CIPA Disease: When a Person Can't Feel Pain

Have you heard about Ashlyan Blockers who is affected by CIPA from her infant stage? Ashlyn is now 12 who suffers from congenital insensitivity to pain with anhydrosis (CIPA), a disorder that avoids her brain from receiving signals that she never experience pain.

CIPA stands for cognitive insensitivity to pain and anhydrosis. It is a rare hereditary disease. It causes affected individuals unable to feel pain and unable to sweat at high temperatures (anhidrosis). In short, an autosomal recessive disorder of the nervous system prevents the feeling of pain or temperature and prevents a person from sweating. So, it is also known as hereditary sensory and autonomic neuropathy type IV (HSAN IV).

The name of the disease (CIPA) is quite descriptive because it elucidates several essential characteristics. Several examples ultimately make you understand what is the mean of descriptive? i.e., sensory neuropathy means that it is a type of nerve disease that majorly affects the nerve (control survival functions of the body). HSAN IV or CIPA majorly affects the autonomic nerve that is responsible for controlling sweat.

I know guys! A thought process is going on, in your mind as such a disease exist in the world? how a person lives his whole life with this disorder? Does he behave normally? or at which age do individuals with CIPA get to die? If a person does not sweat out at a high temperature that means he/she cannot understand the warmness/coldness of other individuals’ hands. If a person with CIPA does not feel any pain that means if any wound happens on his body or slashes by knife, on that period he/she will not feel pain? These types of questions were coming to your mind. Trust me, guys! I also was thinking like that when I listen about this disease from a friend. Let’s discuss further………………………………………………………………………….…………….!!!

Sign & Symptoms

CIPA disease presents at birth and makes people unable to sense pain or temperature and unable to sweat. Symptoms become appears during childhood or it is typically diagnosed during childhood.

· Lack of pain- Most of the people who are affected by CIPA do not complain of pain or sweat. While children with CIPA initially develop injuries or burns without crying or complaining. Parents observe that a child with CIPA is a mild-mannered kid instead of noticing a problem. After a while, parents may begin to wonder why the child does not respond to pain. In that state, a doctor would like to conduct several diagnostic tests for nerve disease.

· Children with CIPA often injure or burn because they do not ignore painful activity. In addition, they can experience infected wounds because they do not impulsively protect their wound from pain. Sometimes, these children with this disease complain of unbearable pain without getting any wound that wonders to parents. Such kind of pain is knowns as ghost pain.

· Anhydrosis- Hydrosis means sweating, while anhydrosis means lack of sweating. Generally, sweat on the body surface helps to cool off the overall body when we get too hot from a high fever or exercise. But, children with CIPA suffer the consequences of anhydrosis.

Diagnosis

There are no simple X-ray tests or blood tests that can determine CIPA. Several people who have a CIPA possess abnormally underdeveloped nerves and lack sweat glands on a biopsy.

Genetic tests conducted for CIPA can be done before birth or during childhood or adulthood. It is a diagnostic test and also known as a genetic abnormality that determines an abnormality of this gene, confirming the diagnosis of CIPA.

· Primary Causes

As we know CIPA is a hereditary disease which means that any person who has CIPA must inherit the gene from both parents. Typically, the parents of an affected child carry the gene but do not have the disease if they only inherit the gene from one parent.

Autosomal recessive disorder, CIPA is generally occurred by a defective gene caused by sensory nerve and some autonomic nerves do not fully develop. Thus, the sensory nerve cannot function properly to send messages of pain and temperature, as well as the body, cannot develop a sweat.

Treatment

Currently, there is no treatment available that can diagnose CIPA disease or replace the missing function of sweating or sensation of pain. Children or adults with the disease have to learn to try to avoid injuries and to monitor injuries intently to prevent infection. Adults with CIPA have to check their BP level, sugar level and other normal tests daily, to understand that everything is going on balanced in their body.

The individual with CIPA has emotional instability so it is essential to be with them and support them to complete their ambitions instead of making them feel miserable.

Stay Happy! Stay Long Live

Best Wishes for the future! 😇😇😇😇✌