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.

 

Messages I received instead of GoFundMe donations

I’ve spent my summer suffocating on laundry fumes. My neighbor values the noxious fragrance of his laundry over my human life, so I was forced to launch a GoFundMe to afford a lawyer and a contractor. Friends and strangers alike have been incredibly generous in their donations, advice, and encouragement. Asking for help was terrifying–it always is–but once again, I was reminded that people care and want to help.

Of course, there were a few inconsiderate exceptions that poked my funny bone.

“Have you tried celery juice?”

Does celery juice pay the bills? If you find a vegetable that can heal my bank account, please let me know.

“There but for the grace of God I go”

Apparently, this means you feel lucky you’re not in my position. Or that God has no mercy on me? Or you don’t have mercy on my brain fog? There but for the grace of GoFundMe I go.

“Do not use western medicine!”

So next time I have an allergic reaction, I should just ignore my EpiPen? Because the last alternative medicine supplement I tried gave me anaphylaxis. I suppose you don’t like to use western money either.

Brain juice is leaking out my ear

Day 23: I’m stranded on an island of pillows and sheets. I think the leak has stopped, but to be safe, I must stay here for a few more days.

While most Minnesotans are floating on lakes, here I lie on bedrest for a cerebrospinal fluid (CSF) leak that started last month.

Day 1: My right ear is taking on fluid. Just the one ear. No sneezing, no coughing, but maybe the start of a headache.

 Day 2: I pooped, then fainted. At least, I was able to pull able my pants before I fainted. I do not want to die like Elvis. 

Over the next two weeks, the pain at the base of my skull and behind my right ear became more severe, like someone had swung a bat at the back of my head. None of my mast cell medications provided relief. As an experienced medical detective, I retraced my steps and remembered, the day before my symptoms started, I had a chiropractic adjustment. The chiropractor had used her activator, a small metal device, on right side of my neck, just below my skull, the same spot as my headache.

My chiropractor specializes in Ehlers-Danlos syndrome (EDS), a connective tissue disorder associated with mast cell disease. Over the past year, I’ve experienced more joint subluxations and my chiropractor gentle guides my bones back in place. However, I know neck adjustments, even gentle ones, can be risky for EDS patients.

Day 13: I think my brain is leaking, I’m just not sure how to tell anyone.

Fully aware nobody likes a self-diagnosing patient, I emailed the facts to my primary care doctor without my hypothesis. She responded, “I have no recommendations at this time. Try more [mast cell medications].” At this point, the pain was causing me to vomit. I only felt relief after laying down. Classic symptoms of a CSF leak.

Day 14: The ER doctor is pretty sure I won’t die tonight. 

In ER, my doctor was concerned about a stroke or vascular dissection. When I suggested a CSF leak, he said that wasn’t a possibility, because I hadn’t experienced a head trauma. When I told him about my chiropractic adjustment and EDS, he paused and then left room, I think to Google. My MRI and bloodwork came back normal, and he sent me home. I really should have asked to see the neurologist on call.

Day 16: I yelled, “Brainjuice! Brainjuice! BRAINJUICE!” But the pain did not stop.

I instituted my own bedrest until I could see a neurologist. It’s not like I really had a choice. The ER doctor prescribed pain medication, but laying down was the most effective way to relieve the pain. My friends with EDS were also convinced I had a CSF leak, while the rest of the world considered I may be losing my sanity.

Day 21: The neurologist says I probably have a CSF leak.

The neurologist believes my CSF leak will heal on its own with more bedrest. It is nice to be validated, but mainly I am tired of enduring this medical circus. My healthy friends aren’t sure what to say, and neither am I. I’ve accepted becoming allergic to the sun, delicious food, and most people. But now if I poop too hard my brain may come through my ear? That’s where I draw the line, folks.

So, I’ll be in bed for the next few days, trying to focus on my gratitude for the Internet. The Internet that provides me with unlimited movies, TV shows, and books without having to lift my head. The Internet that allow me to share my jokes on social media, so I don’t have to laugh alone and continue to question my sanity. The Internet that provides me with research papers and fellow patients who urge me to advocate for myself when my brain juice leaks.

Choose your own (mis)adventure

“You and YOU ALONE are in charge of what happens in this story.”

This is the ominous warning given at the beginning of each Choose Your Own Adventure book, the popular 1980s children’s series. I used to check these books out of my elementary school’s library by the armful, enticed by the idea of being in charge. I carefully weighed the potential risks and rewards of each option at the end of a chapter, imagining my fate. Do I run for the nearest escape or defend myself? Should I ask the sorceress for help? Morocco or Boston?

The authors punished greedy and cautious readers alike. This pissed off my impulsive younger brother, who inevitably succumbed to flipping through the books in search of the most appealing outcomes. Then he would back track, memorize the best choices, and declare himself the winner.

To my disappointment, adult life is a lot less adventurous, aside from an occasional vacation and professionally led excursions. Instead, I am in charge of mundane misadventures. There are no new planets or ancient jewels. Basically, I’m just trying to stay alive and support my Etsy shopping habit.

My recent misadventure in healthcare reminded of these books and their valuable lessons: life is illogical and totally unfair. Oftentimes, health decisions are a gamble. Some options end up being a huge waste of time and money, returning you back to the original situation. Other times, all of the choices suck. To demonstrate what I mean, I present to you…

Choose your own (mis)adventure: Abdominal pain

You can no longer ignore the pain in your upper right abdomen. It has throbbed with increasing ferocity over the last two weeks, but you cannot recall any new changes to your diet or exercise routine. The Internet suggests it could be your gallbladder and upon inquiry, it seems like everyone you know has had their gallbladder removed. One friend warns you of the possibility of a gallbladder rupture. You wonder if this is related to your mast cell disease. Your back begins to spasm.

If you hope the pain can wait until you see can your primary care doctor, click here.

If you go to the emergency room, click here.


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Emergency room

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You check into the emergency room. Forty minutes later, you are ushered into a room and describe your pain to a doctor. The doctor orders an ultrasound and it looks normal. Blood and urine tests are normal, too. The nurse administers mast cell medications, but they have no effect on the pain. The doctor recommends following up with your primary care doctor. Although it cost you $100, at least you’ve confirmed you’re not dying. Right?

Click here to go to your primary care doctor.


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Primary care appointment

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In the clinic, your primary care doctor examines your abdomen and agrees your gallbladder is the likely culprit. She orders a HIDA scan to measure your gallbladder’s functionality. You drink 20 ounces of a milky sludge and lay still in a narrow tube for an hour. The HIDA scan is normal. You pay the $100 imaging deductible.

Meanwhile, the pain is affecting your ability to work and move. You research mast cell related gallbladder issues and discover half of MCAS patients feel better after gallbladder removal. Half of patients feel worse. Your primary care doctor recommends you consult a surgeon.

If you schedule a surgery consultation, click here.
If you decide to give up, click here.


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Surgery consult

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The surgeon acknowledges all your test results are normal, but agrees to remove your gallbladder with the recommendation of a gastroenterologist. You know two things to be certain: surgeons love removing organs, and this was a waste of time and $25.

If you schedule an appointment with gastroenterology, click here.

If you follow up with your primary care doctor in hopes of non-surgical solutions, click here.


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Emergency room 2

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The pain is shooting up to your shoulder and your back is spasming. Near tears, you hurry to the emergency room before the pain renders you unable to walk. The doctor reviews your chart. Blood and urine tests are normal again. The nurse administers pain medication and you have some relief, but no answers. Another $100 emergency room copay.

If you schedule a surgery consultation as recommended by your primary care doctor, click here.
If you get a second option in gastroenterology, click here.


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Primary care follow up

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Your primary care doctor examines your abdomen again. You break out in hives when she pushes in the center of your belly. She reassures you it is not a hernia, but suspects a muscle spasm. She says sometimes Botox injections can help muscle spasms in the abdomen. You hate needles and have no idea if you’re allergic to Botox.

If you give up and accept a life of pain, click here.
If you decided to try Botox in the stomach, click here.


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Gastroenterology

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The gastroenterologist tells you your gallbladder is fine and he doesn’t want to treat you because you have mast cell disease. He informs you that the clinic has hired a gastroenterology psychologist. He suggests she may be able to help you to learn how “food affects your mood.” You just wasted $25 and two hours of vacation time to be referred to a poop shrink. Your current psychologist agrees.

If you give up and accept a life of pain, click here.
If you follow up with your primary care doctor in hopes of a non-surgical solution, click here.


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Botox

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You died from Botox, but at least your stomach isn’t wrinkly. You didn’t leave a will for your poodles.

(Author’s note: It is unlikely you would die from Botox, but you really chose the worst option. Click here to give up and endure instead.)


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Give up and endure

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You swear off all doctors. You can’t afford them anyway. You self medicate with baths, HBO, and ready-bake chocolate chip cookies. If you could just figure out how to never move again, you would be pain free.

Four months later, a friend with MCAS recommends a chiropractor. You decide to go, because the pain is now causing your ribs to subluxate. The chiropractor adjusts your ribs, and you mention the pain in your abdomen. She palpates the pain and tells you its an adhesion. You wince and grit your teeth, as she massages the tender area. When she stops, the pain is gone. Forever.

The End.

(Based on a true story. What the hell.)