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Sickle Cell Disorder Frequency: Hb S with Hereditary Persistence of Fetal Hemoglobin

Approximately 1% of persons with homozygous sickle cell disease also demonstrates hereditary persistence of fetal hemoglobin (HPFH). Persons with Hb S/HPFH have a milder anemia than individuals with SCD who have none to normal levels of Hb F.
Increased fetal hemoglobin protects the cell from sickling because of its higher affinity for oxygen. HPFH may also be present in other hemoglobinopathies and thalassemias or in SCD in combination with other hemoglobins (Hb SC/HPFH) and thalassemia (Hb S/Bthal/HPFH).