Fighting Through the Pain

By Lyn A.E. McCafferty

For 13-year-old Alexa, the pain began in her ankle after a dance class. But unlike a typical ankle sprain, her pain quickly worsened — turning her foot white from lack of blood flow and traveling up her leg, into her thigh and hip. Three months later, Alexa couldn’t move her leg.

Despite extensive testing, doctors near her Long Island home could not identify the cause of Alexa’s pain. They referred her to Children’s Hospital of Philadelphia, where a multidisciplinary team diagnosed her with amplified musculoskeletal pain syndrome (AMPS).

What is AMPS?

AMPS is chronic, non-inflammatory musculoskeletal pain that can affect bones, joints and connective tissue throughout the body.

“AMPS is an overfiring of the nerve fibers in the body to the brain,” says Sabrina Gmuca, MD, MSCE, a pediatric rheumatologist and Director of the Center for Amplified Musculoskeletal Pain Syndrome at CHOP. “Though these nerves are hardwired to protect the body from injury, in AMPS, the nerve fibers have gone haywire — like an abnormal short circuit — causing a pain response and creating a constant pain cycle.”

This short circuit sparks a “fight-or-flight” response in the body, constricting blood flow and oxygen and causing pain. Usually, AMPS is caused by an injury, like Alexa’s ankle sprain, but it  can also be caused by psychological stress or illness, with age, genetics and hormones all playing a role.

Resetting the mind-body connection

For more than three decades, CHOP has pioneered AMPS research and treatment in youth. The Center has repeatedly concluded that pain medications are not ideal treatments for AMPS and therefore doesn’t prescribe them. Instead, CHOP emotionally connects with patients and creates customized treatment plans with input from a multidisciplinary team of rheumatologists, psychologists, occupational therapists and physical therapists.

“One of the most important things Dr. Gmuca tells patients in their initial meeting is that ‘this is not in your head — you’re not crazy,’” says Tami Konieczny, MS, ORT/L, BCP, Occupational Therapy Supervisor. “Saying ‘We believe you,’ goes a long way in building trust with our patients and their families.”

Though no two AMPS patients are the same, there are many similarities among patients: Most are females in their early teens and high achievers in academics, sports or both. Before coming to CHOP, many AMPS patients have been discounted as attention-seeking or “making up” their pain. The reality is quite different.

“Patients are so grateful to finally be seen; to know there’s a name for what’s causing their pain,” Dr. Gmuca says. “By validating the child’s experience and giving them the tools to treat their condition, we help them take the first steps to regain control of their lives.”

Shifting the focus to improve function

“We’ve all been taught that if something hurts on your body, you should rest it,” says pediatric psychologist Jennifer L. Sherker, PsyD. “With AMPS, we sort of turn that advice on its head. Instead of rest, we encourage patients to look past the pain and focus on function.”

The goal of therapy is to encourage patients to resume typical activities, such as dressing themselves, doing homework and walking up stairs, as well as things they used to enjoy, like going out with friends, playing sports or spending time with family. Patients are urged to participate in physical activities like exercise without excess rest periods between repetitions or modifying their body positioning because of the pain.

“We have to get them to do things that are hard, to push themselves through the pain, to do things that hurt and more of it,” Konieczny says. “These activities increase the blood flow in the affected area and flush the buildup of lactic acid out of the body, improving a patient’s overall function.”

Distraction and desensitization play a key role. Physical tools like ice, texture and vibration can help desensitize a patient to over-firing nerves, and positive distractions, such as talking with friends or listening to music, can help divert a patient’s attention.

Psychologists introduce patients to mental health tools to help control their pain, teach coping skills to manage stress, and provide additional support for patients who experience anxiety, depression and other mood disorders, all of which are common in AMPS patients.

“We also help patients reframe their automatic thoughts,” Sherker says. “Instead of thinking, ‘I can’t go to school today because I have pain,’ we want to change their internal narrative to ‘I can go to school this morning and use the strategies I’ve learned to get through each chunk of my day.’”

For patients who need more intensive treatment for AMPS, CHOP offers a therapeutic hospital treatment program in which patients undergo five to six hours of therapy each day with the multidisciplinary team.

Patient advocacy tools and family counseling

“For a lot of our families, the affected child’s pain has been the focus for some time,” Dr. Gmuca says. It may have limited family activities, focused too much attention on one child, and created resentment among family members. For this reason, all AMPS families are encouraged to participate in family counseling.

“We actually give families permission NOT to ask their child about their pain,” Dr. Gmuca says. “By focusing on the pain, parents can actually make kids think about it more often.”

Instead, the AMPS team gives patients the tools to advocate for themselves — at home, at school and in the community.

“Our patients are like Dorothy in The Wizard of Oz,” Konieczny says. “They always had the power [to stop the pain], they just didn’t have the tools. We give them the tools to stop their musculoskeletal pain.”

‘Gave me my life back’

Though treatment for AMPS has a high success rate, some patients experience a new form of the disorder after a new injury or period of increased stress. Such was the case with Alexa. After twice-weekly therapy, her pain subsided, but 18 months later, a lacrosse injury to her hip catapulted her back into the pain spiral again.

This time, the pain was more intense and centered around her abdomen and head. At the height of the COVID-19 pandemic, Alexa came to CHOP for a month of intensive hospital-based treatment.

“This experience has taught me so much,” Alexa says. “Not only did CHOP give me the tools to decrease and manage my pain, they gave me my life back — twice! AMPS no longer decides what I can and can’t do.”