Brain & Nervous System More Neurological Diseases What Is CIPA (Congenital Insensitivity to Pain With Anhidrosis)? A Disorder That Causes a Lack of Pain and Sweating By Heidi Moawad, MD Heidi Moawad, MD Facebook LinkedIn Heidi Moawad is a neurologist and expert in the field of brain health and neurological disorders. Dr. Moawad regularly writes and edits health and career content for medical books and publications. Learn about our editorial process Updated on August 26, 2022 Medically reviewed by Smita Patel, MD Medically reviewed by Smita Patel, MD LinkedIn Twitter Smita Patel, MD is triple board-certified in neurology, sleep medicine, and integrative medicine. Learn about our Medical Expert Board Print Table of Contents View All Table of Contents Symptoms Causes Diagnosis Treatment Coping Frequently Asked Questions Congenital insensitivity to pain and anhydrosis (CIPA) is a rare disease that causes a person to not be able to feel pain or sweat. CIPA is also known as hereditary sensory and autonomic neuropathy type IV (HSAN IV). Here is what the name means: Hereditary because it runs in families Sensory neuropathy because it affects the nerves controlling sensations in the body (which includes pain) Autonomic because it affects the autonomic nervous system (which regulates sweating) This article will go over what causes CIPA. You will learn about the signs and symptoms of CIPA, how it is diagnosed and treated, and tips for living with CIPA. Cavan Images / Getty Images How the Autonomic Nervous System Works Symptoms of CIPA CIPA disease that a child is born with (congenital). A child who has CIPA cannot sense pain or temperature. They do not feel pain and cannot sweat (or do not sweat enough) Since the symptoms of CIPA show up in childhood, the disease is usually diagnosed early in life. Lack of Pain The main symptom of CIPA is not being able to feel pain. In fact, a child with CIPA has no concept of what pain is. Usually, CIPA is discovered when a parent or caretaker notices that their child is unaffected by things that should hurt. For example, you might think your baby is "tough" for not crying when they get a routine vaccination, but that could be a clue that they are not experiencing pain normally. You should tell your provider if your child has a pattern of not responding to typically painful events. There are also other signs of CIPA to watch for. Kids with CIPA often: Have a lot of injuries because they do not get the pain signals that would teach them to avoid things that are dangerous (e.g., the pain of a burn helps them learn not to touch a hot stove) Get infections or have injuries that don't heal because they do not instinctively care for them and try to protect them from things that would cause more pain If a provider notices that your child is not responding to pain normally, they might want to do more tests to see if they could have CIPA or another sensory problem. What Are Congenital Disorders? Anhydrosis (Lack of Sweating) In addition to not feeling pain, children with CIPA cannot sweat or have a decreased ability to sweat (anhidrosis). When to Get Help Heat stroke is a medical emergency. If the following signs and symptoms of heat stroke develop, call 911 or go to the nearest emergency room:Body temperature above 103 degrees FHot, red, dry skinRapid, strong pulseHeadacheDizzinessNauseaConfusionLoss of consciousness What to Do If You Get Heat Stroke Other CIPA Symptoms People with CIPA may have other symptoms, many of which are actually related to not responding to an injury or illness. Other CIPA symptoms include: Slow healing from skin and bone injuries Chronic bone infections Charcot joints (malformations caused by a lack of sensation) Febrile (fever-related) seizures Dental cavities Difficulty with bowel and bladder control Behavior problems Intellectual disability Rating Pain With Scales What Causes CIPA? CIPA is a disease you inherit and are born with. It's autosomal recessive, which means that a child has to inherit the gene from both parents to have the condition. Unless you or a close relative has CIPA, you may not realize that you have the gene until your child is diagnosed with CIPA. The abnormal gene responsible for CIPA, human TRKA (NTRK1), tells the body to make mature nerves. An abnormality in the gene means that your sensory nerves and some autonomic nerves do not fully develop. The underdeveloped sensory nerves cannot send messages to your brain about pain and temperature. For example, your brain tells your autonomic nervous system to make you sweat, but the message is not received. How Some Conditions Are Inherited How Is CIPA Diagnosed? There are not easy tests to identify CIPA, but providers do have a few tools to help them make the diagnosis. For example, they can take a small tissue sample and look at it under a microscope (biopsy) to look for underdeveloped nerves and no sweat glands. The most definitive diagnostic test for CIPA is a genetic test to look for an abnormal TRKA (NTRKI) gene. This can be done before birth or at any time during your life or your child's life. What Can Genetic Tests Tell You? Can CIPA Be Treated? There is no cure for CIPA. There is also nothing that can replace missing pain sensations or make you sweat more. Treatment for CIPA is really about living as safely as possible with the condition. For caregivers of children with CIPA, this means teaching them how to manage their activities and care for themselves since they won't be getting pain or temperature signals to help them out. Lack of Pain Pain is the body's alarm system. Without it, you may not know that you have injuries—even if they're serious. You may re-injure yourself regularly because there's no pain to signal to help you avoid doing so. For example, without pain as a cue, you might not you're doing too much on a broken leg that's trying to heal. If you have CIPA, it's important to learn how to prevent injuries and to monitor any injuries you do get carefully so they don't become infected or more damaged. When children have CIPA, it's up to their caregivers to keep an eye on injuries. It's also important that kids with CIPA learn strategies for staying safe. How to Help Loved Ones Track Pain Lack of Sweat Kids with CIPA also have to learn how to manage their activities when they can't sweat or won't be able to sweat enough to cool their bodies. Many of the same measures that prevent overheating can also help with overheating. For example: Avoid exertion in hot environmentsStay in the shade as much as possible when outdoorsRest often in the heatDrink cool liquid or eat popsiclesWear lightweight, loose, light-colored clothing made of breathable fibers (like cotton)Use air conditioning indoors when possibleUse cooling products (such as neck wraps, bandanas, and vests) or cold compressesTake cool baths (especially before and after exercise)Go to a swimming pool or use a kiddie pool at home If a child with CIPA has a high fever: Give them over-the-counter fever reducers such as Advil (ibuprofen), Tylenol (acetaminophen), or Aleve (naproxen) as approved by their pediatrician Use cold compresses instead of ice baths. While it might seem like ice or cold baths would help a fever, they can cause chills that will increase the body temperature rather than bring it down. How to Treat Fevers in Kids Coping with CIPA Good medical care and lifestyle changes are key ways to live safely with CIPA. These strategies should be implemented as soon as the diagnosis is made and adjusted as needed throughout a child's life. CIPA is a rare disorder, making it challenging for families to cope. Joining a support group—especially one for uncommon disorders that most people don't know about or understand—can be a relief and make you feel less alone. These resources can also help you find practical ways to make living with CIPA easier. Since CIPA is an inherited disorder, adults with the condition will want to discuss it with their partners and providers if they're thinking about planning for a family. How to Find a Support Group Summary CIPA is a rare genetic disorder that makes you unable to feel pain or sweat. It's caused by a defective gene that disrupts the normal development of sensory and autonomic nerves. The lack of pain means you might not know when you get hurt. This can lead to repeated injuries or wound infections. Lack of sweating can make you overheat, and you could get high fevers or other complications, like heat stroke. 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. Frequently Asked Questions How many cases of CIPA are there in the world? CIPA is very rare. It's estimated that there are only a few hundred people with CIPA in the world. How long do CIPA patients live? People with CIPA may not live as long as normal because they are at such a high risk of getting seriously hurt, getting life-threatening infections, and developing conditions like heat stroke that can be fatal.Some researchers have estimated that the life expectancy of people with CIPA is not more than 25 years old. A List of Movies About People With Rare Diseases 9 Sources Verywell Health uses only high-quality sources, including peer-reviewed studies, to support the facts within our articles. Read our editorial process to learn more about how we fact-check and keep our content accurate, reliable, and trustworthy. National Institutes of Health, Genetic and Rare Disease Information Center. Congenital insensitivity to pain with anhidrosis. Centers for Disease Control and Prevention. Warning signs and symptoms: Heat-related illnesses. Franco ML, Melero C, Sarasola E, et al. Mutations in TrkA causing congenital insensitivity to pain with anhidrosis (CIPA) induce misfolding, aggregation, and mutation-dependent neurodegeneration by dysfunction of the autophagic flux. J Biol Chem. 2016;291(41):21363–21374. doi:10.1074/jbc.M116.722587 Shaikh SS, Chen YC, Halsall SA, et al. A comprehensive functional analysis of NTRK1 missense mutations causing hereditary sensory and autonomic neuropathy type IV (HSAN IV). Hum Mutat. 2017;38(1):55-63. doi:10.1002/humu.23123 National Institutes of Health, U.S. National Library of Medicine: MedlinePlus. How to avoid overheating during exercise. National Multiple Sclerosis Society. Heat & temperature sensitivity. Nemours KidsHealth. Fevers. López-Cortés A, Zambrano AK, Guevara-Ramírez P, et al. Clinical, genomics and networking analyses of a high-altitude native American Ecuadorian patient with congenital insensitivity to pain with anhidrosis: a case report. BMC Medical Genomics. 2020;13(1). doi:10.1186/s12920-020-00764-3 Daneshjou K, Jafarieh H, Raaeskarami SR. Congenital Insensitivity to Pain and Anhydrosis (CIPA) Syndrome; A Report of 4 Cases. Iran J Pediatr. 2012;22(3):412-416. By Heidi Moawad, MD Heidi Moawad is a neurologist and expert in the field of brain health and neurological disorders. 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