When 11-year old Grace Cahners broke her foot in July 2015, she received the usual support boot, then casting and several weeks of physical therapy (PT). But instead of getting better, her pain intensified over the course of five months, forcing her to miss the first 54 days of sixth grade. She lost her normally sunny disposition and became crippled by fear.
Grace was diagnosed with complex regional pain syndrome (CRPS), a chronic pain condition in which the brain sends an over-abundance of pain signals to the affected limb. Not a newcomer to pain – Grace was diagnosed with psoriatic arthritis at the age of 13 months – she was using a wheelchair by December.
Leigh Cahners, Grace’s mother, knew that full-day narcotic pain medications and traditional PT would not restore Grace’s ability to walk. “I knew we needed another approach,” she says.
The fear factor
Laura Simons, PhD, had seen this pattern before. For almost ten years, she has been a psychologist in the Pain Treatment Service and the Pediatric Pain Rehabilitation Center at Boston Children’s Hospital. “I was struck that children with CRPS are often very fearful about returning to certain activities after an injury because of the pain it could potentially trigger,” she says.
It turns out that fear actually intensifies pain. Teaming with colleagues David Borsook, MD, PhD, and Lino Becerra, PhD in the Pain Analgesia Imaging Neuroscience (P.A.I.N.) Research Group, Simons learned that the brain responses of adolescents with CPRS are abnormal. Functional MRI images of brain areas associated with emotion, such as the amygdala, showed alterations compared to same-aged healthy peers.
In a small pilot study, brain connectivity patterns could be restored to a more normal-like status after intensive psychological and physical pain rehabilitation. Informed by that study, Simons developed a treatment program for children with CPRS — the Graded Exposure Treatment (GET Living) program — that aims to not just reduce pain but conquer pain-related fear.
Run out of Boston Children’s at Waltham, GET Living teams a PT and a psychologist to work with the children — together — for twice weekly hour-long outpatient sessions for approximately six weeks.
“Normally, the odds that a physical therapist and a psychotherapist would talk to each other during the course of a child’s treatment are pretty low,” says Simons.
At the beginning of the program, patients identify activities they fear returning to in their daily life. In collaboration with the psychologist and PT, they build an activity “ladder” that they tackle during subsequent sessions.
“The therapy progressively exposes patients to activities aimed at getting them to overcome the fear – to live life whether or not the pain shows up,” Simons explains.
With the support of the team’s PT, patients are encouraged to perform progressively more worrisome activities – first in the safety of the treatment setting, then, when they are ready, in real life. Range-of-motion and body mechanics testing measure the progress made.
“I’ve got this”
Grace enrolled in the GET Living Study early in 2016. With PT Eileen Li and psychologist Ellen McGinnis, she began to break down worrisome activities into manageable pieces and to develop coping techniques to help her engage in the activities. These included deep breathing, positive self-talk and facilitators like drawing or listening to music.
“Grace learned that even if she did experience pain, she was able to reduce the nerve response through cognitively talking herself through it,” Leigh explains.
With Grace’s fear named and the coping strategies in place, the GET Living team staged small pain triggering scenarios, all with the goal of readying her to face the trigger in real life.
“In March, we were planning a vacation that would involve getting on a plane,” Leigh recalls. “Grace was so fearful of being in crowded places and getting bumped because it would cause extreme pain.” For several weeks, the group staged the anticipated exposure repeatedly, such as having her walk through a crowded hallway, for example, examining possible difficulties and strategies until the fear of the pain became less and less intense. Ultimately, Grace successfully completed the two plane trips utilizing the tools she and her GET Living team developed together.
Grace moved on to face other triggers, like getting bumped in a crowded hallway at school. By “play acting” anticipated exposures, she was also able to resume swimming, overcoming her fear of cold water that had caused extreme pain.
“By talking it out, then writing it out, then actually performing the task in a closed setting — all of those things are rungs on a ladder that she would simulate and then do in reality,” Leigh says. “The team helped build the structure within her to recognize that she can exposure herself to triggers and know how to respond if pain develops.”
Today, Grace is no longer in a wheelchair. Her mindset now is to go beyond the initial pain and see what happens — to acknowledge that the pain may still exist but in a different fashion that she can control. “The team at Boston Children’s and her local physical therapist gave her the tools to understand that she is in control of her body,” Leigh says.
Grace completed the school year sixth grade earning all As and Bs. Says Leigh, “Her expectation now is ‘I’ve got this’.”
The next step
GET Living has yielded excellent results in most CPRS patients — but not all. “That’s where we need to dig deeper,” says Simons. In some cases, despite concerted efforts from the family and treatment team, the patient continues to struggle.
To better understand potential barriers to treatment response, Simons has a new grant examining learning and memory play in youth with chronic pain. Patients with CRPS, particularly those with strong pain-related fear, have dampened activity in brain areas involved in learning and motivation. This is likely due to dysfunction in the brain’s threat detection system. “Once conditioned to anticipate pain-related fear, it is hard for patients to stop,” Simons explains. “This makes recovery harder and sets them up for pain chronicity.”
A planned second phase of the GET Living study will include an MRI brain scan at the start of treatment and after graduation from the program. If treatment succeeds, Simons and colleagues anticipate seeing more connectivity among the brain circuits responsible for emotion and motivation.
Learn more about the GET Living study.