Juvenile Fibromyalgia Syndrome and Improved Recognition by Pediatric Primary Care Providers

Judith D. McLeod, DNP, RN, CPNP


J Pediatr Health Care. 2014;28(2):e9-e18. 

In This Article

Review of the Literature

JFS is a relatively new diagnosis in pediatrics, with initial identification of the syndrome by Yunus and Masi in 1985. Because of the lack of a diagnostic test, JFS is identified by history and physical examination of the child, which pinpoint the criteria that detect the syndrome.

Criteria for Diagnosis of JFS

JFS is defined as a noninflammatory condition characterized by widespread diffuse pain and specific tender points found on a physical examination. Normal laboratory values and related problems, such as migraine headaches or irritable bowel syndrome (IBS), are usually associated with the condition (Siegel, Janeway, & Baum, 1998). Most studies within JFS relate to the research by Yunus and Masi in 1985, which established the criteria for the diagnosis of juvenile fibromyalgia as 3 months of diffuse pain, five or more tender points, and sleep disturbances. It also may be associated with such minor criteria as anxiety, abdominal pain, or headaches. Gedalia, Garcia, Molina, and Bradford (2000) and other investigators conducted studies in which these criteria were used for diagnosis, which then led to the establishment of these criteria as the standard. These criteria are included in the Guidelines for Management of Fibromyalgia Syndrome Pain in Adults and Children that were established by the American Pain Society. Tender point examination was studied in children by Swain, Kashikar-Zuck, Graham, and Prahalad in 2005. They found that children with JFS may have more than five tender points and determined that using tender points is a valid method for diagnosis of JFS in children.

Musculoskeletal Pain and JFS

Reports of diffuse musculoskeletal pain in children are not uncommon in pediatric practice. The occurrence of musculoskeletal pain syndromes in the general pediatric population of otherwise healthy-appearing schoolchildren and adolescents is 5% to 20%. JFS is associated with about 25% to 40% of these pain syndromes (Anthony & Schanberg, 2001). Juvenile fibromyalgia has a prevalence rate of 6% to 7% in school-aged children, and it is the third most common diagnosis in pediatric rheumatology clinics, representing 15% to 20% of all clients with rheumatology diagnoses. The most prevalent group to be diagnosed in the pediatric population is adolescents aged 13 to 15 years (Brown and Greenwood-Klein, 2001, Buskila, 2009, Yunus and Masi, 1985).

Current Therapies for JFS

Current therapies for JFS are individualized for each patient. The therapy may include medications such as amitriptyline (Elavil) for sleep disturbances and tramadol (Ultram) for pain. Medications such as pregabalin (Lyrica), duloxetine (Cymbalta), and milnacipran (Savella) are approved by the United States Food and Drug Administration for adults but have not yet been approved for children. Clinical trials are ongoing for the use of these medications for JFS in children (Buskila, 2009, McCarberg, 2011; Mease et al., 2009). Cognitive behavior therapy is useful in the JFS population for coping with the syndrome and exercise is encouraged, including physical therapy if indicated. Some patients choose to try alternative therapies such as acupuncture and massage for the pain. The best treatment plan is a combination of all these therapies (Brown and Greenwood-Klein, 2001, Burckhardt et al., 2005, McCarberg, 2011).

JFS Incidence and Differences From Adult Fibromyalgia

The incidence of JFS in adolescents is higher in girls than in boys, and the incidence by gender becomes most apparent at the onset of puberty (Anthony and Schanberg, 2001, Perquin et al., 2000). However, in the younger age groups, gender may be equivalent (Gedalia et al., 2000).

The clinical presentation of juvenile fibromyalgia differs from fibromyalgia in adults in several ways. Children have diffuse pain but have a higher prevalence of sleep disorders and have fewer tender points on examination—usually less than the 11 points required for adults. Only five tender points are required for diagnosis in children. Hypermobility is also strongly associated with fibromyalgia (Buskila, 2009). Mease and Seymour (2008) reported that fibromyalgia was associated with widespread pain even if the number of tender points does not meet the specific criteria for diagnosis. This phenomenon occurs frequently in the pediatric population. The lower occurrence of tender points may be due to the early stage of the disease in children and adolescents, with fewer areas of tenderness manifested. Longer periods between disease occurrence and diagnosis may result in an increase in tenderness and positive tender points (Siegel et al., 1998).

Lack of Provider Recognition

Health care providers see many pain syndromes in children. Of these pain syndromes, JFS is not well understood. The overlap in symptoms with other disorders, such as migraines and chronic fatigue, can lead to a delay in recognition and treatment (Kashikar-Zuck et al., 2007). The difficulty with recognition and diagnosis by pediatric care providers is a primary problem for patients with juvenile fibromyalgia. Children's pain can be overlooked or misdiagnosed. Brown and Greenwood-Klein (2001) found that 60% of the children were initially told by several physicians that they had juvenile rheumatoid arthritis, and 20% were diagnosed with growing pains. Mease et al. (2009) stated that it may take as long as 5 years for a patient with juvenile fibromyalgia to receive an accurate diagnosis, and 42% of those patients are diagnosed after a referral to a rheumatologist. When patients were asked how many providers they visited before a diagnosis was made, 46% had visited three to six providers and 25% had seen more than six providers before they received an accurate diagnosis. The approximate time from the first visit until a pediatric patient with fibromyalgia is referred for treatment is 6 to 12 months or longer (Bennett, Jones, Turk, Russell & Matallana, 2007; Mease et al., 2009).

Ramifications of Delay in Diagnosis

Delay in diagnosis can cause children more difficulty in coping with symptoms. JFS is associated with sleep disturbances, which are characterized by prolonged sleep latency, shorter amounts of sleep during the night, decreased sleep efficiency, and an increase in wakefulness during sleep, with some patients sleeping only 3 or 4 hours a night. These sleep disturbances contribute to chronic fatigue that can interfere with normal functioning, growth, and the ability to deal with stress (Tayag-Kier et al., 2000). In this population, fatigue may also contribute to the development of depression and functional disability (Libby & Glenwick, 2010). Fatigue and depression also may contribute to an increase in school absenteeism. Children with JFS, including the 12% of adolescents who are homeschooled, miss an average of 2.9 days of school a month (Kashikar-Zuck, Johnston, et al., 2010). Children who do not attend school miss social interaction with other children and can fall behind, resulting in a negative impact on their academic success (Libby & Glenwick, 2010). In a study by Conte, Walco, & Kimura (2003), adolescents with JFS have increased levels of stress and anxiety, more temperament instability, and pain sensitivity. They participate less in social activities, sports, and exercise and have an increased tendency toward obesity.

Lack of recognition of the problem in the pediatric population also causes an increase in the cost of visits for the family until a diagnosis can be made. Studies have shown that patients with fibromyalgia use more health care resources, including more visits to medical providers, and their increased cost of pain-related medications is two to three times higher than healthy control subjects. Medical costs for a patient with fibromyalgia are on average $9573 a year, compared with $3291 for healthy patients. In a managed care setting, with an average of $30 to $40 co-pays for care, the patients with JFS will spend an additional $500 to $600 in costs to be diagnosed because of additional visits, medications, and diagnostic tests that might be ordered (Annemans et al., 2008; Berger, Dukes, Martin, Edelsberg, & Oster, 2007; Lachaine, Beauchemin, & Landry, 2010; Palacio et al., 2010; Sicras-Mainer et al., 2009).

Society incurs an increased cost when children miss their regular education, thus having fewer opportunities for social interaction, sports participation, and other extracurricular activities or work, consequently creating more hardship for them compared with normal peers. Kashikar-Zuck, Parkins, and colleagues (2010) found that these patients also will need long-term care for pain, sleep problems, and depression. An adolescent with less ability to cope will become an adult with the same problems (Kashikar-Zuck, Parkins, et al., 2010).

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