Can you Validate Me Please?

My one goal in life with EDS: Hang on.

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Honestly, as a group, we just need to be thought of as a parking passes in need of one thing - validation. Having a disorder that affects collagen, we lack the proper glue to hold our bodies together. Life with faulty collagen is exhausting. All your muscles are constantly engaged in hopes of helping out their slacking friend, collagen. We are known to be Cirque Du Soleil flexible and then as a result, riddled with arthritis later in life. It is not a stretch (pun intended) to think that the medical community would recognize this disorder for what it is - complicated and real. They have the complicated correct, but seem to struggle with the "real" component. Too often the medical community stitches the holes in their knowledge using patient shaming sutures. We become so beaten down by the hail rained down upon us during our medical journey, it leaves us bruised and sometimes hesitant to stand up for ourselves being fearful of having to face yet another medical humiliation. Solace is the cave called home that we return to and hide. I once had an experience with a primary care physician who believed I had Lyme Disease. I remember the after hours calls and the kindness that permeated her voice as she lay out a treatment plan for me. The Lyme diagnosis was incorrect, with its treatment wreaking havoc on the underlying and yet to be diagnosed EDS which is frequently misdiagnosed for years. I often think of my dear friend who overcame cancer. Her journey was quite different. As horrific as it was, she was never demeaned by being told the cancer was "in her head", or that if "she just lost weight her cancer would go away", she didn't need to find her own specialists only to find that they did not participate in insurance because reimbursement was too poor for her type of cancer. Her friends and family didn't judge her when she had to cancel plans because she felt poorly or have them decide the issue was really hypochondria. BTW, I met my high deductible insurance plan's in and out of network deductible  in 3 months this year. The judgement award goes to a stranger who chastised me in a parking lot for not ​ returning my shopping cart to the bin (too many steps away on a hot day my body would not tolerate) When I calmly said I'm not well today, she responded with you don't look sick. I'll take that validation now please.​

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Pass the Gorilla Glue please.

Does this cave have air conditioning?

Seen (for more than 5 min) and heard

​Like so many other people having these genes in their pockets, it took over 10 years for me to receive an official diagnosis. Why? EDS symptoms are weird, inconsistent, pile up and while known to be genetically based, the specific gene has not been identified in all of the EDS subtypes. Also, in the eyes of some doctors (and when I was younger, many), I am just another woman who suffers from hysteria, needs bloodletting and then psychiatric care. I am joking (kinda), but seriously folks, what illness takes 10 years to diagnose?? In the age of rarely earning more than a ten minute appointment with a doctor, unless they are concierge, it is very difficult for a doctor to assess a complicated medical history, consider the a diagnosis then and then determine a course of action. To give you a picture of just how hard it is to be appropriately cared for, here is a list of my various misdiagnoses, how long it took to obtain the correct one and the doctors I saw on my diagnostic journey. This is not a "poor me" story, it is included to provide awareness of the inner workings of EDS.  Here goes:

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• Torn ligament in ankle - one year to diagnose, diagnosed as "normal ankle" - 3 orthopedic specialists. "It's complicated".
• Cerebellum (hind brain) hanging down into my spinal column (nope-not where it belongs) - symptoms started at age 13. I was 40 years old at diagnosis and surgical repair. - diagnosis: "You would benefit from counseling, common migraine - 2 neurologists, 4 primary care physician, 3 neurosurgeons, clearly present on MRI
• Anaphylaxis/Mast Cell Activation issues (MCAS - this has to do with your body's histamine response - histamine cells are everywhere in your body - people with MCAS have "leaky" MAST cells, which can cause rashes, anaphylaxis, belly issues, and more - my symptoms started in my teens, peaked in my 30ies, and I was diagnosed at age 50 - During one particular MCAS flair, I was having an asthma/anaphylaxis attack and sought care from the ER and left alone in behind a curtain, told to stop crying and given a diagnosis of an "anxiety attack"- 3 allergists, 2 ER visits, 1 allergy immunologist, 2 primary care physicians. This disorder is verifiable through a particular battery of tests.
• Gastroparesis - fancy way of saying a belly doesn't work well - took 5 years of intermittent vomiting to diagnose - Diagnosed as bacterial infection - 1 primary care physician, 2 gastroenterologists. Can be clearly diagnosed using a gastric emptying study.
• Traumatic Brain Injury - Missed even though I had 2 concussions within 9 months with a history of crowding (big brain, not so big skull) This was diagnosed 1 full year after the second concussion. Lack of treatment was justified by this repeated phrase: You can't have a TBI as a result of a low impact car accident (you can and do when you have little room for your brain in your skull) - 2 neurologists, primary care physician, 1 neurosurgeon, 2 osteopaths, 1 orthopedic surgeon
• Cerebral Spinal Fluid Flow issues -I have a shunt in my brain to regulate spinal fluid flow, which was diagnosed after 2 years of seeking answers for debilitating symptoms. Prior to that, the diagnosis was "I don't know" - 1 orthopedic, 1 surgeon, 2 neurologists, 1 neuroradiologist. This was indeed complicated, but it took one neurosurgeon to dive into "complicated" and take a chance on a shunt.  I love my shunt more than I love chocolate. 
• Subluxations - In EDS, bones slip out of place or dislocate (remember the skeleton GIF above?) You rarely see these on x-ray, and in a some doctor's minds, if a tree falls in the woods and you don't hear a sound then it never fell - Multiple ER physicians, primary care physician, physical therapists, orthopedic specialists and osteopaths. Don't get me started.

*Side note: I personally researched and sought out all the physicians I saw when I needed to verify a diagnosis. I did not have  any coordination of care. I have not diagnosed myself incorrectly once.

The interesting thing about this list is that all of these issues are classically related to EDS and are documented in medical literature.  Imagine going to your doctor and having them ask you why you are there. In response you say: Well doctor, my head hurts, I'm throwing up, I keep getting random rashes and asthma attacks, my knees feel 80 years old, my fingers keep locking up, I want to pass out if  I stand up too fast, I'm losing my hearing in my left ear, I have this ringing in my ears and I think I might be depressed. I give credence to the difficulty in our diagnostic process, but feel like my intimate relationship with the medical community is often abusive. My years of searching has finally paid off. My doctor hunting has resulted in my team of 5ish doctors that trust my judgement, but I still feel very vulnerable when I have to consult a new physician.

In my experience, meeting various physicians; orthopedic surgeons, physical therapists, neurosurgeons, neurologists, osteopaths, allergists, gastroenterologists, rheumatologists, ophthalmologists and primary pare physicians sheds light on one thing; People who work with EDS all agree that we are a "complicated" lot. Whether they choose to sift through the complicated to learn about EDS and treat or refer on to an appropriate next physician is, in my mind, what makes a trustworthy practitioner. Doctors are taught that if it walks like a duck and quacks like a duck it is indeed a duck.  BUT, when it comes to EDS,  if you hear hooves, and your treatment plan is failing, you need to think Zebra.  In addition, all Zebras have different stripes, so one EDS treatment plan may not suit EDS patients. This is why the Zebra is the mascot for Ehler's Danlos Syndrome.

Not all doctors are McSteamy

It's genetic, this hoof sound means a zebra (not a horse) and all our stripes are unique.

 Lastly, in keeping with EDS awareness month, I would like to leave you with a TicTok. Who doesn't love a TicTok! Even better, I'll top it with a blooper reel.  In true EDS form, I want to let you know that 2 zebras were harmed in the making of this TicTok. Mom zebra subluxed 2 ribs, needing ice, rest and a physical therapist's touch. Daughter zebra promptly passed out after our little project was complete (thank you dysautonomia and POTS). This is not a rare occurrence as many EDSers suffer from Postural Orthostatic Tachycardia, better know as POTS and dsyautonomia. Dysautonomia is the body's inability to regulate what should happen automatically (blood pressure, breathing, heart rate, etc.) These disorders have received more attention of late given their odd occurrences in people who have had COVID. Enjoy; The TikTok not POTS and dysautonomia (should you have them - no fun at all)!

Learn more about Elher's Danlos Syndrome