“It’s complicated” AKA my journey to diagnosis

Lately I've been asked by so many how or when I was diagnosed with POTS. "What were your symptoms? How did they diagnose you? What medicine do you take?" and so much more.

The most disheartening question I get asked? "How did you get doctors to believe you?" It seriously breaks my heart that that's even a question. As you read this you'll learn that so many friends, families, and yes, even doctors, "experts" told me "it was all in my head" that I started to believe them. I'm so thankful that I kept pushing and advocating even during that time- and I hope to encourage others to do the same.

I never thought I would find myself writing such an in depth blog of the last 5(ish) years of my medical journey, but I've decided to share in case it helps someone else manage their own care more appropriately. I remember googling my symptoms more times than I can count and being frustrated at the lack of information out there from fellow chronic illness sufferers. I wanted anecdotal stories and to connect with people who understood, I so badly craved blogs just like this one. I hope that by sharing this I will help others understand what I've been through and what this journey is like. I hope this information will find its way to someone that needs it, someone who can avoid having a diagnostic delay like I did. I hope my words provide hope and encouragement to those who so desperately need it.

Keep in mind that this story is nowhere near over. I still struggle with refractory tachycardia and continue to bounce between specialists, trying to achieve as close to "normal" as we can. Just this week after writing this post I spent 6 hours in the infusion chair trying to get my heartrate under control- buying me a one way ticket BACK to cardiology. Truth be told this is a hard story to tell, mostly because it doesn't have a definite beginning or ending. But without further ado... here is my extremely long-winded and detailed account of my personal journey to diagnosis.

Honestly, looking back, It's hard to pinpoint when my symptoms started or which ones were the first to appear.

I guess the first time I ever remember anyone mentioning tachycardia was in the ER my freshman year of college, so we will start there.

Fall 2010

I had been sick for weeks with all different things. Multiple bouts of strep, 2 cases of epiglottitis (super rare in adults!), and more viruses than I can count. But on this night, I had broken out in a terrible diffuse rash and along with a cough I was suffering from a lot of chest pain - thus, my first ER visit. After a physical exam and chest x-Ray revealed "bacterial mycoplasma pneumonia" (the first misdiagnosis of many), I was told I could be discharged with antibiotics once my heart rate was more regular (under 100 beats per minute at rest). Wait what?! I'd never heard anything about a fast heart rate and had no history of any cardiac issues. After a few liters of fluids and no heart rate change, the doctor released me and said "this must just be your baseline and you never knew it. That, or it's your body's reaction to this bacteria". I didn't give it a second thought and wouldn't hear the word tachycardia again until a couple years later.

After a few rounds of antibiotics with no resolution to my "pneumonia" symptoms, I fought through my first ever college finals week before breaking out in another, different, distinctive rash. I'll always be thankful for that one because it's what eventually led to my accurate diagnosis of Valley Fever. 3 months of anti fungal medications and I finally was back to "normal".I thought this was the end of my foray into the patient world but looking back I can see that this saga really was just the beginning.

2011- 2012 (ish)

Halfway through college I started suffering from nearly daily headaches and occasional debilitating migraines. I started seeing a neurologist who did a full neuro work up which revealed my first of many "chronic" diagnoses: chiari malformation. The neurologist really wasn't impressed by the size and severity and said it was likely an incidental finding and continued to treat my migraines using more conventional treatments and prophylaxis. I tried every rescue med under the sun with no relief - oral medications, at home injections, IM injections at the office, and on and on. Nothing seemed to work.

One day at a normal follow up at the neurologist, it was noted that my heart rate was consistently high when they took my vitals at check-in. The neurologist started me on propranolol- a beta blocker than can also help prevent headaches. "Let's kill two birds with one stone", I remember him saying. Up to this point, I had no cardiac symptoms and had no idea that my heart was beating fast. Fast forward a few months and with no improvement in either heart rate or headaches, I received my first referral to cardiology.

After an extensive cardiac work up (echocardiogram, a million EKGs, a stress test, 48 hour holter monitoring) I was given yet another referral, this time to an electrophysiologist. There, it was made official that I had "inappropriate sinus tachycardia". I remember laughing at the name - surely that can't be a real diagnosis?! In layman terms- my heart was functionally and structurally perfect. But for some reason the electrical system was over firing at all times and causing my heart to beat rapidly. Still to this point I was completely asymptomatic from a cardiac standpoint and felt that all the tests were superfluous. The cardiologist suggested a switch to a different beta blocker that better controls heart rate, along with another cardiac med to add to the shuffle. I remember asking "is this necessary? I feel fine aside from my constant headaches....". Because of the average heart rate found on my holter monitor - I was told being off medication was at this point not an option. I was told that if I continued to let my heart beat this fast with no medical intervention, it could eventually lead to cardiomyopathy. A.K.A. BAD NEWS BEARS. And thus begun the more permanent journey with multiple cardiac meds each day.

Fall 2014

After graduation, I gratefully accepted a Child Life Internship and spent 15 weeks in Southern California learning so much about Child Life and healthcare in general. Looking back, I did have a few "episodes" of dizziness and lightheadedness during these 15 weeks- but being that I was new to such an intense medical environment (especially the burn intensive care unit) I just assumed I was overwhelmed by what I was seeing.

Winter/Spring 2015

As my internship came to an end, I returned briefly to Arizona and had just finished unpacking before accepting a job - my dream job - in Orange County, California. I packed my bags once again and back to California I happily went, so excited for the adventure I was embarking on. Luckily, during this time everything was pretty well controlled with minimal cardiology follow up. I only had to visit the doctor once a year. I was taking 2 cardiac medications a day and experiencing remarkably few symptoms. I would have palpitations and a fast heart rate occasionally, usually in the mornings when I got out of bed. But in general, health wise, I was in a really great spot.

May 2015

In May, I was honored to stand by my college roommate as she married her best friend. During the ceremony, I started feeling terrible. My heart was racing, I was dizzy, and I had spots in my vision. It was an incredibly warm Phoenix day and we had been non-stop busy all day, so this "episode" was chalked up to heat and dehydration, and I didn't think much of it after the fact. At the time, it seemed like a one-time fluke.

Winter 2016

And as all stories go.... little did I know that was all about to change. Almost a year into my Californian adventure, I started experiencing pretty severe and frequent "pre-syncope" episodes of varying lengths. These episodes were similar to the one I had back in May, but they were happening pretty consistently and were much more intense. I would start by feeling suddenly incredibly tired: yawning nonstop, randomly feeling like I hadn't slept at all. Soon after, I would get shaky, flushed, and sweaty. If I didn't sit down right away I would begin to have trouble breathing, lose my vision, and get a loud ringing in my ears. These "episodes" were first very sporadic but then started becoming more and more frequent and were really getting in the way of my active job. In the meantime, my migraines had also increased in both frequency and severity and I found myself making multiple trips to the ED at work, desperate for relief. One day, I had my blood sugar checked on a hunch that I was hypoglycemic, but it was stone cold normal right in the middle of a symptomatic day. On this day, the symptoms were bad enough that for the first time I had to go home "sick" from work. At this point, I knew it was time to seek out a local cardiologist to continue managing my care. Luckily, I was squeezed in for a last minute appointment and a STAT EKG that day where my heart rate was found to be in the 120s. I was out of breath and feeling almost constant palpitations. This, mind you, with a hefty dose of my normal beta blocker already on board. The cardiologist felt pretty strongly that my increase of symptoms was due to the stress of my new career. He increased my beta blocker dose and encouraged me to follow up with a PCP regarding anxiety. Leaving that appointment, I felt completely defeated. I knew deep down that these symptoms and episodes were due to a physiological cause. Sure, feeling like I was going to faint out during an intense trauma assessment or invasive procedure caused me some anxiety, but the physical symptoms came FIRST, and I was determined to get to the bottom of it.

May 2016

Things continued to get worse as I prepared to move back home to Phoenix to accept a full time position in the same field. As things escalated and I got closer to the big move, I began to believe my symptoms might actually be stress related. I was incredibly conflicted about leaving California so soon, and I questioned my decision almost daily. I remember one frustrating day telling myself "if I get home, settled into my new job, and am still experiencing these symptoms, I will find a new doctor." If things improved, I figured, I could know once and for all that this was truly anxiety and that my symptoms had been made worse by this transition into both the workforce and to a new state.

June 2016

Once home and "settled", it quickly became clear that things were not going to improve. In fact, I started feeling even worse. A quick visit to my old primary care provider turned out to be exactly what I was hoping wouldn't happen: a quick once over of my symptoms and the NP quickly assured me, "honey, that's anxiety! You're describing a panic attack"

I PLEADED with her tearfully from the exam table. "I know I have anxiety. I always have. I have never denied being anxious. But I also know that these physical symptoms are being caused by something else. I need you to help me". "You'll do just fine", she said, "stop drinking coffee and start taking Prozac. Get lots of rest and you will feel better".

I left feeling defeated once again, but even more than that, determined. I started taking the prozac as directed, but I knew it was time to advocate for myself and I knew I wasn't taking "no" for an answer.

I called up the cardiologist who had originally given me the IST diagnosis years prior and requested an appointment. I had to wait a month or so to get in to his busy office, but I knew deep down that I needed to go back. Meanwhile, I had daily nausea that prevented me from eating normal meals, and was having to take Zofran more and more often just to function at my new job and be able to take the rest of my oral meds. I had an increase in the frequency of episodes, and they clearly were not related to procedural stress at work: I remember vividly the time I completely lost my vision in the middle of a grocery store. Sadly, I had become accustomed to surviving and even smiling through these near syncope episodes. I started scouting out chairs and exits everywhere I went. I stopped going out with friends for fear I would pass out. I limited my activity and only left the house when it was absolutely necessary. All of this, mind you, without telling anyone around me what I was experiencing.

July 2016

Finally, it was time to visit my Electrophysiologist once again. For the first time in a long time, I left an appointment feeling encouraged and empowered. As soon as I finished telling the Doctor what had been going on for the past few years and especially the months leading up to this appointment he said, "Sarah. This sounds like something completely different than the IST you originally presented with". I felt heard for the first time in a long time. I finally felt like maybe I wasn't crazy, maybe I really wasn't all that "anxious" after all. This doctor ordered a tilt table test to be completed for the next month, and encouraged me in the meantime to drink extra fluids, increase my salt intake, and continue to monitor my vitals at home. I had never even heard of a TTT, but a quick google search showed that it was the "gold standard" diagnostic exam for Dysautonomia patients.

August 2016

The day of the tilt table test (TTT) came and went pretty quickly and smoothly. For those who are unfamiliar with the TTT, it is pretty simple and straight forward. The test begins with the patient laying flat on and strapped down to an exam table. The patient is hooked up to a cardiac monitor, blood pressure cuff, and an IV is inserted. Prior to the test, "baseline" vitals are recorded. Blood pressure, heart rate, and oxygen saturation are the key components of this diagnostic test. In my case, my supine heart rate was somewhere in the mid 70s (a great number, thanks to my beta blockers. ha!) To begin the test, the exam table is tilted upright. The patient is instructed to inform the nurse of any symptoms so that they can be recorded along with the corresponding vital signs at the time of symptom onset. As soon as I was "tilted" I felt incredibly nauseated. I felt like I would throw up right away, but was able to get through that feeling and continue the test. Within a few minutes, I began feeling some very familiar symptoms. First came the yawning. Suddenly, I was lightheaded, flushed, sweaty, and dizzy. I informed the nurse who recorded these symptoms and responded "well, just so you know, your heart rate is 147". um. WHAT!? For reference, a "positive" tilt table test is one in which the patient's heart rate increases my 30 or more beats per minute. Mine increased almost immediately by more than SEVENTY beats per minute. Despite feeling miserable, the test continued. I was given a sublingual dose of Nitroglycerin, thought to help induce further symptoms and to see if the patient will faint. The Nitro made me feel even more terrible, but my blood pressure remained stable and I did not pass out or lose consciousness. Thankfully, the test was finally over! I was given IV fluids and time to 'recover" before I left to go home. I was told pretty immediately that this test would result in a Postural Orthostatic Tachycardia Syndrome (POTS) diagnosis, but was told I would need to follow up with my electrophysiologist for more information and treatment.

Leaving the hospital, I felt as if I had somehow run a marathon. My body was so incredibly exhausted, and my mind was overwhelmed too. I did what every patient is told not to do: I googled. I was terrified at the thought of a new diagnosis, but I was relieved to have my symptoms medically validated for the first time in a long time. All along, I had known that there was something physiologically wrong, and now it had a name: POTS. The information I found terrified me, but I was STARVING for knowledge. I read research articles, blogs, watched YouTube videos, constantly looking for anything that might be useful to me. The amount of different experiences and varying severity of this diagnosis was completely overwhelming, and terrifying. I remember making a conscious decision: I will NOT let this diagnosis define me. I will not become my illness. I will continue to work the job that I love helping others. I will KEEP FIGHTING.

Fall 2016

Being that I now had a "dysautonomic dysfunction" diagnosis rather than a cardiac one, I stopped seeing my electrophysiologist in favor of a local autonomic neurology specialist. At my first visit, this physician confirmed my diagnosis of POTS, and diagnosed me with another syndrome as well: Ehlers Danlos syndrome. This diagnosis, however, didn't change my treatment. I was told to continue with the beta blockers, and was started on some additional medications to support heart rate and blood pressure regulation.

Spring 2017

After nearly a year of managing my new POTS diagnosis with medications, oral fluids, salt, and compression garments, my doctor and I agreed that it was time to be a bit more aggressive. I started Cardiac rehab that same week, an intense 6-day a week program designed to retrain the nervous system and help control the heart rate.

Summer 2017

At my follow up, I mentioned to my neurologist that cardiac rehab was going well, but that I was still symptomatic and struggling to keep up with hydration in the Arizona heat. He prescribed IV fluids, as needed, up to 3 times per week. I was so hesitant to begin this treatment (and I've since written a whole blog detailing that experience. read it here if you're interested!)

And that brings us to today. "Keep fighting" was the goal and keep fighting I sure have. Since I received the POTS diagnosis (2.5 years ago) I have changed medications and dosages more times than I can count. I have started seeing a neurologist (autonomic specialist) and stopped seeing cardiology. I have started and completed a cardiac rehab program. I have started receiving IV infusions up to 3 times a week. I have advocated for IV medications to be ordered PRN to relieve my headaches. I have fought insurance with dozens of phone calls to stay at the infusion center that treats me so well.I am still a "work in progress". I continue to try new things and work hard to be open minded about treatment options. I continue to have persistent and refractory tachycardia, which may soon buy me a whole brand new cardiac workup. At my most recent follow up, my neurologist said "You are the perfect patient. you are doing everything right. I don't have answers to why you are still symptomatic. Your case is..... incredibly complicated". Yeah, not exactly what any patient wants to hear.When I look back on the last few years, it's easy to see what I have lost: Time. Money. Friendships. Freedom. Health. Countless activities. A certain amount of independence. The ability to be spontaneous.But if I look hard enough, I can also see what I've gained: Validation that comes with an appropriate diagnosis. A unique perspective on how to provide the best care for my patients. A voice and a platform from which to share my story. The courage to actually share it. A community of medical support professionals that has rallied around me on the hardest days. The opportunity to educate medical professionals on POTS and Dysautonomia, in an attempt to end the stigma and end diagnostic delay. Countless online "friends" who exchange stories and ideas and support. And probably the best unexpected thing that's come of all this? An incredible infusion care team full of compassionate nurses, some of whom have become my best friends. I don't quite know what I'd do without them or any of the other people that I have met along the way. And for that one small piece of this long journey, I am thankful.

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If you've read this far and you're still with me, THANK YOU. Thank you for encouraging me to share my story . If you are a current POTS patient or believe you might be, check out Dysautonomia International for information, research, and resources. If you are looking for a specialist in your area, there is a comprehensive list of respected specialists HERE. If you are struggling emotionally because of your symptoms and the effect on your life, please, please reach out. You are not alone, and you are not crazy. There are multiple crisis lines you can contact by clicking HERE.

To all the fellow Dysautonomia warriors out there reading this: Advocate for yourself. Don't let doctors convince you of something you know is not true. Ask for further testing. Ask to see a different physician. Record your symptoms so that you have information and data to arm yourself with. Do your own research on medications and treatments. And above all else, KEEP FIGHTING.