Quality of Life and Related Factors in β-Thalassemia Patients

Etemad, Koorosh; Mohseni, Parisa; Aghighi, Mohammad; Bahadorimonfared, Ayad; Hantooshzadeh, Razieh; Taherpour, Niloufar; Piri, Negar; Ghorbani, Sahar Sotoodeh; Malek, Fatemeh; Kheiry, Fatemeh; Khodami, Azimeh; Valadbeigi, Tannaz; Hajipour, Mahmoud

doi:10.1080/03630269.2021.1965617

Cited by 14 publications

(18 citation statements)

References 26 publications

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“…Finally, economic status is an important variable related to HRQOL impairment in developing countries, where psychiatric disorders are widely recognized as disturbing factors in physical and mental domains. Furthermore, regular blood transfusion leads to a high risk of transfusion-mediated infection in thalassemia patients and related HRQOL impairment in developing countries, in addition to common complications, such as iron overload, due to inadequate awareness and poor screening practices ( 13 ).…”

Section: Discussionmentioning

confidence: 99%

“…Fatigue is an independent predictor of low HRQOL that has been associated with lower executive functioning and working memory of affected people (5,(8)(9)(10). Concurrently, regular blood transfusions and disease complications in beta-thalassemia patients affect HRQOL, and iron overload, comorbidities, and fatigue are the most represented etiologies of impaired life functioning (11)(12)(13). Furthermore, patients can encounter stigma for other reasons, such as race, disability, socioeconomic status, and delayed growth and puberty (3)(4)(5)(6)(7)(8)(9)(10)(11)(12)(13)(14).…”

Section: Introductionmentioning

confidence: 99%

“…Concurrently, regular blood transfusions and disease complications in beta-thalassemia patients affect HRQOL, and iron overload, comorbidities, and fatigue are the most represented etiologies of impaired life functioning (11)(12)(13). Furthermore, patients can encounter stigma for other reasons, such as race, disability, socioeconomic status, and delayed growth and puberty (3)(4)(5)(6)(7)(8)(9)(10)(11)(12)(13)(14). The heterogeneous distribution of treatment opportunities and multidisciplinary management worldwide can also influence HRQOL perception in different settings (3,4).…”

Section: Introductionmentioning

confidence: 99%

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Health-related quality of life in hemoglobinopathies: A systematic review from a global perspective

2022

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BackgroundSickle cell disease (SCD) and thalassemia are inherited blood disorders, which can lead to life-threatening events and chronic organ damage. Recent advances in treatments have increased life expectancy, and hemoglobinopathies have become chronic illnesses with social and emotional impairments. Thus, health-related quality of life (HRQOL) assessment has a fundamental role in disease management and treatment, and generic and disease-specific questionnaires are reliable and validated measures to estimate disease burden. The heterogeneous distribution of treatment opportunities worldwide influences physical, social, and emotional disease perception.ObjectivesTo review publications concerning HRQOL for SCD and thalassemia in different areas of the world in order to gather a global perspective of questionnaires used and outcomes evaluated.MethodsA systematic review of the literature was conducted according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines. The Medline databases were searched on 29 September 2021. Inclusion criteria were as follows: (1) studies of HRQOL assessment in SCD and thalassemia patients by using the PROMIS, the SF-36, the SCSES, the PedsQL-SCD, the PedsQOL generic core scale, the ASCQ-Me, and the TranQoL; and (2) every article type, including non-English studies. We excluded studies that were not limited to SCD or thalassemia and studies that were not specific to hemoglobinopathies, and not consistent with the topic of HRQOL assessment. We did not include the gray literature. A total of 102 out of 124 articles from PubMed, Cochrane Library, and Google Scholar were eligible for inclusion (66 SCD articles and 36 thalassemia articles). The quality of studies was assessed through Critical Appraisal tools for use in JBI Systematic Reviews. Data extraction was conducted using a standardized data collection form (authors, year and country of publication, study design, age and number of patients, HRQOL questionnaires, questionnaire language, and clinical outcomes).ResultsThe evaluation of HRQOL was conducted on all continents, but differences in the worldwide frequency of HRQOL assessment were observed. HRQOL of SCD patients was less investigated in Europe. HRQOL of thalassemia patients was less investigated in South-East Asia and Africa. Generic HRQOL questionnaires (PROMIS, SF-36, and PedsQL) were frequently adopted, while disease-specific ones (ASCQ-Me, SCSES for SCD, and TranQoL for thalassemia) were less used. Translation into local languages has been often performed.ConclusionHealth-related quality of life is a complex outcome that has been increasingly incorporated in clinical research and clinical practice worldwide, although with regional differences. Disease-specific outcomes (pain for SCD and transfusion burden for thalassemia) and healthcare system characteristics, particularly in low-income countries, have an impact on HRQOL and should be considered in healthcare plans.

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Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Health-related quality of life in hemoglobinopathies: A systematic review from a global perspective

2022

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“…The WHO ( 16 ) defined the QoL as: “An individual's perception of their position in life in the context of the culture and value systems in which they live and concerning their goals, expectations, standards, and concerns” ( 17 ). Patients with β-TM had a substantially lower QoL in comparison with the general population ( 18 ).…”

Section: Introductionmentioning

confidence: 99%

Predictors of health state utility values using SF-6D for Chinese adult patients with β-thalassemia major

Zhang

Ming

et al. 2023

Front. Public Health

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BackgroundPatients with β-thalassemia major (β-TM), predominantly adult patients, are associated with physical, mental, and social problems, that result in decreased quality of life (QoL). However, there is a paucity of data on QoL and health status utility (HSU) among adult patients with β-TM in mainland China. Our study aimed to evaluate the QoL by short form 36 questionnaire (SF-36) of adult patients with β-TM in mainland China and to estimate their HSU by SF-6D. In addition, we aimed to identify predictors of HSU.MethodsIn this cross-sectional descriptive study, a total of 75 adult patients with β-TM were included by the snowball sampling method that applied involving seven provinces with a relatively high prevalence of thalassemia across mainland China between September 1, 2021 and January 31, 2022. The collected information included social-demographic characteristics, health conditions, treatment, social support (social support rating scale), caregiver burden (Zarit burden interview), and QoL (SF-36). HSU scores were calculated for each adult patient from their SF-36 responses using the SF-6D algorithm with Hong Kong's tariff. The frequency of participants' responses to the SF-6D for each item of the options was described. Mean HSU scores between different subgroups were calculated. Ordinary least squares (OLS) regression modeling was performed to identify factors associated with HSU.ResultsA total of 75 adult patients with β-TM were included in this study. The mean SF-36 score was 50.2 ± 10.70, of which physical and mental scores were 47.57 ± 11.28 and 52.85 ± 14.21, respectively. In addition, the mean SF-6D utility score was estimated to be 0.598 ± 0.112, ranging from 0.391 to 0.962. Univariate analyses showed that interruption of iron chelation treatment significantly affected HSU values (P = 0.038); diagnosis with comorbidity very slightly affected HSU values (P = 0.0996). In the multivariate analysis, diagnosis with comorbidity (P = 0.042) was significantly negatively associated with HSU values; the minimum pre-transfusion hemoglobin concentration (P = 0.047) and social support (P = 0.068) were positively associated with HSU values.ConclusionThis study presents poor QoL and HSU outcomes in Chinese adult patients with β-TM. The study also highlights the importance of social support and treatment compliance, which can increase hemoglobin content and reduce comorbidities, further to ensure the QoL of patients. These findings can be used for future clinical and economic studies.

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“…Thalassaemia is one of the most common monogenic hereditary disorders worldwide. 1 It is an autosomal recessive disorder and offspring inherits this disorder from their parents. 2 This is a chronic haemolytic anaemia where there is abnormal production of haemoglobinresulted from partial or complete deficiency in the synthesis of Alpha and Beta globin chain, followed by excessive destruction of red blood cells.…”

Section: Introductionmentioning

confidence: 99%

Frequency of consanguineous marriage among the thalassaemia major patients in Bangabandhu Sheikh Mujib Medical University

Mahzabin

Islam

Dey

et al. 2022

Bangabandhu Sheikh Mujib Medical University Journal

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Thalassaemia is a commonly occurring hereditary disorder. There is a high prevalence of thalassaemia disease in South-East Asia as well as Bangladesh. It is an autosomal recessive disorder, so consanguineous marriage is a very important factor for this disease. Mutated beta globin gene of haemoglobin from both parents is responsible for this disease to occur. But when the number of thalassaemia carriers is miserably high, then only avoidance of consanguineous marriage can’t prevent the birth of children with thalassaemia major. So, this cross sectional study was conducted among 120 diagnosed (by haemoglobin electrophoresis or high performance liquid chromatography) thalassaemia major patients in the Department of Haematology, Bangabandhu Sheikh Mujib Medical University, Dhaka from July 2019 to May 2020. History from each patient was taken and blood samples were collected from their parents to confirm carrier state by haemoglobin electrophoresis. Blood samples were also collected from patients and their parents toperform complete blood count and peripheral Blood Film. This study showed the mean age of the participants was 15±9.34 and 73 (60.8%) patients were male and 47 (39.2%) were female. About, 71% of the study population lived in urban area, 81 (67%) patients were transfusion dependent thalassaemia patients. 15% thalas- saemia major patients had the history of consanguinity of their parents. Among the parents of the thalassaemia major patients, 62.5% were Beta thalassaemia trait (heterozygous state), 25.83% parents were Hb E trait ( heterozygous state), 7.08% parents were suffering from Beta Thalassaemia Major(homozygous state) and 11 (4.58%) parents were suffering from Hb E/Beta Thalassaemia which is a compound heterozygous state. Though consanguinity is a very influential factor, but the most important causative factor is the presence of high number of thalassaemia carrier in the population. So, it’s a burning issue for the nation to perform a routine carrier screening for all rather than focusing on consanguinity only, to lessen the burden of thalassaemia disease in Bangladesh. BSMMU J 2022; 15(1): 25-28

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Quality of Life and Related Factors in β-Thalassemia Patients

Cited by 14 publications

References 26 publications

Health-related quality of life in hemoglobinopathies: A systematic review from a global perspective

Health-related quality of life in hemoglobinopathies: A systematic review from a global perspective

Predictors of health state utility values using SF-6D for Chinese adult patients with β-thalassemia major

Frequency of consanguineous marriage among the thalassaemia major patients in Bangabandhu Sheikh Mujib Medical University

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