Ardie Pack-Mabien1, Brittany Brown1, Donald E Herbert1, Johnson Haynes1.
Abstract
OBJECTIVE: To assess the prevalence of iron overload in adults with sickle cell disease (SCD) not on a chronic transfusion protocol.
DESIGN: Retrospective chart review. DATA SOURCE: University of South Alabama Comprehensive Sickle Cell Center adult outpatient clinic.
RESULTS: There was no significant difference in units transfused across the four genotypes (HbSS, HbSC, HbSβ(0)-thalassemia, and HbSβ(+)-thalassemia). Only individuals with HbSS (n = 63) met criteria for iron overload with ferritins of ≥1500 ng/mL. Forty-eight had ferritins <1500 ng/mL, eight (13%) had ferritins ≥3000 ng/mL, and seven (11%) had ferritins ≥1500 and <3000 ng/mL. The overall prevalence of iron overload was 9.74% in SCD cohort and 23.8% in the HbSS genotype.
CONCLUSIONS: Our data support that patients with HbSS are at a particularly high risk for inadvertent iron overload as compared to HbSC, HbSβ(0)-thalassemia, and HbSβ(+)-thalassemia. IMPLICATIONS FOR PRACTICE: This study supports the need for healthcare providers to closely monitor the number of red blood cell (RBC) transfusions, RBC units transfused, and serial baseline, steady-state ferritin levels. With closer monitoring, the clinical significance of iron overload in SCD can be established and guide the healthcare provider's management in the prevention of iron overload. ©2015 American Association of Nurse Practitioners.
OBJECTIVE: To assess the prevalence of iron overload in adults with sickle cell disease (SCD) not on a chronic transfusion protocol.
DESIGN: Retrospective chart review. DATA SOURCE: University of South Alabama Comprehensive Sickle Cell Center adult outpatient clinic.
RESULTS: There was no significant difference in units transfused across the four genotypes (HbSS, HbSC, HbSβ(0)-thalassemia, and HbSβ(+)-thalassemia). Only individuals with HbSS (n = 63) met criteria for iron overload with ferritins of ≥1500 ng/mL. Forty-eight had ferritins <1500 ng/mL, eight (13%) had ferritins ≥3000 ng/mL, and seven (11%) had ferritins ≥1500 and <3000 ng/mL. The overall prevalence of iron overload was 9.74% in SCD cohort and 23.8% in the HbSS genotype.
CONCLUSIONS: Our data support that patients with HbSS are at a particularly high risk for inadvertent iron overload as compared to HbSC, HbSβ(0)-thalassemia, and HbSβ(+)-thalassemia. IMPLICATIONS FOR PRACTICE: This study supports the need for healthcare providers to closely monitor the number of red blood cell (RBC) transfusions, RBC units transfused, and serial baseline, steady-state ferritin levels. With closer monitoring, the clinical significance of iron overload in SCD can be established and guide the healthcare provider's management in the prevention of iron overload. ©2015 American Association of Nurse Practitioners.
Entities:
Keywords:
Sickle cell disease; chronic transfusion protocol; intermittent red blood cell transfusion; iron overload
Mesh:
Substances:
Year: 2015
PMID: 25711464 DOI: 10.1002/2327-6924.12221
Source DB: PubMed Journal: J Am Assoc Nurse Pract ISSN: 2327-6886 Impact factor: 1.165