Naturally Nonchalant: An article on CIPA

Introduction

In recent times, people prefer to be “nonchalant” or “sigma” and act like they’re unbothered by whatever is happening around them and in their lives. Although this can be hard to fully master, a very rare genetic disorder called CIPA makes the person almost immune to physical pain, but people with CIPA can still feel emotional pain, such as sadness, happiness, etc.

Inspiration: There’s an episode in the show, House, where a girl with CIPA gains the ability to feel pain after her mom undergoes an accident, but we won’t indulge in such topics, so let’s keep the tone HAPPY!

No context

Body Alarm

Some people would think that having the ability to not feel pain would be a blessing, but instead, you’d risk your life even more without the feeling of pain.

This is simply because feeling pain is important, and as much as we hate to feel pain, it's necessary because the instant we feel pain, it’s like an alarm in our body going off, signaling that we’re pushing our limit and that’s when we stop and prevent ourselves from further injuries.

An example is teething, which is a major developmental process in growing up. Babies have the urge to bite into whatever they find to soothe the pain. In the case of babies with CIPA, because they don’t feel pain, they don’t know when to stop. They might chew through their tongues or bite their fingers until they bleed, and while they can see the blood or their finger bleeding, they won’t feel the pain even slightly.

No pain, No gain (#motivational)

Genetic Mutant

So, how is CIPA caused?

CIPA stands for Congenital insensitivity to pain with anhidrosis, which is a genetic mutation that occurs when the offspring receives a copy of the gene from both parents, but this gene is hidden or recessive in the parents' bodies. The mutation occurs in the NTRK1 gene, which is a receptor for nerve growth factor. A mutation in this gene causes the nerves not to grow properly and causes defects in the functions of the nerves in receiving pain signals.

Anhidrosis is a condition where the body doesn't sweat normally in response to heat or exercise, which causes problems with temperature regulation across the body.

NTRK1 Gene

But to understand this further, we’ll have to discuss how we feel and process pain.

Title of Agony and Despair

How does our body know and realize that we’re in “pain” or that something is going wrong in our body? To understand this, we take a deeper look into our nervous system and the neuron receptors.

Neurons consist of three major parts: dendrites, the cell body, and the axon. Dendrites- Receive messages from other neurons and allow the transmission of messages to the cell body. Cell Body- Contains the basic components such as the nucleus, mitochondria, etc. Axon- Carries electrical impulses from the cell body to the axon terminals that pass the impulse to another neuron.

They also contain a sheath known as the Myelin Sheath, which is an insulating sheath that forms around nerves and helps in generating the message, so the more myelin, the faster the message.

Structure of a Neuron

The basic pathway is that nerves carry messages from the body to the spine, and then the spine carries the messages to the brain.

Peripheral nerves are present in the pathway that are important for feeling pain. They end in receptors that sense touch, pressure, and temperature.

Some of them end in nociceptors, which sense pain. These then send pain messages in electrical impulses along the peripheral nerves, which then travel through the spine and then to the brain.

Nociceptor axons are usually unmyelinated, which means that they are slow. The axons that carry the pain messages from the nociceptors can be either myelinated or unmyelinated, which means that pain messages can travel quickly or slowly.

Which path the pain message takes depends on the type of pain — severe pain travels on the fast path, while less severe pain travels more slowly. Now, the pain threshold varies from person to person, and so does the pathway.

People with CIPA show a decrease or absence of nerve fibers in both unmyelinated and myelinated. This defect causes the brain and body to have no communication so they don't feel pain because there is no message being sent to the brain.

CIPA patients also miss a nerve supply in the epidermis, which is the outermost layer of the skin, and the eccrine sweat glands. Eccrine sweat glands are all over your body and are used for body temperature regulation, but since there are no nerves in the skin and sweat glands, there is no way for the body and the brain to communicate with one another, which results in Anhidrosis.

Opposite of Anhidrosis

Precaution because there IS NO cure

Since there is no cure at the moment for CIPA, parents with children who’ve been diagnosed with CIPA take extra measures to help their child live a safe and healthy life.

These include

• A child might wear protective eyewear so he won’t scratch his eyes.
• Because they have difficulty telling when they need to use the restroom, setting a timer on a wristwatch can help remind them.
• Parents also childproof their houses and keep away anything hot or dangerous.

CYA later

Using CYA felt right because of typing CIPA so many times, but to conclude, this disorder is a very life-threatening and dangerous disease to have, as they can feel pressure, but not pain, so they can injure or mutilate themselves without meaning to and not even shed a single tear.

Learning about this was genuinely super interesting, and trying to figure out a cure will also be challenging but intriguing.

TILL next time, “stay chalant” and cry all you want!!

Outcome of being chalant