Friday, May 19, 2017

This Is What Ehlers Danlos Syndrome Is Like

May is Ehlers Danlos awareness month. In honor of awareness month, I created a video [shown at end of post] depicting a day in the life of someone with Ehlers Danlos Syndrome and its comorbid conditions. The extent of symptoms varies patient by patient. This is how I used to present prior to my secondary Mast Cell Disease becoming severe. I tried to add a humorous twist to the video. Enjoy!


You awake in the morning, rubbing the sleep out of your eyes. Last night was spent tossing and turning, so you are not well rested. You wonder why you expected to feel otherwise when you have Ehlers Danlos Syndrome.

In attempts to make it out of bed, you roll over, but your actions are interrupted when your hip cries a startling "pop." It is another dislocation. A searing pain courses through your skeleton, while you contort your body to adjust the joint back into position.

Since the joint is back in place, the injury requires bracing. You eye the brace from the corner of the room. You are dreading the arduous journey, as you have not yet managed to make it out of bed. Hobbling, you grab the brace and get dressed for the day. You completely bypass the bathroom. A shower is just not in the cards this morning.
Ehlers-Danlos syndrome is a group of disorders that affect the connective tissues that support the skin, bones, blood vessels, and many other organs and tissues. Defects in connective tissues cause the signs and symptoms of Ehlers-Danlos syndrome, which vary from mildly loose joints to life-threatening complications. [ x ] 
Instead, you trek down the hall. Your heart is pounding like a subwoofer, a bass echoing in your ears, and stars dance across your vision. It is just Dysautonomia flaring. The lax vessels formed from faulty collagen are unable to adequately pump blood to the brain. Mid-backout, you surrender to the floor to avoid completely losing consciousness.

Now you head towards the kitchen. It is time to prepare for AM medications and intravenous infusion set up. You are sort of sick to your stomach. Regardless, you need to eat breakfast. Taking pills on an empty stomach is never a practical idea. You realize you can depend on tube feedings, but you prefer not to rely on the formula if you can help it. You choke down a bland piece of toast.

The day has barely started, but you are already exhausted. You grab your bag to head out the door...and pop!!! You partially dislocate your finger. In the interim, you grow even more queasy, proceeding to up chuck in the grass near the front steps of your house. There goes that piece of toast. You also notice remnants of last night's dinner. Darn, Gastroparesis, a paralyzed stomach.
"Each type of Ehlers-Danlos Syndrome is defined as a distinct problem in making or using one of the types of collagen. Collagen is what the body uses to provide strength and elasticity to tissue; normal collagen is a strong protein that allows tissue to be stretched but not beyond its limit, and then safely returns that tissue to normal." [ x ] 
You have a gut feeling something is not right, and it is not just the rotten food from days prior. It is probably not safe to drive, so you need a ride to run your errands. You do not want to inconvenience the same person you called three different occasions last week. You realize you can be a burden; however, rarely do others admit it.

The professor drones on. You finally made it to class! Concentration is difficult. Brainfog is no joke. Your hands shakily scribble your essay. Maybe it is the hypoglycemia. After all, you absorbed little to none of your meals. You got a C on that paper and wonder if you could have gotten a higher grade if you had not had to sacrifice studying for that doctor's appointment.

There are errands to attend to, but you have no energy. Fatigue is evident, despite the 2 PM cat nap. You totally skip lunch. Why bother, since symptoms indicate you will probably puke that up too. You are aware of each inhalation. It is difficult to breathe. Your lungs crave the air that your body is inadequate at giving them.
"An analogy: If one builds a house with bad materials, with cheap nails or only half the wood required, problems will arise. Some problems are more likely to show up than others, but because the bad or missing materials are everywhere and not necessarily visible, one can be surprised where some problems occur." [ x ]
Pressure builds at the base of your skull, right where it attaches to the neck. The tissues are weak and are unable to support your head. An intense ache accompanies the pressure and the room is spinning with nauseating vertigo. You joke that you are bionic woman with your silly neck brace, but are thankful for the reprieve.

That evening, you meet with your family. You enter into your grandma's house when a sense of doom hits you. It is a mast cell reaction to your aunt's perfume. Your skin turns flushed, red, and blotchy. Your limbs tremor and jerk. Parts of your face begin to swell, with your throat quickly following suit. A cousin stabs you with an EpiPen. Mom administers emergency Benadryl before you have the chance to drop into a seizing fit.

A lot has gone on. You give yourself a few hours to recover. Not showering again is not an option. The heat from the shower dilates your blood vessels, exacerbating your Dysautonomia again. You hope you do not faint again. After reaching for the soap, you wash your face that is covered in itchy hives. Oh no, please no rebound reaction.
"It is much the same thing with Ehlers-Danlos Syndrome and collagen. The collagen with which a person with Ehlers-Danlos Syndrome is built is not structured the way it should be, or only part of it is produced. With badly built collagen, the tissues that rely on it can be pulled beyond normal limits and thus be damaged." [ x ]
Once evening medications are over, you connect to your feeding tube in a Benadryl stupor because eating was obviously not a success. Your prayers are pleas for a restful repose. Unfortunately, there will be no soothing lullabies, only formidable tales where your symptoms are incorporated into nightmares. Even in the wildest dreams your brain can conjure, you are never healthy. You close your eyes, knowing tomorrow you will do it all over. Because this is what having Ehlers Danlos Syndrome is like.


Having Ehlers Danlos Syndrome is not all doom and gloom, like this post may suggest. Happiness still exists in a life with chronic illness. However, especially during awareness month, those with Ehlers Danlos Syndrome and its co-morbid conditions often undermine their struggles. We do not want to be thought of as always depressing or always crying poor pitiful me. Hopefully this accurately depicts a day in the life of someone with Ehlers Danlos Syndrome.