Restless in remission

Keeya standing on a paddleboard making the peace sign

You know that feeling when you decide you’re ready to be discharged from the hospital? Like you’re thankful for all everyone has done, but you’re about to rip out your IV and get the hell out of there? As the nurses finalize the paperwork, you’re sitting on the very edge of the bed, next to your packed bag, prioritizing your to do list.

It’s been two months since I went into remission from mast cell activation syndrome (MCAS) and I wake up every morning ready to burst out of my condo. When I started this blog, I worried that I would not be able to write posts regularly because of chronic illness. Ironically, writing during remission feels impossible. After spending five years in pain and isolation, I’m not interested in sitting still.

Of course, I never imagined MCAS remission was a possibility for me. I had heard about it, but knew it was rare. Remission doesn’t mean I don’t have MCAS anymore. It means I don’t have the symptoms of MCAS on my current treatment. Please read my tick saga if you haven’t already to learn about how I went into remission and my current treatment.

Since MCAS remission, I have also stopped my dysautonomia medication and my dysautonomia symptoms have disappeared. My resting heart rate has gone from 81 bpm to 69 bpm! I am more active than ever, yet I have not had a single EDS-related injury or subluxation. Before remission, I experienced subluxations about every three days from walking. Clearly, mast cells impact our nervous systems and connective tissues. I hope my remission will encourage more dysautonomia (e.g. POTS) and Ehlers-Danlos syndrome patients to pursue MCAS testing and evaluation.

Everything is brighter, happier, and more amazing. Not only can I do all the things and eat all the things, but I am raving like a kid, soaking in every experience. I am overwhelmed, overjoyed, and a little lost. For the last five years, every hour was dictated by mast cell disease. There were so many rules, many of them conflicting. I had whittled my life to a strict regime dictated by pain, not joy.

I have been so isolated for so long that I just want to scream, “WHAT ARE MY CHOICES?”

In actuality, when I went to the grocery store and faced all the choices, I grabbed avocados, pasta sauce, and ice cream, before running out to avoid a panic attack. I can eat anything I want without rescue medication or feeling like I’m going to birth a demon, but I’ve also learned drinking four margaritas or eating an entire bag of sour cream and onion chips doesn’t make me feel great either.

My favorite food is cherry tomatoes. I still hide in the corner on my kitchen and binge entire packages like I’m about to get in trouble. Before remission, I would have barfed before getting the second tomato in my mouth, because of histamine. Yet, my stomach pain has disappeared, and I have tapered of Prilosec completely. (Just 8 months ago, I had an endoscopy that showed inflammation and the gastroenterologist said there was nothing more he could do for me.)

Keeya and a big sandwich — I became vegetarian shortly after this sandwich. HA. Part of my spontaneous Florida vacation.

Every day I seek a new adventure. I assumed my muscles would be atrophied from five years of disability, but it appears they mostly just lacked oxygen. I rarely get sore afterwards. I don’t have to worry about one minute of sun or heat making me swell and vomit for the next 24 hours. I have paddled, floated, and swum in lakes, rivers, pools, and the ocean. My daily step average went from 5k to 15k. My cross-training includes weekly running, pickleball, and paddleboard. My lungs have never felt this clear; I have struggled with asthma my entire life. My worst injury has been blisters.

Keeya holding Quixote next to a kayak on a river

How remission how impacted my ability to compete in agility outdoors

See more photos and videos of my adventures on Instagram.

Several people have asked if I worry about remission ending. I don’t. The end of remission would probably be soul-crushing. No one knows how or when anyone’s MCAS baseline will change or if treatments will stop working. I am still susceptible to trauma, infections, stress, and so many other immune system triggers. Everyone is. But I know I can handle it.

Fear limits our joy. (And is terrible for our nervous systems.) I have been given an incredible opportunity to experience the world in a way most people cannot appreciate. My gratitude neutralizes any bubbling fears. I’m too busy yelling, “I can see fish!” from my paddleboard.

Take that dysautonomia! Standing alone used to be a nightmare due to dysautonomia.

It’s really hard to summarize how extensively MCAS, dysautonomia, and Ehler-Danlos syndrome controlled my life before remission, but here are some old posts and an article to provide perspective.

Does everyone have blood in their legs?!

For the past five years, I felt like I was going to lose consciousness any time I was walking or standing. After a year of not passing out, I just tried to ignore the feelings of death and sit whenever possible.

I never strongly pursued autonomic testing, because I hadn’t heard of any appealing dysautonomia treatment options. While beta blockers can lower heart rate and blood pressure, they also can increase mast cell activation. IV fluids always lessen my overall symptoms, but my veins are difficult to stick and react very painfully to IVs. (Drinking water is not as effective.) My symptoms never outweighed the risks of a port or PICC line, a convenient way to deliver fluids and medications.

After autonomic testing, my new MCAS specialist recommended fludrocortisone, a medication that helps control the sodium and fluids, to address my “heavy” feeling, which I was beginning to recognize throughout the day thanks to the tilt table. On the internet, I read that fludrocortisone can raise your blood pressure. My blood pressure spikes when I stand, so I worried the medication would only make it worse. I decided to try it anyway.

My first agility competition on fludrocortisone seemed unremarkable at first. I sleepily walked the course and warmed up my poodle. When it was our turn, we crushed it, winning first place. Maybe a coincidence, I thought. I celebrated with my friends, but something felt off.

I COULD FEEL MY LEGS.

Remember how I didn’t know my legs were numb from the tilt table until I laid back down? I didn’t know I’d been running agility on numb legs until I took the fludrocortisone. My legs didn’t throb and swell afterwards. I leisurely visited with my friends. I took my first picture holding my dog while standing.

And then I realized Quixote and I had just qualified for the AKC National Agility Championship!

BUT WAIT, SO THIS WHOLE TIME I DIDN’T HAVE BLOOD IN MY LEGS?!

I squeezed my 11-year-old pup and fed him some extra chicken. Then I continued to fume.

SOOOO EVERYONE HAS BLOOD IN THEIR LEGS?! AND NOBODY TOLD ME WHAT IT FEELS LIKE! WHAT ELSE AM I MISSING?

Turns out, all organs like blood and oxygen. I had never considered low blood volume might be my problem. My blood pressure actually decreased, probably because my body had been overcompensating from hypovolemia. Over the next few weeks, I felt like I was learning to operate a new body. I really underestimated the impact of dysautonomia on my poop tube. My muscles still brace for pain when I go to the grocery store and I have to remind myself my strength has improved.

AND OH YEAH, TEAM QUIXOTE IS GOING TO THE AKC NATIONAL AGILITY CHAMPIONSHIP IN TULSA ON THURSDAY!

Get in the car; we’re going on a virtual road trip! And by car, I mean YouTube. Please subscribe and buckle up for a good time!

May 2021 Update: MCAS remission eliminated my dysautonomia symptoms completely. Dr. Weinstock estimates MCAS accounts for 1/3 of POTS cases. MCAS can increase vasodilation and blood vessel permeability. While fludrocortisone was helpful, addressing MCAS was the key improving my circulation, reducing my heart rate, and lowering my blood pressure.

The tilt table and other tortures, I mean, tests

When I heard my new specialist did autonomic testing, I should have felt grateful. Many people with mast cell activation syndrome (MCAS) and Ehlers-Danlos syndrome (EDS) also struggle with dysautonomia, dysfunction of the autonomic nervous system. (See also: clusterfuck.) Despite being diagnosed with MCAS five years ago and having the symptoms of dysautonomia, no one referred me for testing.

To be honest, I was scared. While relaying your pee to your refrigerator for 24 hours for MCAS testing is exhausting, I’d heard too many stories about people fainting and vomiting during autonomic testing. While I was so dizzy in the first years of my MCAS diagnosis that I often crawled across my condo, I never actually passed out. Sometimes the fear of not knowing is worse than the experience.

I knew standing increased my blood pressure and made me feel terrible. When the pandemic began, the lines at grocery store deterred me from getting food. Luckily, I can now afford delivery, but some items are not available. If I have to go into a I store, I lean on a shopping cart to avoid losing my balance. After falling down the stairs several times, I stopped using them completely. (On more than one occasion, I’ve been asked if I avoid the stairs because my service dog is too short. Why is it so hard to accept invisible disabilities?!)

Tilt Table Test

Knowing fully well you may be minutes away from passing out or vomiting, the first task after being strapped to the Frankenstein table is to be totally calm to get a baseline reading of your vitals. After five years of MCAS and one epidural blood patch, I excel dissociating from my body. Super handy for medical procedures; not so great for processing emotion. (See also: reasons I need therapy.)

The goal of the tilt table is to measure your blood pressure and heart rate when you go from lying to standing. By using the table, instead of your muscles, we can better understand if the brain signals to the body are lagging.

“Okay you ready?” the doctor asked. I nodded and she pushed the button. The table motor chugged as it hoisted me up to 70 degrees. I braced for the worst.

As the table stopped rising, the pull of gravity took over. Within that split second, I decided:

YEP, I’M FUCKED UP.

What I remember is the heaviness of my body and the doctor’s incessant questioning. I felt like I was wearing a Kevlar suit and about to be sucked through the ground. I decided I would fight the tilt table with deep breathing and calm thoughts. As everything became quiet, dark, and slow, I tried to focus my eyes on a spot on the carpet like they teach you in yoga to keep your balance. Same thing, right?

What actually happened was documented by video. The doctor asked, “How are you doing?”

I responded, “Good.”

I WAS NOT GOOD.

“Any dizziness?” she asked.

“No, I just feel super heavy,” I said.

STOP ASKING ME QUESTIONS.

“Any headache?” she asked.

“Super, super heavy,” I groaned.

SHUT UP. I AM BEING CRUSHED INTO A TINY DIAMOND.

Instead of speaking my mind, I politely closed my eyes.

Within a few minutes, the pressure subsided and I just felt weak.

After 10 minutes, the doctor lowered the table and asked, “How are you feeling?”

“My legs are all tingly,” I said. “There’s pain in my muscles.” Then I realized the pain was the blood rushing back into my legs. They had been numb the whole time I was standing.

As my doctor calculated the results, I recall all the times I had a heavy feeling. I described it in 2018 as almost a paralysis.

“Your heart rate did not jump. This is not a diagnosis of POTS,” my doctor explained. “What we are finding is you had an adrenergic response meaning your blood pressure jumped and you were symptomatic.”

Although I expected the blood pressure rise, I was surprised my heart rate stayed normal. The doctor explained that my results supported the evidence that when I am triggered, my body maintains the reaction. In other words, I get stuck in flight or flight.

I’M NEVER FUCKING STANDING AGAIN.

“So we’ve got three more tests,“ she said.

“Cool,” I lied.

Deep Breathing Test

When my specialist explained the deep breathing test, breathing in and out deeply for one minute with the help of a computer screen, I was like:

I TRAINED FOR THIS MY ENTIRE CHILDHOOD IN THE ASTHMA CLINIC. AND I PLAYED THE TUBA.

I was feeling pretty confident after surviving the tilt table. While my deep breathing performance was perfection and my heart rate was normal, my mast cells protested.

“I’m cold,” I interrupted the doctor’s explanation.

“Like suddenly cold?” she said.

“Yes,” I said.

She retrieved a blanket.

“My chest hurts,” I said, remembering that bronchospasms can often feel like a heart attack.

“This test is like blowing up a teeny, tiny balloon,” my doctor explained. “So, you know how much pressure you have to put on a teeny, tiny balloon?”

“Yeah, until my jaw pops out,” I replied.

*NERVOUS LAUGHTER*

Luckily, my jaw did not pop and my CSF blood patch did not blow, but honestly, this was my least favorite test because worried about my Ehlers-Danlos syndrome (EDS) complications. My results showed a slightly delayed sympathetic response, unlike my tilt table test. Perhaps, my body was starting to wear out from testing. This test triggered a wicked headache that built for 12 hours.

Quantitative Sudomotor Axon Reflex Test (QSART)

After the surviving the tilt table, a little electricity didn’t fluster me at all. Four small plastic cups were taped to forearm, foot, and leg to measure amounts of sweat, or sympathetic response, generated by a mild electrical current. Sympathetic nervous system

“It does feel a little prickly, but I think you’ll be fine,” my doctor said. “How are you feeling?”

“Prickly,” I said.

“Maybe that’s an understatement,” she said.

“Yep,” I said.

WHO THE HELL DESIGNED THESE TESTS?!

For me, this test was tolerable, not really any worse than a MCAS skin reaction. My sweat was slightly low in a few places. No surprise, as I often skip my deodorant stick with no repercussions.

As barbaric as these tests may sound, I am now very grateful I was able to access them.

My doctor diagnosed me with hyperadrenergic dysautonomia. Is it possible I had POTS, another type of dysautonomia, at one time? Very likely. The tests can inform treatment and further testing, but just like MCAS testing, the results can change over time and treatment still requires trial and error. I also tested negative for small fiber neuropathy (think tiny melon ball-like scoops of skin).

I’m looking forward to sharing my major improvements on new treatments– not beta blockers, which can exacerbate mast cell reactions. Aside from treatment, there’s a lot of value in a formal diagnosis for other providers to understand my body’s reactions and to support disability accommodations. For example, one doctor was so obsessed with my high blood pressure that she dismissed my CSF leak. Now I can explain my hyperadrenergic response.

After my long day of autonomic testing, my friend asked, “Do you want to go out and celebrate?”

“Yes,” I said, suffering from one of the worst migraines of my life.

TAKE THE DAMN PICTURE.

May 2021 Update

MCAS remission eliminated my dysautonomia symptoms completely. Dr. Weinstock estimates MCAS accounts for 1/3 of POTS cases. While autonomic testing helped me understand dysautonomia, pursuing MCAS treatment was a better use of my resources.

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Category: Dysautonomia