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Dr Mehran Rezvani pain fellowship anesthesiologist & acupuncturistFibromyalgia in Children and AdolescentsDr Mehran Rezvani pain fellowship anesthesiologist & acupuncturistFibromyalgia is one of a group of chronic soft tissue pain disorders

The etiology and pathogenesis of this disorder are unknown, although data indicate a significant central sensitization component is at the root of the syndromeDr Mehran Rezvani pain fellowship anesthesiologist & acupuncturistIt is characterized by:

Chronic and diffuse musculoskeletal pain

Severe fatigue

Nonrestorative sleepDr Mehran Rezvani pain fellowship anesthesiologist & acupuncturistDEFINITIONDr Mehran Rezvani pain fellowship anesthesiologist & acupuncturistIn adult:The ACR criteria (1990) Widespread pain for at least three months duration and the presence of at least 11 tender points at 18 potential sites

These criteria for children, however, has not been validated

Dr Mehran Rezvani pain fellowship anesthesiologist & acupuncturistAmerican College of Rheumatology (ACR) in 1990

6In children Yunus criteria:Fewer tender points (5 versus 11) Clinical findings beyond diffuse painAbsence of another underlying condition or causeNormal laboratory test resultsDr Mehran Rezvani pain fellowship anesthesiologist & acupuncturistIn Adult :

Dr Mehran Rezvani pain fellowship anesthesiologist & acupuncturist

Dr Mehran Rezvani pain fellowship anesthesiologist & acupuncturistDigital palpation should be performed with an approximate force of 4 kg. For tender pointto be considered "positive" the subject must state the palpation was painful. "Tender" is notto be considered "painful."9

Dr Mehran Rezvani pain fellowship anesthesiologist & acupuncturistYunis and Masi criteria for children

Dr Mehran Rezvani pain fellowship anesthesiologist & acupuncturist

Dr Mehran Rezvani pain fellowship anesthesiologist & acupuncturistAnother set of criteria for the clinical diagnosis of fibromyalgia that do not require a tender point examination was approved by the ACR in 2010 These criteria, which require a widespread pain index >7 and a score of >5 on a symptom severity (SS) scale, recognize the limitations of the 1990 ACR criteria and present a simple but preliminary case definition that was able to correctly classify 88 percent of cases identified by the ACR classification criteria.

These criteria have not been tested in childrenDr Mehran Rezvani pain fellowship anesthesiologist & acupuncturist

Dr Mehran Rezvani pain fellowship anesthesiologist & acupuncturistEPIDEMIOLOGYDr Mehran Rezvani pain fellowship anesthesiologist & acupuncturistLimited data suggest that the prevalence of fibromyalgia syndrome in children and adolescents is between 1 and 2 percent

The age of onset of fibromyalgia is typically in the early adolescent years. Several studies have noted a mean age of onset ranging from 11.5 to 15 yearsIn one study:

Patients with fibromyalgia account for about 7 percent of all cases referred to tertiary pediatric rheumatology centers0 to 4 year 0.5 and 1 % (m & f) 5 to 9 year 1 and 1.4 %10 to 14 year 2 and 2.6 %15 to 19 year 3.5% and 6.2%Dr Mehran Rezvani pain fellowship anesthesiologist & acupuncturistETIOLOGYDr Mehran Rezvani pain fellowship anesthesiologist & acupuncturistAlthough the etiology of fibromyalgia remains unknown, it is currently considered to be a disorder of pain regulation, classified often under the term "central sensitization

Dr Mehran Rezvani pain fellowship anesthesiologist & acupuncturistThe increased incidence of fibromyalgia in first degree relatives of patients with this condition supports genetic susceptibility

Alterations in pain processing with increased levels of pain mediators and increased activation of pain-sensitive areas of the brain as detected by magnetic resonance imaging are observed in patients with fibromyalgiaDr Mehran Rezvani pain fellowship anesthesiologist & acupuncturistFibromyalgia is a disorder of pain regulation due to neuroendocrinologic changes in the central and peripheral nervous systems, which occurs in genetically susceptible individuals possibly in response to environmental triggersDr Mehran Rezvani pain fellowship anesthesiologist & acupuncturistNeurohumoral abnormalities are observed in the hypothalamic-pituitary-adrenal axis at baseline and after provocative testing in patients with fibromyalgia

Sleep and mood disturbances are noted in the majority of patients, suggesting underlying central nervous system dysfunction

Autonomic nervous system dysfunction (eg, orthostatic hypotension) is seen more frequentlyDr Mehran Rezvani pain fellowship anesthesiologist & acupuncturistinsulin tolerance test (ITT)The standard high dose ACTH test (250 mg) (HDT)low dose ACTH test (1mg) (LDT)

21CLINICAL FEATURESDr Mehran Rezvani pain fellowship anesthesiologist & acupuncturistIn a case series from the United States of 59 children22symptomsStudy 1 (59 children)Study 2 (45 children)Diffuse pain97%90%Headaches76%71%Sleep disturbances69%90%Stiffness29%53%Subjective joint swelling24%Fatigue20%62%Dr Mehran Rezvani pain fellowship anesthesiologist & acupuncturistPainDr Mehran Rezvani pain fellowship anesthesiologist & acupuncturistThe cardinal manifestation of fibromyalgia is diffuse musculoskeletal pain involving the arms, legs, back, and neck

Not all parts of the body may be affected simultaneously, but it is important to recognize that this is not a local phenomenon

Affected patients frequently say, "I hurt all over"Dr Mehran Rezvani pain fellowship anesthesiologist & acupuncturistThe pain may be described as an ache or an achy feeling, but terms as varied as "dull," "sharp, "shooting," "excruciating," and "burning" also are used by patients to describe the pain

It is usually constant and chronic, not intermittent

May vary in intensity

Dr Mehran Rezvani pain fellowship anesthesiologist & acupuncturistIt may wake patients from sleep, but as a rule, the disturbed sleep characteristic of fibromyalgia is not caused by pain

Although patients may describe joint pain and stiffness, they will also complain of pain and aching in the muscles, one characteristic that distinguishes fibromyalgia from arthritis.

Do not have swollen or inflamed joints on physical examination, although many will describe subjective perception of joint swellingDr Mehran Rezvani pain fellowship anesthesiologist & acupuncturistTender pointsDr Mehran Rezvani pain fellowship anesthesiologist & acupuncturistThese are highly localized areas of the body ("points") that are consistently sensitive to pressure

Palpation of these points in patients with fibromyalgia usually elicits pain causing a significant reaction in the patient, such as wincing and a withdrawal responseDr Mehran Rezvani pain fellowship anesthesiologist & acupuncturistPalpation of "control points should also be includedThese points should not be tender, or at least should be less tender

Patients are usually not aware of the presence of the tender points

Although a total of 24 tender points have been identified, only 18 have been found to be reliable for use in the ACR criteriaDr Mehran Rezvani pain fellowship anesthesiologist & acupuncturist

Dr Mehran Rezvani pain fellowship anesthesiologist & acupuncturistThe presence of tender points in the absence of chronic pain does not appear to be predictive of later development of fibromyalgia in children

The exact number of tender points present in a child with possible fibromyalgia is probably not as important in making the diagnosis of juvenile fibromyalgia as whether the child's constellation of symptoms is consistent with the diagnosisDr Mehran Rezvani pain fellowship anesthesiologist & acupuncturistFatigueDr Mehran Rezvani pain fellowship anesthesiologist & acupuncturistFatigue is a common and key complaint in children with fibromyalgia, which is similar to adults with this disorder

Yunus found that 91% fatigue The prevalence of fatigue in other series 20 % to 80 to 100 %Dr Mehran Rezvani pain fellowship anesthesiologist & acupuncturistHeadacheDr Mehran Rezvani pain fellowship anesthesiologist & acupuncturistFrequent headaches occur in a majority of patients with fibromyalgia

The reported incidence ranges from 55 to 80 percent of patientsDr Mehran Rezvani pain fellowship anesthesiologist & acupuncturistSleep Disturbance

Dr Mehran Rezvani pain fellowship anesthesiologist & acupuncturistDisturbed or interrupted sleep occurs in at least two-thirds of children

Most often describe difficulty in falling asleep (often take an hour or more to fall asleep)

Even when they do fall asleep, many have difficulty maintaining sleep and wake up during the nightDr Mehran Rezvani pain fellowship anesthesiologist & acupuncturistThere may be overlap with restless legs syndrome

Nonrestorative sleep is a common complaint in children with fibromyalgiaDr Mehran Rezvani pain fellowship anesthesiologist & acupuncturistPsychosocial impactDr Mehran Rezvani pain fellowship anesthesiologist & acupuncturistSleep during the day after not being able to sleep at night

School attendance is often inconsistent or poor or the child is switched to home schooling

Dietary habits may deteriorate, and many patients fail to participate in any form of exercise and become deconditionedDr Mehran Rezvani pain fellowship anesthesiologist & acupuncturistOther symptomsDr Mehran Rezvani pain fellowship anesthesiologist & acupuncturistNumbnessStiffness, and a subjective sense of soft tissue swelling

Feeling cognitively impaired (eg, perception that they are in a "fog")

IBS (which may be quite severe)

Dizziness

Joint hypermobilityDr Mehran Rezvani pain fellowship anesthesiologist & acupuncturistComorbid Neuropsychiatric ConditionsDr Mehran Rezvani pain fellowship anesthesiologist & acupuncturistIt would not be surprising that these conditions are moreprevalent in children with fibromyalgia if the underlying pathogenesis of fibromyalgia is indeed related toneuroendocrine changes in the central and peripheral nervous systems in response to environmentaltriggers in genetically susceptible individuals44Psychiatric comorbidityDepression AnxietyMood, and Behavioral Disorders

Temperamental instability and increased vulnerability to stress

Sleep disturbances Sleep and periodic limb movementsRestless sleepNocturnal arousalsDr Mehran Rezvani pain fellowship anesthesiologist & acupuncturistTemperamental : 45Depression is common among patients with fibromyalgiaStudies in children have noted a prevalence of depression ranging from as little as 7 percent, when depression was defined by the need for treatment by a psychiatrist

55 to 61 percent when less stringent criteria were employedDr Mehran Rezvani pain fellowship anesthesiologist & acupuncturist22 percent and 41 percent of patients with juvenile fibromyalgia hadcurrent or lifetime diagnoses of depressive mood disorders, respectively46DIAGNOSISDr Mehran Rezvani pain fellowship anesthesiologist & acupuncturistA history of Chronic generalized pain Associated features: Fatigue Sleep disturbances Headache Irritable bowel syndrome Depression

Dr Mehran Rezvani pain fellowship anesthesiologist & acupuncturistPhysical examination Excludes other diagnoses, such as infections , connective tissue disorders or neurologic conditions

Arthritis and other autoimmune disorders may coexist with so-called "secondary" fibromyalgia but these disorders must be ruled out prior to making a diagnosis of primary fibromyalgiaDr Mehran Rezvani pain fellowship anesthesiologist & acupuncturistMeasurement of tender pointsApplying pressure equivalent to about 4 kg/cm2 to selected anatomic locations

Applying enough pressure to whiten the examiner's fingernail bed generates approximately 4 kg/cm2 of pressureDr Mehran Rezvani pain fellowship anesthesiologist & acupuncturistThe pressure should be applied gradually over a few seconds and to both right and left sides of the body

Control locations, such as over the thumbnail or the mid-forearm, should also be examined and should not be as tender as the predefined tender pointsDr Mehran Rezvani pain fellowship anesthesiologist & acupuncturistLaboratory tests and Imaging studiesNegative or normal conventional laboratory tests and imaging studies with the possible exception of polysomnography

A small percentage of patients may have a positive test for ANA

But this likely reflects the underlying presence of incidental positive ANA tests in the general populationDr Mehran Rezvani pain fellowship anesthesiologist & acupuncturistNormal results ofCBC U/A ANARFAcute phase reactantsSerum immunoglobulinsare Reassuring and supportive of the diagnosis of fibromyalgiaDr Mehran Rezvani pain fellowship anesthesiologist & acupuncturistDIFFERENTIAL DIAGNOSISDr Mehran Rezvani pain fellowship anesthesiologist & acupuncturistComplex regional pain syndrome type 1

Growing pains

Chronic fatigue syndrome

Rheumatic diseaseDr Mehran Rezvani pain fellowship anesthesiologist & acupuncturistComplex regional pain syndrome type 1Typically presents with moderate to severe pain that is usually localized to an extremityUsually accompanied by AllodyniaEdemaChanges in color or temperature

Dr Mehran Rezvani pain fellowship anesthesiologist & acupuncturist(CRPS; alsoknown as reflex sympathetic dystrophy, posttraumatic sympathetic dystrophy, and Sudeck's atrophy)56May be severe atrophy and trophic changes of the skin, hair and nails, and significant dysfunction of involved muscles and joints

Like fibromyalgia, CRPS occurs more frequently in females than males, and is much more common in adolescents

CRPS are easily distinguished from patients with fibromyalgia because they have localized pain and usually do not have tender points

Dr Mehran Rezvani pain fellowship anesthesiologist & acupuncturistIn addition, sleep disturbance, fatigue, headaches, and otherfeatures of fibromyalgia are not cardinal features of CRPS57Growing painsGrowing pains are common in children, but they are usually seen in school-aged children and not adolescents

Typically affect both legs and sometimes the arms and other areas of the body

Almost always occur at the end of an active day or in the middle of the night, and may wake a child at nightDr Mehran Rezvani pain fellowship anesthesiologist & acupuncturistThey have localized rather than diffuse pain; their pain is intermittent, only occurs at night, is self-limited rather than chronic and persistent, and is not characterized by tender points

Growing pains are also far more likely to be relieved by (NSAIDs)Dr Mehran Rezvani pain fellowship anesthesiologist & acupuncturistChronic fatigue syndrome

CFS is not a pain syndromeHowever, at times patients with CFS can have diffuse pain in addition to severe fatigue, indicating possible overlap between CFS and fibromyalgiaDr Mehran Rezvani pain fellowship anesthesiologist & acupuncturistRheumatic diseaseThe rheumatic disease most commonly confused with fibromyalgia is JIA (Juvenile idiopathic arthritis)

JIA has joint pain and objective signs of synovitis but may has not tenderness in muscles

Back pain is not common in most children with JIA ( although it canoccur in children with spondyloarthropathy)

Dr Mehran Rezvani pain fellowship anesthesiologist & acupuncturistAs a note of caution, fibromyalgia can occur or develop in children with rheumatic diseases such as JIA and SLE, so it is possible for patients to have bothDr Mehran Rezvani pain fellowship anesthesiologist & acupuncturistTreatmentDr Mehran Rezvani pain fellowship anesthesiologist & acupuncturistEDUCATIONDr Mehran Rezvani pain fellowship anesthesiologist & acupuncturistSleep regimen Because of the prevalence of sleep disturbances in these children, it is important to establish a routine sleep regimen with regular times for waking and going to bed

Caffeine should be avoided, especially from the late afternoon hours onward.Dr Mehran Rezvani pain fellowship anesthesiologist & acupuncturistSchool attendanceFor children who have avoided school, efforts should be directed towards a return to attending school on a regular basis

Full time school attendance with minimal accommodations is the ultimate goal, although for children with extended absenteeism, the process of achieving this may be a prolonged one

Dr Mehran Rezvani pain fellowship anesthesiologist & acupuncturistChange in expectationsWhile it is important that the patient and his/her family maintain hope and optimism, realistic goals will help avoid disappointment and frustration

Thus, at least initially, expectations should focus on improvements in sleep, pain, and daily functioning, which are usually achievable as opposed to the longer term goals of complete alleviation or resolution of symptomsDr Mehran Rezvani pain fellowship anesthesiologist & acupuncturistEXERCISEDr Mehran Rezvani pain fellowship anesthesiologist & acupuncturistIn adults with fibromyalgia, strong evidence shows that aerobic exercise has a beneficial effect, resulting in a sense of improved well-being, improved physical functioning, and a reduction in painand tender points

Preliminary data show similar outcomes in juvenile fibromyalgiaDr Mehran Rezvani pain fellowship anesthesiologist & acupuncturistPSYCHOLOGIC INTERVENTIONSDr Mehran Rezvani pain fellowship anesthesiologist & acupuncturistSeveral studies have demonstrated the beneficial effects of cognitive-behavioral therapy (CBT) in improving the symptoms of children with fibromyalgiaDr Mehran Rezvani pain fellowship anesthesiologist & acupuncturistIn one study of 67 children with fibromyalgia, cognitive-behavioral interventions (cognitive restructuring, thought stopping, distraction, relaxation, and self-reward) and patient/parent education(pain management, psychoeducation, sleep hygiene, and activities of daily living) reduced pain, somatic symptoms, anxiety, fatigue, and school absences, and improved sleep quality and reported functional ability in most patientsDr Mehran Rezvani pain fellowship anesthesiologist & acupuncturistPHARMACOLOGIC THERAPYDr Mehran Rezvani pain fellowship anesthesiologist & acupuncturistData on the use of medications in the treatment of juvenilefibromyalgia are limited

However, agents that have shown beneficial effects in adults also have been used in children, and are sometimes effectiveDr Mehran Rezvani pain fellowship anesthesiologist & acupuncturistAntidepressants

Tricyclic antidepressants (eg, Amitriptyline)

Serotonin-reuptake inhibitors (eg, Fluoxetine)

Serotonin-norepinephrine-reuptake inhibitors (eg, duloxetine and milnacipran)FDA approved : Duloxetine and MilnacipranDr Mehran Rezvani pain fellowship anesthesiologist & acupuncturistAmitriptylin : Usual Pediatric Dose for Pain1 to 12 years:Initial dose: 0.1 mg/kg orally at bedtime (investigational).Maintenance dose: May increase as tolerated over 2 to 3 weeks to 0.5 to 2 mg/kg at bedtime

Fluoxetine: 7 to 18 yearsInitial dose: 10 mg orally once a day, increased to 20 mg orally once a day after several weeks if insufficient clinical improvement is observed

milnacipranUsual Pediatric Dose for Fibromyalgia17 years and older:Dosing should be titrated according to the following schedule:Initial dose on day 1: 12.5 mg onceDays 2 and 3: 12.5 mg twice dailyDays 4 through 7: 25 mg twice dailyAfter day 7: 50 mg twice daily

75AnalgesicsNSAIDS and opioids, are ineffective in reducing pain in patients with fibromyalgia

There is some evidence, however, that the combined use of tramadol and acetaminophen may reduce pain in adults with fibromyalgiaDr Mehran Rezvani pain fellowship anesthesiologist & acupuncturistGABA agonistsIn randomized clinical trials, GABA agonists, such as Gabapentin and Pregabalin, help reduce pain in adults with fibromyalgia

Pregabalin was the first drug to be approved by the FDA for treatment of fibromyalgia BUT not recommended for below 18

Whether this drug will be shown to be safe and effective in children remains to be seenDr Mehran Rezvani pain fellowship anesthesiologist & acupuncturistLYRICA :Safety and efficacy not established