Background Sickle cell disease (SCD) is an inherited blood disorder that predominantly affects individuals in sub-Saharan Africa. However, research that elucidates links between SCD pathophysiology and nutritional status in African patients is lacking. This systematic review aimed to assess the landscape of studies in sub-Saharan Africa that focused on nutritional aspects of SCD, and highlights gaps in knowledge that could inform priority-setting for future research. Methods The study was conducted using the Preferred Reporting Items for Systematic Reviews and Meta-Analysis (PRISMA) guidelines. Inclusion criteria comprised original, peer-reviewed research published between January 1995 and November 2020 involving individuals in Africa with any phenotypic variant of SCD and at least one nutritional status outcome. Nutritional status outcomes were defined as those that assessed dietary intakes, growth/anthropometry, or nutritional biomarkers. Databases used were Ovid Embase, Medline, Biosis and Web of Science. Results The search returned 526 articles, of which 76 were included in the final analyses. Most investigations (67%) were conducted in Nigeria. Studies were categorized into one of three main categories: descriptive studies of anthropometric characteristics (49%), descriptive studies of macro- or micronutrient status (41%), and interventional studies (11%). Findings consistently included growth impairment, especially among children and adolescents from sub-Saharan Africa. Studies assessing macro- and micronutrients generally had small sample sizes and were exploratory in nature. Only four randomized trials were identified, which measured the impact of lime juice, long-chain fatty acids supplementation, ready-to-use supplementary food (RUSF), and oral arginine on health outcomes. Conclusions The findings reveal a moderate number of descriptive studies, most with small sample sizes, that focused on various aspects of nutrition and SCD in African patients. There was a stark dearth of interventional studies that could be used to inform evidence-based changes in clinical practice. Findings from the investigations were generally consistent with data from other regional settings, describing a significant risk of growth faltering and malnutrition among individuals with SCD. There is an unmet need for clinical research to better understand the potential benefits of nutrition-related interventions for patients with SCD in sub-Saharan Africa to promote optimal growth and improve health outcomes.

Individuals with sickle cell disease particularly with the homozygous (SS) genotype historically have relatively low blood pressure. Nonetheless, they develop vasculopathy-associated organ dysfunction and the risk of organ dysfunction increases at blood pressures that are normal in the general population. This phenomenon is termed relative systemic hypertension (RSH) with a systolic blood pressure range of 120-139 mmHg, and diastolic blood pressure range of 70-89 mmHg. The significance of RSH lies in its association with renal insufficiency, pulmonary hypertension, stroke and propensity to progress to systemic hypertension. We conducted a retrospective chart review of 1,000 adults with sickle cell disease at the Ghana Institute of Clinical Genetics, to determine the prevalence of RSH in sickle cell disease in Ghana and associated complications. We found a high prevalence of RSH and hypertension with a relatively low frequency of renal insufficiency. Pulse pressure, a predictor of mortality, was higher in males of all ages. We anticipate that providing an estimate of the burden of RSH will heighten its recognition and clinical management among health care providers.

Background: Sickle cell disease (SCD), an inherited blood disorder, predominantly affects individuals in sub-Saharan Africa. Research linking its pathophysiology and nutritional status in African patients has not been previously described. This systematic review aimed to assess the landscape of studies in sub-Saharan Africa focused on nutritional aspects of SCD and highlight gaps in knowledge that could inform priority-setting for future research.Methods: The study was conducted using the Preferred Reporting items for Systematic Reviews and Meta-Analysis (PRISMA) guidelines. All original studies involving individuals with any phenotypic variant of SCD and at least one nutritional status outcome; and were conducted between January 1995 and November 2020 in Africa were included. ‘Nutritional status’ was defined by studies on dietary intake, measurements of growth/anthropometry, or nutritional biomarkers. Studies were included if they involved Databases used were Ovid Embase, Medline, Biosis and Web of Science. Results: The search returned 526 articles with 76 studies included in the final analyses. Most investigations (67%) were conducted in Nigeria. Studies were categorized into one of three main categories: descriptive studies of anthropometric characteristics (49%), descriptive studies of macro- or micronutrient intakes (41%), and interventional studies (11%). Findings consistently found growth impairment, especially among children and adolescents from sub-Saharan Africa. Studies assessing macro- and micronutrients generally had small sample sizes and were exploratory in nature. Only four randomized trials were identified, which measured the impact of lime juice, long-chain fatty acids supplementation, ready-to-use supplementary food, and oral arginine on health outcomes. Conclusions: The findings reveal a moderate number of descriptive studies, most with small sample sizes, that focused on various aspects of nutrition and SCD in African patients. There was a stark lack of interventional studies that could be used to inform evidence-based changes in clinical practice. Findings of investigations were generally consistent with data from other regional settings, describing a significant risk of growth faltering and malnutrition among individuals with SCD. There is an unmet need for clinical research to better understand the potential benefits of nutritional-related interventions for patients with SCD in sub-Saharan Africa in order to promote optimal growth and improve health outcomes.

Expanding services in Ghana for people with sickle cell disease is expected to increase childhood survival and need for transition to adult care. Little is known about patient transition experiences in sub-Saharan Africa. We sought to understand those experiences of adolescents and young adults at an adult sickle cell clinic in Accra, Ghana. Individuals 13 to 22 years of age receiving sickle cell care at the Ghana Institute of Clinical Genetics were interviewed to recall their advance preparation and early experiences in adult sickle cell clinic. Mean age of the 100 participants interviewed was 17.9 ± 2.9 years, 65% female. Most had hemoglobin SS (77%) or hemoglobin SC (20%). Twenty-nine participants recalled pretransition preparation; 93% of them ( 27) had received care at Korle Bu Pediatric Sickle Cell Clinic. Among the remaining 71 who did not recall advance preparation, 54% (34) had received pediatric care at that clinic (P < 0.001). More in the group recalling preparation had positive feelings about needing to transition care compared with those not recollecting preparation (55% vs. 32%, P = 0.04). Our results suggest that pretransition preparation may ease the peritransition experience. Conduct and evaluation of a program for transitioning into adult sickle cell care in Ghana may facilitate the transfer process.