Congenital insensitivity to pain with anhidrosis

Congenital insensitivity to pain with anhidrosis (CIPA) is a very 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 extreme temperatures. "Anhidrosis" means the body does not sweat, and "congenital" means that the condition is present from birth.

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Clinical description

Patients with this disorder are very likely to injure themselves in ways that would normally be prevented by feeling pain. The main features of the disorder are: lack of pain sensation, painless injuries of the arms, legs and oral structures, fever during hot weather because of inability to sweat, 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. People with this disorder may not be able to feel a physical orgasm.^{[citation needed]}

Cause

CIPA is caused by a genetic mutation which prevents the formation of nerve cells which are 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.

Incidence

CIPA is extremely rare. There are only 60 documented cases in the United States and more than 300 in Japan because it occurs more often in genetically homogeneous societies.^{[citation needed]}

It is also found in Gällivare, a Swedish village in Gällivare Municipality in northern Sweden, where nearly 40 cases have been reported; however, the disorder found in Vittangi may be of a different kind because those affected can perspire.^[1]

In Fiction

• In the episode "Insensitive" of House, M.D., Dr. House treats a teenage girl who had various ailments which were masked by her CIPA.

• In the episode "Sometimes a Fantasy" of Grey's Anatomy, a young girl has undiagnosed CIPA and a long string of injuries of her limbs and mouth. But since she feels no pain, she thinks she is a superhero.

• "Ingenious Pain" by Andrew Miller. Main character James Dyer was born unable to feel pain. [1]

• In the third chapter of William Peter Blatty's Legion, a patient of the character Vincent Amfortas has CIPA and dies due to complications associated with infections.

• In the upcoming game "Dark Sector", the main character Hayden Tenno is born unable to feel pain and is able to survive into adulthood (This is also a key element to the story when later infected with a virus that turns most afflicted mad from pain).

• In the Japanese Animation miniseries "Karas", the main character Otoha is unable to feel pain and fights on several occasions despite varying levels of injuries. Higher rates in Japan along with the revealed fact that Otoha is the product of incest imply that he has CIPA.

• In the Japanese animation and comic series "Loveless", four of the main characters are genetically engineered not to feel any pain. Two of these characters (twelve-year-old boys named Natsuo and Youji show traits of CIPA; they never gain the ability to feel pain, they cannot sweat or form goosebumps in response to extreme temperature, and there are multiple times when they unthinkingly do things that people who feel pain would never do (Youji, for example, holds a scalding cup of coffee with his thumb inside the cup).

See also

• Congenital insensitivity to pain
• Familial dysautonomia
• Hereditary Sensory and Autonomic Neuropathy

References

1. ^ Minde J (2006). "Norrbottnian congenital insensitivity to pain". Acta orthopaedica. Supplementum 77 (321): 2-32. PMID 16768023.