Prospective evaluation of targed filter paper screening for sickle cell disease: effectiveness and follow through

Abstract

This study involved evaluation of targeted filter paper screening of newborns with sickle cell disease and the objectives were: to determine the suitability of filter paper screening for sickle cell disease in Louisiana with regards: to false positivity rates and false negativity rates, lower the age of cases determined by the screening programme to below three months from the current practice either at overt disease manifestations or beyond 6-12 months of age, determine in a sub-area whether targeting only to the non-white populations fails in any significant degree to identify cases of sickle cell disease in white populations and the willingness of families found to have newborns with the disease to undertake long-term penicillin prophylaxis. Out of 2,034 newborns, 335 had sickle cell related diseases and 1,593 newborns tested negative for sickle cell disease. The non-compliance frequency stood at 106. The median age at which 59 patients with sickle cell diseases were placed on penicillin prophylaxis was three months. Of the entire 2,034 newborns screened, only one infant, diagnosed with sickle cell died. This study had assisted tremendously in reducing the mortality in early infancy suffered by children with sickle cell disease.