• Sherif M Badawy, Leonardo Barrera, Stephanie Cai, and Alexis A Thompson.
• Department of Pediatrics, Northwestern University Feinberg School of Medicine, Chicago, IL, USA.
• Biomed Res Int. 2018 Jan 1; 2018: 8296139.

BackgroundSickle cell disease (SCD) is a chronic debilitating illness. SCD-related complications result in substantial impairment in quality of life (QOL). Our study objective was to assess the relationship of participants' characteristics, QOL, hydroxyurea adherence, and SCD-related clinical outcomes in youth with SCD.ProcedureA single-center cross-sectional study. Thirty-four youth with SCD enrolled from clinic between January and December 2015. Participants completed PROMIS® measures and ©Modified Morisky Adherence Scale.ResultsParticipants had a mean age of 14.8 (SD 2.9) years and 41% were female. Participants' age correlated with fatigue (rs=0.48; P=0.006), pain (rs=0.32; P=0.07), and anxiety (rs=0.33; P=0.06) scores. Participants with chronic pain had worse upper extremity physical function (P=0.05), pain (P=0.04), anxiety (P=0.05), and depression (P=0.05). Males reported significantly higher hydroxyurea adherence (5.4 versus 3.6, P=0.02) compared to females. Participants with chronic pain had more frequent hospitalizations (P=0.02), emergency room visits (P=0.04), and longer total length of hospital stays over 12-month period (P=0.01).ConclusionsOlder and female participants had worse QOL scores, and males reported higher hydroxyurea adherence. Participants with chronic pain reported significant impairment in different QOL domains and had increased healthcare utilization. Future longitudinal studies examining the relationship between participants' characteristics, QOL, hydroxyurea adherence, and SCD-related clinical outcomes are needed.

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