What Is CIPA?

Congenital Insensitivity to Pain with Anhidrosis is rare autosomal recessive disorder of the nervous system which prevents the feeling of pain or temperature, and prevents a person from sweating.Mental retardation is common.CIPA is the fourth type of hereditary sensory and autonomic neuropathy (HSAN), and is also known as HSAN IV. Pain alters the quality of life more than any other health-related problem, and it is one of the implements of body protection. It interferes with sleep, mobility, nutrition, thought, sexual activity, emotional well-being, creativity, and self-actualization. Congenital insensitivity to pain is a rare disorder, first described in 1932 by Dearborn as Congenital pure analgesia. Congenital insensitivity to pain and anhydrosis (CIPA) is a very rare and extremely dangerous condition. People with CIPA cannot feel pain. Pain-sensing nerves in these patients are not properly connected in parts of brain that receive the pain messages. CIPA is extremely dangerous, and in most cases the patient doesn't live over age of 25. Although some of them can live a fairly normal life, they must constantly check for cuts, bruises, self-mutilations, and other possible unfelt injuries. Self-mutilation is an almost invariable feature of this disorder, most often involving the teeth, lips, tongue, ears, eyes, nose, and fingers. The odds of being born with this condition are about 1 in 125 million. People with CIPA also cannot feel extreme temperatures, or sweat, both creating even more necessary care. However, in a patient with CIPA, the gene encoding the Neurotrophic Tyrosine-Kinase receptor (NTRK1 gene), is mutated in a way that interferes and halts the autophosphorylation process, therefore stopping signals of pain and temperature from being sent to the brain.



A Story About A Painless Girl