When she arrived at Children’s Hospital of Philadelphia (CHOP), Aamira, then 9, was in a wheelchair, and her mother, Melindah, was worried sick. Several months earlier, Aamira slid into base while playing softball and hurt her left ankle. By the time she met with attending physician Addie Eichman, MD last July, Aamira’s pain was severe — and getting worse.

“She would wake up screaming in pain, and she just kept progressing in the wrong direction,” Melindah remembers. “She went from a brace to a boot, boot to crutches, and finally she was completely immobilized in a wheelchair. It was heartbreaking.”

Melindah worried about her daughter’s functional decline. She also worried that Aamira’s pain impacted her school attendance, ability to do the things she loved, including softball and competitive Bollywood dance. (She flourished in both.)

After seeing half a dozen different physicians in four weeks, Aamira was diagnosed with localized amplified musculoskeletal pain syndrome, or AMPS, a rare medical condition that can cause intense intermittent or constant pain for children in a single area or throughout their bodies. The condition is most common among pre-teen and teenage girls; however, because of the wide spectrum of AMPS, many doctors struggle to recognize and diagnose it.

Fortunately, Melindah managed to secure an intake appointment for her daughter at CHOP’s multidisciplinary clinic, the Center for Amplified Musculoskeletal Pain Syndrome. Dr. Eichman recommended Aamira for the center’s unique, hospital-based intensive pain rehabilitation program. Hope was on the way.

Overcoming barriers to diagnosis and treatment

CHOP specialists are the first to acknowledge the challenges to diagnosing AMPS.

“It can be triggered by a physical injury or illness, a physical change that happens to the body where the nervous system responds by increasing pain signals,” says Sabrina Gmuca, MD, MSCE, attending rheumatologist and medical director for the Center for AMPS. “It can also be triggered by emotional or psychological stressors brought on by adverse childhood experiences such as parental separation or living with a household member with a chronic mental health condition. We also see more instances of amplified pain during puberty because it’s a critical time in terms of neurodevelopment and hormonal changes.”

While most patients fall between the ages of 12 and 17 years, there are many patients, such as 9-year-old Aamira, who present symptoms earlier.

“Not one AMPS patient is the same,” Melindah says. “It’s such a wild syndrome. You can’t really understand where the pain’s coming from, what causes it, and there’s no one treatment and no medication to help these children.”

That ambiguity is reflected in the terminology used to identify the condition. While AMPS is the acronym used in the pediatric rheumatology community, other pediatricians refer to it as chronic non-inflammatory musculoskeletal pain, chronic widespread pain or fibromyalgia syndrome.

“There’s a real sense of isolation for people in this medical journey,” Dr. Eichman says. “Doctors don’t know how to label it, and it’s frightening for families.”

Delivering comprehensive care

The Center for AMPS at CHOP consists of both an outpatient multidisciplinary clinic and a hospital-based intensive pain rehabilitation program. For most patients, the clinic is sufficient.

“The first step is the clinic, and we see over 500 patients every year,” senior clinical program operations manager Adriana LaMonte, MPIA, says. “About 20% of patients are referred to the intensive program based on the clinical team’s assessment.”

Aamira entered the intensive program last August.

The program is intentionally designed to mirror a school day. Each weekday morning, parents drop their kids off at CHOP’s Seashore House. Patients participate in five to six hours a day of physical and occupational therapies, individual and group psychological counseling, and creative therapies, including art, music, and dance and movement therapies. You can probably guess which Aamira liked best.

CHOP’s program is unique because its small size — just six patients at a time — and large dedicated team deliver comprehensive treatment that combines psychological counseling, exercise-based therapies and desensitization techniques in one intensive rehabilitation program. Importantly, the team also takes a firm stance against using medications to reduce pain.

“We take a non-pharmacologic approach,” physician assistant Lauren Pianucci, PA-C, MPH, says. “We maintain medications for comorbid medical conditions such as mood disorders, but we do not use medications to treat the pain. Unfortunately, there is no quick fix for AMPS, and pain medications do not help with amplified pain. The hard work of the patient is the key to improvement in treatment.”

Working through pain together

As the name suggests, the program pushes patients to their limits. Aamira said that at least one of her peers almost left the program because she could not bear the pain.

“I had times when I was annoyed. I had no idea what I was doing,” she remembers. “But I saw the others, and they were able to get around way faster than I ever expected. It was good to meet other people experiencing the same things but in different ways.”

There was no alternative but to work through the pain and beat back what her mother called “the AMPS monster.”

The care team had Aamira out of her wheelchair on her second day and walking without crutches by the end of her first week.

“I cried when I saw her walking,” Melindah says.

As her daughter progressed, Melindah relied upon friends, neighbors and colleagues — “Aamira’s squad” — to support her and her child through the program.

“I would come home, and local moms would have done my laundry, cleaned my dishes and prepared meals for us,” she says. “Aamira didn’t like the hospital food, so our community would make her homemade Indian food and bring it to us.”

Aamira found support inside and outside of the program. She received motivational videos from her softball coach, video-conferenced with her best friend, Ziva, and played Exploding Kittens, StarStarter and Pigeon on her iPad with friends back home. She also bonded with the other kids in the program — they still maintain a group chat today — and played softball on the Buerger Center roof deck with her occupational therapist, Tami Konieczny, MS, OTR/L, BCP.

“Aamira flourished in our program,” Dr. Eichman says. “She may have sustained her injury playing softball, but by mid-program she was playing softball and earning praise from some of our best players. You just saw her mood brighten and her personality come out.”

Homecoming

Aamira graduated in late August after three weeks of the program.

“I was pretty excited when I found out that I was going home,” she says. “I was pretty proud of myself.”

Since she got home, Aamira has returned to her softball team and competitive dance. She continued occupational and physical therapy until October, when she celebrated her 10th birthday at an escape room with her friends. Today, Aamira appears to be mostly recovered.

“If pain acts up, I try to clear my mind and focus on that one thing,” she says.

Paying it forward

Aamira’s experience is fortunately fairly typical at the Center for AMPS.

“If a patient succeeds in our intensive program, they can expect to maintain a level of minimal to no pain,” Dr. Gmuca says. “In one of our published studies, one-third of patients reported no pain about a year after completion of the intensive program. If the pain does return, patients can lean on the skills that they learned from the program.”

“It’s extraordinary,” Melindah says. “Aamira got care, and six months later she was walking again. We overcame with the support of our caseworker and the AMPS team at CHOP who helped us navigate every aspect of care.”

Despite raising Aamira and her brothers as a single parent, Melindah is quick to acknowledge her privileges.

“I’m a working professional in the field, blessed with a wonderful career,” she adds. “But what about all the families that don’t have the resources or support? So many families face challenges around the practicalities of food, shelter and jobs.”

Melindah notes that two mothers lost their jobs while caring for their children, and another experienced a nervous breakdown during the program and did not want her daughter to continue. Fortunately, with peer support her daughter was able to complete the program.

If families are fortunate to enroll their child in the intensive program — the wait list can be as long as three or four months — they will need to take time off work and find accommodations for the duration of the program. Many parents stay at the Ronald McDonald House, but availability is limited, leaving some with no alternative but to reserve hotel rooms. In most cases, families live out of suitcases, far from support systems.

Aamira’s care team dreams of doing more to support those families.

“I wish we had funds that could help families defray travel costs or even reimburse them for hotel stays if they cannot get into the Ronald McDonald House,” Ms. Pianucci says.

“I would also love to think about bringing on a part-time nutritionist and psychiatrist, because many patients also have disordered eating,” Dr. Gmuca says. “You cannot participate in exercise if your body’s not well-nourished and if you’re not at a safe weight. Those patients would benefit greatly from support for comorbid medical conditions and individualized plans when it comes to regaining weight that they may have lost.”

The team is committed to improving AMPS diagnosis and treatment outside of CHOP.

“We take a lot of pride in being a world-renowned institution, but at the end of the day, we want every child with chronic pain to have access to care close to home wherever they live,” Dr. Gmuca says. “We’re working to formalize a process through which providers can come to CHOP and shadow our team. We would love to help offset their travel expenses so that they can learn here and return to their home institution to either strengthen existing clinics or start new programs.”

To learn more how to support the AMPS program, contact the CHOP Foundation or contribute directly at the Division of Rheumatology.