CIPA.
The letters look innocuous if you don’t know what they stand for, which if you don’t- that’s a blessing.
Congenital Insensitivity to Pain with Anhidrosis.
I can’t feel pain- to put it simply. There’s a lot more to it, but no one cares for that. You’re a superhero, you’re invulnerable… until people notice the scars on your body, dotting your skin here and there to show where you have been and what you have done that you yourself did not feel or notice until the blood was visible. Burns are bad when you can notice that you touched a hot surface- but without pain… it’s the odd sensation of trying to pull a hand away from a hot stove that you didn’t realize was still more than a little warm that gets you. Not so much a physical sensation, but the absence of such. The wondering of how that sort of pain feels.
You’re not invulnerable with CIPA. The opposite, actually. Because your tactile senses miss a few things here and there, you have to be careful- and even then, small things add up. You don’t heal as quickly from skin injuries and bone fractures are hell to heal from when you can’t tell the difference anyways. The bone infections are… fun. There are other, less pleasant side affects of CIPA, but I think my point has been made.
Oh, I never explained that last word of the acronym- did I? Anhidrosis is a sweating dysfunction. I just… can’t sweat. If you think on that for a bit, especially taking into account the kind of sicknesses kids get when they’re younger, and the benefit that sweat provides to help cool down the body- you might start to understand how this might be a bad situation. Pair that with everything else and you got a veritable shitstorm.
When you’re a kid, it leads to a lot of scars from your parents taking their eyes off of you for merely a second. When you’re a angst-filled teenager or a depressed adult, it leads to a lot of self-caused injuries. Depression… feeling nothing emotionally, mixed with the inability to feel pain… leads to some interesting results. Not good ones. A long, harsh, shiny red gash of a scar grips and tears at my left forearm, twisting from antecubital to the outward-facing part of my wrist.
Usually when something particularly upsetting happens, you might break out in a “cold sweat”, or, from what I have heard- once adrenaline wears off, you feel pain after a traumatic occurrence. When facing down the aftermath of repetitive motion while disassociated from a particularly stressful day while lazing in a bath- I had wondered if all the red in the water would awaken something. If by seeing the shine of fluids, the sinew of muscle, and the glimpses of iridescence of fascia would bring the sensation of pain to life. I didn’t dare tell my therapist of all the thoughts that ran through my mind that night at the curiosity of being “normal” for once… but the pain never came.
It didn’t.
I stumbled along in life- not doing great, but not falling by the wayside either. Some of my scars frightened others, some were drawn in by them. One kid made me smile by gently and ever so professionally taping a single small band-aid over the center of that scar on my arm and declared that I was “all healed!” when I was in a waiting room in the ER for breaking a few fingers from getting a door slammed on them. Thankfully the kid never noticed the fingers.
I was staring down at the twisted lumps of flesh on my hand when I was finally sat on the edge of one of those beds in those private little sterile rooms, waiting to be seen. What did it feel like, I wondered. What did pain feel like? Surely unpleasant, given what I had seen- but it obviously helped prevent situations like this… I had pushed a little too hard on trying to argue with a girlfriend to take me back. I got in her face- I’ll admit that. I just didn’t want to be alone. Now looking at my fingers, I sighed.
“So, what happened today?” A nurse cheerily asked as she looked me over when she walked in the room. She was young. Chipper. New. Definitely new. You can tell easily when they see what happened to you and react far more than you do. I shrugged and held up my hand and watched as the smile almost audibly slid off her face. Gobsmacked. I was used to it.
It took a moment to regain her composure- I waited politely as my fingers darkened in hue with bruising. She then nodded to herself and checked charts and needed information. You could hear the cheap wall clock ticking the seconds by as she read up on my file. I idly kicked my feet, the small sounds of the paper on the hospital table crunching under my jeans as I moved filled the silence and joined the ticking.
“It’s alright. The other nurses here know me- I’m kind of used to being used as a hazing ritual for the newbies.” I admitted, trying to calm her down. It wasn’t unknown to me how rare my disorder was. I had a bad limp in my right leg from an injury that never healed right when I was a kid. The doctors back then didn’t really know what to do with me. A lot of theories, meetings, talks- and I still ended up with a limp and some heavy swelling in that leg.
She gave me a glance over the charts and then excused herself for a moment politely. Soon enough, though I was joined by the kindly white-haired old doc I was used to. Nothing ruffled his feathers these days. “Ah! I’d love to say that it’s nice to see you again, but let’s see what you brought me today.” He spoke warmly, trying to be lighthearted as he sat himself in a wheeled stool and rolled himself over the linoleum floor to get a better look at the problem as I extended my injured hand to him out of reflex. The only thing that was going to hurt about today was going to be the bill. It was the start of the new year, and being how I was, those months before deductable is met are horrendous.
“You know,” He started, deep in thought as his fingertips palpated over pulverized bones of my index, middle, and ring finger. “I recently came across a treatment for CIPA that helped another patient. It took about a year, but it helped. They started to be able to tolerate different temperatures and sweat… they had pain response that helped curb some happenings and incontinence is no longer a risk for them … I didn’t want to bring it up until I knew it was a done deal but- I’d say my other patient has a marked improvement in quality of life with the treatment.” He offered up conversationally, like we were making idle small talk.
“Some kinda gene therapy?” I asked not really believing or engaging with him beyond speaking. I’d been told all my life that there wasn’t a cure- it was an inherited disease, nothing could be done. I’d heard enough crackpot theories from friends and family that I knew how to talk without getting invested. He hmm’d agreeably as he noted down in his chart where my fingers were rightly obliterated and then his eyes twinkled as they found mine.
“It worked.” He stated factually, and those two words hung in the air like a guillotine for me. Do I invest myself in this obviously insane talk, or do I dismiss it?
“Lemme guess, there’s a list I can sign to be put on a wait list for an experimental treatment that isn’t even approved by any association or board?” I muttered and the doctor gave a small, encouraging smile to me before entirely dropping the subject and telling me how they were going to go about fixing my hand. I couldn’t help but notice the way he looked at me as the rest of the day, which was filled with learning new words like “Internal Fixation” and being told to be vigilant and careful while my hand and fingers were healing. You don’t want to know what my hand and fingers looked like.
It worked.
It echoed in my head for five nights while I laid in bed trying to sleep. I thought on it on and off- weighing pros and cons before finally I gave in. I decided I would talk with the doc about it. The next few weeks of check-ins and therapy, I gently questioned him, and finally got answers I needed- hell, the old man offered to put in a good word to have me be seen sooner by the group- not that it would matter too terribly- CIPA was rare. Still, though, I took the offered help and contacted the group through an offered plain business card that gave a nondescript moniker of a company- Something like Red Co. or something. Had a few doctors names on there with numbers, a website address, and even a physical address on the back. I got set up through their online portal, filling out medical information and history until I was fairly certain I was either going to die of boredom, or the paper cuts on my non-medically fucked hand as I searched through my files for medical documentation they requested. It reminded me bitterly that I still needed to catch up with the times and scan my documents into my computer so this could be easier.
Soon enough though, they had everything they needed- every little piece of data of my physical health- and even mental health. They were thorough. A few weeks went by after I was pretty unceremoniously welcomed to the testing group with an email telling me where to go at what time. I found myself staring up at a tall redbrick building that had a paltry amount of parking around it- enough that I was honestly risking getting towed. Hopefully I’d be out in three or four hours before someone took notice of my silver sedan parked alongside the building, next to a flowering crape myrtle and a big sign that warned about parking there. As I walked inside, everything was sterile and white, with maybe a hint of beige on the walls or the slightest of pink in the vases on the countertops holding white flowers that would look right at home at a funeral. The front desk asked for my ID and insurance, and handed me a pen and clipboard that had at least ten sheets of paper on it in exchange. “Please fill all this out, front and back- initial where we have highlighted, sign and date on each page and are you fasting today?” They spoke automatically, unfeeling as I took the pen and clipboard. I nodded to the directions and shrugged, used to procedure and figured they’d want a blood draw before getting started, so I informed them that I hadn’t eaten yet. They noted that down and ushered me to go sit in the waiting room, filling out mostly the same information I had already filled out on the online portal. After I turned it in, the directed me to a locker with a key, telling me to please place personal belongings within until the end of the testing for the day. If you’ve visited hospitals and ERs like I have, you’ll understand how annoying it was to place my phone in there knowing the boredom of old magazines that faced me.
It felt like an hour ticked by until they called me back, and the usual shenanigans of blood draws were the starting point. I was informed of what they needed to test for- which was a rather long list, and a longer list on stickers that had to be affixed to vials waiting to be filled. After all the prep, they tied the band around my arm, told me to make a fist a few times as they poked and finally struck gold on a vein and then I watched numbly as red filled vial after vial after vial after vial. Then I was waiting alone on the usual paper-covered padded table. For what felt like hours. There weren’t even magazines. There wasn’t even any sort of art on the walls or information on pills that the doctors were paid by companies to push. Hell- they didn’t even have a sharps box in here. Just a sterile white room with a metal sink, soap dispenser, paper towels, and cabinets.
I didn’t realize that I was idly chewing on my lip until the door opened and a male doctor, whose handsome face looked incredibly botox’d came in. He looked me over and centered in, pointedly staring at the lower half of my face, which, as I said, I had been chewing my lip. Without a word, he strode over to the paper towels, ripped off a few sheets and brought them to me. I took them and blotted at my lip, finding it to staining the paper towel quite efficiently with blood. It wasn’t anything new or alarming to me, it’s not like it hurt- the only thing about it was that it did ruin my day when I had to rush to clean clothes that got blood on them from shit like this. That being done, he merely stood back, watching me. Not like a sideshow or like I was some kind of freak- but the look in his eyes didn’t seem too trustworthy either. It was more similar to the look on the face of a kid when they have a magnifying glass and are staring down an ant mound with ill-intent.
I figured it was the nerves and discomfort and pushed that odd thought aside. He asked questions about my paperwork, gleaning more information where he needed it, scribbling down on a clipboard as I responded. It was after a long time of this medical interrogation that I realized that he hadn’t even told me his name. I tried to ask as he was in the middle of writing down one of my answers and he merely held up a finger, signaling to give him a moment. After that, he told me that his name was Dr. Jay Smith and that they’d like to run some more tests here in a bit to get a better picture of what they were dealing with in my case. I nodded and was summarily led by some dime-a-dozen pretty nurse into another room, changing into a gown and then going through test… after test… after test… It started out with fairly normal things like CT scans and X-rays, but got progressively more and more odd. I was scratching my head at why they’d need me to do a barium swallow- but I finally snapped when they told me the next procedure was a lumbar puncture to test my CNS fluid. That was when Dr. Smith came back in, all smiles, assuring me with all the medical jargon and charisma in the world that they needed to be absolutely sure of every last detail so that they could make sure that the trial of this therapy would help my condition. I couldn’t get a word in. I ended up finally giving in when a glance at the clock behind him told me that he’d been wearing me down for thirty minutes.
If my car had been towed while I was in here, I would be making them pay the impound for certain.
Finally, at length, and heavily exhausted from being shuttled room to room on a gurney, they brought me into a private room again. It looked like a normal nondescript hospital room, but I finally notice one thing that gave this room some hope for personality. A sign on the wall, in big bold black letters on a plain white background declared: FEEL EVERYTHING
Well, ok.
They hooked me up to some IV that they told me was just some saline, and offered to grab me a drink or a snack if I wanted it. I took them up on that and asked for a Sprite if they had it. Nope. Sierra Mist. I shrugged and agreed to the offered beverage and some chips. I asked if I could have my phone and they informed me that I’d be able to grab it once they were all done. I was painfully bored, tired, and irritable, but I still tried to be nice. I asked for something to read, and found that they had nothing. I asked them what I was supposed to do while I waited and they suggested that I take a nap. I nodded, and gave in, my eyes easing shut as they left- perhaps a little too quickly.
I woke up in the dark, a monitor next to me reading out what my blood pressure’s last recording was, alongside a feed of my heartbeat. I was groggy as the door opened, letting a piercing ray of light into the room which had me flinch and move to block my eyes from the offending light. A young, overly peppy nurse told me that they just needed to take another blood sample as she flipped my room’s lights back on, which had me shrinking further away from the offending fluorescent glare. She rolled a stool up to my side and prepped a needle and my arm with an alcohol swab while I was still trying to make heads or tails out of what was going on. How long had it been? How long had I been asleep? The room didn’t have a clock or windows, so I had no sense of ti-
The needle pierced my skin and I wrenched my arm back protectively, yelping in agony. My eyes were wide with horror as she merely watched me, sighing like I was some tantrum-throwing toddler. She capped the syringe that had torn into my flesh and then took out a fresh one. “Now, I know it’s a little hard to get used to at first, but you’re going to have to behave.” She admonished as her gloved hand pulled my arm gently but firmly back towards herself as she rubbed another alcohol swab over the inside of my elbow, and this time, it stung given the previous needle. It felt like hell lived in that small, tiny little wound. I trembled. I whimpered. My arm shook with the primal need to hold it protectively against myself, fighting with my effort to be compliant. Tears beaded my eyes as the metal slid back in, far too easily to be this painful. I groaned in anguish, feeling like I might faint. I bit my lip, and stopped immediately, pain finding me again as my teeth had effectively sliced into my flesh.
I could feel blood dripping down my chin and the nurse merely looked up at me with a Hallmark-worthy smile, and informed me that they needed another lumbar puncture in an hour…