mastocytosis provides the foundation for understanding MCAS. Finally, you don’t have to have a rare disease to support Rare Disease Day.

MCAS is rarely diagnosed and rarely researched, but please stop calling MCAS rare. Instead, start planning for Mast Cell Disease Awareness Day on October 20. I know I am.

Learn more about Rare Disease Day at http://www.rarediseaseday.org.

Why viruses are scarier with mast cell disease

While the new year promised a fresh start, my mast cells were still reacting to what happened in 2019.

Around Thanksgiving, I caught a virus. I dragged my feverish body to urgent care and asked for a flu test. I needed to know if I should pack my hospital bag, which should not be confused with my emergency room bag. My ER bag is always ready and with me.

“Good news, it’s not the flu,” the doctor said.

“How long do you think this virus will last?” I asked.

“Generally, about 5-7 days and then you should be fine,” he said.

“I’m not worried about the virus,” I said. “I’m worried about my mast cells’ reaction to the virus.”

He sputtered a bit and tried to use “degranulate” in a sentence.

“I have prednisone at home,” I said, as I grabbed my coat.

Most people are used to hearing the dangers of the flu and other viruses for people with weak or suppressed immune systems. The flu is just as dangerous for me, but not because I’m immunocompromised. In fact, just the opposite.

My mast cells fight wars they’ve already won. They swiftly kill the virus, and then proceed to kill me. My immune system doesn’t know when to stop.

After seven days, the fevers waned, but I did not feel better. I attempted to go back to work, but the floor started bouncing. Benadryl every four hours wasn’t enough. I worried the pressure in my head would cause another CSF leak. So, I dug out my favorite poison: prednisone.

Maybe Ebenezer Scrooge just needed some prednisone, because within two days, I was buzzing with the Christmas spirit. As the prednisone tamed my mast cells, I shopped, wrapped, and decorated like one of Santa’s elves. Usually the holidays are a nightmare of unrealistic expectations, but this year I crushed my to-do list with energy to spare, confusing everyone. Don’t get used to it; you’re all getting unscented deodorant and a “bah humbug” next year.

The time between Christmas and New Year’s, when no one needs a calendar or real pants, is the perfect time to taper prednisone. For me, tapering prednisone means laying on the couch and staring at the wall, as optimism drains from my soul. I aimed to get it over with before the new year, because I wanted to start the year off on the right foot. Or left. At least standing.

Within a few days of coming off prednisone, my ear tubes began to ache. My mast cells were still reacting to the virus they killed a month and a half ago. I ignored it, hoping they would calm down, but the congestion in my head continued to build.

One night, after hours of painsomnia, I dreamed I went on a girls’ trip. The five of us innertubed from northern Italy to California (via the Mississippi River, obviously). The water must have been pretty rough, because I woke up with a subluxated jaw. The inflammation in my ear had gotten so bad that my jaw slipped out of the socket. I tried to ignore that pain too, but a spoonful of rice left me crying for another round of prednisone.

So instead of starting the new year motivated, I medicated. Luckily, this was just a cold. In 2016, I caught the flu and needed an ambulance and hospitalization.

I wish my mast cells had a reset button. I wish people kept their germs to themselves. I wish I could take prednisone for the rest of my life and become the most productive person in the history of the universe. Instead, I’ll embrace my solitary confinement and remind you not to kill me.

 

Fighting for MCAS

In many ways, 2019 was my worst year. My home became unsafe due to my neighbor’s laundry fumes. Even with air purifiers and a Vogmask, I couldn’t use my own bathroom without suffering from MCAS reactions. I missed meals and napped on the floor of my office, because I had nowhere to safely make food or sleep. Due to MCAS, I could not stay at a hotel or a friend’s house. Moving was not a reliably safe option either. Besides, I was already drowning in medical debt, living paycheck-to-paycheck despite working full-time at a well-paying job. I cried often and worried I was going to lose everything. The toll on my body resulted in emergency surgery in September.

But that’s not how I will remember 2019.

I was diagnosed with mast cell activation syndrome (MCAS) in December 2015. For the first three years of diagnosis, it seemed like every minute of every day was spent fighting a reaction. Eating, working, and sleeping seemed impossible. Sometimes I couldn’t walk. My body felt like an unruly toddler, throwing tantrums despite every attempt I made to appease it. I quickly learned medications were not enough.

My mast cells wanted a safer environment. So, I resigned to solitary confinement. (Technically not solitary confinement, because I had internet access, but you should still feel sorry for me.) As my mast cells quieted down, I realized my body is not my enemy. My mast cells are simply trying to protect me from a world swirling with chemicals.

This year, I switched from fighting my body to fighting FOR my body.

For me, the worst part of MCAS is not the lack of treatments or the hospitalizations, but people’s unwillingness to accommodate my health: a coworker who sprays perfume at the expense of my lungs, a neighbor who values fragrant laundry over my ability to eat and sleep, or a doctor who refuses to follow my medication protocol because he’s unfamiliar with MCAS.

Our world does not support MCAS patients. It prioritizes chemicals over people. In 2019, I was done feeling like my body was the problem and undeserving of accommodations. Here are some ways I fought for my body this year:

  • Filed a complaint with the Minnesota Board of Medical Practice. In 2018, a hospitalist refused to follow my emergency protocol and cut off my Benadryl and Diluadid in the middle of the night (after my friends left) and ordered morphine instead. Morphine is dangerous for MCAS patients and listed as an allergy in my chart. The board did not discipline the doctor; however, the complaint is on her permanent file. More importantly, the hospital made sure I received excellent care for my emergency surgery this year.
  • Shared my story with my local newspaper. The reporter interviewed me for one-hour, requested supporting documentation, and interviewed two of my friends. The interview was more stressful than I anticipated (hives!), but the front-page story changed my life and my ability to get people to understand my life with MCAS.
  • Asked for financial help. I think I threw up seven times before posting my GoFundMe, but my options were to lose my condo or ask for financial help. My deepest fear was that I wouldn’t receive the help I need, and I would confirm my feelings that I am not worthy enough to keep my home and survive. (Not true!) I raised $3k for a lawyer and safety improvements to my condo. After my newspaper story published, a local family paid my medical bills and I cried until I had to stop and take Benadryl.
  • Requested air fresheners be removed as a disability accommodation. Two businesses I frequent had air fresheners in their bathrooms. This year, I submitted formal ADA requests in writing. Although, the businesses were resistant at first, after my newspaper story published, the air fresheners disappeared forever.
  • Filed a discrimination complaint with the U.S. Department of Housing and Urban Development. I asked for my condo association to implement a fragrance-free laundry product policy as a disability accommodation under the Fair Housing Act. Instead, my condo association recommended I move several times. That is discrimination. HUD is currently requesting my condo association adopt the policy.
  • Drafted state legislation. Can’t wait to share more on this soon!

I wanted to give up so many times this year! I cried until I puked and I screamed into pillows. Other times, I was so tired or in pain that I couldn’t imagine responding to anymore legal letters. When I hit these breaking points, I stopped everything and rested. I reminded myself I’m not just fighting for my life, I am fighting for all people with MCAS. Then I planned the next tiny step I would take when I was ready.

Let me tell you about a few more moments that gave me hope:

  • When I was admitted to the hospital, my nurse was excited to meet another mast cell disease patient. His son had mastocytosis.
  • My pre-op nurse said she knew exactly how to time my pre-medications, because she had a daughter with MCAS.
  • During an emergency room visit for a hemiplegic migraine, a neurologist asked me to give a 10-minute speech on MCAS for the residents. He ended my lecture by saying, “This is why we listen to patients who know how to manage their disease.”

This year, I was grateful and angry, hopeful and scared. I’m taking all of these feelings into 2020 and continuing the fight for MCAS. I hope you’ll join me.