Trends in sickle cell trait and disease screening in the Republic of Uganda, 2014–2019

Abstract

objective Sickle cell disease is an important public health issue that is increasingly recognised as a substantial contributor to morbidity and early childhood mortality in sub-Saharan Africa. We aimed to provide information from large-scale, long-term sickle cell screening efforts in Africa. methods We used nationally representative data from the centralised public health laboratory database in Uganda to examine epidemiological trends in sickle cell screening over a five-year period, comparing age and geographic adjustments to prevalence among different testing cohorts of children aged 0-24 months, and calculating screening coverage within high-burden districts. results A total of 324 356 children aged 0-24 months 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 a cohort of samples co-tested with HIV. In the cohort of 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. The majority of children were screened before age 4 months, but the sickle-specific cohort had a larger proportion of affected children tested between age 5-9 months, coincident with onset of disease signs and symptoms. Successful screening coverage of sickle cell disease births was achieved in several high-burden districts. conclusions Examination and analysis of national sickle cell screening trends in Uganda documents the successes of focused screening strategies as an important step towards universal screening. With this evidence and increased healthcare provider knowledge, Uganda can optimise sickle cell diagnosis and management across the country.

A predictive algorithm for identifying children with sickle cell anemia among children admitted to hospital with severe anemia in Africa

“…A pilot national screening programme, organized through the Uganda Central Public Health Laboratories was established in 2014, which was initially focused on the repurposing of dried blood spot samples collected to screen for the mother-to-child transmission of HIV. 18 , 26 , 27 More recently, new approaches to early detection are starting to become available, including a number of promising rapid diagnostic methods. 28 While such approaches show great promise, for the time being there is still a need for alternative approaches to opportunistic testing.…”

“…While newborn screening would clearly be the best approach and considerable progress in this regard has been made in Uganda during recent years. A pilot national screening programme, organized through the Uganda Central Public Health Laboratories was established in 2014, which was initially focused on the repurposing of dried blood spot samples collected to screen for the mother‐to‐child transmission of HIV 18,26,27 . More recently, new approaches to early detection are starting to become available, including a number of promising rapid diagnostic methods 28 .…”

Sickle Cell Disease in Children and Adolescents: A Review of the Historical, Clinical, and Public Health Perspective of Sub-Saharan Africa and Beyond

“…Universal NBS has not been widely implemented even in Ghana [ 17 ] and Benin [ 59 ] where substantial progress in NBS research capacity and follow-up has been documented since the early 90s. In Uganda, screening for SCD is focused on children aged 24 months or less, and in districts with the highest burden of SCD [ 18 ]. Certainly, we need to acknowledge the numerous limitations to the expansion of the program in SSA [ 62 ].…”

Operational analysis of the national sickle cell screening programme in the Republic of Uganda

“…The sickle specific testing cohort included all samples that had a sickle cell result only. 15 http://www.ajlmonline.org Open Access…”