Although comprehensive and effective, neonatal screening for sickle cell disease was not sufficient to significantly reduce mortality in a newborn screening program. Economic and social development and increase of the knowledge on sickle cell disease among health professionals and family are needed to overcome excessive mortality.

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Despite an effective ongoing comprehensive screening programme, mortality from SCD in Minas Gerais is still high. To decrease mortality rates, socio-economic development and SCD education programmes for health professionals and families are required.

Background Children in developing countries with sickle cell disease SCD have high rates of mortality, especially in some parts of Africa. Aim To compare the 5-year estimated mortality rate in children born between 1999 and 2001 with that of children born between 2009 and 2011. Methods During the period 1998-2012, sickle cell disease was diagnosed in 2591 of 3,617,919 newborns screened in Minas Gerais, Brazil (1 : 1,400). The estimated probability of death [1 - Survival] was calculated by the Kaplan-Meier method. The logrank test was used to compare groups of survival data. Results Of the 2576 children (15 were excluded), 193 died (7.4%): 153 (79.3%) had SS/Sβ-thalassaemia, 34 had SC (17.6%), and six (3.1%) had Sβ thalassaemia. The 5-year estimated mortality was lower for children born between 2009 and 2011 (n=509) than for those born between 1999 and 2001 (n=624), although not significantly [mean (SD) 5.8% (1.1) vs 6.2% (1.0)], respectively). Conclusion Despite an effective ongoing comprehensive screening programme, mortality from SCD in Minas Gerais is still high. To decrease mortality rates, socio-economic development and SCD education programmes for health professionals and families are required.

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