CIPA

Congenital Insensitivity to Pain with Anhidrosis

What is it?

Congenital Insensitivity to Pain with Anhidrosis is a condition involving the nervous system preventing you from feeling physical pain.

The Nervous System

The brain, cranial nerves, spinal cord, spinal nerves, and sensory receptors are just a few of the things that make up the bodies nervous system. There are many different divisions of the nervous system including the central, peripheral, enteric, somatic, and autonomic systems. Axons are found in the peripheral division of the nervous system. They move messages to the brain and the rest of the body. Connective tissue covers the axons which is called endoneurium. They are then bunched together into sections called fascicles. This makes up one nerve. Your nerves allow directions to be sent from other parts of your body to your brain which tells your other body systems what to do.

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Nerve Cell

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The Nervous System with CIPA

For people with CIPA, the connection between nerves is found to be regular. However, nerve fibers are not found which makes communication between the myelinated and unmyelinated receptors impossible. These are sensors on the end of nerves that determine how fast a feeling is sent to the brain. The feeling can't be sent at all because of the lack of nerve fibers.

Infographic

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Onset

To have CIPA someone has to receive a mutated gene from both parents. The mutation targets the autosomal chromosome gene. Doctors believe that as you get older with this disease, it will get worse.

Target Population

This disease is very uncommon and one gender is not targeted more than the other. Only 35 cases are known in the United States. You are born with CIPA and symptoms start to show immediately.

Diagnosis/Signs and Symptoms

There is no specific way to know when CIPA is diagnosed. There is no tests doctors can perform in order to confirm a child has this disease but there are signs. Parents realize something is wrong when their child does not cry or notice when they get hurt. Teething is one of the first signs of this disease because the child won't know when to stop. Once a parent realizes these signs, they go to a doctor but unfortunately there is not much they can do. The obvious sign of getting this disease is the inability to feel extreme temperatures and other painful feelings. Extreme loss of sensory perception is a symptoms as well as the lack of sweat glans which makes it impossible to sweat. In some cases the patient has special needs but in other cases the patient has a regular IQ.
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Treatment

CIPA is not curable yet but there are many things you can do in order to keep yourself safe while dealing with this disease. Parents of children should teach them to come to them if they see any blood on them. You may also want to live in a cooler climate so getting too hot is not a constant problem. Physical therapy for your joints can help too because the child's joints will begin to where down but will not know.

Prognosis

About half of all cases under 3 years of age die, the other half usually do not make it past 25. This is because anhidrosis means the inability to sweat so many patients die from overheating.
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