Life of being an Alphabet

So in my last post I wrote about finally being diagnosed with Ehlers Danlos Syndrome (EDS.) Since that diagnosis I've collected a diagnosis of Postural Orthostatic Tachycardia Syndrome (POTS), on top of the Raynaud's.

EDS is a connective tissue disorder. Anything and everything with collagen can be affected, depending on the type of EDS you have. The three most common types are Hypermobile, Classical, and Vascular. Vascular is considered the most "severe" since it usually has a life expectancy in the 40s and you can have organ ruptures (including the heart.) Classical can have signs and symptoms of both Hypermobile and Vascular. Hypermobile is considered to be the most painful with a higher joint issue. I was officially diagnosed with Hypermobile with Classical tendencies. My skin itself shows signs of either. The reason that it is hard to diagnose is Hypermobile isn't linked to specific genes yet, and Classical only is linked 50 percent of the time. Right now I am in the middle of waiting on genetic testing though to make sure I don't have Vascular type. The good thing, for some parts, is that Vascular usually doesn't have the hypermobile joints as the Hypermobile type, so most likely I'm not. But we are checking to be sure. My main symptoms of the EDS side: joint hypermobility in everything but my elbows (although my hands are getting stiffer every month), my scars both keloid and become cigarette paper scars, I have lots of sprains, strains, and pull tendons, healing time on surgeries and those sprains are a lot longer than normal, stomach issues, and a few other things that coincide with my other disorders.

The Raynaud's is the easiest to describe. Basically my body can't equalize temperature correctly. Due to everything else, my heater in my body and my air conditioning is defective. I always say my thermostat is broken. With the Raynaud's I can't stay warm, get warm when I'm cold, and I always suck heat from other people. My hands often turn purple/blue and then white, like I'm getting frost bit. When they warm up they turn red and feel like they are on fire. My ears like to turn red randomly, which is more annoying than anything else. My feet are usually purple, as I prefer them to be cold than hot. Although when I take a shower they usually look almost black. (Part of this is because of blood pooling too which has caused discoloration of my lower legs and they swell a lot if I'm sitting in a chair/car or standing for a long period of time.) This next part will be slightly graphic. The worst part of the Raynaud's is called a vasospasm and I get it on my breasts. My nipples get hard, turn white, and are extremely tender. This usually happens when my body as a whole gets too cold or when I'm stressing a lot. Pressure helps, but overall pressure, like the feeling of a bra, hurts, like a searing/burning pain. It's straight up agony. I can deal with the rest of the Raynaud's, but that part sucks.

POTS, not the drug and not the opposite of pans. (Although there is a disorder PANS, but that is a whole 'nother story.) Basically anytime I move, get sick, get stressed my autonomic nervous system doesn't act normal. This affects my thermostat too, but a lot more than that. My heart rate increases, my chest gets tight, it's hard to breathe, I feel dizzy, lightheaded, like I'm going to pass out, usually my stomach starts cramping, and I start feeling the like the world is in slow motion and nothing makes sense. Afterward, assuming I don't pass out, I get super tired and confused. This can make me sluggish for a few days afterward. A few times I've had issues where my heart rate dropped too low (we are playing with the adjustment of my meds right now) and I had trouble staying awake and remembering to breathe. Usually with the POTS when I get up my heart rate jumps (30+ is the minimum for a diagnosis) anywhere from 20-50 beats a minute higher. On my meds my resting heart rate is in the mid 60s, without resting is in the 80s, and when I'm sick the resting is high 90s. You can see where a jump in my heart rate can make a huge different. When I was first diagnoses I failed my stress test 3 minutes in, they diagnosed my as POTS with inappropriate sinus tachycardia as my heart rate was 173 and I started fuzzing out. **Fuzzing out is my term for when my eyes go into a dark tunnel vision like sensation, my ears thump to my heart beat and I can't hear anything else, and my legs get weak.)

Just to help explain what I go through beside the things above... I live everyday in pain. I try to ignore it as best as I can. If I complain that I hurt, it's because it is a really bad day. I never sleep well. I have a sleep study next month to hopefully help somehow. I've always had trouble going to sleep and staying asleep. I used to live on naps and naps alone. Now if I don't sleep I drag for days. I am always tired. Sometimes I'm so tired I can't even think, but there is always some degree of tired. I get sick most times that I eat. I take a handful of meds daily to make sure that my stomach doesn't cramp up when I eat, no matter what I eat. I usually spend every day nauseous to some degree. The really bad episodes of it I feel like I can't move without puking. I couldn't live without those meds and mints. Guess I'm not living long in the zombie apocalypse. I also have to be careful when I do daily tasks, like walking. I easily roll ankles and trip. I never know how close I am to walls and end up hitting my hands a lot on walls. I can't walk straight, almost ever.

My biggest thing for all this is a two part struggle. I constantly fear that people don't believe my, my disorders, my struggles every day. And I fear that people judge me for my weakness and that one day I'll have to depend on others. With those two things, I try to always hide my struggles, pretend I'm just a normal fat kid, and push myself too hard. But I'm learning to be open and honest. My friends and family love me for me and I need to stop trying to hide it. I've been blessed with amazing people in my life who are there for me. Some haven't been there for me, and they are no longer a part of my life. I am trying to take control of my body, my life, my disorders. Now that I have the knowledge, I'm ready. Before I was ready to get healthy to do derby. I can't do derby anymore. Too high risk of damage that won't heal for me. So for now I'm a derby lover, that is going to try to get healthy for me and myself. I can do this. I hope.

http://ehlers-danlos.com/

http://www.dysautonomiainternational.org/page.php?ID=30