#I have erythromelalgia which makes my hands and feet burn in response to my POTS. heat. and stress

[dirtytransmasc]

me: sick on top of being chronically ill and in a massive flare, dizzy, bodies on fire, my bones hurt, my heart currently beating out of my chest, etc. so I feel like absolute shit and like I might pass out.

my family (who I'm currently staying with): invites other family, including a small child over

my family: makes me home our big ass dog back so she doesn't tackle said child (she's friendly, that j god, just a bit... overzealous in her affections)

my dad: looks at the multiple able bodied, not sick, not in pain, not on the verge of passing out, people, this g to find someone to help him.

my dad: "hey you, yeah you, the sick one, the one who looks dead on their legs, the one who shouldn't be interacting with family let alone the food they will be eating, come shuck corn, bring it out to the grill (it's like 90 degrees, I thought I was dying the second I opened the door), and complete this list of small house errands (including going into the luckily finished attic, basement, and out to the trash) 😃👍"

me: first off, I hope you all get this bug (besides the baby, everyone else are onlookers who could have stepped in), second off, when I pass the fuck out, it's all your problems.

(small note, I know this sucks and everything, and the nice thing to do seems like it should be comforting words and apologies, but I need dark humor and jokes right now, cause pity and kind words, while assumingly genuine, will only piss me off in my current state, cause it's all I get from my sucky family and it just triggers something deep inside me)

[weirdmageddon]

my rheumatologist has done more for me towards getting a POTS diagnosis than my cardiologist thats kinda sad bro

i came in to the cardiologist the other day (finally after waiting MONTHS) hoping for a tilt table test to see how my heart rate and blood pressure react to orthostasis in a controlled setting. the doctor didnt actually do a thing to test me for it in-office, i was just told to schedule an echo (which is fine), holster (alright) and stress test (why). but i was also prescribed eastern medicine as a treatment....“superbrain yoga”? like i dont want to seem closeminded because she is an indian doctor and there are some things that western medicine hasn’t caught on to but i realy wish i was told why it is supposed to work. like i want to know physiologically how and why it supposedly works. get technical and mechanical with me bro i have le autism, thats my language if you wanna really convince me. if it’s about toning up the muscles in my legs to squeeze the blood into my core upon standing why dont i just do squats? why do i have to do all this really specific stuff like hold my tongue at the roof of my mouth and face east, crossing my arms (right arm must go over left) and maneuver my hands in a certain way to grab my earlobes while doing those squats? is that merely a concentration sort of thing to make your brain focus? if so, why not just let me know what the purpose to these specific movements are (and what does focusing my brain have to do with treating POTS symptoms anyway)?? i’m not a spiritual person so the spiritual aspects of it do nothing for me. but at least i wasn’t given intensive aerobic exercise because i cant do that lol. i was just prescribed core strength training with planks and crunches (fine with me) and “superbrain yoga” (the specifics still confuse me but i’m doing it anyway)

but i didnt even get a tilt table test while i was there, i asked about it and she said “we stopped doing tilt table tests a while ago” and i was like ????????? thats like the gold standard to test for POTS my guy. based on just my symptoms she said i had dysautonomia and i asked “what about POTS?” and she said “it could be” and i was like ? could be? bro you didnt even test for it?

the whole visit just felt really vague and dismissive to my issues (yet again). fucking even my rheumatologist said before this visit to the cardiologist that i “probably have POTS”

so when i left the cardiologist the other day i wrote this up because i was very upset, felt dismissed, and took matters into my own hands to show what kind of medical concepts i’m capable of comprehending and the kind of language i want doctors to talk to me about my conditions in. and today i read it to my rheumatologist during today’s appointment:

the cardiologist says i have dysautonomia, “caused by dysfunction of the small blood vessels”. in the clinic, the nurse measured my laying vs standing blood pressure (which increased rather than decreased) but they didn’t do my heart rate there for some reason. but on my own i’ve measured my heart rate to jump above 30 bpm within 10 minutes of standing, so with all the symptoms lining up exactly with what’s expected of POTS (heart rate increase greater than 30 bpm within 10 minutes of standing, no drop in blood pressure, lightheadedness, brain fog, palpitations, prolonged fatigue, heat intolerance, excessive sweating etc), i’m convinced that the type of dysautonomia i specifically have is POTS, not just the umbrella term “dysautonomia”, and the specific brand of POTS i have is the neuropathic POTS subtype which is thought to be caused by sympathetic denervation (partial autonomic neuropathy) in the lower extremities. this causes the blood vessels in my legs not to constrict as they should when standing, which in turn causes blood to pool in the legs and not return to the heart, causing the heart to have to source its blood supply from elsewhere in the meantime to compensate (with an overall lower venous return), driving up the heart rate and causing lightheadedness. my blood tests also showed i am also very slightly anemic by 0.1 point below the normal range (11.6 g/dL) the resulting denervation hypersensitivity from the sympathetic denervation what is thought to cause erythromelalgia—which i express all the hallmark symptoms of as well in my feet (redness, increased skin temperature, burning sensation (feels like walking on a hot pool deck), cold to touch and bluish purple when not actively flaring, flaring occurs at night, symptoms worsen with exposure to heat and exercise (including walking on feet while flaring) and are relieved with cooling and elevation). i have no response to the cold unlike with what is seen in raynauds. i actually consider cold exposure my savior; the heat is my worst enemy, it makes me feel faint and lightheaded dysautonomia-wise and it makes my feet flare up rheumatologically.

“Several previous investigations have provided clues that patients with the postural tachycardia syndrome have peripheral autonomic dysfunction. Streeten et al. found that patients with orthostatic tachycardia had excessive venous pooling in the legs while standing and suggested that denervation of the legs was a mechanism of the syndrome. This hypothesis was supported by the finding of hypersensitivity to infusion of norepinephrine into the veins of the foot, despite high plasma catecholamine concentrations. [...] These stimuli increased norepinephrine spillover in the arms of both the patients with the postural tachycardia syndrome and the normal subjects, with similar increases in the two groups, but failed to increase norepinephrine spillover in the legs of the patients. [...] The reduced clearance of norepinephrine in the legs, without a similar reduction in the arms, may result from impairment of norepinephrine-reuptake mechanisms due to isolated damage to nerve terminals in the legs. [...] CONCLUSIONS: The neuropathic postural tachycardia syndrome results from partial sympathetic denervation, especially in the legs.” — (https://www.nejm.org/doi/full/10.1056/NEJM200010053431404)

“The laser Doppler flowmetry signal after sympathetic stimulation of reflexes mediated through the central nervous system, was significantly diminished in patients with erythromelalgia as compared with healthy controls. [...] Vasoconstrictor responses involving central sympathetic reflexes were attenuated in erythromelalgia. Local neurogenic vasoconstrictor regulation, vasodilator response to local heating and hyperemic response to ischemia were maintained. [...] The finding of reduced skin perfusion before provocation is in accordance with the clinical observations that many erythromelalgia patients exhibit cold acral skin between attacks. [...] These results indicate that postganglionic sympathetic dysfunction and denervation hypersensitivity may play a pathogenetic role in primary erythromelalgia.” — (https://linkinghub.elsevier.com/retrieve/pii/S0022-202X(15)41629-X)

“Denervation hypersensitivity is a phenomenon peculiar to smooth muscle innervated by the general visceral efferent system. Following denervation there is increased sensitivity of the muscle to neurotransmitters. This is evident in smooth muscle innervated by sympathetic neurons when the postganglionic axon is affected. Such denervated muscle shows hypersensitivity to the application of epinephrine or to circulating epinephrine released during excitement.” — (https://www.sciencedirect.com/science/article/pii/B9780721605616500198)

although my rheumatologist is in no position to give me a POTS diagnosis she very much agreed with the connections i made and said she thinks i am right on the mark with my conditions. she told me im a real academic patient and even that i’d be well suited for going into medicine lol. not only is it refreshing to have a doctor that doesn’t disregard their patient’s knowledge, but it’s good to see what i’ve learned about nerves from my biopsych classes (and in my own time for funsies) paying off in ways concerning my health. my mom who is a nurse also agrees that neuropathic POTS and erythromelalgia are what i have.

anyway the POTS symptoms have been a massive thing for me since puberty and the erythromelalgia developed a year or so after my POTS symptoms started. but i’ve always had freezing cold clammy hands and feet since i was a young child, they just hadn’t started changing colors and flaring until after i hit puberty. i’m not sure what destroyed the sympathetic nerve fibers in my legs (as most POTS happens in teenagers due to some viral illness but i’ve never had that?), i was also just tested for a bunch of autoimmune factors and disorders and my results came back negative. maybe it’s just a genetic factor, who knows, probably something caused by a hormone’s cascading effect gone awry at some point. it seems a lot of autistic afab people have POTS or some other type of dysautonomia for some reason and i’m curious as to why.

anyway i’m really stuck in a liminal space because i have no official diagnosis beyond “dysautonomia” but i’ve been sure of what it is for like over a year and it keeps getting clearer and clearer that i was right all along