Skip to main content
Erschienen in: Quality of Life Research 2/2021

13.09.2020

A multicenter cross-sectional study of the quality of life and iron chelation treatment satisfaction of patients with transfusion-dependent β-thalassemia, in routine care settings in Western Greece

verfasst von: Vassilis Goulas, Alexandra Kouraklis-Symeonidis, Kyriaki Manousou, Vassileios Lazaris, George Pairas, Paraskevi Katsaouni, Eugenia Verigou, Vassiliki Labropoulou, Vassiliki Pesli, Panagiotis Kaiafas, Urania Papageorgiou, Argiris Symeonidis

Erschienen in: Quality of Life Research | Ausgabe 2/2021

Einloggen, um Zugang zu erhalten

Abstract

Purpose

To evaluate health-related quality of life (HRQoL) and satisfaction with iron chelation therapy (ICT) of patients with transfusion-dependent β-thalassemia (TDT) managed under routine care conditions.

Patients and methods

This was an observational, multicenter, cross-sectional study conducted in three hospital-based Thalassemia Units of Western Greece. Patients confidentially completed the 36-item short-form (SF-36) and the “satisfaction with ICT” (SICT) instruments to assess HRQoL and ICT satisfaction respectively.

Results

One hundred and thirty-one adult TDT patients [74 female, median (IQR) age: 41 (36–47) years] were enrolled. Eighty patients (61.1%) were receiving parenteral ICT, with or without oral chelators (Group I), whereas 51 (38.9%) were only receiving oral ICT (Group II). The median SF-36 physical component summary and mental component summary scores were 76.3 and 75.7 among Group I, and 76.9 and 74.5 among Group II patients, not differing between the two groups. In their majority, Group I (84.6%) and Group II (92.9%) patients reported preferring oral ICT. Moreover, Group I patients reported greater perceived ICT effectiveness (median SICT score: 4.3 versus 4.2; p = 0.039), whereas patients receiving deferasirox-containing ICT reported higher treatment acceptance (median SICT score: 4.0 versus 3.6, p = 0.038) and greater satisfaction with the burden of their ICT (median SICT score: 4.4 versus 3.9, p = 0.033).

Conclusion

TDT patients prefer to receive oral ICT and are more satisfied of the burden of deferasirox-containing ICT, even though those receiving parenteral ICT are more satisfied by the effectiveness of their treatment. No differences in HRQoL were not noted between patients receiving parenteral versus oral ICT.
Literatur
1.
Zurück zum Zitat Taher, A.T., Weatherall, D.J., Cappellini, M.D. Thalassaemia. (2018) Lancet, 391(10116): 155–167. Taher, A.T., Weatherall, D.J., Cappellini, M.D. Thalassaemia. (2018) Lancet, 391(10116): 155–167.
2.
Zurück zum Zitat Weatherall, D. J. (2010). The inherited diseases of hemoglobin are an emerging global health burden. Blood, 115(22), 4331–4336.CrossRef Weatherall, D. J. (2010). The inherited diseases of hemoglobin are an emerging global health burden. Blood, 115(22), 4331–4336.CrossRef
3.
Zurück zum Zitat Origa, R. (2017). β-Thalassemia. Genetics in Medicine, 19(6), 609–619.CrossRef Origa, R. (2017). β-Thalassemia. Genetics in Medicine, 19(6), 609–619.CrossRef
4.
Zurück zum Zitat Koutelekos, J., & Haliasos, N. (2013). Thalassaemia. Perioperative Nursing, 2, 101–112. Koutelekos, J., & Haliasos, N. (2013). Thalassaemia. Perioperative Nursing, 2, 101–112.
5.
6.
Zurück zum Zitat Bonifazi, F., Conte, R., Baiardi, P., Bonifazi, D., Felisi, M., Giordano, P., et al. (2017). Pattern of complications and burden of disease in patients affected by beta thalassemia major. Current Medical Research and Opinion, 33(8), 1525–1533.CrossRef Bonifazi, F., Conte, R., Baiardi, P., Bonifazi, D., Felisi, M., Giordano, P., et al. (2017). Pattern of complications and burden of disease in patients affected by beta thalassemia major. Current Medical Research and Opinion, 33(8), 1525–1533.CrossRef
7.
Zurück zum Zitat Vichinsky, E., Neumayr, L., Trimble, S., Giardina, P. J., Cohen, A. R., Coates, T., et al. (2014). Transfusion complications in thalassemia patients: a report from the Centers for Disease Control and Prevention (CME). Transfusion, 54(4), 972–971.CrossRef Vichinsky, E., Neumayr, L., Trimble, S., Giardina, P. J., Cohen, A. R., Coates, T., et al. (2014). Transfusion complications in thalassemia patients: a report from the Centers for Disease Control and Prevention (CME). Transfusion, 54(4), 972–971.CrossRef
8.
Zurück zum Zitat Cappellini, M. D., Porter, J. B., Viprakasit, V., & Taher, A. T. (2018). A paradigm shift on beta-thalassaemia treatment: How will we manage this old disease with new therapies? Blood Reviews, 32(4), 300–311.CrossRef Cappellini, M. D., Porter, J. B., Viprakasit, V., & Taher, A. T. (2018). A paradigm shift on beta-thalassaemia treatment: How will we manage this old disease with new therapies? Blood Reviews, 32(4), 300–311.CrossRef
9.
Zurück zum Zitat Chonat, S., & Quinn, C. T. (2017). Current Standards of Care and Long Term Outcomes for Thalassemia and Sickle Cell Disease. Advances in Experimental Medicine and Biology, 1013, 59–87.CrossRef Chonat, S., & Quinn, C. T. (2017). Current Standards of Care and Long Term Outcomes for Thalassemia and Sickle Cell Disease. Advances in Experimental Medicine and Biology, 1013, 59–87.CrossRef
10.
Zurück zum Zitat Shah, F. T., Sayani, F., Trompeter, S., Drasar, E., & Piga, A. (2019). Challenges of blood transfusions in β-thalassemia. Blood Reviews, 37, 100588.CrossRef Shah, F. T., Sayani, F., Trompeter, S., Drasar, E., & Piga, A. (2019). Challenges of blood transfusions in β-thalassemia. Blood Reviews, 37, 100588.CrossRef
11.
Zurück zum Zitat Scalone, L., Mantovani, L. G., Krol, M., Rofail, D., Ravera, S., Bisconte, M. G., et al. (2008). Costs, quality of life, treatment satisfaction and compliance in patients with beta-thalassemia major undergoing iron chelation therapy: the ITHACA study. Current Medical Research and Opinion, 24(7), 1905–1917.CrossRef Scalone, L., Mantovani, L. G., Krol, M., Rofail, D., Ravera, S., Bisconte, M. G., et al. (2008). Costs, quality of life, treatment satisfaction and compliance in patients with beta-thalassemia major undergoing iron chelation therapy: the ITHACA study. Current Medical Research and Opinion, 24(7), 1905–1917.CrossRef
12.
Zurück zum Zitat Gan, G. G., Hue, Y. L., & Sathar, J. (2016). Factors Affecting Quality of Life in Adult Patients with Thalassaemia Major and Intermedia. Annals of the Academy of Medicine, Singapore, 45(11), 520–523.PubMed Gan, G. G., Hue, Y. L., & Sathar, J. (2016). Factors Affecting Quality of Life in Adult Patients with Thalassaemia Major and Intermedia. Annals of the Academy of Medicine, Singapore, 45(11), 520–523.PubMed
13.
Zurück zum Zitat Borgna-Pignatti, C., & Marsella, M. (2015). Iron Chelation in Thalassemia Major. Clinical Therapeutics, 37(12), 2866–2877.CrossRef Borgna-Pignatti, C., & Marsella, M. (2015). Iron Chelation in Thalassemia Major. Clinical Therapeutics, 37(12), 2866–2877.CrossRef
14.
Zurück zum Zitat Kwiatkowski, J. L. (2016). Current recommendations for chelation for transfusion-dependent thalassemia. Annals of the New York Academy of Sciences, 1368(1), 107–114.CrossRef Kwiatkowski, J. L. (2016). Current recommendations for chelation for transfusion-dependent thalassemia. Annals of the New York Academy of Sciences, 1368(1), 107–114.CrossRef
15.
Zurück zum Zitat Ware, J. E., Kosinski, M., & Keller, S. D. (1994). SF-36 Physical and Mental Health Summary Scales: A User's Manual. Boston, MA: The Health Institute. Ware, J. E., Kosinski, M., & Keller, S. D. (1994). SF-36 Physical and Mental Health Summary Scales: A User's Manual. Boston, MA: The Health Institute.
16.
Zurück zum Zitat Pappa, E., Kontodimopoulos, N., & Niakas, D. (2005). Validating and norming of the Greek SF-36 Health Survey. Quality of Life Research, 14(5), 1433–1438.CrossRef Pappa, E., Kontodimopoulos, N., & Niakas, D. (2005). Validating and norming of the Greek SF-36 Health Survey. Quality of Life Research, 14(5), 1433–1438.CrossRef
17.
Zurück zum Zitat Rofail, D., Viala, M., Gater, A., Abetz-Webb, L., Baladi, J. F., & Cappellini, M. D. (2010). An instrument assessing satisfaction with iron chelation therapy: Psychometric testing from an open-label clinical trial. Advances in Therapy, 27(8), 533–546.CrossRef Rofail, D., Viala, M., Gater, A., Abetz-Webb, L., Baladi, J. F., & Cappellini, M. D. (2010). An instrument assessing satisfaction with iron chelation therapy: Psychometric testing from an open-label clinical trial. Advances in Therapy, 27(8), 533–546.CrossRef
18.
Zurück zum Zitat Hays, R. D., & Stewart, A. L. (1992). Sleep measures. In A. L. Stewart & J. E. Ware (Eds.), Measuring Functioning and Well-being: The Medical Outcomes Study Approach (pp. 235–259). Durham, NC: Duke University Press. Hays, R. D., & Stewart, A. L. (1992). Sleep measures. In A. L. Stewart & J. E. Ware (Eds.), Measuring Functioning and Well-being: The Medical Outcomes Study Approach (pp. 235–259). Durham, NC: Duke University Press.
19.
Zurück zum Zitat Porter, J., Bowden, D. K., Economou, M., Troncy, J., Ganser, A., Habr, D., et al. (2012). Health-Related Quality of Life, Treatment Satisfaction, Adherence and Persistence in β-Thalassemia and Myelodysplastic Syndrome Patients with Iron Overload Receiving Deferasirox: Results from the EPIC Clinical Trial. Anemia, 2012, 297641.CrossRef Porter, J., Bowden, D. K., Economou, M., Troncy, J., Ganser, A., Habr, D., et al. (2012). Health-Related Quality of Life, Treatment Satisfaction, Adherence and Persistence in β-Thalassemia and Myelodysplastic Syndrome Patients with Iron Overload Receiving Deferasirox: Results from the EPIC Clinical Trial. Anemia, 2012, 297641.CrossRef
20.
Zurück zum Zitat Haghpanah, S., Zarei, T., Zahedi, Z., & Karimi, M. (2014). Compliance and satisfaction with deferasirox (Exjade®) compared with deferoxamine in patients with transfusion-dependent beta-thalassemia. Hematology, 19(4), 187–191.CrossRef Haghpanah, S., Zarei, T., Zahedi, Z., & Karimi, M. (2014). Compliance and satisfaction with deferasirox (Exjade®) compared with deferoxamine in patients with transfusion-dependent beta-thalassemia. Hematology, 19(4), 187–191.CrossRef
21.
Zurück zum Zitat Cappellini, M. D., Bejaoui, M., Agaoglu, L., Porter, J., Coates, T., Jeng, M., et al. (2007). Prospective evaluation of patient-reported outcomes during treatment with deferasirox or deferoxamine for iron overload in patients with beta-thalassemia. Clinical Therapeutics, 29(5), 909–917.CrossRef Cappellini, M. D., Bejaoui, M., Agaoglu, L., Porter, J., Coates, T., Jeng, M., et al. (2007). Prospective evaluation of patient-reported outcomes during treatment with deferasirox or deferoxamine for iron overload in patients with beta-thalassemia. Clinical Therapeutics, 29(5), 909–917.CrossRef
22.
Zurück zum Zitat Taher, A., Al Jefri, A., Elalfy, M. S., Al Zir, K., Daar, S., Rofail, D., et al. (2010). Improved treatment satisfaction and convenience with deferasirox in iron-overloaded patients with beta-Thalassemia: Results from the ESCALATOR Trial. Acta Haematologica, 123(4), 220–225.CrossRef Taher, A., Al Jefri, A., Elalfy, M. S., Al Zir, K., Daar, S., Rofail, D., et al. (2010). Improved treatment satisfaction and convenience with deferasirox in iron-overloaded patients with beta-Thalassemia: Results from the ESCALATOR Trial. Acta Haematologica, 123(4), 220–225.CrossRef
23.
Zurück zum Zitat Arian, M., Mirmohammadkhani, M., Ghorbani, R., & Soleimani, M. (2019). Health-related quality of life (HRQoL) in beta-thalassemia major (β-TM) patients assessed by 36-item short form health survey (SF-36): a meta-analysis. Quality of Life Research, 28(2), 321–334.CrossRef Arian, M., Mirmohammadkhani, M., Ghorbani, R., & Soleimani, M. (2019). Health-related quality of life (HRQoL) in beta-thalassemia major (β-TM) patients assessed by 36-item short form health survey (SF-36): a meta-analysis. Quality of Life Research, 28(2), 321–334.CrossRef
24.
Zurück zum Zitat Seyedifar, M., Dorkoosh, F. A., Hamidieh, A. A., Naderi, M., Karami, H., Karimi, M., et al. (2016). Health-Related Quality of Life and Health Utility Values in Beta Thalassemia Major Patients Receiving Different Types of Iron Chelators in Iran. International Journal of Hematology-Oncology and Stem Cell Research, 10(4), 224–231.PubMedPubMedCentral Seyedifar, M., Dorkoosh, F. A., Hamidieh, A. A., Naderi, M., Karami, H., Karimi, M., et al. (2016). Health-Related Quality of Life and Health Utility Values in Beta Thalassemia Major Patients Receiving Different Types of Iron Chelators in Iran. International Journal of Hematology-Oncology and Stem Cell Research, 10(4), 224–231.PubMedPubMedCentral
25.
Zurück zum Zitat Floris, F., Comitini, F., Leoni, G., Moi, P., Morittu, M., Orecchia, V., et al. (2018). Quality of life in Sardinian patients with transfusion-dependent Thalassemia: a cross-sectional study. Quality of Life Research, 27(10), 2533–2539.CrossRef Floris, F., Comitini, F., Leoni, G., Moi, P., Morittu, M., Orecchia, V., et al. (2018). Quality of life in Sardinian patients with transfusion-dependent Thalassemia: a cross-sectional study. Quality of Life Research, 27(10), 2533–2539.CrossRef
26.
Zurück zum Zitat Rofail, D., Abetz, L., Viala, M., Gait, C., Baladi, J. F., & Payne, K. (2009). Satisfaction and adherence in patients with iron overload receiving iron chelation therapy as assessed by a newly developed patient instrument. Value in Health, 12(1), 109–117.CrossRef Rofail, D., Abetz, L., Viala, M., Gait, C., Baladi, J. F., & Payne, K. (2009). Satisfaction and adherence in patients with iron overload receiving iron chelation therapy as assessed by a newly developed patient instrument. Value in Health, 12(1), 109–117.CrossRef
27.
Zurück zum Zitat Trachtenberg, F. L., Gerstenberger, E., Xu, Y., Mednick, L., Sobota, A., Ware, H., et al. (2014). Relationship among chelator adherence, change in chelators, and quality of life in thalassemia. Quality of Life Research, 23(8), 2277–2288.CrossRef Trachtenberg, F. L., Gerstenberger, E., Xu, Y., Mednick, L., Sobota, A., Ware, H., et al. (2014). Relationship among chelator adherence, change in chelators, and quality of life in thalassemia. Quality of Life Research, 23(8), 2277–2288.CrossRef
Metadaten
Titel
A multicenter cross-sectional study of the quality of life and iron chelation treatment satisfaction of patients with transfusion-dependent β-thalassemia, in routine care settings in Western Greece
verfasst von
Vassilis Goulas
Alexandra Kouraklis-Symeonidis
Kyriaki Manousou
Vassileios Lazaris
George Pairas
Paraskevi Katsaouni
Eugenia Verigou
Vassiliki Labropoulou
Vassiliki Pesli
Panagiotis Kaiafas
Urania Papageorgiou
Argiris Symeonidis
Publikationsdatum
13.09.2020
Verlag
Springer International Publishing
Erschienen in
Quality of Life Research / Ausgabe 2/2021
Print ISSN: 0962-9343
Elektronische ISSN: 1573-2649
DOI
https://doi.org/10.1007/s11136-020-02634-y

Weitere Artikel der Ausgabe 2/2021

Quality of Life Research 2/2021 Zur Ausgabe