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11 Articles in Volume 14, Issue #10
Combating Opioid-Induced Constipation: New and Emerging Therapies
Updates on Smoking and Low Back Pain
Unraveling the Psychological Mechanisms Behind Smoking in Chronic Pain
Addressing Psychosocial Factors in Pain Management in the Emergency Department
Long-Term Outcomes and New Developments in Juvenile Fibromyalgia
Pain Management in the Elderly: Etiology and Special Considerations
Using Pharmacogenetic Testing in a Pain Practice
Editor's Memo: Care With Caution
Ask the Expert: HIPAA Rules
Ask the Expert: DMARDs and Opioids
Letters to the Editor: November/December 2014

Long-Term Outcomes and New Developments in Juvenile Fibromyalgia

Recent findings indicate that symptoms persist throughout adolescence and young adulthood for the majority of fibromyalgia patients. Recommended treatments continue to be interdisciplinary and may involve parental guidance and family support for lifestyle changes.

Juvenile fibromyalgia (JFM) is a chronic rheumatologic condition characterized by persistent widespread musculoskeletal pain, fatigue, and sleep disturbances. Additional symptoms are common and include chronic headaches, gastrointestinal problems, anxiety and stress, mood problems, muscle stiffness, cognitive symptoms, and dysautonomia.1 JFM, which affects between 2% to 6% of school-age children, is more prevalent in girls compared to boys.1-6

Although JFM is one of the most understudied pain syndromes in children, in recent years more has come to light about the clinical characteristics, long-term outcomes, and effective treatments for managing the many symptoms of JFM.7 Recent findings indicate that symptoms of JFM persist throughout adolescence and young adulthood for the majority of JFM patients.

There is little evidence to support pure pharmacological management of JFM, particularly as a long-term solution. Recommended treatments continue to be interdisciplinary and may involve parental guidance and family support for lifestyle changes. With this in mind, more effective and long-lasting new treatments need to be developed to treat chronic symptoms.

What Causes JFM?

It is now thought that patients with fibromyalgia (FM) have a ramping up of their pain perception. Research on the neurobiology of FM supports the prevailing theory that heightened sensitivity to pain signals in the brain and disturbances in descending inhibition of pain, also known as central sensitization, are a primary underlying mechanism for the pain symptoms in FM.8,9 In other words, patients with FM may be unable to modulate pain signals, which results in excessive and persistent pain.

The fact that chronic pain disorders, particularly FM, tend to co-occur in families10 lends credence to theories that there may be a genetic predisposition to pain sensitivity in such conditions. Other potential explanations for heightened pain sensitivity include increased physiologic “memory” for pain, hormonal imbalances, or an infectious trigger. (See Kashikar-Zuck and Ting for a more comprehensive review of research into multiple potential biological causes for FM.11)

Recently, there has been some skepticism about the role of central sensitization as the primary underlying pathology in FM, particularly due to mixed findings for central nervous system dysfunction as well as overlap found between symptoms of FM and those of newly identified peripheral anomalies, such as small-fiber polyneuropathy.12,13 Small-fiber polyneuropathy is caused by the dysfunction and degeneration of the small-diameter unmyelinated C-fibers and thinly myelinated A-δ peripheral nerves that initiate pain perception. Recent findings indicate that adults with FM have increased evidence of small-fiber neuropathy14 compared with healthy control subjects.12,13 One study that examined children, adolescents, and young adults with juvenile-onset chronic widespread pain also found evidence of definite small-fiber neuropathy in 59% of the sample, and probable or possible neuropathy in an additional 39%.15 Further, markers of immune dysfunction were found in many of the patients with FM and chronic widespread pain with small-fiber neuropathy, suggesting a possible mediating process.12,13,15

There are several biological causes of small-fiber neuropathy (eg, diabetes); therefore, it is possible that a subset of patients with FM may have a potentially treatable underlying cause for neuropathy.13,14 Anecdotally, treatment with corticosteroids or immunoglobulin therapy was reported to be effective in patients with chronic widespread pain,15 as well as in patients with FM and chronic polyneuropathy.16 The findings of these studies should be interpreted with caution because small-fiber neuropathy has been identified in only a subset of FM patients, and no research has been done thus far in JFM. Additionally, the underlying cause of neuropathy in adults and children with FM is unknown, as is the effectiveness of treating neuropathy to manage FM pain.

Long-Term Outcomes in JFM

New studies are beginning to document the longitudinal outcomes of youth diagnosed with JFM, thereby shedding more light on the prognosis for JFM patients. Using longitudinal data from the national Childhood Arthritis and Rheumatology Research Alliance dataset, Connelly et al examined outcomes in children with JFM, comparing findings at the patients’ initial clinic visits and at follow-up visits 0.25 to 2.5 years later.17 They found that pain intensity, well-being, and functioning worsened during the follow-up period. Widespread pain was less frequent, but associated symptoms (eg, headache, disrupted sleep, irritable bowel symptoms, paresthesias) did not change over time, regardless of treatment modality or adherence to treatment. These findings suggest that JFM symptoms are persistent even at early stages of the condition, and that currently offered treatments are not very effective in reducing symptoms or their impact on functioning.

In fact, it appears that symptoms of JFM continue into adulthood for the majority of patients. In a 6-year follow-up study of young adults diagnosed with JFM during adolescence, patients with JFM had significantly higher pain scores, poorer physical functioning, and a greater number of medical visits compared with healthy control subjects. The majority of JFM patients (80%) continued to experience FM symptoms into early adulthood, and half (51.1%) met American College of Rheumatology criteria for adult FM.18

In addition to physical symptoms, mood difficulties also seem to persist in many patients with JFM. Symptoms of anxiety19 and depression20,21 are higher in youth with JFM compared with healthy controls. These symptoms tend to remain comorbid with FM into young adulthood.18 The young adult years seem to be a particularly vulnerable time for increasing anxiety and mood difficulties (see Table 1); this is true for young adults in general but is, perhaps, even more so for youth with JFM as they struggle with increasing social and vocational demands and expectations for greater independence from family. The literature on psychiatric comorbidity in FM indicates that rates of current major depression are lower in young adults with JFM than in older adults with FM, whereas rates of lifetime major depression and dysthymia are higher for those who have FM starting in their childhood. Additionally, rates of anxiety disorders have been shown to be high across the lifespan in FM patients.22,23 Considered together, these findings highlight the crucial importance of targeting mood symptoms early in the course of treatment, with particular emphasis placed on the management of anxiety and assessment of risk for depression.

The role of the family and peer relationships is important to consider, given that youth with JFM can become socially isolated from their peers,24 and the family environment can influence how well they are able to function in their daily lives.25 Adolescents with JFM from controlling family environments are at an increased risk for poorer emotional functioning (particularly depression). Therefore, it is important that behavioral and family interventions encourage independent and active coping among adolescents with JFM so that they experience self-efficacy in independently managing their symptoms.25

Clinically, when multiple family members have JFM/FM, there seems to be greater empathy and awareness within the entire family. However, it is important to stress to parents and children that each individual’s experience of pain and coping varies; thus, symptoms may not be alike. Nevertheless, it is important to encourage family members to support each other both physically and emotionally (eg, as coaches or cheerleaders) to create a positive and constructive environment for successful symptom management.

Given the multitude of factors that may influence the development and maintenance of symptoms (eg, neurochemical, physical, and psychosocial factors), it is important for interventions to be interdisciplinary and powerful enough to provide sustained effects.

Treatment Options

JFM primarily is treated with education, cognitive-behavioral therapy (CBT), and physical therapies and exercise, with a relatively minor role for pharmacologic treatment.26,27 There is consensus among evidence-based treatment recommendations for FM that self-management techniques should be promoted (eg, aerobic exercise and psychological interventions teaching stress management and relaxation skills), rather than passive (eg, massage, medication only) management strategies.27 Unfortunately, there are few guidelines specifically for treating JFM.

The majority of young adults diagnosed with JFM report using medication and psychotherapy, with the most common treatment used over the past 2 years being antidepressant medication. Overall, patients reported that treatments were effective; however, medications were variable in their effects.28 There appears to be a gap between the recommended active treatments (ie, self-management strategies) for FM and those used most often by young adults with JFM (medication). Many basic lifestyle changes including improved sleep hygiene, regular physical exercise, and pacing activities are relatively straightforward but can be difficult for JFM patients to implement because they require ongoing commitment and practice. These changes are challenging enough for most individuals but can be even more so for adolescents who just wish to be “normal” and fit in with their peers. Therefore, recommendations such as regular bedtime routines often are met with resistance. Furthermore, mood symptoms, fatigue, and pain coupled with limited coping skills directly affect motivation to make adaptive changes. Hence, structured therapies such as CBT can be of enormous benefit.

Medications

There is limited support for medications for treating JFM.26,27 Medications may play a role in the management of JFM symptoms; however, they do not provide long-term solutions. There are ongoing clinical trials in patients with JFM evaluating the use of pregabalin (Lyrica) and duloxetine, both of which are approved for use in adult FM.29,30 A trial of the selective serotonin reuptake inhibitor fluoxetine in patients with JFM recently was completed and results are pending.31

Opioids are not recommended for JFM management,27 given limited efficacy and the potential addiction risks associated with chronic use. However, tramadol, a weak µ-opioid receptor agonist and serotonin and norepinephrine reuptake inhibitor, has been evaluated in a small trial in adult FM patients and may be an option for JFM patients for whom other medications are not helpful. Additional medication management in JFM commonly is targeted towards managing symptoms of sleep disruption, irritable bowel symptoms, headaches, dysmenorrhea, etc.11

Though the evidence does not provide clear recommendations, the following could be considered in clinical practice: For sleep, initial medication recommendations could include melatonin or medications such as amitriptyline or muscle relaxants such as cyclobenzaprine. A small (n=36) randomized controlled trial performed in adults with FM found cyclobenzaprine to be of benefit for sleep.32 Amitriptyline may have the added benefit of preventing headache (insufficient evidence to date but a large pediatric trial is ongoing33) and reducing functional abdominal pain.34 Non-steroidal anti-inflammatory medications and oral contraceptive agents may provide additional pain relief when appropriate.

Cognitive Behavioral Therapy

A recent Cochrane review (2013) found that CBT for patients with FM (mostly based on adult studies) provided a small incremental benefit over control interventions in reducing pain, negative mood, and disability at the end of treatment and at 6-month follow-up.35 Specifically in the treatment of adolescents with JFM, CBT demonstrated relatively robust improvements in functional disability and depressive symptoms36 as well as improvement in pain coping, pain catastrophizing, and pain coping efficacy that were sustained over 6 months.37 Adolescents with greater initial disability and higher coping efficacy were especially likely to benefit from CBT and experienced a clinically significant improvement in functioning.38

The increased use of specific coping strategies learned during CBT was thought to be the primary reason CBT was effective. However, it appears that CBT results in more global changes in psychological perceptions of pain (cognitive appraisals) and the ability to manage pain, thereby reducing the impact of pain in patients’ lives.39 Targeting maladaptive thought patterns related to pain and functioning is particularly important to remove barriers to lifestyle changes and to provide JFM patients with a sense of self-efficacy.

CBT is available for children and adolescents, and education about pain coping skills can be modified based on developmental level. Most CBT therapists with pediatric experience are able to adapt the therapy for different ages as well as for boys versus girls. For example, younger children may benefit from more concrete directions about how to distract themselves during pain flares and from increased parent involvement and coaching, whereas adolescents should be working toward managing their pain more independently, and are better able than children to apply cognitive restructuring skills, such as identifying maladaptive thought patterns about pain.

Boys tend to prefer more behavioral strategies (such as activity pacing and problem solving), whereas girls are likely to respond well to strategies such as deep muscle relaxation, guided imagery, and cognitive skills (identifying thoughts and feelings). The published trial on CBT for JFM showed high engagement of adolescents (11-18 years of age) with CBT, with more than 85% of patients completing the treatment.37 Across all age groups, it is important for the parents to demonstrate understanding and willingness to have their child/teen participate in treatment and to learn how to support them through positive feedback as they learn adaptive coping skills.

Alternative Therapies

Alternative therapies such as massage, yoga, and acupuncture often are sought as non-pharmaceutical approaches to pain control. There are few trials testing the effectiveness of these therapies in JFM. In children and adolescents, complementary treatments are used less often than medical management or psychotherapy, but when such treatments are used, they often consist of yoga or massage.28 Females with JFM generally are more likely to use these alternative therapies compared with males.40

Physical Exercise Interventions

Physical activity is an important component of pain management for individuals with FM. The American Pain Society recommends 30 minutes of moderate to vigorous exercise 2 to 3 times per week (eg, brisk walking, swimming, sports) for individuals with FM.41 For adolescents with JFM, we often phrase this as “any sustained physical activity that makes your heart beat faster, your breathing get harder, and/or causes you to sweat.” Aerobic exercise and strength training have been found to be effective for reducing pain in adults with FM,42-44 and one study conducted in adolescents with JFM demonstrated short-term benefit from an aerobic exercise program45; however, it is not known whether patients continued to exercise after the 12-week program, which would be necessary to obtain continued benefit.

Despite these recommendations and promising findings, objective physical activity monitoring has documented that adolescents with JFM are very sedentary and, therefore, are at greater risk for becoming deconditioned and experiencing increased pain.46 Patients with JFM have altered biomechanics, which might be a barrier to initiating physical activity, putting them at increased risk for injury or pain after exercise.47

Specifically, one study showed that adolescents with JFM demonstrated mild deficits in walking gait, significant strength deficits in knee extension/flexion and hip abduction, and deficits in functional performance compared with their matched healthy peers. These deficiencies became more pronounced as functional physical tasks increased even slightly in difficulty level (eg, performing a vertical jump task). Despite relatively mild objective functional deficits, adolescents with JFM had significantly greater fear of movement than healthy teens. The resulting activity avoidance may exacerbate physical deconditioning over time.

Early intervention to correct these deficits and, more importantly, increase their confidence in exercise could help prevent further deconditioning and improve functional outcomes.47 Adolescents typically respond well to a gradual reintegration into activities that they previously enjoyed, such as sports, playing outside with friends, or taking walks with family members or pets.

Skilled physical therapy with a professional familiar with JFM also is beneficial for building core strength and stamina. Low-impact aerobic activities, such as swimming or aquatic therapy, can be very useful and often are recommended. However, although there are numerous options for physical exercise, a high level of motivation and persistence is required to maintain these activities.

Need for Novel, Integrated Treatments

Given the chronicity of JFM symptoms and the disruption that occurs in normal functioning and development, novel interventions that target healthy lifestyle and self-management techniques to provide sustained effects are necessary.27 A single modality of treatment may not offer all the necessary components. For example, although CBT results in improvements in daily functioning and increased well-being, CBT alone does not increase physical activity/exercise or offer marked pain relief.48 CBT emphasizes cognitive pain coping skills and behavioral interventions, such as relaxation and activity pacing, which do not inherently encourage vigorous activity. As noted earlier, physical exercise can be beneficial, but effects cannot be sustained without a high level of motivation and persistence. Thus, studies have combined CBT with behavioral activation and/or exercise training in adults with FM; these studies have shown beneficial effects, but this approach has not been tested in adolescents with JFM.49,50

Recognizing that increased physical activity and exercise is an important target but that engagement in regular exercise is difficult in patients with JFM, especially in light of evidence showing biomechanical deficits and fear of physical activity, interventions need to be designed with the unique needs of JFM patients in mind.47 Clearly CBT has shown great promise in treating adolescents with JFM, but incorporating an exercise component into treatment requires a tailored approach. Our research group has discovered that neuromuscular training programs51 based on pediatric exercise science principles to prevent injury52 and improve fundamental physical skills is an ideal approach for JFM patients.45,51,52 This training can be modified to gradually increase the level of challenge to minimize delayed-onset muscle soreness.4

Neuromuscular training primarily is focused on teaching safe exercising techniques by improving gait, posture, balance, strength, and functional movement, which also increases participants’ confidence in increasing activity levels. This type of training improves body biomechanics and allows patients to begin engaging confidently in more vigorous activities. Importantly, when beginning any exercise program, JFM patients need careful education to learn to differentiate muscle soreness from a pain flare. Slight increases in soreness are inevitable before muscles become conditioned and strong, and it is important for patients to be able to overcome this initial discomfort to achieve eventual pain reduction.8

An integrated program combining CBT and specialized physical exercise may provide the most effective way to promote self-management skills and achieve long-lasting positive treatment outcomes.53 Such a treatment approach may improve the motor skills and confidence that youth with JFM require to engage in activity, while simultaneously addressing psychological contributors to enhance motivation and adherence; this tailored approach affords patients the opportunity to integrate regular exercise into their lifestyle and achieve better pain management.47

Our research team is testing an integrated CBT and neuromuscular training program for teens with JFM. The 8-week training program is conducted by 2 trainers (a CBT therapist and an exercise trainer) in small groups of 4 to 5 patients with JFM. Group members learn new neuromuscular exercises and coping skills as well as how to support one another in a positive and encouraging environment (see Figures 1 and 2). Parents attend some of the sessions and learn coaching skills to enhance their teens’ practice outside of sessions. Preliminary evidence demonstrates that the intervention is very well received by participants. They report feeling stronger and more confident in their movement, in addition to having more energy and motivation to participate in physical and recreational activities. Additionally, teens appreciate participating in a group treatment format with other JFM patients, which affords them the opportunity to meet other teens with JFM and offer support to one another.54

Conclusions

Symptoms in JFM are varied and include physical and psychological complaints that persist into adulthood. Research suggests that there are potential factors that contribute to the development and maintenance of JFM symptoms and the best treatments available are interdisciplinary and promote active self-management techniques. Interventions for JFM need to be more powerful than those currently available, and they also need to be long lasting. An intensive integrated CBT and specialized physical exercise intervention holds promise because this approach promotes positive pain coping skills while establishing sound biomechanical fundamentals that allow JFM patients to engage in physical and recreational activities more comfortably and confidently. If the effects of such interventions can be sustained, then disability associated with JFM likely will decrease as pain control and quality of life improve.

Last updated on: June 15, 2015
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