Neuropathic Pain in Children with Sickle Cell Disease: The Hidden Side of the Vaso-Occlusive Crisis

Abstract

The majority of hospitalizations of patients with sickle cell disease (SCD) are related to painful vaso-occlusive crises (VOCs). Although the pain of VOC is classically nociceptive, neuropathic pain (NP) has also been demonstrated in SCD patients. The aim of our study is to specify the prevalence of NP during VOCs in SCD children using a dedicated scale and to measure its characteristics. We performed a prospective study that included SCD children hospitalized for an acute VOC. The presence of NP was sought with the DN4 scale on the second and fourth days of hospitalization. A total of 54 SCD children were included in the study. Overall, 41% of the patients (n = 22) experienced neuropathic pain during the VOC, mostly at an early stage (Day 2). The median age, the sex ratio, the location of the pain, and the morphine consumption were similar for patients with and without NP. Our study shows that neuropathic pain is very common during VOCs in SCD children. The absence of identified risk factors should prompt us to be vigilant regardless of the patient’s age, sex, and clinical presentation.

Prevalence of neuropathic pain in adolescents with sickle cell disease: A single‐center experience

“…Aligned with this limitation, we are currently pursuing an institutional initiative, which will address improving screening and evaluation for NP component during inpatient admissions for SCD‐related pain. 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 .…”

“…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 . 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 28 .…”

“…Our study uniquely investigated the NP scoring concurrently in the inpatient and outpatient settings and suggested that outpatient and inpatient populations behave differently with respect to painDETECT scores. In general, studies on NP in patients with SCD have focused either on testing for NP in the inpatient setting during admissions for pain 21 or in the outpatient setting at baseline. 5,8,10,20,22 Our study is uniquely positioned to evaluate the population in both settings, based on the occurrence of admissions for pain for the duration of the study and the population of patients tested at their baselines in the clinic setting during routine visits.…”

“…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][24][25] These mechanistic sequelae can contribute to further enhancement of NP pathophysiology.…”

“…[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. 28 Further examination of NP with clinical tools (painDETECT) and qualitative sensory testing evaluations concurrently would contribute to better characterization of NP in adolescents with SCD-related acute and/or chronic NP.…”