A Cost-Effectiveness Analysis of a Pilot Neonatal Screening Program for Sickle Cell Anemia in the Republic of Angola.

OBJECTIVE: To assess the cost-effectiveness of a pilot newborn screening (NBS) and treatment program for sickle cell anemia (SCA) in Luanda, Angola. STUDY DESIGN: In July 2011, a pilot NBS and treatment program was implemented in Luanda, Angola. Infants identified with SCA were enrolled in a specialized SCA clinic in which they received preventive care and sickle cell education. In this analysis, the World Health Organization (WHO) and generalized cost-effectiveness analysis methods were used to estimate gross intervention costs of the NBS and treatment program. To determine healthy life-years (HLYs) gained by screening and treatment, we assumed NBS reduced mortality to that of the Angolan population during the first 5 years based upon WHO and Global Burden of Diseases Study 2010 estimates, but provided no significant survival benefit for children who survive through age 5 years. A secondary sensitivity analysis with more conservative estimates of mortality benefits also was performed. The costs of downstream medical costs, including acute care, were not included. RESULTS: Based upon the costs of screening 36,453 infants and treating the 236 infants with SCA followed after NBS in the pilot project, NBS and treatment program is projected to result in the gain of 452-1105 HLYs, depending upon the discounting rate and survival assumptions used. The corresponding estimated cost per HLY gained is $1380-$3565, less than the gross domestic product per capita in Angola. CONCLUSIONS: These data demonstrate that NBS and treatment for SCA appear to be highly cost-effective across all scenarios for Angola by the WHO criteria.

An accurate and inexpensive color-based assay for detecting severe anemia in a limited-resource setting.

Severe anemia is an important cause of morbidity and mortality among children in resource-poor settings, but laboratory diagnostics are often limited in these locations. To address this need, we developed a simple, inexpensive, and color-based point-of-care (POC) assay to detect severe anemia. The purpose of this study was to evaluate the accuracy of this novel POC assay to detect moderate and severe anemia in a limited-resource setting. The study was a cross-sectional study conducted on children with sickle cell anemia in Luanda, Angola. The hemoglobin concentrations obtained by the POC assay were compared to reference values measured by a calibrated automated hematology analyzer. A total of 86 samples were analyzed (mean hemoglobin concentration 6.6 g/dL). There was a strong correlation between the hemoglobin concentrations obtained by the POC assay and reference values obtained from an automated hematology analyzer (r=0.88, P<0.0001). The POC assay demonstrated excellent reproducibility (r=0.93, P<0.0001) and the reagents appeared to be durable in a tropical setting (r=0.93, P<0.0001). For the detection of severe anemia that may require blood transfusion (hemoglobin <5 g/dL), the POC assay had sensitivity of 88.9% and specificity of 98.7%. These data demonstrate that an inexpensive (<$0.25 USD) POC assay accurately estimates low hemoglobin concentrations and has the potential to become a transformational diagnostic tool for severe anemia in limited-resource settings.

A prospective newborn screening and treatment program for sickle cell anemia in Luanda, Angola.

Over 300,000 infants are born annually with sickle cell anemia (SCA) in sub-Saharan Africa, and >50% die young from infection or anemia, usually without diagnosis of SCA. Early identification by newborn screening (NBS), followed by simple interventions dramatically reduced the mortality of SCA in the United States, but this strategy is not yet established in Africa. We designed and implemented a proof-of-principle NBS and treatment program for SCA in Angola, with focus on capacity building and local ownership. Dried bloodspots from newborns were collected from five birthing centers. Hemoglobin identification was performed using isoelectric focusing; samples with abnormal hemoglobin patterns were analyzed by capillary electrophoresis. Infants with abnormal FS or FSC patterns were enrolled in a newborn clinic to initiate penicillin prophylaxis and receive education, pneumococcal immunization, and insecticide-treated bed nets. A total of 36,453 infants were screened with 77.31% FA, 21.03% FAS, 1.51% FS, and 0.019% FSC. A majority (54.3%) of affected infants were successfully contacted and brought to clinical care. Compliance in the newborn clinic was excellent (96.6%). Calculated first-year mortality rate for babies with SCA compares favorably to the national infant mortality rate (6.8 vs. 9.8%). The SCA burden is extremely high in Angola, but NBS is feasible. Capacity building and training provide local healthcare workers with skills needed for a functional screening program and clinic. Contact and retrieval of all affected SCA infants remains a challenge, but families are compliant with clinic appointments and treatment. Early mortality data suggest screening and early preventive care saves lives.