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Congenital Insensitivity to Pain with Anhidrosis (CIPA) Print E-mail

Dr. Kennedy This is an extremely rare inherited disorder of the nervous system which prevents the sensation of pain, heat, and cold. A person with CIPA cannot feel pain or differentiate even extreme temperatures. "Anhidrosis" means the body does not sweat. This conditionis present from birth. Patients with CIPA are very likely to injure themselves in ways that would normally be prevented by feeling pain. These people suffer fever during hot weather (because of inability to sweat), painless injuries of the arms, legs and oral structures, sometimes mental retardation, infection and scarring of the tongue, lips and gums, chronic infections of bones and joints, bone fractures, multiple scars, osteomyelitis and joint deformities, which may lead to amputation.

CIPA is caused by a genetic mutation which prevents the formation of the nerve cells responsible for transmitting signals of pain, heat, and cold to the brain. Overheating kills more than half of all children with CIPA before age 3. The genetic mutation is in the gene encoding the receptor tyrosine kinase (NTRK1 gene).

Incidence

According to Wikipedia CIPA is extremely rare, on the order of one out of 357,000 and there are 84 documented living cases in the United States and more than 300 in Japan. Only one case is documented in New Zealand, while 2 cases have been documented in Morocco. 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 sweat 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. A high percentage of the individuals who make it past three years suffer from mental retardation and even during their infant years, retardation is likely because higher body temperature allows more harmful bacteria and virus attacks to occur. In some cases, when infections or viruses attack, blood vessels can swell and cause aneurysms, all of which a CIPA patient will not even feel.

Treatment

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 including the absence of sweat glands, nerve fibers, ulcers, and other conditions.



The information in this article is not meant to be medical advice.�Treatment for a medical condition should come at the recommendation of your personal physician.

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