Diagnosis patterns of sickle cell disease in Ghana: a secondary analysis using cohort data

Abstract Background: Despite having the highest prevalence of sickle cell disease (SCD), Sub-Saharan Africa lacks a robust screening program. We sought to capture the diagnosis patterns of SCD, particularly age and mechanism at SCD and age of first pain crisis, in Accra, Ghana. Methods We administered a survey to parents of offspring with SCD between 2009–2013 in Accra as a part of a larger cohort study and analyzed a subset of the data to determine diagnosis patterns. Univariate analyses were performed on diagnostic patterns; bivariate analyses were conducted to determine if patterns differ by offspring’s age, or their disease severity. Pearson’s chi-squared were calculated. Results Data was collected on 354 unique participants from parents. 44% were diagnosed with SCD by age four; 46% had experienced a pain crisis by the same age. 66% were diagnosed during pain crisis, either in acute (49%) or primary care (17%) settings. Younger (< 18 years) offspring were diagnosed with SCD at an earlier age (74% by four years old); in the adult ( > = 18 years) group, 30% were diagnosed by four years ( p  < 0.001). Few were diagnosed via newborn screen. Half with severe disease were diagnosed by age four, compared to 31% with mild disease ( p  = 0.009). Conclusions These findings reflect a reliance on diagnosis in acute settings, and a relative underutilization of systematic screening programs. By understanding current patterns, opportunities remain to more effectively detect and treat SCD in this high prevalence population.