Abstract

Objectives

To assess the health-related quality of life (HRQoL) of children with sickle cell anemia (SCA) in Uganda and Malawi during steady disease states and sick attacks and to examine the impact of malaria chemoprevention with weekly dihydroartemisinin-piperaquine compared to monthly sulfadoxine-pyrimethamine.

Methods

This cohort study was nested within a clinical trial comparing weekly dihydroartemisinin-piperaquine with the standard of care (monthly sulfadoxine-pyrimethamine) among children with SCA. HRQoL was assessed using EQ-5D tools during steady states and sick attacks. Ordinary least squares regression identified factors associated with HRQoL.

Results

A total of 633 children with SCA were enrolled (mean age 8.1 years, standard deviation [SD] 3.7). HRQoL was higher during steady states but deteriorated during sick attacks, with the most problems reported in pain and discomfort. Older children (ages 12-16: mean difference [MD] = 0.09, P children hospitalized 1 to 3 times (MD = −0.27, P = .01) or ≥4 times (MD = −0.24, P = .02) had substantially lower HRQoL. There were no HRQoL differences between treatment arms.

Conclusions

The HRQoL of children with SCA was relatively high during steady states but declined substantially during sick attacks, especially because of severe pain and discomfort. HRQoL is influenced by a child’s age and frequency of hospitalization. Results provide information for calculation of quality-adjusted life years for future economic evaluation.