2020
DOI: 10.1182/hematology.2020000143
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Optimizing the management of chronic pain in sickle cell disease

Abstract: Chronic pain in sickle cell disease (SCD) refers to pain present on most days lasting over six months. It can start during childhood and the prevalence increases with age. By adulthood, over 55% of patients experience pain on over 50% of days; 29% reporting pain on 95% of days. The true prevalence of chronic pain in SCD is likely underappreciated as it is mostly managed at home. Patients with chronic pain and SCD frequently seek acute care for exacerbation of underlying chronic pain difficult to distinguish fr… Show more

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Cited by 27 publications
(23 citation statements)
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“…Relatively little research has been done evaluating NP in the setting of VOC, but our study supports the finding by Sigalla et al., which establishes an NP component to VOC in pediatric SCD patients 21 . Not only are patients in this group more likely to have NP during acute pain events, they are also more likely to be treated with opioids, which can trigger mechanisms of opioid hyperalgesia, opioid tolerance, and central sensitization 23–25 . These mechanistic sequelae can contribute to further enhancement of NP pathophysiology 1,11,21,24,26,27 .…”
Section: Discussionsupporting
confidence: 87%
See 1 more Smart Citation
“…Relatively little research has been done evaluating NP in the setting of VOC, but our study supports the finding by Sigalla et al., which establishes an NP component to VOC in pediatric SCD patients 21 . Not only are patients in this group more likely to have NP during acute pain events, they are also more likely to be treated with opioids, which can trigger mechanisms of opioid hyperalgesia, opioid tolerance, and central sensitization 23–25 . These mechanistic sequelae can contribute to further enhancement of NP pathophysiology 1,11,21,24,26,27 .…”
Section: Discussionsupporting
confidence: 87%
“…21 Not only are patients in this group more likely to have NP during acute pain events, they are also more likely to be treated with opioids, which can trigger mechanisms of opioid hyperalgesia, opioid tolerance, and central sensitization. [23][24][25] These mechanistic sequelae can contribute to further enhancement of NP pathophysiology. 1,11,21,24,26,27 When NP was evaluated by qualitative sensory testing in 24 children and adolescents at baseline and again during admission for pain, increased cold and mechanical pain sensitivity were found during pain crisis.…”
Section: Discussionmentioning
confidence: 99%
“…Although vaso-occlusive episodes (VOE) were included as an acute event in the final list of disease attributes, it is only one dimension of pain [ 52 ]. Chronic pain, which includes neuropathic pain and pain of other etiologies, can vary in intensity and frequency over time but is a near universal feature of SCD that significantly impacts health-related quality of life (HrQoL), medical and non-medical resource use [ 52 , 53 ]. To reflect this, we will model chronic pain separately from other chronic disorders.…”
Section: Resultsmentioning
confidence: 99%
“…Pain is the hallmark of SCD, and it accounts for most of the emergency room visits in patients living with the disease [ 21 ]. Those living with SCD describe their pain as unimaginable, agonizing, and sometimes impossible to describe [ 22 ]. Pain in SCD, acute or chronic, reduces the quality of life in several ways and is not limited to altered mood, irritability, depression, anxiety, and sleep disturbance [ 23 ].…”
Section: Discussionmentioning
confidence: 99%