Overview
Congenital Insensitivity to Pain with Anhidrosis (CIPA) is a rare medical condition in which an individual cannot feel pain or temperature, and does not have the ability to sweat. Also known as hereditary sensory and autonomic neuropathy type IV, this condition usually manifests during early infancy and can lead patients to suffer from repeated injuries.
How can CIPA affect patients?
On hearing about this condition for the first time, people tend to think that having such a condition might give the patient an advantage since he/she does not have the capacity to feel pain, and as a result, this may be beneficial. However, this is not the case, as patients suffering from CIPA are at risk of injuring themselves without noticing it.
Pain is a major factor in determining a person’s behavior and people usually tend to avoid situations that can lead to pain. When an individual experiences pain, it is a sign of a traumatic event or indicate the presence of an underlying disorder. To ease such discomfort or pain, individuals normally seek medical help, and when facing extreme temperatures, they can take the necessary steps to counter these conditions. An example of this situation is wearing a jacket during winter season.
Patients suffering from CIPA will overlook such situations and unintentionally get themselves injured. Examples of such injuries include severely biting the lips, tongue, fingers. In certain extreme cases, the patient may accidentally even end up amputating these parts of the body.
What causes CIPA?
CIPA is known as an autosomal disorder recessive disorder, and it is a condition that is passed on genetically if each of the parents’ autosomal chromosome is mutated.
The primary cause of this condition is due to the mutation of the NTRK1 gene. The NTRK1 gene helps in binding a receptor protein to another protein called NGFB, and it’s vital in the proper functioning of neurons. This particular gene is located in the surface of neurons that primarily transmit touch sensations like pain, temperature and tactile sensations.
Patients suffering from CIPA have mutations in the NTRK1 gene, which results in the neurons not being able to transmit any touch sensations, leaving patients without the capability to feel pain or temperature. This mutation in the gene also causes the loss of function for neurons located in the sweat glands, causing patients to not sweat.
What are the treatment/management options for CIPA?
Since this is a genetic condition, there has been no cure discovered yet, which means patients and their families will need to learn how to manage this condition. Children suffering from CIPA need to be watched closely, as they could end up burning or hurting themselves without feeling any pain. Children with CIPA may end up fracturing their arms or legs, and not even notice it due to the lack of pain.
Another risk that faces children with CIPA is joint deterioration. Children may tend to exert themselves unnecessariy, or strain certain joints for an extended period of time without noticing it.
Children suffering from CIPA also run the risk of ‘overheating’. Sweating helps regulate body temperature; hence the lack of sweating can lead to sudden fevers, febrile seizures, and even fatalities.
Each patient suffering from CIPA will need to follow their own individualized health plan. Common tips that help CIPA patients manage their condition include the following:
- Childproofing the patient’s home is important in avoiding injuries
- Wearing protective gear, like protective eyewear, helps prevent self-injury
- Teaching patients to seek medical help when they see blood or injuries
- Physical therapy is helpful, especially in dealing with joint deterioration
- Moving and living in a cooler climate can help a CIPA patient avoid overheating
Conclusion
CIPA is a rather rare medical condition that is currently undergoing research in diagnosis and treatment. While there is no cure to this condition, patients and their families can still manage the symptoms of this condition and modify their living environments to safeguard the patient’s wellbeing.
One important aspect that’s required for patient wellness is to create and spread awareness of this condition. While patients need to operate in an environment that is safe and free of hazards, spreading awareness of the condition and how to monitor any signs of injuries or temperature changes is important for effectively managing this condition.
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