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

Abstract

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/Sβ⁰-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.

Keywords:

• Sickle cell disease
• Epidemiology
• Haemoglobinopathies
• Death rate
• Neonatal screening
• Socioeconomic status

Acknowledgments

We wish to thank all who agreed to be interviewed, despite having suffered the loss of a child, and Ana Paula Fernandes for her guidance. We are grateful for financial support from CNPq Brazilian Research Council and Nupad UFMG Ncleo de Aes e Pesquisa em Apoio Diagnstico, Federal University of Minas Gerais, Brazil.