Prevalence of Sickle Cell Trait and Reliability of Self-Reported Status among Expectant Parents in Nigeria: Implications for Targeted Newborn Screening

Abstract

Background/Aims: Sickle cell disease (SCD) is a life-threatening, autosomal recessive blood disorder prevalent in sub-Saharan Africa. We identified the prevalence of sickle cell trait (SCT) among pregnant women and their male partners in Enugu State, Nigeria, and determined the accuracy of self-reported sickle cell status and its reliability for identifying high-risk newborns for targeted screening. Methods: We conducted a nested cohort study of expectant parents enrolled in the Healthy Beginning Initiative (HBI). The HBI is a cluster-randomized trial of a congregation-based approach designed to increase HIV testing. Participants completed a survey regarding self-awareness of their sickle cell genotype and consented to genotype screening by cellulose acetate electrophoresis. Results: SCT prevalence (HbAS) was 22% (746/3,371). Only 50% of participants provided an accurate self-report. Self-report accuracy was significantly different (p < 0.0001) between individuals who reported having SCT or SCD (61% accuracy) versus those who reported not having SCT or SCD (86% accuracy). Demographic variables including gender, age, household size, employment, education, and home location were significantly associated with providing an accurate self-report. Conclusions: Low numbers of accurate parental self-reports, coupled with a high SCT prevalence in Nigeria, could limit the efficacy of targeted newborn screening. However, our data indicate that it is feasible to integrate sickle cell screening for pregnant women with existing, community-based health care programs developed by the President's Emergency Plan for AIDS Relief (PEPFAR), such as the HBI. Expanding screening programs could enable the development of targeted newborn screening based on maternal genotype that could identify all newborns with SCD in resource-limited settings.

Global Emergency Medicine: A Review of the Literature From 2016

“…The 2016 global EM articles selected for full review are listed in Table 4. [16][17][18][19][20][21][22][23][24][25][26][27][28][29][30][31][32][33][34] Nineteen articles were selected for inclusion, of which 15 (79%) were categorized as emergency care, three (16%) were categorized as development, and one (5%) was categorized as disaster response articles. Eighteen (95%) articles were original research manuscripts and one (5%) was a review article.…”

“…The use of the Emergency Triage Assessment and Treatment (ETAT) tool for pediatric patients continues to spread, with Kapoor et al 33 demonstrating the dissemination of ETAT from the referral hospitals down to the primary health center level in Guatemala. In Nigeria, Burnham-Marusich et al 18 used a nested design to conduct two-step screening for sickle cell disease, which due to pain crises and acute infections poses a heavy burden on the emergency care system in sub-Saharan Africa. In another article studying anemia, Sawe et al 26 demonstrated that clinical gestalt was a fair alternative to laboratory testing in diagnosing anemia in resource-limited settings.…”

A Patient-Held Smartcard With a Unique Identifier and an mHealth Platform to Improve the Availability of Prenatal Test Results in Rural Nigeria: Demonstration Study

“…It can be used to increase access to health information and reduce turnaround times for receipt of laboratory test results by skilled birth attendants. Studies of mHealth apps to improve maternal health in low-income countries have focused primarily on using mobile phones for data collection, appointment reminders, health promotion and education, and provider-to-provider or person-to-person communication [ 12 , 13 ]. Specifically in Nigeria, mHealth has been used by community health workers for decision support (prenatal care decision support algorithm), health education, and data collection, as well as by clients for appointment reminders [ 25 , 26 ].…”

“…Our rationale for focusing on HIV, HBV, and SCD for mHealth is based on several factors: (1) we found high prevalences of HIV, hepatitis B surface antigen, and sickle cell trait of 2% [ 10 ], 5%, and 22% [ 13 ], respectively, in pregnant women in the communities where HBI was implemented; (2) an integrated approach to screen for multiple diseases rather than a single condition was widely acceptable by the community; and (3) the integration of both infectious and chronic diseases highlights the double burden facing sub-Saharan Africa.…”

Prevalência de hemoglobina S em doadores de sangue do Hemocentro de Passo Fundo, Rio Grande do Sul, Brasil

“…A prevalência da heterozigose para HbS é maior nos países africanos, e, na Nigéria, atinge aproximadamente 20% 10 . Em afro-americanos e na Jamaica este número chega a 10% 9 .…”