Organ damage in sickle cell disease study (ORDISS): protocol for a longitudinal cohort study based in Ghana

Abstract

IntroductionSickle cell disease is highly prevalent in Africa with a significant public health burden. Nonetheless, morbidity and mortality in sickle cell disease that result from the progression of organ damage is not well understood. The Organ Damage in Sickle Cell Disease Study (ORDISS) is designed as a longitudinal cohort study to provide critical insight into cellular and molecular pathogenesis of chronic organ damage for the development of future innovative treatment.Methods and analysisORDISS aims to recruit children aged 0–15 years who attend the Kumasi Centre for Sickle Cell Disease based at the Komfo Anokye Teaching Hospital in Kumasi, Ghana. Consent is obtained to collect blood and urine samples from the children during specified clinic visits and hospitalisations for acute events, to identify candidate and genetic markers of specific organ dysfunction and end-organ damage, over a 3 year period. In addition, data concerning clinical history and complications associated with sickle cell disease are collected. Samples are stored in biorepositories and analysed at the Kumasi Centre for Collaborative Research in Tropical Medicine, Ghana and the Centre for Translational and International Haematology, University of Pittsburgh, USA. Appropriate statistical analyses will be performed on the data acquired.Ethics and disseminationResearch ethics approval was obtained at all participating sites. Results of the study will be submitted for publication in peer-reviewed journals, and the key findings presented at national and international conferences.

The SickleGenAfrica Network

“…We sought to address this knowledge gap by leveraging a longitudinal cohort study of sickle cell disease in Ghana called ORDISS to develop a continentwide genomics network, when the second phase funding of H3Africa was announced in late 2016. 5 We launched the Sickle Cell Disease Genomics of Africa (SickleGenAfrica) Network in April 2018 in Accra, Ghana, with a vision of aligning the survival of individuals who have sickle cell disease in Africa with national norms. The network is organised into three research projects and multiple cores working from universities and hospitals in Ghana, Nigeria, Tanzania, and South Africa in collaboration with the University of Pittsburgh, to test the overarching hypothesis that genetic variation affects the body's defence against intravascular haemolysis and the development of organ damage in sickle cell disease (appendix p 2).…”

“…We sought to address this knowledge gap by leveraging a longitudinal cohort study of sickle cell disease in Ghana called ORDISS to develop a continent-wide genomics network, when the second phase funding of H3Africa was announced in late 2016. 5 …”

Evaluation of Current Clinical Research Spectrum of Sickle Cell Anemia to Approach New Innovative Developmental Techniques for Enhancing Sudanese Health Systems

“…Novel therapeutic methods that use medications to upgrade the downstream sequelae of HbS polymerization have not veri ed to be as active as hydroxyurea (HU) which has an "anti-sickling" consequence on induction of embryonic hemoglobin (HbF, α2γ2). In addition to great advances in HSCT and gene therapy, new pharmacological anti-sickling approaches have developed beside introducing up to date molecular based technologies (22,23).…”

Evaluation of Changes in Haematological Parameters of Sickle Cell Anaemia Subjects in Rivers and Bayelsa States

“…According to global estimates, approximately 5% of the population has some type of haemoglobin variant, and more than 300,000 babies are born each year with haemoglobinopathies, with the homozygous sickle cell disease (HbSS) being the most prevalent type [3][4][5].…”