Chronic Illness Life with Ehlers-Danlos Syndrome: Postural Orthostatic Tachycardia Syndrome (POTS)

Two years ago life changed as I knew it.

The  Reasons: A genetic condition called Vascular Ehlers-Danlos Syndrome (vEDS) and a disorder of the autonomic nervous system known as Postural Orthostatic Tachycardia Syndrome (POTS).

While I talk incessantly on EDS, I should talk more about POTS because it’s a syndrome. This means POTS occurs for a number of reasons.

Syndrome is defined as "a group of signs and symptoms that occur together and characterize a particular abnormality or condition."
[IMage Description: Pexels.com photograph edited for free use. The photograph is black and white showing a person lying face down on the ground amongst a busy walkway, slightly out of focus from the viewer. A test overlay from OnlyinthisHead.com Chronic illness life with Ehlers-Danlos Syndrome and Per Merriam-Webster, Syndrome is defined as “a group of signs and symptoms that occur together and characterize a particular abnormality or condition.”]
POTS is why I can’t stand and vEDS is what causes my POTS as well as almost all my other medical issues over the years. From Multiple Sclerosis to Traumatic Brain Injury, dysautonomia presents differently for everyone with POTS. In short, dysautonomia keeps the body’s fight or flight reaction stuck in an “all-systems-go” mode, creating multi systemic effects.

"Dysautonomia is a term for a group of diseases that include postural orthostatic tachycardia syndrome (POTS), multiple system atrophy, autonomic failure, and autonomic neuropathy. In these conditions the autonomic nervous system (ANS) does not work properly."
[Image Description: Pexels.com photo edited for free use. Photo graph shows one black chess piece sitting upright and a white chess piece lying on its side. The background of the photograph is white Overlayed in black text is OnlyinthisHead.com Chronic Illness life with Ehlers-Danlos Syndrome. There is a quoted definition per Wikipedia, “Dysautonomia is a term for a group of diseases that include postural orthostatic tachycardia syndrome (POTS), multiple system atrophy, autonomic failure, and autonomic neuropathy. In these conditions, the autonomic nervous system (ANS) does not work properly.”]
Unpredictable levels of disability result. While a time-limited frustration for some, in others POTS, presents dysfunctional processes building years of damage atop an already weakened system. The latter often results in early debility with a hefty helping of insult to multiple injuries.

My Personal POTS Story

In my case, the exhausting POTS dance is longstanding. I remember POTS symptoms since ten or eleven years old. What I mistook for normal is ANYTHING BUT!

Symptoms Associated with POTS
[Image description: Pexels.com photo edited for free use. The photograph depicts a close up of an incandescent light bulb glowing bright orange red and broken glass and smoke represent the light bulb is burning out. Overlaid in white text on the photo is Symptoms of an Autonomic Nervous System Burnt Out with POTS. The symptoms are listed in a white, chiller font: Racing Heart when Up, Fatigue, Headaches, Migraines, Lightheadedness, Heart Palpitations, Exercise Intolerance, Nausea, GI Problems, Brain Fog, Tremulousness, Fainting or Near-Fainting, Cold, painful extremities,
Chest pain, Shortness of Breath, Orthostatic Intolerance.]  OnlyinthisHead.com | Chronic Illness Life with Ehlers-Danlos Syndrome
POTS symptomatology sprinkled my medical records since my early twenties. Signs dismissed under common mental illness diagnoses went to the wayside when my gastrointestinal system flared royally at twenty-six years old. My primary focus became taming those symptoms.

Insidiously, POTS cloaked itself as a primary result of mental illness. Then as a secondary effect of GI-related metabolic issues for several years.  Truly, I see POTS as a master deceiver to the diagnostician. My lengthy narrative stands as one of many.

Regrettably, only after a major work injury came recognition of processes complex, difficult to manage, and eluding diagnosis connecting the tandem effects of vEDS and POTS. Between 2015 and now, vESDs and POTS knocked me further still than I’ve yet to reascend. 

Picture from the hospital admission that lead to my officially POTS diagnosis in 2016.
[Image Description: Selfie of author in hospital in January 2016 for a POTS exacerbations and nutritional malabsorption. In the photo is a woman in her mid-thirties with long brown hair and a gray streak, parted to the side. She looks tired and pale with dark eyes. She is not smiling and has her head tilted to the side. She is dressed in a hospital gown with EKG patches and a central line access and dressing visible.]

On a Positive Note

I’m now under POTS specialty management, an integral part of care with autonomic nervous system dysfunction.

Thankfully, completing this extremely important part allows for unequivocal evidence of what I have and  how one manages it. A consult like this follows you throughout the journey as a safeguard when encountering less informed practitioners. Locally, the practitioners continually state the limits on managing my case. Over the last six months of local practitioners basically saying “You’re too complicated and need to go to (insert preferred university hospital here).” I finally get the hint. I accept the referral but the fact my local system lacks skill needed for complex health conditions troubles me deeply.

Grievously, so much irreversible damage is done for the years unmanaged and each set back I encounter seems to drive me down even further. I cannot recoup these losses. It’s enough for most all practitioners giving the grim “You’ll Never Get Better” speech over and over. I hear ya Doc, I can only expect mild improvement and I’m not a candidate for surgery…blah, blah, blah…tell me something I don’t know. 

What Diagnosis Did for Me

POTS and vEDS caused me multiple health crises over fifteen years local doctors never cared to understand.

After transversing the referral gauntlet to no avail, revealing the cause before the effect provides validation for the suffering. I only entertain thoughts knowing “sooner” may equate with a better outcome to motivate my awareness efforts. Dwelling in sadness only begets more despair. Plausibly, finding answers is the most bittersweet moment of my life in understanding the “Why” behind my lifelong relationship with pain and strife.

My official Ehlers-Danlos Syndrome and Postural Tachycardia Syndrome diagnoses ended one journey and began another. Where I struggled for appropriate referrals in the past, now there is no question. Be that as it may, new challenges present reflecting lack of education on new research concerning POTS as well as EDS types. For this reason, my cardiologist referred me to the most revered POTS specialist for the region. In my research, I knew countless POTS patients traveled to see this doctor from all around the nation.

The following link is an affiliate link meaning if you use this link to purchase this book I make a small commission. Please see the Disclosures Page for more information.

The Calling by Blair Grubb M.D. | This book is a collection of memoirs from the well-renowned Autonomic Nervous System Disorder and POTS Specialist. In the short stories, Dr. Grubb reveals the meaningfulness of his journey through life as well as healthcare. Many of the stories brought tears to my eyes, helping me understand there’s is beauty, even in suffering. Click here to purchase on Paperback or Kindle via Amazon!

After nearly a year on the waitlist, we made it to Dr. Grubb’s Autonomic Nervous System Disorders Clinic at the University of Toledo for more functional POTS care so maybe I can stand and walk again or at least not feel so worn out from the surges of tachycardia unmanaged currently.

It was everything we’d hoped for and more! In the last few years of losses, this appointment gave us hope Life will get easier to manage and if not, at least we’ve got the right specialists on our team!

If you’re struggling with POTS symptoms, ask your doctor about a TILT table test. This confirms the diagnosis when read by an electrophysiologist skilled in autonomic conditions affecting the cardiac and vascular systems. Despite common misconceptions, blood pressure changes neither confirm nor deny a POTS diagnosis. Blood pressure changes vary depending on processes behind the syndrome.

TILT Table Test to Confirm POTS
Image Credit: Mayo Clinic 2016 [Image description: drawing of the tilt table test used to diagnose POTS. The patient is strapped to a table raised to a vertical position. The healthcare providers measure blood pressure pulse oxygen saturations and signs of orthostatic intolerance. A POTS diagnosis is confirmed with an increased, sustained heart rate greater than 30 beats per minute with postural change.

Typically, an increase equal or greater than 30 heart beats per minute wth postural changes coupled with symptoms of orthostatic intolerance reproduced within thirty minutes of the position change confirms a POTS diagnosis.

Affirming diagnosis with a TILT test opens access for POTS and genetics specialist consults, perhaps the most important move after ruling out time-limited causes. At this point, it’s extremely important knowing your patient right for second opinions. I found charting my symptoms, vital signs and triggers in a note I request included in my chart expedited the process. Genetics helps determine the cause behind POTS and the POTS specialist helps coordinate therapeutics treatments along with recommendations for relevant follow-ups from other practitioners.

The appointment was approximately two hours with a thorough review of my medical records and subjective history. The important points in my medical history other practitioners previously dismissed received recognition during this appointment. With that emerges access to better-suited treatment modalities. I felt understood and finally confident all the appointments leading me to this one were somehow worth it.

Do what you must to get the right help!

It will take time but you’ll get there!

If I did it you can too!

A Moment of Gratitude

Certainly, getting this far was no small feet I could accomplish on my own.

Without the everlasting loving support from my soulmate, love of my life, and best friend Seth, I wouldn’t have made it this far in getting my health issues sorted out. Thank you from the bottom to the top of my tachycardic heart for all your sacrifices.

Another person who helps by all mean necessary is my ma-in-law, Leslie. Her support and love takes us many miles. She’s a compassionate soul I am thankful for every day.

Additionally, continued support from my parents (Rick & Linda) makes a time of isolation and loss one of the good memories, interesting conversations, and a lot of laughs!!! My folks went many miles to ensure I wasn’t alone in my health crises over the years. Their love and willingness to help is a safe haven I am honorably grateful for. Everlasting love for my Ma and Pa.

Of course, without my kiddos (Emarie, Jordan, and Gavin), my drive in deconstructing this puzzle might use a lot omf! Aside from the obvious benefits, I did it so my children might know what troubles to watch out for. The thought of them going through the painstaking processes I did for answers breaks my heart! I love them with every bit I have to give!

The journey is Far from Over

Conclusively, things are falling into place. Thirty plus practitioners from 2014 to 2017 and still counting. Then fifteen plus in the decade before that. this is typical for both POTS and vEDS. No one should wait so long! 

Raising awareness with my story succeeds the purpose even if only one person benefits from the retelling!

I share my story because I want to help other people, like me with health problems no one could explain. Trust your gut. Get a second opinion. It’s not only in your head! Don’t delay as these problems demand attention left festering alone! Please share to help spread awareness and thank you for reading about my journey ❤

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