Healthcare access inequalities in Quilombola rural and riverside communities in the Brazilian Amazon: Associations with social, organizational, and territorial factors.

Disease-related neurodevelopmental and cognitive issues are common among children with sickle cell disease (SCD) and have lasting effects on academic, occupational, health, and quality of life outcomes. SCD is known to exert diffuse effects on cognition, with patients demonstrating poorer performance on tests of intelligence (IQ) and subcomponent abilities including verbal reasoning, visual-spatial and fluid reasoning, executive functioning, and processing speed relative to healthy peers and unaffected siblings. However, deficits tend to be most pronounced in executive functions (i.e., attentional control, working memory) due to the predominance of cerebral infarcts that occur in the frontal cortex. Children with SCD with neurodevelopmental disorders and cognitive difficulties are more likely to exhibit problems with academic achievement, experience grade retention, and need academic modifications to instruction. Cognitive issues also limit one’s ability to obtain employment as an adult, particularly among individuals with SCD who do not graduate from high school. Beyond effects on academic outcomes and employment, cognitive issues also have implications for health and well-being. Youth with SCD with cognitive deficits demonstrate poorer medication adherence, higher rates of pain-related emergency department visits and hospitalization, less successful transitions to adult healthcare, and lower health-related quality of life. As a result, it is critical to monitor for neurodevelopmental and cognitive issues in order to connect patients to early intervention services, advocate for academic supports, and counsel patients and families about disease-modifying and potentially curative therapies. The American Society of Hematology’s (ASH) 2020 guidelines on the prevention, diagnosis, and treatment of cerebrovascular disease in SCD recommend that clinicians conduct regular surveillance using simplified signaling questions to monitor for concerns about neurodevelopmental disorders and cognitive deficits. More formal screening by a psychologist or qualified provider was recommended for patients with abnormal surveillance results. While a few studies have described strategies for cognitive screening in pediatric SCD, there is a dearth of guidance on conducting surveillance using signaling questions. The ASH guidelines’ authors provided examples of signaling questions that may be used; however, empirical research characterizing the sensitivity and specificity of these questions is lacking. As a result, there is uncertainty about the degree to which the proposed signaling questions yield reliable estimates of cognitive impairment and whether the same signaling questions can be used across childhood or whether developmental considerations affect the questions’ performance.