title: Relationship among chelator adherence, change in chelators, and quality of life in thalassemia.
abstract: OBJECTIVE
  Thalassemia, a chronic blood disease, necessitates life-long adherence to blood transfusions and chelation therapy to reduce iron overload. We examine stability of health-related quality of life (HRQOL) in thalassemia and adherence to chelation therapy over time, especially after changes in chelator choice.
  
  
  METHODS
  Thalassemia Longitudinal Cohort participants in the USA, UK, and Canada completed the SF-36v2 (ages 14+) and the PF-28 CHQ (parents of children <14 years). Chelation adherence was defined as self-reported percent of doses administered in the last 4 weeks.
  
  
  RESULTS
  Two hundred and fifty-eight adults/adolescents (mean 29.7 years) and 133 children (mean 8.5 years) completed a mean of 2.8-years follow-up. Children made few chelator changes, whereas a mean of 2.2 changes was observed among the 37% of adults/adolescents who made chelator changes, mainly due to patient preference or medical necessity. Physical HRQOL improved among those with lower iron burden (better health status) at baseline who made a single change in chelator, but declined among participants with multiple changes and/or high iron burden (worse health status). Mental health improved among participants with lower iron burden, but iron overload was negatively associated with social functioning. Adherence did not significantly change over follow-up except for an increase after a change from deferoxamine (DFO) infusion to oral deferasirox (p = 0.03). Predictors of lower adherence for adults/adolescents at follow-up included side effects, smoking, younger age, problems preparing DFO, increased number of days per week DFO prescribed, and lower physical quality of life .
  
  
  CONCLUSIONS
  Strategies to balance medical needs with family, work, and personal life may assist in adherence.
authors.affiliation: New England Research Institutes, 9 Galen Street, Watertown, MA, 02472, USA, ftrachtenberg@neriscience.com.
authors.forename: Felicia L
authors.initials: FL
authors.lastname: Trachtenberg
authors.forename: Eric
authors.initials: E
authors.lastname: Gerstenberger
authors.forename: Yan
authors.initials: Y
authors.lastname: Xu
authors.forename: Lauren
authors.initials: L
authors.lastname: Mednick
authors.forename: Amy
authors.initials: A
authors.lastname: Sobota
authors.forename: Hannah
authors.initials: H
authors.lastname: Ware
authors.forename: Alexis A
authors.initials: AA
authors.lastname: Thompson
authors.forename: Ellis J
authors.initials: EJ
authors.lastname: Neufeld
authors.forename: Robert
authors.initials: R
authors.lastname: Yamashita
mesh_terms: D000293:Adolescent; D000328:Adult; D015913:Chelation Therapy; D002648:Child; D002675:Child, Preschool; D002908:Chronic Disease; D015331:Cohort Studies; D005260:Female; D006801:Humans; D007223:Infant; D007502:Iron Chelating Agents / Q000008:administration & dosage*; D019190:Iron Overload / Q000188:drug therapy / Q000209:etiology; D008137:Longitudinal Studies; D008297:Male; D008875:Middle Aged; D011788:Quality of Life / Q000523:psychology*; D013789:Thalassemia / Q000188:drug therapy* / Q000378:metabolism; D055815:Young Adult; D016903:Drug Monitoring
chemical_list: D007502:Iron Chelating Agents