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Pain in sickle cell disease 

Pain in sickle cell disease
Chapter:
Pain in sickle cell disease
Author(s):

Carlton Dampier

and Lamia Barakat

DOI:
10.1093/med/9780199642656.003.0025
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date: 18 June 2019

Sickle cell disease (SCD) presents a complex pain disorder to clinicians. Pain from vaso-occlusion of sickle erythrocytes can occur in multiple musculoskeletal locations, several internal viscera such as the spleen, as well as the penis. Such pain is typically intermittent in childhood, shares features of acute pain with other pain disorders, and often responds to non-steroidal anti-inflammatory drugs (NSAIDs) and opioid analgesics. Adolescents with SCD often experience more frequent pain, and those with bone disease in spine, hips, or shoulders may experience chronic pain. Like other chronic pain disorders, this pain often responds poorly to opioids, but there is limited current clinical or research data to support alternative medications. Many cognitive-behavioural strategies are helpful as part of multidisciplinary pain management, particularly in adolescents, who may also benefit from psychological support to treat coexistent mood disorders, to increase coping skills, and to support appropriate school and family functioning. Future advances in pharmacological and psychological therapies are needed to ameliorate the substantial burden of pain in children and adolescents with SCD.

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