What to do

1. Test the father now
   • CBC with red-cell indices plus Hb analysis, preferably HPLC/capillary electrophoresis or hemoglobin electrophoresis.
   • This helps determine the risk of HbSS, HbSC, HbS-beta thalassemia, etc.
2. Do newborn screening for hemoglobinopathies
   • Use a routine newborn dried-blood-spot heel prick, ideally after 24-48 hours of life and before discharge, according to local newborn-screening policy.
   • HPLC, isoelectric focusing, or capillary electrophoresis are preferred newborn methods. They can detect HbS even though HbF predominates in neonates. Newborn screening is specifically designed to identify affected infants early. Newborn hemoglobinopathy screening guidance
3. Do not wait until 6 months to screen
   • The initial Hb testing should be done in the newborn period, ideally as part of universal newborn screening.
   • If the newborn screen suggests sickle cell disease, arrange confirmatory hemoglobin testing and pediatric hematology review urgently, within the first month, so preventive care can begin before 2 months of age. Early follow-up recommendations
4. When to repeat Hb electrophoresis
   • If newborn Hb testing is abnormal or equivocal, confirm by a reference-laboratory method and/or molecular testing in early infancy, generally by 2-3 months.
   • A repeat hemoglobin electrophoresis/HPLC at about 6 months of age can better define the final phenotype because fetal hemoglobin has declined substantially by then. This is particularly helpful when distinguishing HbSS from HbS-beta-thalassemia or when the newborn pattern is uncertain.
   • If the infant received a blood transfusion, the hemoglobin result may be unreliable. Use molecular testing or repeat hemoglobin testing about 3 months after the last transfusion.

Follow-up based on results

• If newborn screen shows an affected pattern, such as FS, FSC, or FSA:
  • Refer to pediatric hematology promptly.
  • Begin infection-prevention planning, including penicillin prophylaxis by 2 months if clinically significant SCD is suspected, and ensure immunizations are up to date.
  • Educate parents that fever in an infant with confirmed/suspected SCD requires urgent medical evaluation.
• If the infant has sickle trait only, such as an FAS pattern:
  • The child is generally healthy and needs no SCD prophylaxis.
  • Provide parental counseling and document the trait status for future reproductive counseling.
• If routine newborn screening is unavailable, send hemoglobin fractionation by HPLC/capillary electrophoresis or isoelectric focusing now, rather than waiting for symptoms or for 6 months.