Ankita Chattopadhyay, Amity University Kolkata
CIPA refers to a rare hereditary disease-Congenital insensitivity to pain and anhydrosis. This name is very descriptive as it reveals several important characters of the disease itself and is also called hereditary sensory and autonomic neuropathy type IV (HSAN-IV) and is a nerve disease that targets the nerves controlling sensation. It affects the autonomic nervous system which controls the surviving function of the body. In simple words, a person who feels no pain and never sweats is the victim of CIPA.
Symptoms-
It is characterized by no sense of pain, no sweating, mental retardation, periodic fever, and self-mutilations. The symptoms are obvious in childhood and diagnosed early. The height of the patients is below normal of their age. Children with CIPA experience burning without crying, complaining, or even noticing. While parents will for sure consider their child to be normal like rest but later with time realize that it’s different. After consulting a doctor and going through several tests for nerves, they realize its CIPA. The victims are very prone to injuries or burns as they never avoid painful activity. They might develop infected wounds as they do not instinctively protect their wounds.
Anhydrosis means a lack of sweating. Generally, sweat on the surface of the skin helps cool off the body when it’s too hot but CIPA uses it as its consequence. The consequence of anhydrosis is excessive fever as they “cool off” protection that sweating provides.
CIPA has less than 60 cases recorded in medical history.
Cause-
It is hereditary and is autosomal recessive. Typically both of the parents of the affected child carry the gene but the child does not have it if only one of the parents inherits it. The gene responsible is the human TRKA (gene developing mature nerve). It promotes the growth of a specific nerve by encoding a receptor called tyrosine kinase. Due to this incapable gene, sensory and some autonomic nerves do not develop, leading to making it incapable to sense messages of pain and temperature.
Diagnosis-
No simple X-ray or blood test can identify. The most absolute test is a genetic test that can be done before childhood, during childhood. The known genetic abnormality is the TRKA gene located on chromosome 1.
Treatment-
Considering the recent time, no treatment cures CIPA or can replace the missing sensation. Victims should learn to prevent injuries and it should be monitored carefully to avoid infection. Parents should have a constant eye on the children as they shall try new things without knowing the potential significance of the physical injury.
Sources-
- Melamed I, Levy J, Parvari R, Gelfand EW. 2000 Mutation in the syndrome of (CIPA)
doi.org/10.4103/idoj.IDOJ_86_17
- Daneshjou K, Jafarieh H, Raaeskarami SR..2012 Congenital Insensitivity to Pain and Anhydrosis (CIPA).
doi:10.4103/idoj.IDOJ_86_17
- An I, Ucmak D. 2018 Congenital Insensitivity to Pain and Anhydrosis Syndrome.
doi:10.4103/idoj.IDOJ_86_17
- Heidi Moawed. 2020 CIPA Disease- When a person can’t feel pain
https://www.verywellhealth.com/cipa-disease-when-a-person-can-t-feel-pain-4122549
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It will really help many people to understand symptoms of CIPA.
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