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Association between reported medication adherence and health-related quality of life in adult patients with haemophilia

International Journal of Clinical Pharmacy volume 43, pages 1500–1507 (2021)Cite this article

Abstract

Background Medication adherence is an important issue, not just health-related, for patients with haemophilia. Poor medication adherence to long-term therapies limits the potential of effective treatments to improve patients’ health-related quality of life. Objective The aim of this study was to investigate the association of reported medication adherence and health-related quality of life in patients with haemophilia. Setting Data were collected from patients at University Hospital Centre Zagreb, Croatia and at University Medical Centre Ljubljana, Slovenia. Method Adult male patients with severe or moderate haemophilia receiving prophylactic treatment were eligible for the study. Main outcome measure Implementation phase of medication adherence was assessed with the self-reported VERITAS-Pro instrument and health-related quality of life with SF-36v2. Results A total of 82 participants were included in the study (median age was 44.50, range 18–73 years). The majority of our participants reported being adherent to medication (83%). Participants showed better health in the mental health domains and Mental Component Summary than in the physical health domains and Physical Component Summary. After controlling for demographic, socioeconomic and clinical predictors, better reported medication adherence explained an additional 4–6% of better health variance in Bodily Pain and Social Functioning domains and Mental Component Summary. Conclusion We found that reported medication adherence can contribute to better health-related quality of life in patients with haemophilia. Since life with a chronic condition is demanding, it is an important finding that medication adherence to replacement therapy can improve life conditions for patients with haemophilia.

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References

  1. Srivastava A, Brewer AK, Mauser-Bunschoten EP, Key NS, Kitchen S, Llinas A, et al. Guidelines for the management of hemophilia. Haemophilia. 2013;19:e1–47.

    Article  CAS  Google Scholar 

  2. Stonebraker JS, Bolton-Maggs PHB, Soucie JM, Walker I, Brooker M. A study of variations in the reported haemophilia A prevalence around the world. Haemophilia. 2010;16:20–32.

    Article  CAS  Google Scholar 

  3. Stonebraker JS, Bolton-Maggs PHB, Michael Soucie J, Walker I, Brooker M. A study of variations in the reported haemophilia B prevalence around the world. Haemophilia. 2012;18:e91–4.

    Article  CAS  Google Scholar 

  4. Orphanet. The portal for rare diseases and orphan drug. https://www.orpha.net/consor/cgi-bin/OC_Exp.php?Lng=GB&Expert=448. Accessed 29 Mar 2021.

  5. Atilla B, Güney-Deniz H. Musculoskeletal treatment in haemophilia. EFORT Open Rev. 2019;4:230–9.

    Article  Google Scholar 

  6. Van Vulpen LFD, Holstein K, Martinoli C. Joint disease in haemophilia: pathophysiology, pain and imaging. Haemophilia. 2018;24:44–9.

    Article  Google Scholar 

  7. O’Hara J, Walsh S, Camp C, Mazza G, Carroll L, Hoxer C, et al. The impact of severe haemophilia and the presence of target joints on health-related quality-of-life. Health Qual Life Outcomes. 2018;16:1–8.

    Article  Google Scholar 

  8. Zupancic Salek S (eds) Smjernice za dijagnostiku i liječenje hemofilije Zagreb Fotosoft d.o.o 978-953-7064-21-1 (2013)

  9. World Health Organization. Adherence to long-term therapies: Evidence for action. Sabate E, editor. Geneva: World Health Organization; 2003. ISBN: 92 4 154599 2

  10. Jüngst C, Gräber S, Simons S. Medication adherence among patients with chronic diseases: a survey-based study in pharmacies. QJM. 2019;112:505–12.

    Article  Google Scholar 

  11. Llewellyn CD, Miners AH, Lee CA, Harrington C, Weinman J. The illness perceptions and treatment beliefs of individuals with severe haemophilia and their role in adherence to home treatment. Psychol Health. 2003;18:185–200.

    Article  Google Scholar 

  12. Torres-Ortuño A, Cuesta-Barriuso R, Nieto-Munuera J, Galindo-Piñana P, López-Pina JA. The behaviour and perception of illness: modulating variables of adherence in patients with haemophilia. Vox Sang. 2018;113:577–83.

    Article  Google Scholar 

  13. Krishnan S, Vietri J, Furlan R, Duncan N. Adherence to prophylaxis is associated with better outcomes in moderate and severe haemophilia: results of a patient survey. Haemophilia. 2015;21:64–70.

    Article  CAS  Google Scholar 

  14. Ho S, Gue D, McIntosh K, Bucevska M, Yang M, Jackson S. An objective method for assessing adherence to prophylaxis in adults with severe haemophilia. Haemophilia. 2014;20:39–43.

    Article  CAS  Google Scholar 

  15. Tran DQ, Barry V, Antun A, Ribeiro M, Stein S, Kempton CL. Physician trust and depression influence adherence to factor replacement: a single-centre cross-sectional study. Haemophilia. 2017;23:98–104.

    Article  CAS  Google Scholar 

  16. De Moerloose P, Urbancik W, Van Den Berg HM, Richards M. A survey of adherence to haemophilia therapy in six European countries: results and recommendations. Haemophilia. 2008;14:931–8.

    Article  Google Scholar 

  17. Conn VS, Ruppar TM, Enriquez M, Cooper P. Medication adherence interventions that target subjects with adherence problems: systematic review and meta-analysis. Res Social Adm Pharm. 2016;12:218–46.

    Article  Google Scholar 

  18. Limperg PF, Terwee CB, Young NL, Price VE, Gouw SC, Peters M, et al. Health-related quality of life questionnaires in individuals with haemophilia: a systematic review of their measurement properties. Haemophilia. 2017;23:497–510.

    Article  CAS  Google Scholar 

  19. Varaklioti A, Kontodimopoulos N, Katsarou O, Niakas D. Psychometric properties of the Greek Haem-A-QoL for measuring quality of life in Greek haemophilia patients. BioMed Res Int. 2014;2014:968081.

    Article  Google Scholar 

  20. Faranoush M, Shahverdi E, Ghorbani R, Moghaddam M. Health-related quality of life in Iranian adult men with severe hemophilia. Blood Coagul Fibrinol. 2017;28:638–41.

    Article  Google Scholar 

  21. Aledort L, Bullinger M, von Mackensen S, Wasserman J, Young NL, Globe D, et al. Why should we care about quality of life in persons with haemophilia? Haemophilia. 2012;18:e154–7.

    Article  CAS  Google Scholar 

  22. Oladapo AO, Epstein JD, Williams E, Ito D, Gringeri A, Valentino LA. Health-related quality of life assessment in haemophilia patients on prophylaxis therapy: a systematic review of results from prospective clinical trials. Haemophilia. 2015;21:e344–58.

    Article  CAS  Google Scholar 

  23. Lindvall K, Von Mackensen S, Berntorp E. Quality of life in adult patients with haemophilia—a single centre experience from Sweden. Haemophilia. 2012;18:527–31.

    Article  CAS  Google Scholar 

  24. Duncan N, Shapiro A, Ye X, Epstein J, Luo MP. Treatment patterns, health-related quality of life and adherence to prophylaxis among haemophilia A patients in the United States. Haemophilia. 2012;18:760–5.

    Article  CAS  Google Scholar 

  25. PASS 15 Power Analysis and Sample Size Software [Computer software]. NCSS, LLC. Kaysville; available from: https://ncss.com/software/pass (2017). Accessed 15 Jun 2017.

  26. Cohen J. Statistical power analysis for the behavioral sciences. 2nd ed. Hillsdale: Lawrence Erlbaum Associates; 1988.

    Google Scholar 

  27. Duncan N, Kronenberger W, Roberson C, Shapiro A. VERITAS-Pro: a new measure of adherence to prophylactic regimens in haemophilia. Haemophilia. 2010;16:247–55.

    Article  CAS  Google Scholar 

  28. Beaton DE, Bombardier C, Guillemin F, Ferraz MB. Guidelines for the process of cross-cultural adaptation of self-report measures. Spine. 2000;25(24):3186–91.

    Article  CAS  Google Scholar 

  29. Miesbach W, Kalnins W. Adherence to prophylactic treatment in patients with haemophilia in Germany. Haemophilia. 2016;22:e367-374.

    Article  CAS  Google Scholar 

  30. van Os SB, Troop NA, Sullivan KR, Hart DP. Adherence to prophylaxis in adolescents and young adults with severe haemophilia: a quantitative study with patients. PLoS ONE. 2017;12(1):e0169880.

    Article  Google Scholar 

  31. Maruish ME (Ed.). User’s manual for the SF-36v2 Health Survey (3rd ed.). Lincoln, RI: QualityMetric Incorporated. ISBN: 1–891810–28–6

  32. Babic-Banaszak A, Kovacic L, Kovacevic L, Vuletic G, Mujkic A, Ebling Z. Impact of war on health related quality of life in Croatia: population study. Croat Med J. 2002;43:396–402.

    PubMed  Google Scholar 

  33. Juresa V, Ivankovic G, Vuletic G, Babic-Banaszak I, Srcek I, Mastilica M, et al. The croatian health survey—SF-36: I. General quality of life assessment. Coll Antropol. 2000;24:69–78.

    CAS  PubMed  Google Scholar 

  34. Maslic Sersic D, Vuletic G. Psychometric evaluation and establishing norms of Croatian SF-36 health survey: framework for subjective health research. Croat Med J. 2006;47:95–102.

    PubMed  PubMed Central  Google Scholar 

  35. Logar Zakrajšek B, Bren A, Sočan G, Pajek J. Pilotna raziskava psihometričnih lastnosti vprašalnikov SF-36v2 in ESRD-SCL-TM za merjenje z zdravjem povezane kakovosti življenja bolnikov po presaditvi ledvice. Psihol Obz. 2018;12:1–11.

    Google Scholar 

  36. IBM Corp. IBM SPSS Statistics for Windows, Version 26.0. Armonk, NY: IBM Corp. Released 2019.

  37. Ryu E. Effects of skewness and kurtosis on normal-theory based maximum likelihood test statistic in multilevel structural equation modeling. Behav Res Methods. 2011;43:1066–74.

    Article  Google Scholar 

  38. Kim HY. Statistical notes for clinical researchers: assessing normal distribution (2) using skewness and kurtosis. Restor Dent Endod. 2013;38:52.

    Article  Google Scholar 

  39. Dolatkhah R, Fakhari A, Pezeshki MZ, Shabanlouei R, Tavassoli N, Gholchin M. Social determinants and health-related dimensions of quality of life in adult patients with haemophilia. Haemophilia. 2014;20:376–81.

    Article  CAS  Google Scholar 

  40. Cuesta-Barriuso R, López-Pina JA, Nieto-Munuera J, Sagarra-Valls G, Panisello-Royo JM, Torres-Ortuño A. Effectiveness of the Medtep Hemophilia online platform for adherence to prophylactic treatment in haemophilia patients: results from a 1-year observational study. Haemophilia. 2018;24:452–9.

    Article  CAS  Google Scholar 

  41. Kempton CL, Recht M, Neff A, Wang M, Buckner TW, Soni A, et al. Impact of pain and functional impairment in US adults with haemophilia: Patient-reported outcomes and musculoskeletal evaluation in the pain, functional impairment and quality of life (P-FiQ) study. Haemophilia. 2018;24:261–70.

    Article  CAS  Google Scholar 

  42. Windyga J, Lin VW, Epstein JD, Ito D, Xiong Y, Abbuehl BE, et al. Improvement in health-related quality of life with recombinant factor IX prophylaxis in severe or moderately severe haemophilia B patients: results from the BAX326 Pivotal Study. Haemophilia. 2014;20:362–8.

    Article  CAS  Google Scholar 

  43. Stirratt MJ, Dunbar-Jacob J, Crane HM, Simoni JM, Czajkowski S, Hilliard ME, et al. Self-report measures of medication adherence behavior: recommendations on optimal use. Transl Behav Med. 2015;5:470–82.

    Article  Google Scholar 

  44. Guedes VG, Corrente JE, Farrugia A, Thomas S, Wachholz PA, de Oliveira Vidal EI. Comparing objective and self-reported measures of adherence in haemophilia. Haemophilia. 2019;25:821–30.

    Article  Google Scholar 

  45. Tiede A, Bonanad S, Santamaria A, Goldmann G, Canaro M, Palomero A, et al. Quality of electronic treatment records and adherence to prophylaxis in haemophilia and von Willebrand disease: systematic assessments from an electronic diary. Haemophilia. 2020;26:999–1008.

    Article  CAS  Google Scholar 

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Acknowledgements

The authors would like to thank all the participants who took part in the study. The authors would like to thank the Indiana Hemophilia & Thrombosis Center, Inc. for providing VERITAS-Pro and OptumInsight Life Sciences, Inc. for providing SF-36v2 for use in this study. The authors would also like to thank Bianca Dobson for language editing of this publication.

Funding

No special funding was obtained.

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Authors and Affiliations

  1. Andrija Stampar Teaching Institute of Public Health, Zagreb, Croatia

    Martina Bago & Ivana Prga

  2. Faculty of Humanities and Social Sciences, University of Zagreb, Zagreb, Croatia

    Ana Butkovic

  3. University Medical Center Ljubljana, Ljubljana, Slovenia

    Irena Preloznik Zupan & Barbara Faganel Kotnik

  4. Faculty of Medicine, University of Ljubljana, Ljubljana, Slovenia

    Irena Preloznik Zupan & Barbara Faganel Kotnik

  5. Clinical Hospital Dubrava, Zagreb, Croatia

    Vesna Bacic Vrca

  6. Faculty of Pharmacy and Biochemistry, University of Zagreb, Zagreb, Croatia

    Vesna Bacic Vrca

  7. University Hospital Centre Zagreb, Zagreb, Croatia

    Silva Zupancic Salek

  8. Faculty of Medicine, University of Zagreb, Zagreb, Croatia

    Silva Zupancic Salek

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  1. Martina Bago
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  2. Ana Butkovic
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Correspondence to Martina Bago.

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Bago, M., Butkovic, A., Preloznik Zupan, I. et al. Association between reported medication adherence and health-related quality of life in adult patients with haemophilia. Int J Clin Pharm 43, 1500–1507 (2021). https://doi.org/10.1007/s11096-021-01270-x

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  • DOI: https://doi.org/10.1007/s11096-021-01270-x

Keywords

  • Haemophilia A
  • Haemophilia B
  • Medication adherence
  • Quality of life