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

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.

Caregivers' knowledge, attitude, and perception toward pneumococcal infection prophylaxis in pediatric sickle cell disease patients in Kumasi, Ghana

“…Children up to 3 years are expected to visit every 2 months, and those above 3 years are scheduled for visits every 3 months. In addition, clinic attendees must undergo routine health evaluations such as complete blood counts with reticulocyte count and kidney and liver function tests 24 . The caregivers of the clinic attendees comprised the study population for this study.…”

“…In addition, clinic attendees must undergo routine health evaluations such as complete blood counts with reticulocyte count and kidney and liver function tests. 24 The caregivers of the clinic attendees comprised the study…”

Penicillin V prophylaxis uptake among children living with sickle cell disease in a specialist sickle cell clinic in Ghana: A cross‐sectional study

“…Based on the standard operating procedure for the management of SCD at the clinic, SCD patients below 3 years and those 3 years and above are scheduled for a visit every 2 and 3 months, respectively. Also, routine laboratory tests such as complete blood counts with reticulocyte counts, kidney function test, liver function test, and eye examination are done 25 . In terms of routine regimen, SCD patients are given folic acid, hematinic, hydroxyurea (SS genotype and some SC genotype with severe form of crisis), and penicillin V (being prescribed to all age groups).…”

“…Also, routine laboratory tests such as complete blood counts with reticulocyte counts, kidney function test, liver function test, and eye examination are done. 25 In terms of routine regimen, SCD patients are given folic acid, hematinic, hydroxyurea (SS genotype and some SC genotype with severe form of crisis), and penicillin V (being prescribed to all age groups).…”

Global perspectives on cellular therapy for children with sickle cell disease

“…The formation of several regional academic clinical and research networks has led to advocacy, knowledge sharing, and advances in care for SCD in SSA. Networks such as the African Research and Innovative Initiative for Sickle Cell Education (ARISE) [32], the International Hemoglobinopathy Research Network (INHER-ENT) [33], the Re ´seau d'Etude ´de la Dre ´panocytose en Afrique Centrale (REDAC) [34], the Sickle Pan-African Research Consortium (SPARCO) [35], and others have brought together leading clinicians and scientists on the continent to critically appraise and address the challenges of SCD care. Furthermore, increased recognition of the contribution of noncommunicable diseases to mortality in LMICs through the Millenium Development Goals has added to the demand for improvements in care for SCD [36].…”