A case‐control and seven‐year longitudinal neurocognitive study of adults with sickle cell disease in Ghana

Abstract

Sickle cell disease (SCD), an inherited blood disorder that is the most common genetic disorder worldwide, primarily affects people of African descent. [1][2][3] The SCD genetic polymorphism produces mutant haemoglobin molecules that polymerize within the erythrocyte during deoxygenation, resulting in sustained haemolytic anaemia and painful vaso-occlusive

Mind the gap: trajectory of cognitive development in young individuals with sickle cell disease: a cross-sectional study

“…This study was conducted prior to the introduction of disease-modifying therapies, e.g., hydroxyurea. A Ghanaian study in adults aged 18–50 years with follow-up data after 7 years found a significant decline in verbal, non-verbal and global PIQ on the Revised Quick Cognitive Screening Test, as well as significant decreases in visuospatial abilities, processing speed, and EF ( 28 ). The rarity of these types of longitudinal evaluations in high and low resource countries is in part due to the logistical difficulties and expense.…”

Commentary on the ASH 2020 guidelines on cognitive screening and intervention in sickle cell disease

“…Individuals with sickle cell disease (SCD) experience progressive neurocognitive dysfunction resulting from cerebrovascular disease 1 , 2 and environmental disparities. 1 , 3 , 4 In 2020, the American Society of Hematology (ASH) published guidelines outlining the standard care for the prevention, diagnosis, and treatment of cerebrovascular disease in SCD, based on a systematic review of the literature and expert panel consensus. 5 Recommendations 8 and 9 describe guidance pertaining to screening and interventions to address cognitive deficits, respectively.…”