Abstract

Purpose: Sickle cell disease (SCD) potentially causes numerous acute and chronic complications across the lifespan. The majority of patients with SCD live in sub-Saharan Africa and 1 in 50 Ghanaian newborns are affected. Foetal hemoglobin (Hb F) level is a known modulator of the severity of SCD. High levels of Hb F inhibit sickling and correlate with reduced disease morbidity and mortality. Hb F monitoring is also used to assess adherence and response to disease modifying (hydroxyurea) therapy. Standard hemoglobin electrophoretic techniques are unable to quantify Hb F. In resource-limited settings like Ghana, the use of high performance liquid chromatography (HPLC) for Hb F quantification is often challenging due to high cost and unavailability of laboratory equipment and skilled technicians. We report on the performance of a low-cost, point-of-care, microchip based cellulose acetate electrophoresis “Gazelle” compared to HPLC for Hb F quantification in Ghanaian children with SCD. GazelleTM is a fast (<8 minutes), easy-to-use test which can be performed by minimally trained personnel using only a finger-prick volume of blood. Materials and methods: The study was conducted at the paediatric SCD Clinic in Korle Bu Teaching Hospital, Accra, Ghana. Children (age ≥ 1 year to 16 years) known to have sickle cell disease who are on hydroxyurea therapy, and followed at the paediatric sickle cell clinic were enrolled in the study. The study is still ongoing. Results: A total of 110 children were included in the analysis. Gazelle showed a correlation of 0.93 when compared to HPLC (Figure 1). Conclusion: Haemoglobin variants are quantified by Gazelle offering the ability to monitor disease modifying therapy in SCD such as hydroxyurea, where quantification of HbF levels is the key to assessing treatment adherence and determining response to therapy. Gazelle has the potential to be utilized as a POC test for quantification of foetal haemoglobin (Hb F) levels in monitoring hydroxyurea therapy for children with SCD.Hb Quantification Performance of Gazelle compared to HPLC C. SEGBEFIA declares a conflict of interest: Consultancy, Expert: Novartis - Advisory Board / Steering Committee Member Global Blood Therapeutics - DSMB Member P. THOTA declares a conflict of interest: Other: Employee of Hemex Health

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