Trends in mortality and hospital admissions of sickle cell disease patients before and after the newborn screening program in Maranhão, Brazil

OBJECTIVE: To assess the impact of the implementation of neonatal screening on hospitalization and death rates due to sickle cell disease in patients from the state of Maranhão, Brazil. METHODS: A descriptive study was performed of all inpatients and deaths of patients with a diagnosis of sickle cell disease in Maranhão between 1999 and 2012. Data were collected from the Hospital Information System of the Brazilian National Health Service (SUS) and the Death Information System of the Ministry of Health. The implementation of newborn screening tests in Maranhão took place in 2005, and so the periods 1999–2005 (pre) and 2006–2012 (post) were analyzed for trend analysis using a multiple linear regression model. Fisher's exact test was used for the analysis of categorical variables and the Kruskal–Wallis test for continuous variables. RESULTS: The rate of hospitalization increased from 0.315 (pre) to 1.832 (post), indicating 5.82 times more admissions (p-value = 0.04). The mortality rate increased from 0.115 to 0.216, that is 1.88 times higher, but this was not statistically significant (p-value = 0.586). The median age at admission dropped from 11.4 years to 8.7 years (p-value = 0.0002), whereas the median age at death increased from 10 years to 14 years (p-value = 0.665). CONCLUSION: The increases in the rates of hospitalization and death after the implementation of neonatal screening suggests that previously there was an underdiagnosis of sickle cell disease and that screening, along with other factors, increased “visibility” in the state of Maranhão.