title: Multicenter COMPACT study of COMplications in patients with sickle cell disease and utilization of iron chelation therapy.
abstract: BACKGROUND
  Over the past few decades, lifespans of sickle cell disease (SCD) patients have increased; hence, they encounter multiple complications. Early detection, appropriate comprehensive care, and treatment may prevent or delay onset of complications.
  
  
  OBJECTIVE
  We collected longitudinal data on sickle cell disease (SCD) complication rates and associated resource utilization relative to blood transfusion patterns and iron chelation therapy (ICT) use in patients aged ≥16 years to address a gap in the literature.
  
  
  METHODS
  Medical records of 254 SCD patients ≥16 years were retrospectively reviewed at three US tertiary care centers.
  
  
  METHODS
  We classified patients into cohorts based on cumulative units of blood transfused and ICT history: <15 units, no ICT (Cohort 1 [C1]), ≥15 units, no ICT (Cohort 2 [C2]), and ≥15 units with ICT (Cohort 3 [C3]). We report SCD complication rates per patient per year; cohort comparisons use rate ratios (RRs).
  
  
  RESULTS
  Cohorts had 69 (C1), 91 (C2), and 94 (C3) patients. Pain led to most hospitalizations (76%) and emergency department (ED) (82%) visits. Among transfused patients (C2+C3), those receiving ICT were less likely to experience SCD complications than those who did not (RR [95% CI] C2 vs. C3: 1.33 [1.25-1.42]). Similar trends (RR [95% CI]) were observed in ED visits and hospitalizations associated with SCD complications (C2 vs. C3, ED: 1.94 [1.70-2.21]; hospitalizations: 1.61 [1.45-1.78]), but not in outpatient visits.
  
  
  CONCLUSIONS
  Although the most commonly reported SCD complication among all patients was pain, patients who received ICT were less likely to experience pain and other complications than those who did not. These results highlight the need for increased patient and provider education on the importance of comprehensive disease management.
authors.affiliation: Department of Epidemiology and Public Health, University of Miami, Miller School of Medicine , Miami, FL , USA.
authors.forename: Lanetta
authors.initials: L
authors.lastname: Jordan
authors.forename: Patricia
authors.initials: P
authors.lastname: Adams-Graves
authors.forename: Julie
authors.initials: J
authors.lastname: Kanter-Washko
authors.forename: Patricia A
authors.initials: PA
authors.lastname: Oneal
authors.forename: Medha
authors.initials: M
authors.lastname: Sasane
authors.forename: Francis
authors.initials: F
authors.lastname: Vekeman
authors.forename: Christine
authors.initials: C
authors.lastname: Bieri
authors.forename: Matthew
authors.initials: M
authors.lastname: Magestro
authors.forename: Andrea
authors.initials: A
authors.lastname: Marcellari
authors.forename: Mei Sheng
authors.initials: MS
authors.lastname: Duh
mesh_terms: D000328:Adult; D000755:Anemia, Sickle Cell / Q000150:complications / Q000175:diagnosis / Q000453:epidemiology / Q000628:therapy; D001803:Blood Transfusion / Q000379:methods / Q000706:statistics & numerical data; D015913:Chelation Therapy / Q000379:methods / Q000706:statistics & numerical data; D019468:Disease Management; D042241:Early Diagnosis; D061345:Early Medical Intervention; D005260:Female; D006760:Hospitalization; D006801:Humans; D007501:Iron / Q000627:therapeutic use*; D008297:Male; D008501:Medical Records, Problem-Oriented; D010146:Pain / Q000209:etiology / Q000517:prevention & control; D012189:Retrospective Studies; D055502:Secondary Prevention; D014131:Trace Elements / Q000627:therapeutic use; D014481:United States / Q000453:epidemiology
chemical_list: D014131:Trace Elements; D007501:Iron
keywords: Blood transfusions; Complications; Iron chelation therapy; Iron overload; Sickle cell disease