Ehlers-Danlos Syndrome Presents… Me!

If I had only joined the circus, I would have been one of the freaky back benders. Eww.

My small joints are my biggest problems. Feet, hands, spine, ouch, ouch, ouch.

The first place I felt a ripping pain in my body was in my feet. I mean, other than the abdominal hernias I was born with that were so severe, I needed surgery at two months old. I don’t remember what that felt like. Probably a similar ripping pain. I was told I was a very fussy baby!

Marfan hand. I ❤️ YOU JUSTIN BIEBER!! I’M HERE FOR YOU WHEN YOU GROW UP!!!!!!

When I was 14 and growing fast, one day I could hardly walk up to my math class in junior high, due to that ripping pain in my feet. Very soon after, I was distracted from my ripping feet by the pain and snapping in my knees. My low back came to the pain party around the same time. It was a big party. So distracted by pain was I, I was spared the emotional agony of adolescence. My clique of junior high friends: agony, isolation, loneliness. They are total bitches, but they were the only BFFs I had. I got a head start on being old and wise.

A little lasering on my painful feet. So nice!

Trouble was brewing in my abdomen then, too. My old hernia tore on one side. On the other, my funny body was building crazy metastasizing scar tissue, just because. Two emergency surgeries are coming for those situations, but they can wait for now. I’ll live with the pain. In the meantime, everyone please stay away from me. 

Fast forward.

All grown up and diagnosed, I get checked for aneurysms every now and then. This is my favorite machine in town, the super mighty Siemens at Westwood Open (there’s a laugh) MRI on Wilshire Blvd. It is the best machine made for looking at blood flow.

That’s Hector. He totally thinks I’m high-maintenance. I totally think he should be better at telling me when I can start breathing again. That thoracic magnetic resonance angiogram is the worst. To map the blood flow in the chest, you have to hold your breath repeatedly. This goes on for like 15 minutes, and then comes a flush of dye at the end. It’s horrible.

The brain scan is not that hard. No brain aneurysm in sight, I’m happy to know.

Every couple of years, I get x-rayed from top to bottom. Just curious about how the joint damage is progressing and what we can do about it. A body like mine will not last forever. 

My heart is in good shape. Two weeks of holter monitoring can tell you a lot. I see my cardiologist every six months, only because I enjoy him so much. I can tell the feeling is mutual. He appreciates that I keep my weight down, exercise, wear my compression hose and listen to him complain about his joint problems. 

My skin tolerated the Zio patch for two whole weeks! This is why I am smiling.

I have other joint deformities that are a little bit unusual for Ehlers-Danlos. I have the legs and feet of someone with Stickler Syndrome – shout out to anyone with an EDS clinical diagnosis with Stickler features. Isn’t it cute how collagen disorders overlap?

I’ve got plenty of Marfan features, too, which is obvious from my height. I’ve got the deformed ribcage and those long spider fingers. I love being part Marfan. I think Michael Phelps has Marfan or at least MASS phenotype. That guy is totally my boyfriend! He’s like Lance Armstrong if Lance were the world’s best cyclist, not the world’s meanest cheater. I read It’s Not About the Bike a few times, it was so inspiring! Ha ha. Now I read books about how Lance cheated, then go ask my doctor for testosterone and EPO. Michael’s super power is his hypermobility and that was a gift from God, not doping from a shady European doctor. Michael also has amazing spirit. Those 2008 Beijing Olympics were the best.

I achieved a perfect score of 9/9 on the Beighton scale. Or is that the mark of total failure? Either way, I’m quite proud. That's the test where the geneticist bends your joints backwards and measures how far they go. 

Rib dislocations... big problem. Is this what it feels like to get shived in prison?

Compressing my dislocated rib with the belt from the hotel robe, in beautiful Cabo San Lucas.

Growing up, I did not know what was wrong with me. I could see the other kids had something I lacked. But was it? Stable collagen, I would find out, when I was 33, even though I’d been going to doctors my entire life, very obviously presenting with Ehlers-Danlos Syndrome. I mean, other than my Stickler legs, I could be the picture in the medical textbook. But whatever. I can’t get in a tizzy about everything that has gone wrong. I’d have no time for anything else. I’ll write more about medical hell growing up another time. They are some funny and awful stories. They were not funny at the time.

I thought a lot about suicide when I was diagnosed. I think anyone receiving a hopeless diagnosis and certainly one so debilitating and pain centric would. But by then, I had endured so much, it seemed like the wrong time to give up. I could always give up later. Death awaits all of us. Seemed like a boring choice, after all. What might happen if I stuck around. Seriously, of course I came up with a suicide plan. I’m a planner. And I put it on the shelf.

Overdid things at Comic-Con last year. Time to go home!

Pack in ice before shipping. I always travel with Ziploc freezer bags.