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Journal Article Epublish 32 citations

PubMed: 35281803 DOI: 10.3389/fgene.2022.802355

Establishing a Sickle Cell Disease Registry in Africa: Experience From the Sickle Pan-African Research Consortium, Kumasi-Ghana.

Front Genet 13:802355

Authors: Vivian Paintsil, Evans Xorse Amuzu, Isaac Nyanor, Emmanuel Asafo-Adjei, Abdul Razak Mohammed, Suraj Abubakar Yawnumah, Yaa Gyamfua Oppong-Mensah, Samuel Blay Nguah, Paul Obeng, Elliot Eli Dogbe, Mario Jonas, Victoria Nembaware, Gaston Mazandu, Kwaku Ohene-Frempong, Ambroise Wonkam, Julie Makani, Daniel Ansong, Alex Osei-Akoto

Affiliations

  • Directorate of Child Health-Komfo Anokye Teaching Hospital, Kumasi, Ghana.
  • Department of Child Health-Kwame Nkrumah University of Science and Technology, School of Medicine and Dentistry, Kumasi, Ghana.
  • Department of Pathology, Division of Human Genetics, Faculty of Health Sciences, University of Cape Town, Cape Town, South Africa.
  • Sickle Cell Foundation of Ghana, Kumasi, Ghana.
  • SPARCo, Sickle Cell Programme, Muhimbili University of Health and Allied Sciences, Dar Es Salam, Tanzania.

Abstract

Sickle cell disease (SCD) is the most common clinically significant hemoglobinopathy, characterized by painful episodes, anemia, high risk of infection, and other acute and chronic complications. In Africa, where the disease is most prevalent, large longitudinal data on patients and their outcomes are lacking. This article describes the experiences of the Kumasi Center for SCD at the Komfo Anokye Teaching Hospital (KCSCD-KATH), a Sickle Pan-African Research Consortium (SPARCO) site and a SickleInAfrica Consortium member, in establishing a SCD registry for the evaluation of the outcomes of patients. It also provides a report of a preliminary analysis of the data. The process of developing the registry database involved comprehensive review of the center's SCD patient medical records, incorporating data elements developed by the SickleInAfrica Consortium and obtaining ethical clearance from the local Institutional Review Board. From December 2017 to March 2020, 3,148 SCD patients were enrolled into the SCD registry. Enrollment was during the SCD outpatient clinic visits or through home visits. A significant proportion of the patients was from the newborn screening cohort (50.3%) and was males (52.9%). SCD-SS, SCD-SC, and Sβ thalassemia were seen in 67.2, 32.5, and 0.3% patients, respectively. The majority of the patients were in a steady state at enrollment; however, some were enrolled after discharge for an acute illness admission. The top two clinical diagnoses for SCD-SS patients were sickle cell painful events and acute anemia secondary to hyperhemolysis with incidence rates of 141.86 per 10,000 person months of observation (PMO) and 32.74 per 10,000 PMO, respectively. In SCD-SC patients, the top two diagnoses were sickle cell painful events and avascular necrosis with incidence rates of 203.09 per 10,000 PMO and 21.19 per 10,000 PMO, respectively. The SPARCO Kumasi site has developed skills and infrastructure to design, manage, and analyze data in the SCD registry. The newborn screening program and alternative recruitment methods such as radio announcement and home visits for defaulting patients were the key steps taken in enrolling patients into the registry. The registry will provide longitudinal data that will help improve knowledge of SCD in Ghana and Africa through research.

MeSH

Registries, Cells, Infant, Newborn, Anemia, Sickle Cell, Incidence, Anemia, Neonatal Screening, Diagnosis, House Calls, Research

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