Abstract

Neuropathic pain is associated with poor health-related quality of life (HRQL) in pain conditions other than sickle cell disease (SCD); this relationship in SCD is unknown. We investigated this relationship and hypothesized neuropathic pain is associated with poor HRQL in adolescents with SCD. We conducted a cross-sectional study of patients with SCD ages 13-18years during baseline health. Primary outcome was HRQL, assessed by the PedsQL SCD Module (child self-report, parent proxy report). PedsQL is scored from 0 to 100, with higher scores indicating better HRQL. Neuropathic pain was assessed using the painDETECT questionnaire (scored 0-38); higher scores indicated greater likelihood of neuropathic pain. All completed both PedsQL SCD Module and painDETECT questionnaire. Descriptive statistics were used and associations between painDETECT and PedsQL Total Score, Pain Impact, Pain and Hurt, and Pain Management and Control Scores were determined via Pearson correlation. Significance was P<.05. Twelve patients were enrolled. Median (interquartile range [IQR]) age was 15 (14-16.5) years, 75% were female, and 83% were on hydroxyurea. Higher painDETECT scores were significantly associated with lower PedsQL SCD Module child self-report Pain and Hurt Scores (r=-0.68, P=.01). Higher painDETECT scores were also significantly associated with lower PedsQL parent proxy-report Total Scores (r=-0.64, P=.03) and Pain and Hurt Scores (r=-0.67, P=.02). These data suggest that adolescents with SCD and neuropathic pain have poor HRQL even in their baseline state of health. Prospective, larger studies are needed to confirm this preliminary finding and explore a multimodal approach for pain assessment in SCD.

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