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Paeds Casesrheumatology-musculoskeletal-and-sports

Paeds Cases · rheumatology-musculoskeletal-and-sports

Pain amplification, juvenile fibromyalgia and complex regional pain syndrome: Case

Clinical long case of a thirteen-year-old girl with juvenile fibromyalgia and her fourteen-year-old sister with complex regional pain syndrome, covering the central sensitization mechanism, the 2010 American College of Rheumatology criteria, the Budapest clinical criteria and the multidisciplinary management.

paediatric rheumatology long case
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Target exams

RACP DCEMRCPCH ClinicalRCPSC Pediatrics

Target exams

RACP DCEMRCPCH ClinicalRCPSC Pediatrics
Prompt
A thirteen-year-old girl presents with widespread musculoskeletal pain for nine months affecting her neck, back, shoulders and legs, profound fatigue that is not relieved by rest, and unrefreshing sleep with morning unrefreshment. She has headaches and intermittent abdominal pain. She has attended school only intermittently for three months. Her full blood count, C-reactive protein, erythrocyte sedimentation rate, creatine kinase, thyroid function and vitamin D are normal. Her Widespread Pain Index is nine and her Symptom Severity Scale is seven. She scores her pain at eight out of ten. Her mother, who has chronic back pain, is very distressed and convinced the child has a serious undiagnosed illness. The task is to confirm the diagnosis, address the family's concerns, and build the multidisciplinary management plan.

Framing the case

This thirteen-year-old girl with the widespread pain, the fatigue, the unrefreshing sleep and the school absence, whose investigations are normal and whose Widespread Pain Index is nine, has the classic juvenile fibromyalgia by the 2010 American College of Rheumatology criteria. The framework that organises the case is the central sensitization, the amplified processing of the nociceptive signals in the nervous system, and the management is the multidisciplinary rehabilitation that addresses the pain, the function, the sleep, the mood and the family. [3][7]

The diagnosis and the classification

The diagnosis is made by the 2010 American College of Rheumatology preliminary diagnostic criteria, validated in the adolescents by Ting and colleagues in 2016. The criteria require the symptoms for at least three months and no other disorder explaining the pain, plus a Widespread Pain Index of seven or more with a Symptom Severity Scale of five or more, or a Widespread Pain Index of three to six with a Symptom Severity Scale of nine or more. This girl meets the first alternative with the Widespread Pain Index of nine and the Symptom Severity Scale of seven. The fatigue, the unrefreshing sleep, the headaches and the abdominal pain are the central features, and the normal blood tests confirm that no other disorder explains the pain. [1][3]

The exclusion of the red flags

The inflammatory and the malignant disease are excluded before the amplified pain label is applied. The juvenile idiopathic arthritis is excluded by the absence of the morning stiffness and the objective synovitis and the normal inflammatory markers. The leukaemia is excluded by the normal full blood count and the absence of the cytopenias and the systemic features. The thyroid disease, the vitamin D deficiency and the anaemia are excluded by the first-line panel. The absence of the objective synovitis, the persistent fever, the weight loss, the night pain and the neurological deficit confirms that the primary amplified pain diagnosis is appropriate. [3][7]

The central sensitization mechanism for the family

The explanation of the central sensitization is the first and the most powerful treatment for this family. The mother, who has the chronic back pain and the conviction that the child has a serious undiagnosed illness, is the key to the engagement. The explanation validates the pain as real, explains that the pain is generated by the amplified nervous system rather than the ongoing tissue damage, and reassures that the movement is safe. The parental catastrophizing is the modifiable driver of the child functional disability, and the family intervention that addresses the maternal response to the pain is the essential component. [8]

The multidisciplinary management plan

The management is the multidisciplinary rehabilitation built on the four pillars. The education on the central sensitization, the graded aerobic exercise begun at the level she can tolerate and increased steadily through the pain, the cognitive behavioural therapy that addresses the catastrophizing and the fear and the anxiety, and the sleep hygiene with the regular schedule and the reduction of the screen time. The low-dose amitriptyline at around zero point three to zero point five milligrams per kilogram at night is the adjunct for the sleep. [3][6]

The prolonged school absence is the predictor of the poor outcome

This girl has attended the school only intermittently for three months, and the prolonged school absence is the predictor of the poor outcome and the chronic disability. The school reintegration is the treatment and not the reward for the recovery, and the plan for the gradual return is built early. The family is counselled that the child who waits for the pain to resolve before the return to the school waits indefinitely, and the functional goals replace the pain intensity goals.

[7][8]

The school reintegration and the functional goals

The school is contacted early, the modified programme is negotiated, and the plan for the gradual return is built with the clear functional milestones. The return to the full attendance, the social activities and the sport are the goals, and the pain intensity score is the secondary measure that is expected to fall as the function recovers. The multidisciplinary team of the rheumatologist, the physiotherapist, the psychologist, the nurse and the school liaison is assembled around the family, and the coordinated plan is delivered. [7]

The escalation if refractory

If this girl does not respond to the outpatient management and the school absence remains prolonged, the intensive functional restoration programme is the escalation. The Sherry model delivers the daily physiotherapy, the psychology and the schooling over several weeks, and it achieves the return to the full function in the great majority of the children. The opioids and the surgery are avoided, because they are ineffective for the central sensitization and they add the harm. [7]

Communication and the family

The family is counselled honestly on the juvenile fibromyalgia, the central sensitization mechanism and the biopsychosocial model. The mother's distress and her conviction of the undiagnosed illness are addressed with the validation and the education, and the family intervention targets the parental catastrophizing. The child is prepared in the age-appropriate way, and the self-management skills are taught. The long-term plan includes the relapse management, the comorbidity screening and the transition to the adult care in the adolescence. [8]

The explanation of the central sensitization is the first treatment

This girl and her mother who understand that the pain is real, that it is generated by the amplified nervous system, that the movement is safe and that the recovery comes through the function are the family who engage with the rehabilitation. The explanation, given with the validation of the pain and the optimism for the recovery, unlocks the willingness to move and to return to the school. The candidate who carries this principle demonstrates the understanding that the management of the amplified pain begins with the words.

[3][7]

References

  1. [1]Ting TV, Barnett K, Zelikovsky N, et al 2010 American College of Rheumatology Adult Fibromyalgia Criteria for Use in an Adolescent Female Population with Juvenile Fibromyalgia J Pediatr, 2016.PMID 26545727
  2. [3]Kashikar-Zuck S, Ting TV Juvenile fibromyalgia: current status of research and future developments Nat Rev Rheumatol, 2014.PMID 24275966
  3. [6]Eccleston C, Palermo TM, Williams AC, Lewandowski A, Morley S Psychological therapies for the management of chronic and recurrent pain in children and adolescents Cochrane Database Syst Rev, 2012.PMID 23235601
  4. [7]Sherry DD, Sonagra M, Milojevic D The spectrum of pediatric amplified musculoskeletal pain syndrome Pediatr Rheumatol Online J, 2020.PMID 33046102
  5. [8]Dougherty BL, Zelikovsky N, Schurman JV, Williams SE Longitudinal Impact of Parental Catastrophizing on Child Functional Disability in Pediatric Amplified Pain J Pediatr Psychol, 2021.PMID 33491076