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Congenital Insensitivity to Pain with Anhidrosis

Discussion in 'Neurology' started by waleed, Sep 8, 2011.

  1. waleed

    waleed Moderator

    Aug 12, 2011
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    Life is full of a lot of different emotions. Some are good, some are bad, and some we could do without. When it's 90 degrees outside, we sweat in order to create a cooling effect and regulate our body temperature. What if you couldn't sweat? How about freezing temperatures? What if you couldn't feel how cold it was outside?

    Have you ever had a time where you were in a hurry and wanted to grab something to eat, but had to wait till it cooled down so it wouldn't burn your mouth? What if you couldn't tell how hot or cool it was? And what about pain? What if you could never feel a headache, or a paper cut, or even a broken arm?

    This condition is called Congenital Insensitivity to Pain with Anhidrosis or CIPA. This is a very rare inherited disorder that I could never imagine living with. This disorder prevents the nervous system from feeling the sensations that are accompanied with pain, heat, and cold.

    Congenital is a term meaning that from the day this person is born, they can not feel pain or even know the meaning of the word pain because they have never felt it. Pain is normally a feeling that come from the nervous system and may have a sharp or dull sensation. The inability to feel this can cause various health problems. Normally when you go to the doctor, the doctor asks you where it hurts. However in this situation, the patient will normally say they feel fine. They feel fine because, they can't tell you where it hurts because they can't feel where it hurts. Congenital Insensitivity to Pain with Anhidrosis is a very hard condition to treat.

    So what exactly causes this to happen? CIPA is a mutation that has occurred in the genetic make up during the formation of nerve cells that their jobs are to send the brain signals of pain, heat, and or cold.
    Congenital Insensitivity to Pain with Anhidrosis, is a lot different then Congenital Insensitivity to Pain. Adding the term Anhidrosis to the condition adds the symptoms of lacking the ability to sweat, this ultimately links the inability to recognize hot or cold. The Anhidrosis portion of CIPA is caused by the under activity of the sympathetic nervous system. The Sympathetic Nervous System is a branch of the nervous system that becomes active during times of stress.

    In the United States, there are only 35 citizens that have Congenital Insensitivity to Pain with Anhidrosis. Since this disease had been reported back in 1983, less than 60 cases have ever been reported. Most of the babies that carry this disease do not live past 3 years of age, if they make it past 3 years old, many do not make it past 25. The reason behind the short life span is because when it comes to babies, based upon size and the inability to sweat, overheating kills them easily. Without proper actions, such as monitoring vital signs and temperatures, the parent or guardian will never know the baby is hot. The inability to seat normally leads to recurrent fevers that are unexplained and can be fatal due to hyperthermia. Fevers are normally seen as a time frame of your bodies defenses kicking in to fight infections, but if not treated, it can cause febrile fits and death.

    Those who survive their infant years have a high percentage of becoming mentally retarded and even during their infant years; this has a probability of happening because the higher your body temperature goes the more harmful bacteria and virus attacks can occur. When infections or viruses attack, in some cases blood vessels can swell and cause aneurysms, all of which a CIPA patient will not even feel.
    I believe that paranoia at some point would have to occur at some point in time with this disorder. I was not able to find out any information on paranoia linked to Congenital Insensitivity to Pain with Anhidrosis, however if you think about it... suffering from this type of condition, where you have to check your heart rate four or five times a day, check to make sure your body temperature hasn't risen above 98.6 degrees Fahrenheit several times a day, as well as constantly monitoring every inch of your body for any signs of cuts, scraps, or bruises that you could never feel happening. If you think about it, paranoia would have to set in.

    I could see the paranoia being so extreme, that this condition could cause one to feel imprisoned by their own fear. Fear of turning on the hot water, but not being able to feel the temperature, to wash your hands or to shower, then examining your body for burns just in case you didn't turn the cold water on far enough to make it a decent temperature because you couldn't feel the temperature.

    I could only imagine the paranoia of the children's parents. Watching the child when they eat, just to make sure the child doesn't bite their tongue through when they eat, or making sure that there are no sharp objects in sight.

    Treatments for CIPA do not always work however there are some cases where naloxone may be used as a treatment. Naloxone is a chemical that acts within the nervous system of the body by blocking the nervous system from causing the inactions that occur within the group of cells that receives the message to initiate the sensation of pain, heat, or cold.

    Most treatments are hard to narrow down for this condition because each CIPA patient may have other conditions attached to it. Such as; the absence of a sweat gland, nerve fibers, ulcers, and other sub-conditions. Recently I had viewed an episode of "House" where a young girl suffered from CIPA and wasn't put under any type of anesthesia for a procedure. This of course is something that has to be done. Even thought the patient is lacking the sensation of pain, some patients may have tactile hyperesthesia, which means they may not be able to feel pain and sweat, but they are sensitive to touch. Anesthesia use is also required because CIPA patients already have problems with managing their temperature, so the anesthesia is necessary to avoid accidental fractures as well as relaxing the muscles. One of the most obvious reasons to make sure anesthesia is in use is to calm the patient; I highly doubt someone would be comfortable enough watching themselves be operated on or the sight of blood that could be involved.

    Japan has more than 300 cases of CIPA that have been documented. Many surveys have been distributed to those suffering from CIPA and have shown that of 34 questionnaires all of the patients had damaged sweat glands, little sweating was verified in eight of the 34 patients and seven patients stated that they could not sweat at all. Most of those who filled out the questionnaires stated to have some degree of mental deficiency.

    In conclusion, if you are patient sufferings from CIPA, this is not a condition that you should be taking lightly. Seek medical assistance and always, always be careful.


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  2. kkEspana

    kkEspana Well-Known Member

    Jun 3, 2012
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    you are right

    Yes, you are right!

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