#ceds

so, first, what is ehlers danlos syndrome?

ehlers-danlos syndrome is a genetic connective tissue disorder, sometimes inherited but it doesn’t have to be. it’s caused by faulty genes that control collagen (and tenascin in classical-like EDS.) there’s 13 types, 12 have genes that are proven to cause them and hypermobile EDS is entirely a clinical diagnosis while researches try to find the gene that causes it.

now, what about cEDS?

classical ehlers-danlos, which is what i have, is caused by mutations with the COL5A1, COL5A2, and/or COL1A1 (although the latter is rare, most cases of cEDS are caused by defects with the COL5A1 or COL5A2 genes. 10% of cEDS is caused by an unknown gene and the cEDS diagnosis they’re given is clinical.

what are the symptoms?

the diagnostic criteria requires fitting both major criteria or one major criteria and three minor criteria, however many people fit both major criteria and minor criteria. i am one of these! i will also put an asterisk on the symptoms i have.

major criteria:

1. skin hyperextensibility and atrophic scarring* (atrophic scars don’t have to be wild and dramatic! most of mine just barely make dents in my skin that aren’t visible with the naked eye until my scars are in bright light, but they’re still atrophic! my skin is also considered moderately hyperextensible. it stretches by an inch on the back of my hand, almost an inch on my forearms, an inch on my face, and two inches on my neck and upper arms. my knees and feet have mild hyperextensibility.)

2. generalized joint hypermobility* (this is diagnosed by a test called the beighton score. a score of 5 is considered generalized joint hypermobility if you’re a teen or adult under 50.) my score fluctuates between 6 to 8, as my knees can get tight and lose mobility sometimes. this is also a symptom of eds!)

minor criteria:

1. easy bruising* (i always had this. fun fact growing up i went to a hematologist and an oncologist because we weren’t sure if i had a bleeding disorder or leukemia. i tested negative for both. and half my bruises are spontaneous, i get bruises without injury. and the other half is wide bruises from mild injury.)

2. soft, doughy skin* (for me this feels like a marshmallow when you press down, fold or stretch my skin, my skin can be literally kneaded over like dough. my skin also feels like a tie between a fitted sheet and a peach when people run their fingers across it.)

3. skin fragility* (my skin literally breaks easily without bleeding or genuine injury… just barely a scratch leaves a wide wound that doesn’t even bleed because the skin above the layer that bleeds is extremely fragile. and i also bleed easily anyway once the layer that bleeds is hurt.)

4. molloscuoid psuedotumors (i dont have these but they’re little bumps that randomly appear on wounds that seem kinda bony)

5. subcanteous spheroids (i don’t have these either but they’re also little bumps but they appear inside the fat because of subcutaneous fat lobules losing their blood supply and then calcifying.)

6. hernia (or history thereof. because of how lax the connective tissue is throughout the body it’s easy for people with any type of EDS to get hernias.)

7. epicanthic folds* (nobody in our family has them except me, so they’re definitely caused by lax connective tissue on my face)

8. complications of joint hypermobility (sprains*, strains* dislocations, and subluxations*.i have a long history of all these except full dislocations. i’ve never had a full dislocation but ik i might in the future)

9. family history of a first degree relative who meets clinical criteria (haha no. im the only one in my family who has any of these symptoms)

some symptoms of eds that aren’t in the criteria but many people with it have:

dysautonomia, usually POTS but doesn’t have to be (i have pots, and in my case it’s caused by the blood vessels being too stretchy to send blood back to the upper body so the body panics and makes the heart rate jump by 30 or more bpm upon changing positions and releases stress hormones to make up for the low blood volume, causing dizziness, fainting (although fainting is only in 30% of POTS patients, im one of them) digestive issues and if you have hyperadrenergic the symptoms will mimic a panic attack instead of the typical floaty dizzy faint feeling seen in standard pots. any form of pots causes extreme thirst and frequent urination because in POTS the body dumps water because pots causes the body to struggle to hold onto electrolytes that pin the water in your cells.

mast cell activation syndrome: im believed to have this but haven’t been diagnosed. mast cells produce allergic reactions, and in MCAS they can be randomly overactive and cause skin reactions to certain things that happen only once, recurrent hives, and in extreme cases anaphylaxis.

digestive issues: the most common are slow transit and gastroparesis because loose connective tissue can struggle to push food through, but people can have diarrhea or ibs with eds it’s just more common for people with eds to have slow transit or gastroparesis. i have ibs-m meaning i have slow transit sometimes and fast transit others (alternating between constipation and diarrhea) and a lot of stomach pain and gas.

trouble with sensing where you are in space: sometimes people with eds don’t know where they or their joints are in space and bump into things easily. it’s another reason why i get bruises

blood vessel fragility: i constantly have visible bursted blood vessels they look like tiny red freckles. and i easily get them and spontaneously get them.

>wake up

>ouch

>go to handicap shower-its taken. You now wait over 90 minutes for whoever was in the shower talking on the phone to be done. Finally, they are done, you get in and oh wow shaving ON the shower seat. Thanks. Christmas came early. You get to go out of your grouchy withdrawled way to clean their body hair off of your seat. You earn yourself a cigarette and a blunt.

>10am class time, in the elevator; everyone stares at you like you haven't been here for a year now, and crowds you. They start holding the door open to have a conversation between floors. why. just bring the person with you. now you are late for class. everyone rushes and cuts in front of you, you, the only person that NEEDS to be in that elevator, is the last one out.

>You have to pee in class, so you get up to find an accessible bathroom because right now you've been dealing with two compounding issues and need one to help get yourself up, you now have a ten minute walk to the bathroom because some chud decides to cut in front of you to rip his geekbar in the accessible bathroom and you cant even get mad because youre doing that too.

>You are also young, thin, sort of con. attractive, and fem so creepy males (derogatory) will go out of their way to hold doors open for you so they could have the possibility you rub against them by accident. This makes you feel trapped, and you get to use your voice line 3 times today; "My arms work just fine you know" you also upset 2 people and this makes you happy, you are not their charity work; you have a better degree than them, a better job than them, and a much higher GPA, you are nowhere near helpless.

>People never move out of you way, you stopped caring. If they want to be in their own world so bad, you get to body check them and crush their toes with your cane.

>You finally get to your dorm and are waiting for the elevator, the waiting room starts to fill up, and after 5 minutes the elevator arrives. You are swarmed, a chud pushes you out of his way so he can be in the elevator before you because it is full. You are now forced to wait another 5-10 minutes for the single working elevator in your dorm to come back, because you live on the fourth floor, and cannot thug it up that many stairs.

omg we loveeee complaining about our body its amazing its my favorite <3

anyways. i am so fucking tired of having cEDS. i'm so tired all the time. i'm in so much pain all the time. i can never catch a fucking break.

earlier, i was just having a nice time talking with my partner when i suddenly felt something pop in my head and i collapsed onto my desk. no headache, no slurred speech, no numbness, just down. i'm terrified of brain aneurysms literally all the time--I know I'm at higher risk for them because all of my fucking everything is stretchy and fragile.

turns out, i managed to pop my occipital junction. per my mom, it's a thing that happens pretty often with EDS i've just managed to avoid thus far--you just pop the bone holding up your head. no big deal. i'm still dizzy, but i am wholly uncertain if it's because of the occipital pop or the hour long panic attack because i had no idea if i was dying.

because i don't know! i don't know what can kill me! i'm in pain literally constantly! i don't know what the normal amount of pain is! is this a life threatening medical emergency? or is it just a new and annoying symptom to add to the list? how do i tell the difference?

and i'm constantly finding new things wrong with me. my ribs pop out of place. my hips pop out of place. my fingers. my shoulders. my knees. constant abdominal pain. constant bleeding--there is never a time where i am not bleeding from SOMEWHERE. i have to be so careful handling paperwork for my job because if something tears, i'm bleeding all over these important forms, or my keyboard, or a coworker--so many times i've been mid conversation and looked and oh, oops, looks like i bumped my arm into something, grab me the wound kit i'm making a mess :) i'm covered head to toe in scars with no stories behind them. and that's just the external shit. until i went on my new meds a couple months ago, i was constantly at risk of going into hypovolemic shock due to the fact that i was passing palm sized blood clots every single fucking day for months.

sitting hurts. standing hurts. walking hurts, with or without a cane. lying down hurts. looking at screens hurts. reading books hurts. eating hurts! showering! having sex! playing a video game! holding a pencil! being hugged! driving! sleeping! breathing!

and i can barely process it half the time, because my fucking chiari malformation gave me a brain injury, so the fog and the buzzing is so so so high, and i have the fucking migraines, and hallucinations, not to mention all of the fucking. hand wave motion system shit my therapist is trying to help me ignore or otherwise deal with. and my immune system is fucked, and i never know if my MCAS is going to act up and give me a reaction to something, and i get sick so easy, and i'm slowly going blind in one eye for some reason, and my short term memory is fucked, and every time i get pneumonia my lungs are fucked even worse, and i just

i could handle it! i could, really! i'm functional (ish) most of the time! but i can't physically show I'm in pain, or people get weird about it! and i'm scared all the time! because i have no idea how long i have! i get so scared that my fucking hair falls out, and i feel sick, and the sickness makes it worse, and i can't manage my health anxiety because the chances of something actually being very wrong are so much higher for me than other people and if i'm not careful or if i don't pay attention it could be lights out and i just

im so tired

i wish i didnt have this. i wish my body worked. i wish i could do the things i love. i wish i could hold a thought in my head long enough to write right now. i wish i could go outside. i wish i could dance again. i wish i could sing with my whole chest. i wish i could try new things. travel. read. sit in the sunshine. talk about my daily life without having to add disclaimers that, actually, this is what a good day looks like.

and i'm so grateful to be alive. i didn't used to be. a lot of my life has been spent so miserable that i couldn't even comprehend what a life where i feel fulfilled looks like. now i wake up in the morning and i'm grateful for every day, even the bad ones--in part because I have no idea how many I have left. and i have people who love me and understand when i can't push through the pain to make things happen. and when i die, i wont have any regrets for how i lived, and what the experience of being me was like.

but there's so much more to experience and know. and i grieve so much that i'm going to miss out on a lot of it because my body is collapsing underneath me. and there is no version of me that isn't sick, and it doesn't helo anything to dream about what that person would look like.