the unique samantha

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Why You Should Care . . .

You can’t know me without knowing my journey with EDS. My journey helped me become the woman I am today. The woman I was at 8 and the child I was yesterday.

So What Is It?

EDS stands for Ehlers-Danlos Syndrome. It’s a collection of 13 connective tissue disorders. EDSers are born with faulty collagen, and unfortunately collagen is needed to hold the body together. It’s like glue. Of these 13 types of connective tissue disorders, some are moderately common and some are extremely rare. Each type has certain symptoms that are different from other types, but a few symptoms are present across the spectrum.

Now that that’s out of the way, I thought you might want to know why it holds an important place in my life.

Yes, It’s Crazy. But It’s True . . .

First let me get one thing out of the way right at the beginning. I have two types of EDS, which is supposed to be impossible, but here I am. I inherited Classical EDS from my Mom, which was obvious from the beginning of life. I also inherited Hypermobile EDS from my Dad, which was diagnosed later.

Here’s the absolute crazy thing: neither of my parent knew they had EDS. My parents, like so many other EDSers, had slight symptoms, but doctors couldn’t quite figure it out. So, yes it’s crazy, two people with different types of EDS meet and pass on their faulty gene to me.

I know a lot of people might flood my comments with how it’s impossible to have two types of EDS. Bu my two types have been confirmed by four different geneticists.

And no, my friends, it is not fun being one in a million.

Ok, So Now That’s Out Of The Way . . .

According to my parents, my health and development issues were never great. My Mom had been around babies all of her life. She knew what the milestones were. I was late meeting every single one of mine.

At the same time, my parents struggle with my severely fragile skin. It was bruised most of the time and would tear at the smallest bump. There was clearly something wrong with my health, but the doctors didn’t understand what.

So There Is A Name . . .

At the old of 18 months, I was still not walking well. My left foot was floopy and didn’t want to stay in place. So, my parents were directed to an orthopedic surgeon to see what could be done. It turned out that he was the most important doctor in my life.

This doctor was the first one to suggest to my parents that I might have Ehlers-Danlos Syndrome. He didn’t want to make the official diagnosis because he didn’t think he had the qualifications to do so.

I was shipped off again, this time to the Shriner’s Hospital For Children. There, a doctor finally told my parents that I have Type I Ehlers-Danlos Syndrome, now called Classical EDS

There was only one problem, even the doctors at the Shriner’s didn’t know much about EDS. To make things more interesting no one had ever heard of a toddler being diagnosed.

So we left the Shriner’s Hospital For Children with more questions than answers. There just wasn’t enough information in 1973.

Cigarette Paper Skin . . .

As a child, my skin was often referred to as Cigarette Paper Skin because it was so fragile. Any fall would justify a trip to the Emergency Department for stitches. Unfortunately, the doctors in the ED in my small Maine town didn’t know anything about Ehlers-Danlos.

The doctors would spend hours stitching my thin, fragile skin. Then, later, the stitches would pull through my skin. This caused extremely slow healing and giant scars.

I have tons of scars. The cover my body from my head to my toes. Some are bright purple and some have stretched so much that cross my whole leg. But they are mine, and I earned them.

Floppy Joints . . .

As I had mentioned before, my foot was pretty floppy and didn’t want to stay in place. Well, most of my other joints were not much more stable.

When I was about eight, my joints began to painfully sublux and dislocate for no reason. Again, the doctors in the local Emergency Department didn’t understand how I could dislocate my knee during my sleep or my finger tying my shoe.

My feet were very unstable and I need orthopedic shoes from the very beginning. I didn’t know any other shoe as a young child. But wearing orthopedic shoes with a dress when you’re in middle school spell social humiliation.

Actually all of my joints were unstable. I could barely hold a pencil or a heavy book. My elbows and shoulders hurt when I tried to pick myself up off of the floor.

I only became aware that most people’s knees didn’t hurt every day when I was about 10. EDS and unstable joints lead to unrelenting, chronic pain at a very, very young age.

Wait, There’s More . . .

EDS is not just skin and joints though. Slow healing is a big part of every type. When a doctor says he’ll take the stitches out in seven days, they’ll actually be ready in 14.

I get sick if I’m around anyone who sneezes or coughs. I’ve had Scarlet Fever, several bouts of pneumonia, fevers of 103, and my annual bronchitis and sinus infection.

On top of all of that fun stuff, I have other connective tissue related issues. My heart and lungs are weak. I had to have a full hysterectomy when I was only 24. I can’t even think about spicy food without my GERD yelling at me. I have mild hearing loss, and my eyes like to change prescription a few times a year.

Ugh, Surgeries . . .

To date, (I’m currently 53) I’ve had 43 surgeries. Some have been routine, like tonsils and appendix. However, some have been considered advanced or even experimental. My feet have been cut up so much that I have two completely different sized and shaped feet. Sadly, most of these surgeries have failed withing a few years, if not months.

Evolution . . .

Ehlers-Danlos is progressive. I’ve had to give up things I once loved. I can’t sew or knit anymore. I really shouldn’t be around most animals any longer because of their unpredictability. I can’t even hold a fork and spoon correctly and haven’t cut my own food in years!

And I have learned to live with pain of every minute, of every hour, of every day, of every week, of every month, of every year.

Displaced . . .

At the young age of 43, I moved from my safe haven on a hill in the woods into the local nursing home. I just couldn’t care for myself any longer. I couldn’t cook or clean. I could barely shower, and needed help washing my hair. I had begun to use a wheelchair full time, and my little cabin was not set up for one.

So I packed a bag and moved from the silence of a dead end dirt road to Main Street.

The Sunny Side . . .

Even with all of my physical issues, I’m actually content most of the time. I know my limits and stay withing them while creatively adopting activities to keep a great quality of life. I write, read, research curious little things, and make tiny wall hangings from string.

I have a wonderful support system. I have an amazing family and loving friends. I still love people, and I still create, and I still learn..

The Unique Samantha . . .

This is my life’s journey so far. It’s made me as unique as your journey has made you.