Let's Talk People Who Are Immune To Pain; CIPA

in steemstem •  17 days ago


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It seems Steemit has been stabilised for quite some time now. That's a relieve considering how much time I have wasted on video games last week waiting for the system to become usable again. Now, my holidays were already over (sad face) but I'm always researching stuff for my blog. I have invited one of my friends last week to join this community and it seems that he still thinking about it. We are now entering the final posting before completing our training period (housemanship) and I would, of course, seek opportunities to continue my master, maybe in Paediatric as soon as I can, we will see. Today, I'm going to talk about one of the least known conditions in the world called CIPA, congenital insensitivity to pain. Sounds like a good superpower, I mean if you are immune to pain, you can achieve lots of things, right? Well, it's partly awesome but for most of it, it can be your greatest weakness. I would explain that statement later.

Being one of the few people with this "gift" can be quite frustrating, emotionally and physically speaking. Feeling pain is super important as it gives a clue to our body that something is happening somewhere in the system which potentially could affect us in a certain way. We will respond to that kind of threat by committing a specific action that could potentially save our lives. Think about if someone were to stab you in the back, people with CIPA couldn't feel anything which would have put their lives in jeopardy. Think about how your children will react if they can't feel anything that other children could. Curiosity can be dangerous sometimes. Most of the documentation regarding patients with CIPA indicate that they were often presented with a few childhood scars which could have been caused by their own curiosity. Slicing their own finger, body or even face can be a normal (even though it is not) response from people with CIPA as they were trying to discover why they couldn't feel pain or temperature changes.

Let's take a case study as an example to illustrate the struggle bears by people who were having CIPA and how it can affect people around them. In 2012, a 12 years old boy who had chronic osteomyelitis was admitted into the paediatric ward of the Imam Khomeini hospital located in Tehran. He was referred from another medical centre for treatment of his right foot's heel sore which couldn't be managed properly by the patient himself since he was and currently is, mentally challenged (plus he has CIPA). That imposed a certain level of difficulty as people who were mentally challenged can't be educated or managed properly regarding their own condition thus they would depend heavily on their caregivers. This would also mean that they were less afraid of certain consequences of mutilating themselves and even ignore whatever injury they could have; infections are therefore inevitable. Now, the heel sore was not the only significant finding revealed by physical examination on this kid. He was also found to have disfigurement of his mandible and multiple scars from self-mutilation. Managing this kid can be quite difficult and often people with CIPA would require a multidisciplinary approach so that every system would be covered.

Even though it is not known whether this condition can only be inherited by children with parents who are consanguineous, in most of the cases (if not all), they are. The mode of inheritance is autosomal recessive and that means, the child has to inherit defective genes from both of their parents; if they inherit only one, they would be called as the carrier of the disease, often shows no symptom and live rather healthily with an intact sense of pain and temperature. There is no standard treatment protocol and the most definitive way of diagnosing CIPA is through genetic study. We can diagnose it rather easily from clinical points of view but sometimes, losing sensitivity to pain can be psychosomatic exhibited by children who underwent a traumatic experience, for example, child abuses. Self-mutilation in people with CIPA can be rather severe (some patients presented with amputated fingertips) or it can be as mild as cuts (yeah, that's mild for CIPA) but according to a few articles, people with CIPA can lead a rather normal life/childhood as long as they/people around them (parents for example) are committed to taking care of them as best as they could.


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Now, the reason why people with CIPA can't feel a thing even if we inflict the most painful stimuli to them can be related to a gene called SCN9A which plays an important role in peripheral pain's signalling as they code for voltage-gated sodium ion channels. They are important in relaying nociceptive signal (pain) and thus, like anyone could have guessed, people with CIPA inherit a defective SCN9A gene which indirectly affects their nociceptive signalling, hence, no pain of the sort. It's really not like what all of us imagine and people with CIPA can bleed internally without them knowing about it; they can be unconscious or worse, die anytime if they were not managed properly. Some of them would have a biweekly medical checkup to ensure everything operates within normal parameters. I remember seeing a quote in a TV series (I can't remember which one) which stated that "pain is the only thing that made us humans", so what are people with CIPA? Of course, we can't take that seriously since it was probably fictional anyway.

CIPA And Anaesthetic



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One of the most interesting topics to be discussed with academicians regarding things related to CIPA is anaesthetics. Just imagine, people with CIPA can't feel pain and the main purpose of giving people anaesthetic before a surgery is to achieve that kind of objective, so do they need any kind of anaesthetic or we can just gut them while they watch? That innate anaesthetic seems like cool but eventually, they would need anaesthetic because controlling peri-operative pain is just a part of the overall objectives of giving people anaesthesia. Some patient's might deemed unsuitable for surgery because they can't tolerate it physically but the main concern for people with CIPA is the autonomic part. Even though it seems like they have a selective autonomic dysfunction, they could have developed further complications such as regurgitation of gastric content, aspiration and worse pneumonia. If you remember one of the component of general anaesthesia that I wrote a few months back, giving people some potent analgesic such as fentanyl can improve patient's condition during surgery as they suppress pain but in people with CIPA, the aim of giving fentanyl is to eliminate stress while the patient is being intubated and all.

I did mention the risk of CIPA patients to get gastric content regurgitation, did I? As there were abnormalities in the autonomic function, people with CIPA respond quite similar to people who have uncontrolled diabetes mellitus in terms of gastric emptying rate; they were quite slow. This would predispose them to aspiration which can lead to pneumonia if let say those stomach content regurgitate into the lung. As the condition of patients with CIPA can be rather unpredictable, they were usually considered as someone who was having a full stomach which would require a different anaesthetic technique called rapid sequence induction. I would write about it somewhere in the future but it is basically for people who were high risk for regurgitation which include pregnant women, obese people, emergency cases etc. Preparation is important for a successful surgery but whatever happens during the surgery can be quite unpredictable. People with CIPA seems to have a higher incidence of bradycardia compared to a normal, healthy individual. It seems that there is no definite explanation to explain whatever lead to the bradycardia incidence but in some people, it can be due to cardiac instability as a result of autonomic dysfunction.

It really difficult to say that we definitely sure what kind of anaesthesia that people with CIPA would require as it would depend on their clinical condition, however, most of the papers which discussed this kind of topic agreed that people with CIPA would have the least complications or problems pertaining to their post-operative care. If you ever watch the medical series entitled "House MD", you would notice that he encounters a patient with CIPA in the season 3. One of the most epic scenes that might make this one of the most interesting cases is how he gutted the patients without anaesthesia and pull out a 6-metres tapeworm from her intestine. Looks cool but totally unrealistic to the real clinical scenario. Well, take the educational stuff and leave the totally unethical practices. He is a good and competent doctor but like anybody else, he still a human.

Sources



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This is quite interesting, I always thought that it would be so dope to have CIPA haha so many things you can achieve! But your post made me see the other side, the real one, thanks for the valuable infos. Also wish you luck for your master studies I'm just starting mine in French Litterature!

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Thanks for your wishes. Best of luck in your study!

I upvoted your post.

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the aim of giving fentanyl is to eliminate stress while the patient is being intubated and all.

I remember years ago when I underwent surgery, the doctor insisted I not be aware. I was very skittish and she didn't want to deal with my stress during surgery. This wasn't so much about pain as my ability to be calm and cooperate.

A very interesting article. I'll have to check out some of those sources.

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Thanks. Do check those sources and let me know if you have anything to say so that I can improve.

This reminded me of Tim Cridland - "Zamora" (the torture king). He doesn't feel pain and could even perform incisions on his body on live audience without feeling a damn thing... Mehn, I wouldn't mind that kind of mutation. But sometimes, pain can actually help you to survive.

Nice piece buddy

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I'm not sure who is he but you are right; pain does help us in a certain way. It's like a warning system for us to do something or we're screwed.

Woo! Love your post - we've given you a full upvote with a chance to be featured in the weekly curation. Hope this helps you continue to write such amazing posts. We've also resteemed this! We encourage you to use the #naturalmedicine tag so we all can connect and find each other.

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Very interesting write up I must say. It sort of reminded me of the main villain in Deadpool 1 who couldn’t feel pain lol. If I am drawing conclusions from this, I would say Man had CIPA. Funnily, his own was due to some sort of mutation that occured in his life. It wasn’t inherited in an autosomal recessive fashion.

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That was hilarious. I remember that movie. Yeah, when I watched it, that's the first thought that crossed my mind too. Pity, he got CIPA. He can die at any time, anywhere if he got internal bleeding; he won't feel it.

You have chosen a very interesting topic. Triggers some associations and memories. I read something about hypnosis surgery. The pain sensation topic is really fascinating, because it raises the question of how a patient who does not tolerate anaesthesia (as I have heard somewhere before) can be operated on painlessly. I think they did experiments with it in France.
You know that you don't feel much pain when you're exposed to a lot of adrenaline and also when you're in shock, as far as I know. Also that it hurts more when you count on the pain (the psychological aspect of the pain expectation).

I looked for one source on research in hypnosis:

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5428569/

It's interesting what the researchers say about that. I thought it wasn't easy to understand (at least not from my point of view).

Then your article reminded me of an SF novel, I think it was a scene where a woman with built-in blades in her fingers was carrying out a dangerous mission and was seriously injured. She injected so much painkiller into her blood that she could complete her actions despite a broken leg. The bad damage she caused herself could only be cured in the further course of the Science Fiction story. :) I think it was "Neuromancer", but I'm not quite sure anymore.

In any case, your article is very worth reading.


edit: it was Neuromancer from William Gibson

Molly, an assassin and former prostitute, carries various body modifications, such as fixed lenses with residual light amplifiers and retractable scalpel blades under her fingernails. She works for Armitage as a mercenary for the rough (called street samurai in the novel). She quickly develops an affection for Case. She tells of her former love, Johnny, a data courier who was killed by the Yaks (Yakuza). This is obviously Johnny Mnemonic, the main character from Gibson's most famous short story, which was the basis for a movie.

unfortunately the English wiki entry is much shorter than the German one. So I made a translation of the Molly-chapter.

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Thanks for the detailed feedback, @erh.germany. Did you know some of the medical officers asked patients to cough while they were giving them injections? The moment they cough, the doctor would stick the needle in and the patient won't feel a thing. It seems, our mind can be too preoccupied with task hence ignore pain. It's like a cut, if you didn't realize it while doing something interesting, you won't feel it but once you see it, with your own eyes, you started to feel pain. It's interesting, isn't it?

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Very much so! I experienced it myself. Even I burnt a good deal of my skin between the little and the ring finger - guess from the oven when I pulled out a casserole - but didn't realize it until the next day.

HaHa! Now this reminded me on Arthur Dent, who is able to fly in that second before he falls face to the ground when some pretty female legs distract him just right on time! Maybe you know Hitchikers through the Galaxy from D. Adams :)