Angie Friesen didn’t think much of it when her sixth-grade daughter, Annelise, told her she’d hit her elbow on a desk at school.
Nothing was broken.
The pain continued. Within a few months, it had spread to Annelise’s shoulder blade. Another X-ray and a nerve study revealed no damage.
Finally, a pediatrician gave a diagnosis.
“I hate to even say the word,” Friesen remembers the doctor saying. He suspected her daughter had a condition called CRPS, or complex regional pain syndrome. In adults, the condition can be devastating, with burning pain that spreads, discoloration of a limb and the loss of function.
Friesen’s pediatrician apparently didn’t know it at the time, but children with CRPS often respond well to treatment, especially in rehabilitation programs dedicated to treating chronic pain syndromes, sometimes called amplified musculoskeletal pain.
But early stages of the condition can perplex families and physicians. There is no simple test: A diagnosis, when it is made, is based on symptoms. When conventional treatments provide no relief, these symptoms are exacerbated and patients can slip into a cycle of pain and loss of function, driven by overfiring nerves and constricting blood vessels.
When children stop doing what they usually do — going to school or walking without assistance, for example — nerves become even more sensitive, creating a feedback loop comparable to what happens when a microphone is set against a speaker. Just as a small sound grows louder, the child’s pain is amplified.
Cara Hoffart, director of the Rehabilitation for Amplified Pain Syndrome Program (RAPS) at Children's Mercy Hospital in Kansas City, Mo., said the typical patient is a teenage girl with a "10 out of 10 pain." Many are high achievers who maintain good grades even when they miss school. Those not in wheelchairs, or whose pain comes and goes, are often misunderstood and dismissed.
“Every patient I have ever met has been made to feel that the pain is all in their head,” she said. “Someone doesn’t believe them.”
But the pain is real.
Annelise’s pediatrician in Andover, Kan., said there was not much he could do and sent the family to a neurologist who put the middle schooler on a drug used for epilepsy. It made Annelise extremely sleepy, and the pain spread to the tips of her toes. By the second week of seventh grade, her entire body hurt, a sensation she described as being cold and on fire at the same time. Her skin turned blue. The wind at her brothers’ football games became excruciating; even the air produced when people spoke was a problem.
“She asked us to face a different direction because our breath hurt and caused her pain,” her mother said.
Soon, she needed a wheelchair. She stopped talking and sank into a depression.
“I just want to be with Jesus,” she told her mother. “There is no reason for me to live.”
Children with amplified pain are often bounced around from pediatrician to rheumatologist to neurologist to orthopedic surgeon and to the emergency room, according to Hoffart. Most undergo unsuccessful treatments, including exploratory surgery, steroids, opioids, nerve blocks, epidural morphine and electric nerve stimulation.
“People can’t agree on what to call it, let alone how to treat it,” Hoffart said.
At the Mayo Pediatric Pain Rehabilitation Program, medical director Tracy Harrison uses the terms chronic pain syndrome and central sensitization, but she says that amplified pain is a fair description of an oversensitivity within the nervous system that allows chronic nonmalignant pain to persist.
The incidence of amplified pain in children is not well studied. It includes localized pain and diffuse pain, including juvenile fibromyalgia, which is estimated to affect 2 to 6 percent of children and is categorized by general pain, fatigue, poor sleep, headaches and irritable bowel symptoms.
Friesen heard about the Kansas City program by chance, while talking to friends at a ballgame. Then she faced a four-month battle with her insurance company to get Annelise a spot because it was not willing to cover this type of treatment. “They told us that this was not medically necessary to go to treatment and denied all our claims,” she said.
“My daughter is going to die,” she said, finally breaking down on the phone with an insurance representative. Annelise, she said, was in so much pain she wanted to kill herself.
In what Friesen calls a miracle, they got approval. Mother and daughter drove three hours from their home and moved into a basement apartment near Children’s Mercy for seven weeks of treatment.
The RAPS clinic at Children's Mercy is one of eight in the country that are modeled after one at the Children's Hospital of Philadelphia. The CHOP program is led by David Sherry, who has been studying and treating amplified pain and fibromyalgia for 20 years, developing a system of therapy focused on aerobic exercise, physical and occupational therapy, and counseling. In style and substance, he is a bit unusual.
Dressed in blue jeans and Chuck Taylors and with a white beard straight out of “The Lord of the Rings,” Sherry speaks to his patients in riddles and writes on their skin with marker. As a pediatric rheumatologist, he treats many patients in pain, but those with amplified pain suffer the most, he believes.
Early in his career, he said, he saw a mentor treat amplified pain with physical therapy instead of drugs such as corticosteroids or nerve blocks.
“That’s cool,” he remembers thinking. “I like illnesses I can fix.” Ultimately, he said, he gives children the tools to fix themselves.
Treating chronic amplified pain is far different from treating acute pain, which is a reflexive response designed to protect the body from further danger, Harrison says. To break the cycle of amplified pain, patients must get back to doing the things they did before they lost function even if — and especially if — it hurts, Hoffart says.
Sherry’s program is intense, quickly removing medication, crutches and wheelchairs and starting patients on five to six hours of physical and occupational therapy a day.
Children who have not been able to walk may be told to ride a stationary bike and do water aerobics. The body is desensitized with towel rubs, vibration, touch, compression and fanning, Sherry says. The point is to retrain the nerves and blood vessels. Function will come back long before the pain goes away.
Jeanine Miles described the treatment as “seven weeks of hell.” Her daughter, Taylor, entered the program with amplified pain in a leg, a condition that left her relying on an electric scooter for mobility. Within two hours in the clinic, she was walking — but also “calling me every name in the book,” Miles said of her otherwise “sweet little fifth-grader.”
“If it hurts to do something, that’s what you should do,” said Hoffart, who did a fellowship with Sherry at CHOP. It’s a theme that extends to psychological counseling, where Sherry tells his patients to “talk about what you don’t want to talk about” during their sessions.
Sherry began considering connections between the psychological aspects of stress and physical pain 20 years ago at the Children’s Hospital of Seattle. Now, art and music — forms of emotional expression that can reinforce coping tools — are essential parts of the CHOP program, along with stress management and counseling.
Living with chronic pain is an emotional experience, Hoffart said, because you “lose what you are and what you love.” This emotional stress fosters more physical pain, affecting hormones, revving up the nervous system and reducing blood flow through the body.
Dustin Wallace, who runs behavioral health programs at the RAPS clinic in Kansas City, said amplified pain often begins when a child is dealing with stress, such as a mild learning disability, a family move or pressure with sports. Then, they experience an illness or injury, such as a broken leg or bad case of mono. Ultimately, with both stress and a focus on health, the child’s physical activity level changes, and the cycle of amplified pain begins.
But the “weirdest” part of treatment, he said, relates not to managing stress but to the sensory cortex.
“We have got to make pain less dangerous to the brain,” he said.
The brain learns from behavior. To make pain less dangerous, a patient needs to go back to activities and reduce its significance. That means going back to school and sports and time with friends and not making decisions based on pain. For parents, this often goes against every instinct to protect their children.
Wallace said there are different approaches to treating amplified pain and some controversy about the use of medication and interventions before reaching rehabilitation programs. There can be relapse, and it does not always produce 100 percent pain-free patients. Harrison said success depends a great deal on effort, sticking to coping strategies and making sure function never takes a “back seat” to pain.
It is not as simple as “come for three weeks and now your life is all better.”
Based on anecdotal evidence of waiting lines at clinics and the observations of pediatric rheumatologists, Hoffart speculates that pediatric amplified pain is becoming more common, partly as a result of societal changes. Many children are overscheduled and placed under a great amount of stress. Most of her patients are Type A overachievers, she says, as were the people trying to help them. “All of us were one stone’s throw away from being a pain kid,” she tells her colleagues.
For several weeks, Friesen picked Annelise up from long days of treatment to find her face puffy from crying. They spent a lot of time at pet stores petting puppies, she said. After seven weeks, Annelise went home “80 percent mobile.” They continued physical therapy at home and followed Wallace’s instruction to not let pain factor into decisions.
One night that November, Annelise asked her mom for a blanket at bedtime.
“I covered her up and started crying,” Friesen said. It was the first time since her pain began that her daughter had used a blanket.