Congenital insensitivity to pain
Encyclopedia
Congenital insensitivity to pain (CIP), also known as congenital analgesia, is one or more rare conditions where a person cannot feel (and has never felt) physical pain
. The conditions described here are separate from the HSAN
group of disorders, which have more specific sign
s and etiology
.
and sensation are otherwise normal; for instance patients can still feel discriminative touch (though not always temperature), and there are no detectable physical abnormalities.
Children with this condition often suffer oral cavity damage both in and around the oral cavity (such as having bitten off the tip of their tongue
) or fractures to bones. Unnoticed infection
s and cornea
l damage due to foreign objects in the eye are also seen. Because the child cannot feel pain they may not respond to problems, thus being at a higher risk of more severe diseases or otherwise.
In some people with this disorder, there may be a mild intellectual disability, as well as an impaired corneal reflex
.
Hansen's Disease, an infectious illness, can result in the progressive destruction of the nerves; the disease can be passed on to offspring, sometimes remaining dormant except for nerve damage. This scenario can mimic the truly hereditary diseases described above.
.
For example, it is found in Vittangi
, a village in Kiruna Municipality
in northern Sweden
, where nearly 40 cases have been reported.
Also, Ashkenazi Jews have been found to have a higher risk, though it is still unusual.
's Millennium series, former boxer Paolo Roberto discovers that Ronald Niederman was born with congenital analgesia and is therefore, as he describes "invulnerable" physically. ("He just keeps on going!") Even so, Roberto continues that his inability to perceive pain is a severe danger because Niederman has no way of understanding when his body is being put in mortal danger by way of punches, blows, burns, or even gunshots. It is suggested that human awareness of painful stimuli is an evolutionarily necessary component of vulnerability to injury and death.
The Grey's Anatomy
episode Sometimes a Fantasy
features a young girl named Megan Clover that suffers from congenital analgesia believing herself to be a superhero.
The episode of House, M.D. entitled Insensitive also features a young girl who suffers from congenital analgesia, making her sickness extremely difficult to diagnose.
In the 2011 slasher film
Wrong Turn 4: Bloody Beginnings
, the characters Three Finger, One Eye and Saw Tooth are said to have the disorder by the head of the sanatorium
they are in in the portion of the film set in 1974, with it being theorized that the condition was the result of inbreeding
. Due to their inability to properly register pain, the three were depicted as being prone to self harm in their youth, mutilating, severing and eating some of their own body parts.
In the play "Almost, Maine" by John Cariani, the vignette "This Hurts" is about a man with Congenital Analgesia, or "Hereditary Sensory Neuropathy Type 4", who is clocked in the head with an ironing board, and in the end feels the pain of love.
Pain
Pain is an unpleasant sensation often caused by intense or damaging stimuli such as stubbing a toe, burning a finger, putting iodine on a cut, and bumping the "funny bone."...
. The conditions described here are separate from the HSAN
Hereditary sensory and autonomic neuropathy
Hereditary sensory and autonomic neuropathy or hereditary sensory neuropathy is a condition used to describe any of the types of this disease which inhibit sensation.They are less common than Charcot-Marie-Tooth disease....
group of disorders, which have more specific sign
Medical sign
A medical sign is an objective indication of some medical fact or characteristic that may be detected by a physician during a physical examination of a patient....
s and etiology
Etiology
Etiology is the study of causation, or origination. The word is derived from the Greek , aitiologia, "giving a reason for" ....
.
Presentation
For patients with this disorder, cognitionCognition
In science, cognition refers to mental processes. These processes include attention, remembering, producing and understanding language, solving problems, and making decisions. Cognition is studied in various disciplines such as psychology, philosophy, linguistics, and computer science...
and sensation are otherwise normal; for instance patients can still feel discriminative touch (though not always temperature), and there are no detectable physical abnormalities.
Children with this condition often suffer oral cavity damage both in and around the oral cavity (such as having bitten off the tip of their tongue
Tongue
The tongue is a muscular hydrostat on the floors of the mouths of most vertebrates which manipulates food for mastication. It is the primary organ of taste , as much of the upper surface of the tongue is covered in papillae and taste buds. It is sensitive and kept moist by saliva, and is richly...
) or fractures to bones. Unnoticed infection
Infection
An infection is the colonization of a host organism by parasite species. Infecting parasites seek to use the host's resources to reproduce, often resulting in disease...
s and cornea
Cornea
The cornea is the transparent front part of the eye that covers the iris, pupil, and anterior chamber. Together with the lens, the cornea refracts light, with the cornea accounting for approximately two-thirds of the eye's total optical power. In humans, the refractive power of the cornea is...
l damage due to foreign objects in the eye are also seen. Because the child cannot feel pain they may not respond to problems, thus being at a higher risk of more severe diseases or otherwise.
In some people with this disorder, there may be a mild intellectual disability, as well as an impaired corneal reflex
Corneal reflex
The corneal reflex, also known as the blink reflex, is an involuntary blinking of the eyelids elicited by stimulation of the cornea , or bright light, though could result from any peripheral stimulus. Stimulation should elicit both a direct and consensual response...
.
Causes
In some cases, this disorder can be in the voltage-gated sodium channel SCN9A (NaV1.7). Patients with such mutations are congenitally insensitive to pain and lack otherHansen's Disease, an infectious illness, can result in the progressive destruction of the nerves; the disease can be passed on to offspring, sometimes remaining dormant except for nerve damage. This scenario can mimic the truly hereditary diseases described above.
Types of congenital pain indifference
There are generally two types of non-response exhibited.- Insensitivity to pain means that the painful stimulus is not even perceived: a patient cannot describe the intensity or type of pain.
- Indifference to pain means that the patient can perceive the stimulus, but lacks an appropriate response: they will not flinch or withdraw when exposed to pain.
Incidence
The disorder is primarily found in homogeneous societiesSpecies homogeneity
Homogeneous means consisting of parts or people which are similar to each other or are of the same type. Homogeneity means all of the same or similar kind or nature. In ecological terms it can also be expressed as a lack of, or reduction in biodiversity. Species richness is the fundamental unit...
.
For example, it is found in Vittangi
Vittangi
Vittangi is a locality situated in Kiruna Municipality, Norrbotten County, Sweden with 789 inhabitants in 2005.The locality is very notable because it houses a cluster of people suffering from congenital analgesia, an otherwise extremely rare illness....
, a village in Kiruna Municipality
Kiruna Municipality
Kiruna Municipality is a municipality in Norrbotten County in northernmost Sweden. Its seat is located in Kiruna...
in northern Sweden
Sweden
Sweden , officially the Kingdom of Sweden , is a Nordic country on the Scandinavian Peninsula in Northern Europe. Sweden borders with Norway and Finland and is connected to Denmark by a bridge-tunnel across the Öresund....
, where nearly 40 cases have been reported.
Also, Ashkenazi Jews have been found to have a higher risk, though it is still unusual.
Media depictions
In Stieg LarssonStieg Larsson
Karl Stig-Erland Larsson , who wrote professionally as Stieg Larsson, was a Swedish journalist and writer, born in Skelleftehamn outside Skellefteå. He is best known for writing the "Millennium series" of crime novels, which were published posthumously...
's Millennium series, former boxer Paolo Roberto discovers that Ronald Niederman was born with congenital analgesia and is therefore, as he describes "invulnerable" physically. ("He just keeps on going!") Even so, Roberto continues that his inability to perceive pain is a severe danger because Niederman has no way of understanding when his body is being put in mortal danger by way of punches, blows, burns, or even gunshots. It is suggested that human awareness of painful stimuli is an evolutionarily necessary component of vulnerability to injury and death.
The Grey's Anatomy
Grey's Anatomy
Grey's Anatomy is an American medical drama television series created by Shonda Rhimes. The series premiered on March 27, 2005 on ABC; since then, seven seasons have aired. The series follows the lives of interns, residents and their mentors in the fictional Seattle Grace Mercy West Hospital in...
episode Sometimes a Fantasy
Sometimes a Fantasy (Grey's Anatomy)
"Sometimes a Fantasy" is the third episode of the third season of the ABC series, Grey's Anatomy. The episode was written by Debora Cahn and was directed by Adam Arkin. It originally aired on October 5, 2006, running for 43:12 min.-Starring:...
features a young girl named Megan Clover that suffers from congenital analgesia believing herself to be a superhero.
The episode of House, M.D. entitled Insensitive also features a young girl who suffers from congenital analgesia, making her sickness extremely difficult to diagnose.
In the 2011 slasher film
Slasher film
A slasher film is a type of horror film typically involving a psychopathic killer stalking and killing a sequence of victims in a graphically violent manner, often with a cutting tool such as a knife or axe...
Wrong Turn 4: Bloody Beginnings
Wrong Turn 4: Bloody Beginnings
Wrong Turn 4: Bloody Beginnings is an American horror film and the fourth installment in the Wrong Turn horror/slasher franchise that began with Wrong Turn then continued with Wrong Turn 2: Dead End and Wrong Turn 3: Left for Dead. The fourth film serves as a prequel to the previous three...
, the characters Three Finger, One Eye and Saw Tooth are said to have the disorder by the head of the sanatorium
Sanatorium
A sanatorium is a medical facility for long-term illness, most typically associated with treatment of tuberculosis before antibiotics...
they are in in the portion of the film set in 1974, with it being theorized that the condition was the result of inbreeding
Inbreeding
Inbreeding is the reproduction from the mating of two genetically related parents. Inbreeding results in increased homozygosity, which can increase the chances of offspring being affected by recessive or deleterious traits. This generally leads to a decreased fitness of a population, which is...
. Due to their inability to properly register pain, the three were depicted as being prone to self harm in their youth, mutilating, severing and eating some of their own body parts.
In the play "Almost, Maine" by John Cariani, the vignette "This Hurts" is about a man with Congenital Analgesia, or "Hereditary Sensory Neuropathy Type 4", who is clocked in the head with an ironing board, and in the end feels the pain of love.
See also
- Hereditary sensory and autonomic neuropathyHereditary sensory and autonomic neuropathyHereditary sensory and autonomic neuropathy or hereditary sensory neuropathy is a condition used to describe any of the types of this disease which inhibit sensation.They are less common than Charcot-Marie-Tooth disease....
- Familial dysautonomiaFamilial dysautonomiaFamilial dysautonomia is a disorder of the autonomic nervous system which affects the development and survival of sensory, sympathetic and some parasympathetic neurons in the autonomic and sensory nervous system resulting in variable symptoms including: insensitivity to pain, inability to produce...
- Congenital insensitivity to pain with anhidrosisCongenital insensitivity to pain with anhidrosisCongenital insensitivity to pain with anhidrosis is an extremely rare inherited disorder of the nervous system which prevents the sensation of pain, heat, cold, or any real nerve-related sensations ; however, patients can still feel pressure. CIPA is the fourth type of hereditary sensory and...
- Familial dysautonomia
- HypoalgesiaHypoalgesiaHypoalgesia or hypalgesia denotes a decreased sensitivity to painful stimuli.Hypoalgesia occurs when nociceptive stimuli are interrupted or decreased somewhere along the path between the input , and the places where they are processed and recognized as pain in the conscious mind...
External links
- The Facts of Painless People A website about two adults on separate sides of the world who were born with CIP.
- A Life Without Pain The website of a documentary about three children who have CIP or similar diseases
- A child with congenital insensitivity to pain on The Oprah Winfrey ShowThe Oprah Winfrey ShowThe Oprah Winfrey Show is an American syndicated talk show hosted and produced by its namesake Oprah Winfrey. It ran nationally for 25 seasons beginning in 1986, before concluding in 2011. It is the highest-rated talk show in American television history....
. - Related CIP support organisation
- A child with congenital insensitivity to pain on The Oprah Winfrey Show