In 1932, a clinician described a fifty-four year-old man who reported never having felt pain, despite a list of injuries including “a blow in the face with a pickaxe, a bullet through a finger, a broken nose, severe laceration of the knee, and a burned hand—all without apparent pain.” The man, named Edward Gibson, made ...
Congenital insensitivity to pain and anhydrosis (CIPA) is a very rare and extremely dangerous condition. People with CIPA cannot feel pain [1]. Pain-sensing nerves in these patients are not properly connected in parts of brain that receive the pain messages.
Most people afflicted with the disorder do not live past age 3, though not all deaths are due to the lack of pain. In fact, half of CIPA deaths are due to overheating because of the person's inability to produce sweat. This causes hyperthermia, or extremely elevated body temperature, which then leads to death.
CIPA is characterized by loss of pain and thermal sensation accompanied by mental distress. Other sensory modalities such as touch, pressure, and vibration are not affected.
There is no cure for CIPA and the treatment is mostly about staying safe. It's important to avoid injuries and monitor any wounds for infection. Support groups can be helpful for social support and tips for living with CIPA.
CIP is often diagnosed in early childhood due to a lack of typical pain response (wincing or crying) during standard medical procedures like vaccination and treatment for common childhood injuries like falls.
Loss of sensory neurons leads to the inability to feel pain in people with CIPA. In addition, people with CIPA lose the nerves leading to their sweat glands , which causes the anhidrosis seen in affected individuals.
Since the abilities of CIPA patients to perceive taste and smell were not basically impaired, despite their lower sensitivity to capsaicin, it was suggested that their dietary habits were only minimally affected, except for intake of pungent foods.
Congenital insensitivity to pain with anhidrosis (CIPA), also referred to as hereditary sensory and autonomic neuropathy type IV, is a rare inherited autosomal recessive disease. The incidence of this disorder is about 1 in 125 million, with few cases reported worldwide to date [1].
Clinical Comments
A distinct form of congenital insensitivity to pain with anhidrosis (CIPA) has been identified, also known as hereditary sensory and autonomic neuropathy type IV. Nearly 20% of patients with this disorder die within the first 3 years of life because of hyperpyrexia.
“This helps us to understand why 30% of COPD can occur in people who never smoked.” With normal aging, lung function declines, so people who already have low lung function to begin with may develop COPD later in life, even if they don't smoke, he explained.
Painful stimuli usually provoke either withdrawal or emotional changes in healthy individuals but fail to do so in individuals with CIPA.
dogs with diabetes mellitus
The cardiac index of parasympathetic activity (CIPA) was determined for each dog over a period of 2600 heartbeats. The mean, median and modal CIPA values were significantly lower in the diabetic dogs than in the healthy dogs.
Channelopathy-associated congenital insensitivity to pain is a rare condition, though its exact prevalence is unknown. It is one of about 10 disorders characterized by a lifelong inability to sense physical pain.
Frequency. Congenital insensitivity to pain is a rare condition; about 20 cases have been reported in the scientific literature.
Worldwide several hundred cases have been reported, but the exact prevalence is unknown. The condition is inherited and is most common among Negev Arabs aka Negev Bedouins. For Japan, the prevalence is estimated at 1/600,000-950,000. Approximately 20% of people with CIPA die of hyperthermia by age 3.
CIPA is caused by changes in the NTRK1 gene. Inheritance is autosomal recessive. Resource(s) for Medical Professionals and Scientists on This Disease: RareSource offers rare disease gene variant annotations and links to rare disease gene literature.
Despite a lack of peripheral pain sensation, CIPA patients still respond to airway manipulation, and a certain depth of anesthesia is needed to reduce the hemodynamic response to endotracheal intubation (8).
COPD does not usually become noticeable until after the age of 35 and most people diagnosed with the condition are over 50 years old. See your GP if you have the following symptoms: increasing breathlessness when exercising or moving around.
Interesting to mention is the recent discovery of the existence of patients suffering from what has been defined Congenital Absence of Pain with Hyperhidrosis (also called Congenital analgesia with hyperhidrosis or Congenital indifference to pain with hyperhidrosis), pathology somewhat opposite to note CIPA.
Jo Cameron only realises her skin is burning when she smells singed flesh. She often burns her arms on the oven, but feels no pain to warn her. That's because she is one of only two people in the world known to have a rare genetic mutation.
The link between pleasure and pain is deeply rooted in our biology. For a start, all pain causes the central nervous system to release endorphins – proteins which act to block pain and work in a similar way to opiates such as morphine to induce feelings of euphoria.
Similarly, dogs can tell if you're feeling pain or discomfort, and may try to soothe your inflamed joints with licking and cuddles. Our furry friends are very observant of our body language and facial expressions, and notice when their humans aren't feeling well just by viewing any subtle changes in our daily routines.
Dogs are notoriously good at hiding signs of pain, which is great as a survival tactic in the wild but not so good for the owners of domesticated dogs wanting to ensure their dog's quality of life and wellbeing.
CIPA is mainly caused by mutations in the neurotrophic tyrosine kinase receptor type 1 gene (NTRK1).