My Life With Ehlers Danlos Syndrome, A Synopsis: UPDATED
Updated: Mar 20
Update: As a result of my vascular EDS, I had 2 strokes in 2018 - the first occurring around June (was considered mild, but resulted in unsettling hallucinations,the likes I'd never known), the 2nd stroke - a massive one - occurred on October 12th. I went to sleep that night and woke up hours later in a hospital bed unable to move the left side of my face, my left arm, and only slightly move my left leg.It took a team of neurologists to consult on my case, because I was the first vascular EDS stroke patient. in the history of Southdale Hospital.
I was able to recover my memory in 2 days - still don't remember much from that entire day, even before the stroke. After a a short while in the hospital I went to a Temporary Care Unit (TCU) for therapy and rehab, where, once again, I was the first vascular EDS stroke patient. in the history of their facility. Within a week of being at the TCU, The road back to walking was much more difficult - and took much longer.
I was told by my in-home physical and occupational therapist that I would never walk again without the use of a walker or cane... that was over a year ago. Since that time,I've needed NO cane, walker, crutches etc. to walk. I guess I hated the idea of anybody telling him what I could or couldn't do. Nevertheless, it was extremely hard work fighting for each inch; each step; each movement. As you read the myriad of health issues outlined below, imagine how much more grueling and painful it made my rehabilitation and how miraxulous my level of recovery is.
I made a promise when I was diagnosed with this disease at the Mayo Clinic in Rochester, Minnesota, back in 2014. I promised that I would bring greater awareness to it and that i would talk about my journey. Thanks for reading.
To those of you who have just become my friend recently, you may notice that my cover photo is that of a zebra with the acronym JADWE: Just Another Day With EDS. That acronym within the acronym stands for Ehlers Danlos Syndrome. Ehlers Danlos Syndrome is a genetic connective tissue disease - more specifically, a deficiency in the protein of the collagen in my connective tissue.
The best way that I've been able to explain it, is like this: think of your connective tissue like you would the cement between bricks in a building, no matter how strong the bricks, the structure will not be able to hold up and will eventually fall if the cement is deficient and/or substandard.
Connective tissue surrounds the ENTIRE body - inside and out. So throughout my life, I've been highly-prone to sprains, breaks, fractures, torn ligaments, tendons & muscles; tearing of the skin, the need for stitches etc.
As my disease has progressed, I've been more susceptible to subluxations, dislocations, IBS (internal organs are covered by connective tissue) and the like. For example, I've dislocated my shoulders just by stretching; I've subluxated and dislocated my ribs merely by pulling my shirt off - or putting it on.
I have muscle spasms so strong that they literally dislocate my joints; my ligaments and tendons in my hand have been so lax that my knuckles have sunk to the bottom of my hand and my height can change three inches on any given day because the tendons and ligaments in my legs can become so lax, as well, that they sink to the bottom of my feet. I am no longer able to get around outside without the aid of my Batmobile - my motorized wheelchair.
The laxity of the connective tissue also amplifies whatever sickness I may fall prey to: cold, respiratory infection, flu, pneumonia etc. Our Mayo geneticist said that it could take months for a person with EDS to shake off something that it would usually take an average person a couple of weeks to get over.
I experience this same laxity in the tendons & ligaments in my legs which causes my height to fluctuate, up and down by 3 to 4 inches. Hot & humid days... shorter; cooler days, taller.
Ehlers Danlos Syndrome, presently, is the reason I currently suffer from high-grade tears (50% or more tear) in my rotator cuffs in BOTH shoulders.
I also live daily with the understanding that those with Vascular Ehlers Danlos Syndrome have a life expectancy of 48 years.
An Ehlers Danlos Syndrome diagnosis, many times, carries with it other disorders such as MCAS Mast Cell Activation Syndrome - the symptoms mimic, very much, autism in its ability to make one hyper-sensitive to outside stimuli.
Another diagnosis that those who suffer from EDS are, by and large, afflicted with, is Postural Orthostatic Tachycardia Syndrome (POTS). POTS is a disorder that has OI as its most common symptom. When you have POTS, most of your blood stays in the lower part of your body when you stand up.
This makes your heart beat faster to try to get blood to your brain. Your heart rate can go up by 30 beats or more a minute after you stand up. As that happens, your blood pressure is likely to drop.
Out of all the things that I suffer with, or can suffer with, on a daily basis however, nothing is quite so daunting as the pain and fatigue. The pain can be mind-numbing and immobilizing and the fatigue, well it's... fatiguing. The weariness is, many times, the by-product of dragging around a body that has difficulty "pulling its own weight" - think of it as dragging wet clothes in a big plastic bag.
Now back to the zebra. There's an old medical proverb that says "when you hear the beating of hooves, think horses, not zebras." In other words, diagnoses usually don't come down to the rare and the exotic, but the common and ordinary.
I live daily with the understanding that the unpredictability of my disease is the most predictable thing in my life.
Well I, along with my sons Ezekiel, Clifford and many others, are that zebra. It took a long time to get this diagnosis - thank you Mayo Clinic - and when I did - when we did - we made a promise to raise more awareness about this disease. So here I am, telling you, our RMG family, my story. JADWE.