Newborn screening for sickling hemoglobinopathies. Houston, 1976 to 1980.

Fifty of 52 infants with cord blood hemoglobin electrophoresis patterns indicative of a possible sickling hemoglobinopathy (SH) were followed up prospectively. A retrospective group of 39 children whose mothers were contacted two to four years after the birth of a child with a positive newborn screening test formed a comparison group. During an average follow-up period of 30 months, children of mothers who were informed early of a positive cord blood test were hospitalized more readily for complications of an SH. During this same period, five (6%) of 88 infants with fetal and sickle hemoglobin (FS) or fetal, sickle, and C hemoglobins (FSC) in their cord blood died, all before 6 months of age. All five deaths occurred in infants whose parents were unaware of their child's disease; four of the five deaths may have been caused by sepsis. These results alone did not prove that screening significantly reduces mortality but did show that newborn screening increases parental awareness of complications and may, therefore, improve the prognosis in sickle cell disease in infancy.