Ehlers-Danlos Syndrome

What’s Wrong with Her?

“She’s in a wheelchair one week and dancing on the beach the next. Obviously, she’s just a hypochondriac in a victim mindset, who wants people to notice her!” Oh how I wish that were true, because then I’d have a cure, especially considering all the years I’ve spent in therapy. When a celebrity, like Celine Dion, has a rare disorder, Stiff-Person Syndrome, people swoon about how courageous she is. When a non-celebrity has a rare disorder, like Ehlers-Danlos Syndrome, people accuse them of seeking attention. That’s not a dis to Celine Dion. Who doesn’t love Celine Dion?!? It’s just an example of how the general public treats someone who is suffering who happens to be a celebrity versus someone who is suffering who is a non celebrity, not unlike the celebration of the butterfly versus the disdain of the moth. Disability can happen to anyone: you, me, or a celebrity. Both disorders are marked by spasms, chronic pain, and can be progressively debilitating. Both deserve more research, greater care and deeper understanding.

I’ll tell you what’s wrong with her (and by her, I mean me). It’s it an “invisible illness,” what’s called a “dynamic disability,” and in the iconic words of the Maybeline campaign, ‘Maybe she’s born with it!’” Living with the rare, life-threatening genetic disorder, Vascular Ehlers Danlos Syndrome, is like living with a domestic abuser. I never know when the next hit is going to come, how much of my life I will lose when it happens, how severe the pain will be, or how long it will last. I rarely talk about it because it’s the problem that can’t be solved. No one is going to rescue me from this abuser or believe the pain they can’t see; it’s simply easier to ignore. This disorder makes my body a rag doll prone to daily subluxations, weekly spasms, and monthly dislocations that can come from something as simple as a sneeze. There is no cure and little treatment, unless you count sarcasm and gallows humor which I’ve learned to survive on.

Beside my bed, I have a complete collection of braces for every part of my body, two neck massagers, a red-light therapy belt, an array of pain-relievers, and a partridge in a pear tree. Some women wear Chanel N°5, while my signature scent is Tiger Balm, mixed with freezer bag aroma from daily ice packs, which contrast with the nightly musk of a 35-year-old heating pad and Icy Hot menthol sticky patches for my back.

Everything in my environment is to ease pain: from the hospital bed and mobile desk I am writing this on, to my shower chair, to the tush cush I tend to take with me to sit on wherever I go, to the wheelchair, walker, canes, or other mobility devices I may need. Even my wardrobe, undergarments, and shoes all need careful consideration, because something as simple as a push up bra can dislocate my ribs, cause a full body spasm, and have me bedridden for a week, recovering on muscle relaxers. Talk about dressed to kill.

Having vEDS has made me a full-time medical advocate for myself, my child, and my grandmother before us. It took me over 200 doctors, across three states, five major hospitals, and over 20 years to get a diagnosis; and a diagnosis creates all the difference in the world for treatment and mental health. After years of being told “It’s all in your head” by medical doctors and being scolded by my spiritual community that my soul chose this pain and that my thoughts were somehow creating this genetic mutation on COL3A1, I was relieved to discover it was a genetic disorder and not in fact, something I, or the Witches of Eastwick, conjured up in a spell. I was finally able to stop blaming myself.

I was diagnosed with Ehlers-Danlos syndrome, EDS in 2013. There are several types of EDS, and I won the pain lotto with three: vascular, hypermobile, and periodontal (as if going to the dentist wasn’t hard enough). It’s a defective collagen disorder, which weakens connective tissue and thereby impacts the entire body; so it comes with many other comorbidities. The official website for rare diseases (GARD) shows that there are 94 symptoms, and counting. Everything is more challenging when you have an underlying chronic health condition: “normal” flu/colds, travel, social engagements; especially for those of us who are also known as an HSP (Highly Sensitive Person). As Greta Gerwig’s iconic Barbie monologue so perfectly expresses, those who suffer get told a lot about how important it is to be grateful for all we have and reminded that things could always be worse. Consolation prizes on game shows are better than those in real life—I’ll take a new washing machine over ridiculous social standards, any day!

After 15 years of infusions and the past seven years of in-home health services that provide nightly physical therapy to keep me out of the wheelchair as much as possible, I’ve lived six years beyond my life expectancy, so I’m looking for a hand to hold that isn’t a scythe. Having survived six strokes, while living with three aneurysms, I live a very tick, tick, boom existence with daily gratitude and unconditional love, as my ride or die (pun intended) companions.

In a world where over half the population suffer from at least one chronic condition, according to the American Hospital Association, the question we should be asking is, not, “What’s wrong with her?” But rather, “What’s wrong with a society that’s in this much pain and suffering and judges when they could be offering compassion instead?”

For more information on Vascular Ehlers-Danlos Syndrome, please check out GARD, the Genetic and Rare Disease Information Center, at rarediseases.info.nih.gov.

To end this on a positive, upbeat note, which is expected of all people who dare to share the truth about their pain, please check out this sunshine parade of hope. It’s a piece I wrote about how I’ve managed to outlive my shortened life expectancy, entitled How to Heal Almost Anything.

Sage Justice is an award-winning playwright and critically acclaimed performing artist who has appeared on stages from Madison Square Garden in New York City, to The Comedy Store in Hollywood, California. Ms. Justice is the author of Sage Words FREEDOM Book One, an activist, and a member of the Screen Actors Guild. She is a co-founder of The Unity Project which fuses activism with art, to educate and inspire, with a special emphasis on community engagement to end homelessness. She has a series of short reels about living with EDS that you can find in one of the mini circles (highlight reels) on her Instagram page @SageWords2027.