Newborn screening for sickle cell disease: necessary but not sufficient.

ickle cell disease is a global health problem that ffects > 300,000 newborns per year, predominantly in Subaharan Africa,1 where mortality is estimated to be > 50% y age 5 years for those with homozygous hemoglobin S HbSS).1 In Brazil, there are estimated to be > 30,000 indiiduals with sickle cell disease and 2,500 newborns per ear, making this the most common genetic disorder in the ountry.2 Advances in the management of sickle cell disase in the United States have led to a decline in mortality rom about 26% to approximately 1-2% in the first 18 years of ife, with the greatest progress demonstrated by a flattenng of the mortality peak in the ages of 1-4 years from 13% o 2%.3,4 Much of this progress resulted from the implemenation of universal newborn screening in all 50 states in the .S. following publication of the results of the prophylactic enicillin study, which demonstrated a dramatic reduction n sepsis and mortality due to S. pneumonia in the first five ears of life.5 Remarkable progress has also occurred in Brazil over the ast two decades, during which newborn hemoglobinopathy creening was initiated in Campinas (1992), Minas Gerais 1998), Rio de Janeiro (2001), Pernambuco (2003), and