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My goal is to help provide EDS & Dysautonomia patients with resources to discuss with their healthcare team.

POTS? Ehler's Danlos Syndrome? (Journey Part 1)

POTS? Ehler's Danlos Syndrome? (Journey Part 1)

If you have found my blog, it's for one of two reasons. 1 - you're a friend or family member  and want to know more about my conditions 2 - you or someone you know has recently been diagnosed with Ehler's Danlos Syndrome (EDS) or POTS. Either way, thank you for reading :) I hope I can shed some light on the conditions. I have the Hypermobility type of EDS; this means that my joints are incredibly flexible in comparison to the norm. The test (Beighton Score) for EDS is simple and I've pictured it below. I scored a 7/9, it turns out my elbows are the only stable joint in my body.

I have been doing these 'party tricks' for years. I can also pop out my shoulders on command (which has always made raising my hand in class difficult). My hips are not stable either and my knees have had chronic pain since I was 16. But this has been mynormal. I knew I was hypermobile (that my joints move more than they should) but no one in my life understood this wasn't normal and that I needed medical care.

I didn't put all of the pieces together until almost a year after my initial diagnosis of Postural Orthostatic Tachycardia Syndrome.

Let me back it up a little bit...I went from being in the best shape of my life, I was weight lifting 3-5 days a week, eating clean, working 20+ hours a week, and taking 18 credits to finish up my undergraduate degree in communication. Working out was my life and my stress relief

At the age of 26, I went from running in the Spartan race in November to being bedridden from January until March (when my health insurance finally kicked in and I could see a doctor). I knew something was wrong but I had no idea what it was. I was nauseous all the time (the mouth-watering-feel-like-you're-about-to-puke kind), I got dizzy when I'd stand up, I could feel my heart pounding in my chest (heart palpitations), and things would get dark and hazy.

The night of March 1st, I went to the ER because I knew something was wrong with my body and I couldn't handle the debilitating nausea any longer. They put me in a wheelchair and rolled me back to a room. They ran some tests because of my heart palpitations (chest X-ray, blood work, and an EKG). They gave me Zofran for the nausea and a total of 3 liters of saline because I was dehydrated. They took my blood pressure (BP) and the nurse said I had really good blood pressure because it was around 90/60. Little did she know how wrong she was. Afterwards they told me to make an appointment with my general practioner (I didn't have one yet...I'd been without health insurance for about 2 years at this point) and sent me home with care instructions for 'anxiety' and dehydration...I was feeling defeated at this point.

I knew something was physiologically wrong with me. It wasn't all in my head. Almost passing out every time I stood up, wasn't normal. Being nauseous all the time, wasn't normal. What was wrong with me?

I searched and found a doctor that was a short drive from my apartment. She wasn't sure what was wrong either. My symptoms varied so much: nausea, dizziness, chronic pain in my joints, heart palpitations, insomnia, and sleeping too much at times.

She made some referrals - orthopedist (for the pain in my wrist and knees), an ENT (to see if there was an imbalance in my ears causing my dizziness), and a head CT to see if something was going on in my brain.

2 weeks passed - Head CT came back normal

3 more weeks passed - Orthopedist told me I had to pick either my right wrist or my right knee because they could only treat one per appointment... Gotta love Western medicine, because clearly my issues are compartmentalized. I chose my wrist because it had been bothering me more than my knee. He took X-rays and manipulated my wrist around. I showed him how when it clicks I can get relief and that I feel pressure building before the click. He said that he saw some extra mobility in my wrist but that my only option would be surgery, which could cause a loss of mobility. I chose to accept the wrist brace he gave me for bad pain days and never went back.

Another month passed - The ENT did a bunch of weird tests that involved me on an exam table while he tipped my head a bunch of weird ways quickly. He also looked in my ears, nose, and down my throat. Everything came back normal, none of my symptoms had been replicated. He had me sit down in the chair as we talked at the end of the appointment. Had me breathe in and out quickly (almost like hyperventilating), he called this a poor-man's vasovagal test, for about 2 minutes. Afterwards, he asked if I was experiencing any symptoms. I said yes, but not as severe as normal. He said "you need to schedule an appointment with a cardiologist as soon as possible." Finally, I was starting to get somewhere.

I talked to my General Pracitioner (GP) again and told her what the ENT had said. She gave me a referral to see a cardiologist a few days later.

The cardiologist did an EKG (so many sticky leads on my body and the adhesive left marks on my body that didn't go away for 2 weeks) and then did something called Orthostatics. This is where he took my BP and Heartrate while lying down, sitting up, and then standing for a total of 10 minutes. I felt nauseous, uncomfortable, and my symptoms had definitely returned. He told me he wanted to schedule a tilt-table test and that he might know what condition I have (the tilt table test would confirm if he was right). Things started moving quickly now, and he was able to secure my appointment for 9am the following morning at the hospital.

I wasn't allowed to eat or drink anything that morning and I drove myself because the appointment had been last minute. Biggest mistake of my life. I arrived to the hospital later than I was supposed to because I had overslept at my brother's house but was still early enough to have my test done (they wanted more time for me to fill out paperwork but it turns out I'm a pro at paperwork and only needed a few minutes).

The nurse came in, and told me she was going to get my IV of saline started. I told her I pass out at the sight of my own blood, so I wouldn't be watching what she did. We made small talk to distract me for what she was doing. She stuck my arm wrong and I could feel the blood pooling on my hospital gown. I tried not to think about it.

A little while later, the nurse put me in a wheelchair (ughhh, I don't like being in a wheelchair. They're always too large for me and I can't place my feet comfortably; not a big fan of spreading my legs in public so I can put my feet on the foot rests) and they wheeled me into the testing room.

They had me lie down on a table and explain they were continuing my IV of saline, would tilt me up, that I was to report my symptoms, they would not talk to me throughout the test (so they wouldn't interfere with the results), and then if I didn't experience all of my symptoms - they would introduce norepinephrine into my IV. They also hooked me up to several leads. I don't even know how many sticky things they put on my body. (The next day I was covered in red marks and it looked like I had weird hickies all over). The staff of about 6 people (including my cardiologist in the adjoining room, behind a glass window) monitored my blood pressure and heart rate.

They tilted me up and I told them I felt uncomfortable and that normally when I felt like this, I would lie down and my symptoms would subside. I was stuck feeling uncomfortable, nauseous, slight heart palpitations, and an overall feeling of desperately wanting relief. It felt like these 10 minutes took forever, it was torture.

Next they told me they were switching my IV over to the norepinephrine. Almost instantly I felt 100x worse. I told them I was getting overheated, I could feel sweat pooling on my lower back, things started going black, and then I said "I'm gonna....I'm gonna... I'm gonna pass out..." They kept me in this almost passing out state for probably less than 30 seconds (but it felt a lot longer) as they told me to hang on so they could monitor my body's reaction.

They tilted the table back down so my feet were now slightly above my head. They told me I did great, switched my IV back to the saline, and let me lie there until I felt ready to get back in the wheelchair.

The nurse wheeled me back to my room and gave me a menu of hospital food to order from. I picked the breakfast burrito and she brought me orange juice.

I was still disoriented and not feeling well but I wanted to know what the test had concluded. I don't remember much (because I had just passed out 20 minutes prior) but they handed me a packet of information on Postural Orthostatic Tachycardia Syndrome, told me to drink more water, gave me a prescription for Flornef (to increase my blood pressure) and another for Metoprolol (to decrease my tachycardia-fast heart rate) and that I needed to increase my daily salt intake to 4x that of a normal adult. My cardiologist joked and said "you won't hear this often from a cardiologist but you really need to increase your salt intake. Salt Everything."

*About a year later, I went back to my cardiologist and wanted to know the details of what happened during my tilt table test. Upon being tilted up, my heartrate increased by more than 30bpm (beats per minute), which is a diagnosis by itself for POTS. My BP hovered around 90/60 for most of the test (normal is 120/80). When they introduced the norepinephrine, initially my heartrate (HR) skyrocketed and then my BP and HR plummeted to 60/30 and 30bpm. This rapid drop is called Neurocardiogenic Syncope*

I finally had an answer, Postural Orthostatic Tachycardia Syndrome and Neurocardiogenic Syncope. But what did this mean for my daily life? I'd never taken daily medications. I'd always been relatively healthy. How was I supposed to carry around at least a gallon of water with me every day? Would my symptoms finally subside? These were questions I wouldn't find answers to until I made a post on Facebook to update my friends and family on medical journey.

I posted that I finally had a diagnosis and one of my friends responded "Welcome to the Spoonie Club". What? Spoons? What does that have to do with POTS? Was this just a pun on the acronym? Nope. She sent me a link to The Spoon Theory (www.butyoudontlooksick.com)

I asked her so many questions and tried desperately to figure out what my new normal was going to look like. She told me to look on Facebook for support groups and I was pleasantly surprised with how many people were on them and active! My friend warned me that for some people it was their only place to vent and to be careful not to get caught up in the negative posts (not to wallow in their misery) because not every day is a bad day. And to remember that no matter what, I am capable of whatever I push myself to do (I just needed to understand my physical limitations so I don't burn out).

This began my journey of finding my new normal. I'll post more about it next time. I hope you have enjoyed reading the beginning of my complicated diagnostic journey. There's more to come and I will do my best to let you in on what I've learned from all of this.

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The Journey Continues (Journey Part 2)

The Journey Continues (Journey Part 2)