MORBIDITY & MORTALITY IN SICKLE CELL DISEASE INFANTS

Abstract

All infants born in NYC are screened for sickle cell disease (SCD). This study was conducted to observe the morbidity and mortality among sickle cell infants and to study the correlation between mortality and early comprehensive care provided to the patients (pts. ) and their families. In 1982 (1/1-12/31) the Newborn Screening Lab identified 173 newborns with SCD on repeat testing. 168 pts. were followed in sickle cell/hematology clinics over an 11-23 mos, period. 5 pts, were reported to be lost for follow-up despite the counseling given to all parents of the importance of early entry in a comprehensive care system. Written protocols with the infant's name and the screening diagnosis were sent to providers to whom the infants were initially referred. Interesting features reported by the providers were that 7 families in the study had one or more children previously diagnosed with SCD. 6 with “ SS” and 1 “ SC” and in 2 families the older sibling with “ SS” had died. Two mothers had “ SS” disease, and one mother gave birth to an “ SS” infant after knowledge of the hemoglobinopathy by prenatal diagnosis. 33 infants were reported to have had one or more complications. One infant had salm. sepsis, 2 had pneumo. sepsis, 4 had possible sepsis, one had sequestration crisis, 5 had pneumonia, 8 had hand-foot syndrome, and 12 had high fever. One infant from the study group died at the age of 12 mos. as a result of sepsis and D.I.C. No other death was reported by either the parents or by the Bur. of Vital Statistics, NYC Health Dept. Our data suggests that low mortality could be associated with early entry of SCD pts. in a comprehensive care system.

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