Newborn Screening for Sickle Cell Disease in Liberia: A Pilot Study

Background In malaria‐endemic countries in West Africa, sickle cell disease (SCD) contributes to childhood mortality. Historically, Liberia had regions wherein hemoglobin S and beta‐thalassemia trait were mutually exclusive. Data on hemoglobinopathies in the Monrovia, the capital, are outdated and d...

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Bibliographic Details
Published in:Pediatric blood & cancer 2016-04, Vol.63 (4), p.671-676
Main Authors: Tubman, Venée N., Marshall, Roseda, Jallah, Wilhemina, Guo, Dongjing, Ma, Clement, Ohene-Frempong, Kwaku, London, Wendy B., Heeney, Matthew M.
Format: Article
Language:English
Subjects:
Anemia, Sickle Cell - diagnosis
Anemia, Sickle Cell - epidemiology
epidemiology
Feasibility studies
Female
Hematology
Hemoglobin
hemoglobinopathies
Humans
Incidence
Infant, Newborn
Isoelectric Focusing
Liberia - epidemiology
Male
Medical screening
Neonatal Screening
Oncology
pediatric hematology/oncology
Pediatrics
Pilot Projects
Public health
Sickle cell disease
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Summary:Background In malaria‐endemic countries in West Africa, sickle cell disease (SCD) contributes to childhood mortality. Historically, Liberia had regions wherein hemoglobin S and beta‐thalassemia trait were mutually exclusive. Data on hemoglobinopathies in the Monrovia, the capital, are outdated and do not reflect urban migration. Updating the epidemiology of SCD is necessary to plan a public health and clinical agenda. Neither newborn screening (NBS) nor screening tools were available in country. This pilot study aimed to determine the feasibility of NBS using a South–South partnership and define the incidence of sickle cell trait (SCT) and SCD in Monrovia. Procedure This descriptive epidemiologic feasibility study collected dried blood spots from 2,785 consecutive newborns delivered at a hospital in Monrovia. Samples were analyzed by isoelectric focusing at a regional reference laboratory. Infants with SCD were referred for preventive care. Results SCT occurred in 10.31% of infants screened. SCD occurred in 33 infants screened [1.19% (95% confidence interval [CI]: 0.79–1.59%)] (FS: 28/33, FSB: 2/33, FSA: 2/33, FSX: 1/33). There were no infants with FSC phenotype observed. Nonsickling hemoglobin phenotypes “FC” and “F” were each present in three infants screened. Seventy‐six percent of infants with SCD were brought to care, demonstrating the feasibility of our approach. Conclusions The incidence of SCD and other hemoglobinopathies remains high in Liberia. Additional studies are needed to clarify sickle genotypes and identify the contribution of silent beta‐thalassemia alleles. By developing regional partnerships, countries similar to Liberia can acquire current data to inform NBS as an important public health initiative toward improving child health.
ISSN:1545-5009
1545-5017
DOI:10.1002/pbc.25875