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. 2022 Mar 16;6(6):1844–1853. doi: 10.1182/bloodadvances.2021004675

Table 4.

Patient characteristics significantly associated with reduced HRQoL or greater fatigue by HRQoL or fatigue instrument in adults and children

Characteristic Adults Children
EQ-5D FACT-An PROMIS SF-7a PedsQL 4.0 Peds-FACIT-F PROMIS Child SF-10a
Older age X
Female sex X X X
Non-Amish X X X X
Splenectomized X A
Regularly transfused X X X
Iron overload: any chelation use X X X A
Iron overload: ferritin > 1000 ng/mL X A
Pulmonary hypertension X X X
Nonmissense/nonmissense genotype X X
Hemoglobin < 8 g/dL X
≥18 total transfusions A

A, significant only when the Amish population was excluded from analysis; X, significant in entire cohort.