We will never really comprehend what individuals who are pain-free go through, despite the fact that practically everyone thinks that being pain-free is a blessing.Yes, you read that correctly.There are people who have never felt pain and who will never be able to feel pain. "Response to stimuli" is a crucial component that keeps us alive and is only partially present in CIPA sufferers. Response to stimuli refers to experiences with pressure, temperature, vibration, and pain. CIPA (congenital insensitivity to pain with anhidrosis) patients, on the other hand, are unable to feel pain, heat, or sweat, which is one of the most vital processes for getting rid of waste and regulating body temperature. The illness known as CIP impairs one's capacity to feel physical pain. Affected people never experience pain when harmed in any region of their bodies from birth. People who have this condition are unable to distinguish between hot and cold, sharp and dull, but they can detect the burning sensation on their tongue when drinking something hot, for example.. The buildup of wounds, bruises, broken bones, and other health problems that could go unnoticed is frequently caused by this lack of pain sensitivity. Young children with congenital insensitivity to pain may repeatedly self-bite, leading to mouth or finger wounds as well as multiple burn-related injuries. People with congenital insensitivity to pain frequently have shorter life expectancies as a result of these repeated injuries. Congenital insensitivity to pain is frequently accompanied by an absence of all sense of smell (anosmia). Additionally, those who have CIPA heal from bone and skin injuries slowly.. Repeated trauma can result in Charcot joints, a disorder where the bones and tissue surrounding joints are damaged, or chronic bone infections (osteomyelitis). Since it is a genetic disease, there is no outside cause. Congenital pain sensitivity is caused by mutations in the SCN9A gene. One component of a sodium channel called NaV1.7, the alpha subunit, is made according to instructions from the SCN9A gene. Sodium channels are essential for a cell's ability to produce and send electrical signals because they allow positively charged sodium atoms (sodium ions) to enter cells. NaV1.7 sodium channels are present in nociceptors, which are nerve cells that send pain signals to the brain and spinal cord.The olfactory sensory neurons, which are nerve cells in the nasal cavity that provide signals linked to smell to the brain, also include the NaV1.7 channel.
Congenital insensitivity to pain is caused by mutations in the SCN9A gene, which produce nonfunctional alpha subunits that cannot be integrated into NaV1.7 channels. The channels cannot form as a result. NaV1.7 channels are necessary for the proper transmission of pain signals from the lesion site to the brain, which prevents persons who lack them from feeling pain. Anosmia is most likely caused by the loss of this channel in olfactory sensory neurons, which affects how smell-related impulses are transmitted to the brain. Both copies of the gene in each cell have mutations because this disorder is inherited in an autosomal recessive way. One copy of the defective gene is carried by each parent of a person with an autosomal recessive disorder, although usually, neither parent exhibits the disease's signs and symptoms. A 12-year-old boy was admitted with osteomyelitis that had persisted. He was the fifth child of a consanguineous Iranian marriage. His birth certificate showed a low Apgar rating. The family was free of any inherited or familial diseases. The pregnancy was regular. As a result of osteomyelitis, heel ulcers, fever, and convulsions, he was admitted to the hospital numerous times. He was unable to sense heat or discomfort, and he had no reaction to suffering. Results from the brain CT scan and lumbar puncture were both normal. Results from the TORCH and metabolic investigations were negative. He had an intellectual disability. Both the electromyography (EMG) and the nerve conduction velocity (NCV) were normal. All of the tests, including serum uric acid levels, blood gas analysis, nitroblautetrazolium (NBT) testing, immunological globulins, viral indicators including HBV, HCV, and HIV testing, and complements, were normal. A 12-year-old child with CIPA syndrome exhibits osteolysis of the right fingertip and painless heel sores on the left.

This patient was brought in to get a 10 mm by 10 mm hill sore ulcer treated. We did our best to control the infection by administering the appropriate antibiotics and by debridement of necrotic areas in order to avoid limb amputation and maintain his quality of life as much as possible. . Additionally, there was severe osteolysis in his mandible, where attempts were made to perform the necessary dental procedures. It was clear that he had self-mutilated, notably, the tips of his fingers were observed. Radiographic also revealed osteolysis in his digit rum. The other sites such as the lung, heart, abdomen, and eyes were normal in physical examination. Now that we think about it, it is a curse that can never be undone. A disease with no cure. But studies have proved that we can develop medications that can suppress severe pains with the help of CIPA  patients .so be thankful and fulfilled with your beautiful body .have a great day guys.