One of the worst pains I have ever felt in my life was, undoubtedly, having a kidney stone. Between feeling like I was being stabbed in the stomach to nearly passing out from my heart racing, I would not wish the pain I felt on anyone. However, in a strange way, I am glad I was in so much pain, because it alerted me to the fact that something was wrong with my body.
Pain has long been thought of as an evolutionary mechanism which allows us to detect issues in our body to increase our chances of survival. Despite how unpleasant it is to be in pain, it is important for us to be cognitively aware of our ailments so we can seek medical attention or treatment if necessary.
So, what happens if someone cannot feel physical pain at all? Welcome to the world of congenital insensitivity to pain, or congenital analgesia. CIP is a type of peripheral neuropathy where those afflicted cannot feel pain, often leading to an accumulation of broken bones, wounds which do not heal and untreated disorders and diseases. There have only been about 20 cases reported in scientific literature, according to MedlinePlus.
It is important to note those who suffer from CIP can sense the differences in dull versus sharp touch and cold versus hot temperature, but they lack conscious awareness about the pain associated with these extremities, such as if they accidentally cut themselves with a knife (a typically painful experience).
Some of those afflicted can also suffer from CIP with anhidrosis, which is an inability to sweat. CIPA is marked by frequent high fevers and seizures as a result of incredibly high body temperatures because the body does not have a mechanism by which to cool off.
Stefan Betz is one of the only people in the world known to be afflicted with CIP. In 2017, then 21-year-old Betz was featured in a BBC News article, where he talked about his experiences with CIP and how his diagnosis came to be. Scientists believe Betz’s condition is so “rare” because most individuals afflicted with it do not live long enough to be diagnosed. They succumb far earlier in childhood due to unknown injuries or devastating illnesses.
“We fear pain, but in developmental terms from being a child to being a young adult, pain is incredibly important to the process of learning how to modulate your physical activity without doing damage to your bodies, and in determining how much risk you take,” said Dr. Ingo Kurth, who works at the Institute of Human Genetics in Aachen, Germany.
““We fear pain, but in developmental terms from being a child to being a young adult, pain is incredibly important to the process of learning how to modulate your physical activity without doing damage to your bodies, and in determining how much risk you take.”Dr. Ingo Kurth, Institute of Human Genetics
Betz told BBC that people often think of him as some sort of “superhero” and want to be able to not feel pain either. Without the ability to feel pain, however, Betz’s life has become increasingly more difficult with uncertainties at every turn.
“People assume that feeling no pain is this incredible thing and it almost makes you superhuman,” Betz said. “For people with CIP, it’s the exact opposite. We would love to know what pain means and what it feels like to be in pain. Without it, your life is full of challenges.”
Betz has had his fair share of injuries: At the age of five, he bit off the tip of his tongue without any apparent painful response. Days later Betz proceeded to jump down a flight of stairs, fracturing his right metatarsal in his foot. At the time of the interview, Betz was recovering from a tibial fracture he sustained while skateboarding and had recently developed osteomyelitis in the same leg.
“You learn that you have to pretend you have pain to prevent yourself from being reckless,” Betz said. “Which isn’t easy when you don’t know what it is. I now try to be vigilant otherwise one day my body will just give out.”
Others afflicted have not been so lucky, according to Dr. Kurth. He recalled a story of a Pakistani boy who worked as a street performer and routinely stepped on hot coals and stabbed knives into his arms without any painful reaction. The boy died in his early teens after jumping off the roof of a house. Other cases – usually those involving men – have ended with these sufferers killing themselves through performing dangerous acts or committing suicide due to depression and other comorbidities.
Steven Pete, another sufferer of CIP, wrote to BBC News in 2012 about his condition and how it has affected his life. Although his childhood was a mess of constant injuries, casts and crutches, Pete said now he is more hypervigilant than the average person.
“There was one time, at the roller-skating rink. I can’t recall all of the details, but I know that I broke my leg. People were pointing at me because my pants were just covered in blood from where the bone came out. After that, I wasn’t allowed to roller skate until I was much older,” Pete said. “When I was five or six years old, I was taken away from my home by child protective services. Someone had reported my parents for child abuse.”
While in the care of the state, doctors noticed Pete had managed to break his own leg, and gradually they began to realize Pete’s parents were not lying about his condition. Since then, he has injured his left leg frequently enough to the point where doctors have warned he will eventually need an amputation. His brother Chris, also afflicted with CIP, died by suicide after being told he would soon need to be in a wheelchair due to frequent back injuries.
““There was one time, at the roller-skating rink. I can’t recall all of the details, but I know that I broke my leg. People were pointing at me because my pants were just covered in blood from where the bone came out. After that, I wasn’t allowed to roller skate until I was much older.”Steven Pete, sufferer of Congenital Insensitivity to Pain (CIP)
“He [Chris] was an ‘outdoors man.’ He liked to be outside, to fish and to hunt. But he tried to see about getting some sort of financial disability assistance once this would all happen,” Pete recalled. “And pretty much what the judge told him was: ‘If you’re not in pain then you have no reason to be on any type of assistance.’”
Pete said the gross misunderstanding of the disease and the psychological importance of pain is part of the problem. Just because he is otherwise healthy does not mean his lack of an essential bodily sensation is not harrowing and dangerous.
“The thing is, with our condition, a lot of people see us and they might assume that we’re healthy. But they have no idea that my body could give out at any time, that I ache all over. I have severe arthritis in my joints. It’s not painful – I don’t feel pain – but it’s hard to move around sometimes. It feels like a compression, a throbbing compressed feeling in my joints,” Pete said. “It limits your mobility and your joint isn’t able to move as much as it should. They [doctors] just don’t understand the human component of it – the psychology of what can happen when you grow up not being able to experience pain.”