Survival of children with sickle cell disease in the comprehensive newborn screening programme in Minas Gerais, Brazil

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(0)-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.