Adherence to hydroxyurea, health-related quality of life domains, and patients' perceptions of sickle cell disease and hydroxyurea: a cross-sectional study in adolescents and young adults

Badawy, S. M.; Thompson, A. A.; Lai, J. S.; Penedo, F. J.; Rychlik, K.; Liem, R. I.

Health Qual Life Outcomes. 2017 Jul 7; 15(1):136


BACKGROUND: Sickle cell disease (SCD) patients have impaired domains of health-related quality of life (HRQOL). Hydroxyurea is safe and efficacious in SCD; however, adherence is suboptimal, and patients' perceptions are poorly understood amongst adolescents and young adults (AYA). Study objectives were to: (1) examine patients' perceptions of SCD and hydroxyurea; and (2) explore the relationship of their perceptions to clinical characteristics, HRQOL domains and hydroxyurea adherence. METHODS: Thirty-four SCD patients on hydroxyurea (>/=6 months) participated in a single-institution study. Study measures included Brief-Illness Perceptions Questionnaire, (c)Modified Morisky Adherence Scale 8-items, and Patient Reported Outcomes Measurement Information System (PROMIS(R)). We assessed the relationship of patients' perceptions to hydroxyurea adherence using Wilcoxon rank-sum test, the number of hospitalizations using Kruskal-Wallis test, and the number of ED visits, adherence level, HRQOL domain scores using Spearman's rho correlations. We conducted a sub-analysis in HbSS patients to evaluate the relationship of patients' perceptions to laboratory markers of hydroxyurea adherence. RESULTS: Participants were 59% male and 91% Black, and had a median age of 13.5 (range 12-18) years. Participants with >/=4 hospitalizations over 1-year prior (using electronic medical chart review) reported more negative perceptions of SCD-related symptoms and emotional response, and perceived hydroxyurea as less beneficial; all p-values

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