Date of Award

12-2019

Degree Name

Doctor of Philosophy (PhD)

Advisor(s)

CHARLES DARKOH

Second Advisor

RUSSELL E. WARE

Third Advisor

WENYAW CHAN

Abstract

Sickle cell anemia contributes substantially to childhood morbidity and mortality in sub-Saharan Africa, where there are scarce health resources and inadequate awareness among healthcare providers and the community. Without early diagnosis and initiation of preventive treatments, most infants will die of acute complications before their fifth birthday. Information on the mortality associated with sickle cell anemia is excluded from global statistical summaries due to the lack of accurate data, making sickle cell anemia an invisible killer of children on the African continent. Information from long-term large-scale sickle cell screening efforts is not yet available in Africa; therefore, a mixed-methods study was conducted using five years of data from Uganda’s national sickle cell screening program and from interviews with 23 sickle cell healthcare providers. This was achieved with the following objectives: (i) characterize the epidemiology of sickle cell disease in Uganda; (ii) evaluate the centralized sickle cell screening laboratory in Uganda; and (iii) describe healthcare providers’ experiences with sickle cell screening in Uganda. ix A total of 324,356 children were screened for sickle cell trait and disease from February 2014 to March 2019. A high national burden of sickle cell disease (0.9%) was confirmed. Among samples referred specifically for sickle cell testing, the overall prevalence of sickle cell disease was 9.7% and particularly elevated in high-burden districts where focused screening occurred. A large proportion of affected children were tested between 5-9 months of age, coincident with onset of disease signs and symptoms. With the use of crude birth rate data, a high screening coverage of newborns was observed several high-burden districts. Median turnaround time from sample collection to result reporting was 16 days (IQR 11, 24). Predictors affecting prolonged turnaround time were sample volume, health facility level, and testing month. Cost per test was $4.46 and cost per case detected was $483.74. Barriers to screening were identified, including the need for initial and ongoing training for healthcare providers on sickle cell screening and management; healthcare system capacity issues, such as resources of staff and supplies, and system fragmentation; and the knowledge and awareness gap of sickle cell disease in the community. Focused sickle cell screening has been a time- and cost-effective approach to begin to confront Uganda’s large burden of disease. However, as the adverse impact of sickle cell disease on the population becomes more fully realized, a shift toward earlier and more widespread screening through healthcare provider training, community education, and improved sickle cell care models will be most advantageous. The experiences in Uganda are instructive for all countries in sub-Saharan Africa with a large sickle cell burden.

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