Title: Quality of Life in Latino and Non-Latino Youth aged 8-18 Years with Sickle Cell Disease
Description: While sickle cell disease (SCD) primarily affects those of African heritage, Latinos, the second most commonly affected group, are often not included in studies of youth with SCD. The purpose of this mixed methods study was to complete the linguistic translation validation of the PedsQL SCD Module, a recently validated disease specific quality of life (QOL) instrument, for use in Spanish speaking parents and youth with SCD (Aim 1). Using this instrument, QOL of Latino and African American youth with SCD who participated in an NIH funded study to improve adherence to hydroxyurea therapy (R21 NR013745) were compared (Aim 2) and factors associated with QOL examined (Aim 3). For Aim 1, 10 Latino youth with SCD (n = 5 age, 8-12 years; n = 5 age, 13-18 years) and their parents completed a demographic survey, Spanish version of PedsQL SCD Module and an audio-taped cognitive interview. Across age groups, all reported that the translated PedsQL Sickle Cell Disease Module was easy to understand and had minimal suggestions for further improvement.

For Aims 2 and 3, secondary baseline data from 28 youth (mean age 13.6  2.4 years) with sickle cell disease and their parents who participated in the HABIT feasibility trial were analyzed using descriptive statistics, Wilcoxon Signed Rank and Mann-Whitney test, and linear regression modeling. Latino youth reported higher QOL scores than non-Latino youth for all QOL measures except for the Worry II subscale of the disease-specific QOL measure while Latino parents reported higher QOL scores than non-Latino parents for all subscales except for three: the disease-specific Worry I, Worry II, and Communication I subscales. Poorer disease specific QOL was predicted by greater youth-parent discordance regarding sickle cell disease responsibility for parents (β = -3.07, p = 0.04) but not youth. Poorer disease-specific QOL was predicted by greater number of both emergency room visits during the prior year for both youth (β = -2.89, p = 0.005 [self-report]; β = -5.07, p = 0.002 [electronic medical records]) and parents (β = -3.41, p = 0.002 [self-report]; β = -6.93, p = <0.001 [electronic medical records]) and hospitalizations during the prior year (youth β = -5.72, p = <0.001 [self-report]; β = -7.56, p = 0.03 [electronic medical records]; parents β = -6.48, p = <0.001 [self-report]; β = -9.16, p = 0.02 [electronic medical record]).

Based on these findings, greater youth-parent discordance regarding sickle cell family responsibility and greater utilization of emergency rooms and/or hospitals were associated with poorer disease-specific QOL.