ATLANTA—Cognitive-behavioral therapy (CBT) can be highly effective for reducing disability and depressive symptoms in patients with juvenile fibromyalgia (JFM), according to recent research. But experts on the syndrome say that progress in treating JFM has been hindered by clinicians’ reluctance to diagnose it.
“It’s important that, if we strongly feel they have fibromyalgia, to give them the diagnosis,” said Tracy Ting, MD, MSc, assistant professor of pediatrics at Cincinnati Children’s Hospital, in a session, “Juvenile Fibromyalgia,” here at the 2010 ACR/ ARHP Annual Scientific Meeting. [Editor’s note: This session was recorded and is available via ACR SessionSelect at www.rheumatology.org.] “It’s a good stepping point which they can take back to their schools, talk to their friends and their family who are also questioning what’s going on with them.”
JFM is a chronic noninflammatory musculoskeletal pain condition characterized by widespread pain, and multiple tender points, according to the session’s other presenter, Susmita Kashikar-Zuck, PhD, director of the of the pediatric pain research program at Cincinnati Children’s. It primarily affects adolescent girls. The dilemma for physicians is that patients often present with symptoms that have lasted several months, having consulted several specialists who have found no explanatory cause. Diagnostic tests are usually unremarkable and clinical symptoms are often vague and diffuse. As a result, anxious families frequently shuttle between specialists and their pediatrician without a resolution.
But evidence is building that JFM has physiological underpinnings, Dr. Kashikar-Zuck said. Meanwhile, there are more signs that treatment is effective, further underlining the importance of making a diagnosis.
Lives Turned Upside Down
At least half of Dr. Ting’s JFM patients have had their “lives turned upside down” by some insult, she says, whether it is an illness such as mononucleosis or an injury. The incident sets them up for a vicious cycle of pain, poor sleep, and fatigue.
The exact etiology of JFM is unknown, Dr. Ting said. But fibromyalgia is related to a disordered pain regulatory process. An unpleasant or noxious stimulus that would produce only mild or momentary discomfort in a normal subject can be experienced as intensely painful by someone with fibromyalgia, providing the basis of months or years of pain. One theory behind JFM is that the “pain gate” surrounding the nociceptor fibers in the spinal cord opens in response even to mild stimulation such as pressure. New research using functional magnetic resonance imaging shows that fibromyalgia patients experience pain differently than controls. However, other factors appear to amplify pain perceptions and contribute to the condition. Genetics appears to play a role in pathogenesis, as do mediators such as the presence of Substance P, environmental factors such as stress or anxiety, and psychological factors such as anxiety or depression.
Treatment begins with a diagnosis and moves on to education. Dr. Ting said she typically educates patients about sleep hygiene and prescribes physical activity. Exercise is key to reducing the pain response, but it must be graded to be sustainable. Symptom management includes medication as needed for sleep and mood disorders, headaches, irritable bowel syndrome, and other associated symptoms. Pain, however, has proven to be more intransigent. “If I tell [patients] right up front there’s no proven medication, no quick fix for their pain, I’m going to lose them immediately,” Dr. Ting says. “They’re going to shut down and not listen to a word I say.” Dr. Ting tries to bring up pain treatments and their limited effect last in the discussion.
Dr. Ting also suggested treading carefully around psychological issues, because many patients have been told that the condition is purely psychological. But she also suggested consulting with psychologists or psychiatrists early in the process, if necessary, given the potential effectiveness of CBT. Patients may be receptive to the idea that there are coping skills and distraction techniques to help them deal with the pain. Finally, she urged physicians to inform the patient’s school of the diagnosis.
Daily and Long-Term Impacts
JFM can have a dramatic impact on daily life, noted Dr. Kashikar-Zuck. Her team conducted a sociometric study that found that the patients and their peers perceived patients with JFM as more socially isolated and less well liked than others. In another study of family life, she found that JFM patients had poorer family functioning and more conflicted family relationships. (Mothers also reported twice as many pain conditions and were five times more likely to have fibromyalgia.) In a third study, she found a strong association between JFM and anxiety, mood, and behavioral disorders. Fifty-seven percent met the criteria for at least one anxiety condition, 41% reported mood disorders, and more than 20% had a behavioral diagnosis.
The long-term outcomes for adolescents with JFM are not well studied. Community-based studies tend to show that individuals with chronic widespread pain improve over time, while clinical studies have found that the majority of JFM patients show a less positive prognosis. Dr. Kashikar-Zuck is following a cohort of adolescents diagnosed in her clinic through young adulthood. In a Web-based questionnaire of patients who had been diagnosed three to four years earlier, 60.7% reported that they continued to experience all the cardinal features of fibromyalgia. More than 70% reported some of the cardinal symptoms. (The study has been accepted for publication in Rheumatology.)
CBT and Coping
CBT has been shown to be useful for pain management in adult chronic pain conditions, Dr. Kashikar-Zuck says, and shows promise in the treatment of pediatric chronic pain. CBT teaches self-management and coping skills to reduce disability, distress, and pain.
The protocol for CBT for JFM includes relaxation training, distraction techniques, activity pacing, cognitive techniques such as calming statements, and problem solving. As part of the protocol, parents should be coached in how to support the patient’s efforts, Dr. Kashikar-Zuck said. It’s also important to provide patients with the rationale for the behavioral techniques. Dr. Kashikar-Zuck discusses the “pain gate” in an age-appropriate way to her adolescent patients.
In a small study of 30 patients led by Dr. Kashikar-Zuck, patients were randomly assigned to eight weeks of CBT or self-monitoring.1 Functional disability scores declined by 38.8% overall. Other studies also showed positive results.
Recently, Dr. Kashikar-Zuck decided to test the thesis further by comparing eight weeks of CBT to eight weeks of one-to-one fibromyalgia education from a therapist. During the session at the ACR meeting, Dr. Kashikar-Zuck presented preliminary results of the randomized, blinded study of 114 children and adolescents. Patients receiving CBT showed an improvement of nearly 40% in Functional Disability Inventory scores at a six-month follow-up visit compared with about a 10% improvement for patients receiving fibromyalgia education alone. Scores in the children’s depression inventory improved 35% with CBT, compared with about 25% for the education group.
Reduction in pain from CBT was small but significant. While pain reduction is not a primary objective of CBT, Dr. Kashikar-Zuck found it encouraging that the treatment could improve functioning and increase activity without any increase in pain. “The general trend is for parents to say, ‘My child will start doing things as soon as the pain gets better.’ My reaction is to say, We’re going to do things the other way [around]. We’re going to have your child start doing things first and allow the pain to get better. We don’t want to let her life to start falling apart while we wait for the pain to go away.”
The path to wellness for these adolescents starts with a physician who feels comfortable dealing with chronic pain, Dr. Kashikar-Zuck told the audience. “Patients are going to come in and see their physician first. If they feel a sense of discomfort, rejection or abandonment, you’ve already set up a situation where they’re not going to respond well to treatment. Remember, these patients have usually been through the wringer before they ever get to you. So if it is possible to listen, and to speak to them in a reassuring way, and give the impression you’re able to understand and at least tell them what is known in an honest way, and let them know what you can and can’t do for them, patients will engage. And it is our goal to engage early, when they’re adolescents. Because all the research we’re doing shows this is something that can and should be managed early—and when left untreated can essentially lead to some long-term problems.”
Richard Sine is a medical journalist based in Atlanta.
