Sickle-Cell Disease and Stroke: Quality of Life of Patients in a Chronic Transfusion Regimen from the Caregivers' Perspective

Strokes affect up to 10% of children with sickle-cell disease (SCD). The most commonly used strategy to prevent a first-time stroke or its recurrence is to perform periodic red blood cell transfusions. This article aims to evaluate the quality of life (QoL) of children and adolescents with SCD undergoing a chronic transfusion regimen (CTR) for stroke prophylaxis, according to their caregivers' perception. A cross-sectional study was conducted using a sociodemographic interview with an application of a validated instrument (Pediatric Quality of Life Inventory) involving 16 caregivers of patients with SCD aged <18 years undergoing CTR in a reference center. The data were processed using STATA version 13.0. The caregivers were predominantly the mothers of the minors that were part of the study cohort (87.5%), an income of <2 minimum wages (81.2% of cases) and >8 years of schooling (56.2%). The patients had a mean age of 10.4 years, 68.8% were male, 75% were mixed-race and came from small towns and rural areas (68.8%). The overall mean QoL was 45.8 (95% confidence interval [CI] 42.5-49.2). Female patients and those aged <12 years had lower levels of overall QoL. The emotional dimension of the children was the least compromised as per the caregivers' perception. The mean QoL of children with SCD on a CTR is lower than the estimated global mean QoL reported in the literature. It is possible that the occurrence of a stroke enhances the caregivers' negative perceptions about the QoL of patients with SCD.