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Relationship among chelator adherence, change in chelators, and quality of life in Thalassemia

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Abstract

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.

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Acknowledgments

This work was supported by the following NIH-NHLBI cooperative agreements: U01-HL65232 and NIH/NCRR UL1-RR-024134 to the Children’s Hospital of Philadelphia, U01-HL72291 and by Harvard Catalyst CTSC U-01RR025758 to Children’s Hospital, Boston, U01-HL65233 to University Health Network Toronto General Hospital, UL1RR024131-01to Children’s Hospital & Research Center Oakland, U01-HL65244 and CTSC UL1-RR024996 to Weill Medical College of Cornell University, and U01-HL65238 to New England Research Institutes. Its contents are solely the responsibility of the authors and do not necessarily represent the official views of the NHLBI.

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Authors and Affiliations

  1. New England Research Institutes, 9 Galen Street, Watertown, MA, 02472, USA

    Felicia L. Trachtenberg, Eric Gerstenberger & Yan Xu

  2. Children’s Hospital Boston, Boston, MA, USA

    Lauren Mednick, Amy Sobota & Ellis J. Neufeld

  3. Children’s Hospital of Philadelphia, Philadelphia, PA, USA

    Hannah Ware

  4. Lurie Children’s Hospital, Chicago, IL, USA

    Alexis A. Thompson

  5. California State University, San Marcos, CA, USA

    Robert Yamashita

Authors
  1. Felicia L. Trachtenberg
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  2. Eric Gerstenberger
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  3. Yan Xu
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  4. Lauren Mednick
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  5. Amy Sobota
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  6. Hannah Ware
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  7. Alexis A. Thompson
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  8. Ellis J. Neufeld
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  9. Robert Yamashita
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Corresponding author

Correspondence to Felicia L. Trachtenberg.

Additional information

For the Thalassemia Clinical Research Network.

This is publication number 32 of the Thalassemia Clinical Research Network (TCRN). A list of TCRN member institutions and staff appears in ‘Appendix.’

Appendix

Appendix

The following institutions and researchers contributed to the Thalassemia Clinical Research Network Thalassemia Longitudinal Cohort data reported in this paper.

Children’s Hospital, Boston: Ellis Neufeld, MD, PhD, Principal Investigator, Jennifer Braunstein, NP, Research Nurse, Amber Smith, Study Coordinator, Latoya Lashley, Study Coordinator; Satellite: University of Texas Southwestern Medical Center at Dallas, Charles Quinn, MD, MS, Principal Investigator, Deborah Boger, RN, MSN, PNP, Study Coordinator, Leah Adix, Study Coordinator, Sandra Richardson, Study Coordinator; Children’s Healthcare of Atlanta, Jeanne Boudreaux, MD, Principal Investigator, Leann Hassen, Study Coordinator; Baylor College of Medicine, Brigitta Mueller, MD, Principal Investigator, Bogden Dino, Study Coordinator. Weill Medical College of Cornell University: Patricia Giardina, MD, Principal Investigator, Dorothy Kleinert, RN, Research Nurse; Satellite: Winthrop University Hospital, Mark Weinblatt, MD, Principal Investigator, Linda Skelly, Study Coordinator. The Children’s Hospital of Philadelphia: Janet Kwiatkowski, MD, Principal Investigator, Marie Martin, RN, Research Nurse, Sage Green, Study Coordinator; Satellite: Children’s Memorial Hospital, Chicago, IL, Alexis Thompson, MD, Principal Investigator, Janice Beatty, RN, Research Nurse, Diane Calamaras, RN, CPNP, Research Nurse, Pauline Hess, study coordinator. Children’s Hospital at Oakland: Elliott Vichinsky, MD, Principal Investigator, Dru Foote, NP, Research Nurse, Nancy Sweeters, Study Coordinator, Olivia Vega, Study Coordinator; Satellites: Children’s Hospital of Los Angeles, Thomas Coates, MD, Principal Investigator, Susan Carson, RN, Research Nurse, Eun Ha Pang, Study Coordinator, Rachna Khanna, Study Coordinator; Stanford Hospital, Michael Jeng, MD, Principal Investigator, Kokil Bakshi, Clinical Research Associate; Children’s and Women’s Health Center of British Columbia, John Wu, Principal Investigator, Heather McCartney, RN, Research Nurse, Colleen Fitzgerald, Study Coordinator, Stephanie Badour, Study Coordinator. Toronto General Hospital, Toronto, Ontario, Canada: Nancy F. Olivieri, MD, Principal Investigator, Vivek Thayalasuthan, Study Coordinator; Satellite: Hospital for Sick Children, Isaac Odame, MD, Principal Investigator, Manuela Merelles-Pulcini, RN, Study Coordinator. University College London, John Porter, MD, Principal Investigator, Cindy Bhagwandin, Study Coordinator; Satellite: Whittington Hospital, Farrukh Shah, MD, Principal Investigator. NHLBI oversight, Kathryn Hassell, MD. Data Coordinating Center: New England Research Institutes, Sonja McKinlay, PhD, Principal Investigator, Lisa Virzi, RN, MS, MBA, Project Director, Felicia Trachtenberg, PhD, Senior Statistician.

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Trachtenberg, F.L., Gerstenberger, E., Xu, Y. et al. Relationship among chelator adherence, change in chelators, and quality of life in Thalassemia. Qual Life Res 23, 2277–2288 (2014). https://doi.org/10.1007/s11136-014-0671-2

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  • DOI: https://doi.org/10.1007/s11136-014-0671-2

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