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Beyond The Pain
Irfan Suleman and his colleagues bring relief—and healing—to young people whose lives have been turned upside down by debilitating chronic pain.
Photos by Alison Creasy
The Technologies Ahead
Norah Daly’s journey into the often-mysterious world of chronic pediatric pain began with a pair of routine childhood mishaps. In October 2015, the then-8-year-old from Lynchburg, Virginia, injured her right foot during a gymnastics session. Not long after, she broke two bones in her left foot while riding a scooter.
She was fitted with a cast and then had to hobble around for four weeks. There weren’t any signs of trouble until Norah complained of pain when that cast was removed. Her left foot seemed stuck in a contorted position. Patches of skin on her foot had a strange coloration.
The doctors she saw in this early period were mystified; they couldn’t locate a physical source for the pain the girl was reporting. Norah’s mother, Theresa Daly, was concerned at this point, but not panicked. She remained hopeful that the next recommended step—physical therapy sessions, starting in January 2016—would get things back on track. But Norah didn’t get better. In fact, she got worse. First, the pain spread up her left leg, far beyond the site of that original injury. Then, she started feeling pain in her other foot, where she had endured that gymnastics injury.
By February, she couldn’t walk at all. She was spending most of her days in bed, sobbing.
“This is a happy, upbeat, energetic child we are talking about,” Theresa Daly says. “It was so unlike Norah, to be down like that all day, every day.”
The Dalys went to see one specialist after another. That journey eventually brought Norah and her mother to Johns Hopkins to see an interdisciplinary team of experts in pediatric chronic pain rehabilitation. By the time Norah arrived in a clinic at the Kennedy Krieger Institute in May 2016, she was in a wheelchair. She couldn’t operate the chair without help. She couldn’t dress herself. She needed help eating meals. She had lost control of her bladder, so she was wearing diapers.
She was still crying her way through most of every day. For Theresa, the most heartbreaking moment on the family’s journey had come one night while helping her daughter get ready for bed. When it came time to say prayers, Norah asked God to just let her die.
A Long Journey
The epidemiology of pediatric chronic pain is still a work in progress. Estimates of its prevalence vary widely, as do the definitions used in different papers on the topic. In surveys, roughly one in three children report symptoms that sound like chronic pain. One in five say they feel abdominal pain at least once a month, for example, and one in four say the same about back pain.
But the bulk of these children are not experiencing problems on a level that has them looking for serious medical help. A 2016 paper published in the journal Children puts the number of pediatric chronic pain cases that are serious enough to warrant intensive rehabilitation at 3 percent.
The cases in this last group are the ones that find their way to the Pediatric Chronic Pain Rehabilitation Program, a joint effort of the Johns Hopkins Children’s Center and the Kennedy Krieger Institute. The children here are clearly in a great deal of trouble. Many have all but given up on sports, socializing and other trappings of a normal childhood. Many are not attending school anymore. And many are spending their days shuffling among bed, bathroom and couch.
“When families arrive here, they are often in a very frustrated state,” says Cynthia Ward, director of cognitive behavioral therapy services for the program. “Many of them have been to see specialists all over the region, and even the country. It’s been a long journey, and they tend to get to us at a point where they just don’t know what to do.”
The first thing the experts in the pain program want those families to do is return to square one. In a two-hour-plus evaluation, the families backtrack through the child’s physical history with a physician and a physical therapist, and then review their child’s social and psychological status with a cognitive behavioral therapist.
“These patients are used to spending about 20 minutes in a doctor’s appointment,” says Irfan Suleman, a pediatric anesthesiologist at the Children’s Center who serves as medical director for the program. “Most of them have never experienced this level of interaction with physicians, physical therapists and behavioral therapists. This is one reason they start to trust us right from the beginning.”
For Norah Daly and her family, this initial session did not go smoothly. Norah was in no mood that day to deal with another round of doctors and therapists. She cried during the exam. She responded to questions with stony silence. She ignored her mother’s pleas to calm down and cooperate. Such behavior would have been unthinkable from Norah before the onset of her pain.
“It was only after many months of this suffering that Norah started to have these difficult days now and again,” Theresa Daly says. “She was just a loving and delightful girl before this all started.”
Theresa was impressed by the way the chronic pain rehabilitation team interacted with her daughter on the worst of her days. “Everyone was just so kind and patient,” she recalls. “No one thought we were making everything up.”
Chronic pain can pop up across a number of different conditions. In Norah’s case, the team identified the culprit as complex regional pain syndrome (CRPS), a condition where pain lingers, and often worsens, after the physical injury where it originated has been resolved. That discoloration on Norah’s skin is an indicator for CRPS. So is the immobility of Norah’s foot after her cast came off.
In most CRPS cases, the pain remains focused on the area around the original injury. But in some cases, such as Norah’s, the pain can spread through a whole leg or arm and perhaps show up in a second leg or arm as well. The syndrome can have unpredictable effects on muscle coordination, with Norah’s loss of bladder control being a case in point.
Two seemingly contradictory things are true about CRPS patients. First, there is no clear physical cause for their pain, and second, that pain is not a figment of their imagination. This latter news had Theresa breathing a sigh of relief. She and her daughter had been told several times that Norah was making up her pain. One specialist had suggested in no uncertain terms that it was high time the Dalys stop “enabling” their daughter’s problems.
The precise workings of CRPS are not perfectly understood. The pain that remains after an injury like Norah’s broken bones most likely centers on some sort of glitch in nerve signaling. In young bodies that haven’t had a chance yet to learn very much about dealing with pain and bouncing back from injury, anxiety and fear may be involved. These emotions trigger the production of certain hormones in the body that can amplify pain signaling.
But there are other possible culprits. Abnormalities in peripheral nerves have been found in some CRPS patients. There is a thread of evidence indicating that the syndrome can be influenced by genetics.
Whatever the precise workings, Suleman explains, the bottom line is this: “When the healing process is done, the pain signals the body sends to the brain should get turned off. Unfortunately, that doesn’t happen all the time.” This brand of pain becomes chronic in an official diagnostic sense after three months.
Suleman has been fascinated by such cases since his days training in pediatric anesthesiology at Arkansas Children’s Hospital. He completed a fellowship in pediatric pain at Harvard in 2014, then set out in search of a position with an institution that he felt had the mix of vision and commitment that could lead to groundbreaking advances in the field of chronic pain. Today, in addition to his other titles, he is director of pediatric interventional pain management at the Johns Hopkins Children’s Center, one of just a handful of such programs in the country.
Media stories about groundbreaking advances in medicine often revolve around the search for some sort of “magic pill,” a medication or procedure that instantaneously cures a health problem once and for all. That kind of magic does happen on occasion in pediatric chronic pain. But the more likely road ahead for patients like Norah is a long, grueling run of hard work in physical and behavioral therapy.
That run may never come to an end, actually. Chronic pain can linger in the body for years, decades and even a lifetime. In such cases, the goal of the chronic pain team is to help patients develop new tools that empower them to manage their pain levels and return to a more normal life—and to do so without the help of long-term pain medications.
“Our model involves a shift in thinking for many patients and families,” says Christopher Joseph, the director of physical therapy for the program. “They come into the system saying, ‘Where is my pain coming from, and how can you make it go away?’ We try to get them to a place where they’re saying, ‘I’m going to have this pain for a long time. Can you help me deal with it?’”
On the day she brought Norah to the Chronic Pain Rehabilitation Clinic for evaluation, Theresa felt like she was ready to make that shift. The question was whether her daughter was ready too. Eventually, someone posed the question to Norah: Are you ready to spend six weeks in here working hard to try and get better?
Norah didn’t say anything, but she nodded her head.
“No One Could Figure It Out”
Pediatric chronic Pain pops up in a number of conditions other than CRPS. In Ehlers-Danlos syndrome, the pain is linked to an extreme hypermobility of ankles, knees, fingers or elbows. The resulting lack of stability in these joints can leave young patients in pain from a seemingly endless series of recurring sprains, hyperextensions and other injuries.
In postural orthostatic tachycardia syndrome, patients experience abrupt, dramatic changes in heart rate as they move from one postural position to another, and many of these patients report the discomfort they feel when this happens as pain.
In addition, a small but growing number of patients are finding their way to the chronic pain program suffering from sensory processing disorders, which manifest in a sort of sensory defensiveness caused by hypersensitivity to certain everyday environmental occurrences—bright light, perhaps, or noise, touching or some other physical phenomenon (see “Making Sense of SPDs”).
Chronic pain can also surface without any connection at all to a broader syndrome or condition. Suleman sees a good number of patients battling chronic pain in backs and abdomens. Sometimes these cases come on in the wake of a physical injury; other times, the starting point remains a mystery.
Polly Bramsen’s pain is one such case. The teen from Arlington, Virginia, was on a family vacation in England in fall 2016 when she began feeling intense abdominal pain for no apparent reason. She got through the last two weeks of that vacation, mostly by lying in bed, and then endured the long flight back. When she got home, Polly embarked with her mother on a search for a specialist who could help take her abdominal pain away.
“We went to so many doctors’ appointments I can’t even remember how many,” Polly says. “No one could figure it out.”
Polly had been the picture of adolescent health all the way up through her junior year of high school. Now a senior, she was mostly homebound. Walking around the block had become a major ordeal for her. Attending classes at the private Howard Gardner School in Alexandria was out of the question.
“It really did change my life,” she says. “It changed who I was as a person and the way I acted around other people.”
Polly was blessed with a strong support system on every front. Her mother, Georgina Bramsen, helped keep her spirits up, even on the worst of days. School friends texted and called daily to check on her. Her teachers kept her on track toward an on-time graduation.
That graduation was approaching by the time Polly found her way to the Pediatric Chronic Pain Rehabilitation program. Her evaluation session did not solve the mystery of where her pain began, but it ended with Suleman suggesting a surgical procedure called a transverse abdominis plane (TAP) block. Performed on an outpatient basis with the patient under sedation, it involves inserting a needle precisely between two layers of abdominal wall and releasing a local anesthetic.
The idea here is akin to the workings of an electrical breaker. The anesthetic is designed to temporarily flip off the pain signals going to the brain. When it wears off, those signals will flip back on, but they often do so at a recalibrated, lower level. Before the TAP block, Polly had rated her pain at 6 on a scale of 10. Afterward, she put her pain level at 1.
The TAP block is one of a variety of medical interventions that Suleman can offer his patients. In cases of back pain, he may recommend epidural steroid or corticosteroid injections. Where headache and neck pain are involved, he may try an occipital nerve block. Because chronic pain patients tend to be needle-phobic, he generally performs all of these injections under sedation, his needle placement guided by either ultrasound or fluoroscopy.
Other surgical options at Suleman’s disposal are procedures that aim to desensitize nerves that have somehow become irritated—for example, by getting trapped in the scarring that is associated with a past surgery. Suleman is actively looking at various ways to expand the range of clinical options he can offer patients in the coming years (see “The Technologies Ahead”).
There are occasions when these medical interventions do, in fact, provide patients with a “magic pill” that takes away their pain altogether. More often, as in the case of Polly, the pain returns over time at a new, lower level.
Two weeks after her procedure in spring 2017, Polly felt her pain level jump a notch higher, but it quickly settled back down. Since then, her pain has climbed back near that level of 6 a couple of times, but those episodes have been short-lived.
Polly has been working in outpatient fashion with a therapist to learn tools, such as meditation and breathing exercises, which can help her better manage her pain. If the TAP block is like a breaker, these tools are akin to a dimmer switch, letting Polly reduce her anxiety levels and refocus her mental attention in ways that bring her pain down to a manageable level.
“It’s been such a relief to feel better,” Polly says. “It went on for so long, and it was so mysterious every step of the way that it got really hard to keep going, hard to keep hoping that things would improve.”
Now a high school graduate, Polly hasn’t decided on her next steps. She may take some classes in the coming months, but she is not going to jump into college on a full-time basis. “What I’m really interested in right now is getting out into the world and just sort of starting to live my life,” she says. “I’m so grateful to Dr. Suleman because he quite literally changed my life.”
In the inpatient physical therapy unit at Kennedy Krieger, Norah Daly’s days were long, arduous affairs. Her mornings, afternoons and evenings were filled with sessions with physical, occupational and cognitive behavioral therapists. The downtime she had was generally spent in a “playroom,” where many of the games and distractions are designed to reinforce the lessons of therapy sessions.
This kind of intensive regimen right out of the gate is typical for inpatients in the chronic pain program. The exceptions to this rule tend to be patients who need help at the outset on issues that Ward describes as “foundational”—anxiety levels, sleeping habits, depression and the like. Family counseling that aims to build a more productive support network is another foundational issue that occurs on occasion.
The therapy plans that emerge are highly individualized affairs. In general, those plans share the common goal of helping patients learn to use that dimmer switch—developing a variety of skills, exercises and strategies that will help them manage their pain.
Whenever possible, those plans point toward a future free of pain medications. Narcotics, in particular, are almost never used in the chronic pain program. That’s because they sap patients of the energy they need in rehab and leave them unable to recognize and properly react to acute pain that might strike due to new injuries.
Oftentimes, physical therapy sessions in the program are relatively straightforward affairs. But there are other sessions where Joseph and his colleagues are performing what he calls “a different kind of physical therapy.”
“In those sessions, we are really pushing patients to work against what their own brain is telling them,” says physical therapy director Joseph. “It can be very difficult for them, but we need to show them that they have the ability to push through things and sort of reset their pain signaling in the process.”
On the cognitive behavioral therapy side, the team aims to give patients a plethora of pain management options, everything from breathing exercises and meditation to guided imagery and journaling. There are iPhone apps available nowadays that are designed to make some of these strategies more accessible to young patients.
“After their time with us is done, they should have an assortment of eight or nine tools that they’ve learned,” says Ward, the cognitive behavioral therapy director. “That’s going to give them a lot of options to decide what fits their needs in any given situation.”
But Suleman, Joseph and Ward all emphasize the importance of bringing interdisciplinary expertise to bear in a multimodal way that includes genuine, frequent interplay among physicians, physical therapists, occupational therapists and cognitive behavioral therapists. During inpatient care, that team can also encompass social workers, nurses, child life specialists and other experts.
“You really need to treat these patients according to a bio/psycho/social model of pain,” Ward says. “There is the physical pain, but there is everything around it too—anxiety, mood, school, family. Yes, we tease these pieces apart and think about their relative influence, but our program is built to look at them and deal with them as a whole too.”
Another key is giving patients access to clinicians with specialized training and experience in chronic pain. The supply of such expertise has been growing in recent years but still falls short of the need, Ward says.
“A mental health provider who treats anxiety and mood in the general patient population is probably not going to understand the special complexities of children with chronic pain,” she explains. “The way the pieces fit together for these patients is different. Their family dynamics are different. The fact that there is always going to be an ebb and flow to their progress—that’s different too.”
Joseph and Ward both use the word “puzzle” in describing the work of tailoring treatment regimens to patients and then adjusting them as the patients move through the program.
“We are always looking for that spark, the thing that really interests a patient,” Joseph says.
Physical therapists on Joseph’s team often find that spark by taking advice from colleagues on the cognitive behavioral therapy side, as they share ideas gleaned through individual or family therapy. Early on in Norah’s case, it became apparent that working with therapy dogs would help bring that spark to her eye.
From her mother’s point of view, the progress Norah made during her time as an inpatient happened with astonishing speed. In a matter of days, she was dressing and feeding herself. After a week, she was able to operate her wheelchair on her own. After a month, she put on a special Father’s Day show, getting out of her chair and walking in front of her father and siblings.
“The last week there, Norah was riding a bike down the hallway—it was just so incredible to see,” Theresa Daly recalls with a laugh.
Some 18 months after that bike ride, Norah remains fully mobile. She is back up to speed with her school lessons, and the activities of a more normal childhood have returned to her day-to-day life.
She still grapples with pain on a daily basis, however. While that pain is no longer as debilitating as it once was, it can still get quite intense. The watchwords in the Daly household at such times are taken straight from Norah’s sessions with her cognitive behavior therapists: “acknowledge” and “redirect.”
She relies on different tools in different circumstances. She finds relief in a swimming pool on many days. Other times, she turns to deep breathing exercises or to a desensitization exercise that involves rubbing her body down with different cloth fabrics.
“There are still days when her brain and her body are telling her to just lie down and stop everything,” Theresa says. “But she is going against that. She’s doing a great job of taking what she’s learned in therapy and always moving forward and staying really positive. She’s a fighter now, and she’s fighting the fight really well.”
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‘‘Most [patients] have never experienced this level of interaction with physicians, physical therapists and behavioral therapists. This is one reason they start to trust us right from the beginning.”
Making Sense of SPDs
Intrigued by the number of patients who are showing up at the Pediatric Chronic Pain Rehabilitation Program with sensory processing disorders (SPDs), Christopher Joseph is in search of private funds for a small proof-of-concept research project that could ultimately point the way to better diagnosis and treatment.
In an effort to describe how SPDs work, the program’s director of physical therapy offers the example of a healthy person who deals with occasional bouts of acrophobia, the fear of heights. “If it’s a serious case, that can be a really disorienting thing, right?” he asks. “OK, now imagine that person is back on solid ground, but he or she feels like that all the time.”
Various sensitivities can be involved in an SPD. Some patients can’t handle loud noises, while others have trouble with bright lights, soft touches on their skin or brushes running through their hair. One indicator that a case of chronic pain may have roots in SPD is a patient’s tendency to offer up a scattershot description of their pain, identifying it simultaneously as shooting, burning, aching and throbbing.
“A lot of times, these kids don’t seem to have a vocabulary to describe the discomfort they’re going through, so they end up calling it pain,” he says.
These sensitivities often cause SPD patients to withdraw from human interaction and do poorly in social situations. By the time they reach the age of 11 or 12, Joseph says, it’s too late to help them reverse the sensitivity, and therapists need to focus instead on “helping them adapt to the situation.”
An important early step in the research Joseph plans to conduct is looking at how and why SPD patients show up at the clinic. “How are these kids presenting?” Joseph asks. “Are they coming through a diagnosis of anxiety disorder? Are they coming through some other diagnosis? Are there more of them out there, or is it just that more of them are ending up with us? Once we know these sorts of things, we can start looking at other questions. How do we find these kids out there in the system? What solutions can we offer them?” JD
Polly, left, and Georgina Bramsen