PURPOSE: 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.
PURPOSE: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 Cohortparticipants 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: A Sobota; R Yamashita; Y Xu; F Trachtenberg; P Kohlbry; D A Kleinert; P J Giardina; J L Kwiatkowski; D Foote; V Thayalasuthan; J B Porter; A A Thompson; L Schilling; C T Quinn; E J Neufeld Journal: Am J Hematol Date: 2011-01 Impact factor: 10.047
Authors: Luciana Scalone; Lorenzo G Mantovani; Marieke Krol; Diana Rofail; Simona Ravera; Maria Grazia Bisconte; Caterina Borgna-Pignatti; Zelia Borsellino; Paolo Cianciulli; Domenico Gallisai; Luciano Prossomariti; Ippazio Stefàno; Maria D Cappellini Journal: Curr Med Res Opin Date: 2008-05-27 Impact factor: 2.580
Authors: Farrukh Shah; Paul Telfer; Mark Velangi; Shivan Pancham; Robert Wynn; Sally Pollard; Elizabeth Chalmers; Jonathan Kell; Angela M Carter; Joe Hickey; Clark Paramore; Minesh Jobanputra; Kate Ryan Journal: EJHaem Date: 2021-09-08
Authors: Victoria F Wagner; Hope Northrup; S Shahrukh Hashmi; Joanne M Nguyen; Mary Kay Koenig; Jessica M Davis Journal: J Genet Couns Date: 2017-08-13 Impact factor: 2.537