Clinical and health-related quality of life outcomes of transfusion-dependent thalassaemia patients in Singapore

Lam, Joyce Ching Mei; Lee, Shir Ying; Koh, Pei Lin; Fong, Sing Zern; Abdul-Kadir, Nur Insyirah; Lim, Chun Yi; Zhang, Xiao; Bhattacharyya, Rahul; Soh, Shui Yen; Chan, Mei Yoke; Tan, Ah Moy; Kuperan, Ponnudurai; Ang, Ai Leen

doi:10.1016/j.bcmd.2021.102547

Cited by 11 publications

(17 citation statements)

References 13 publications

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“…In Singapore, increasing age and reception of social assistance were the only factors that were significantly associated with lower overall TranQoL scores. Endocrine complications and older age were associated with lower TranQoL scores; however, they did not show statistical significance ( 114 ).…”

Section: Resultsmentioning

confidence: 99%

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: Resultsmentioning

confidence: 99%

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

2022

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“…Singapore has a thalassemia carrier rate of about 4.49%. 8 Although TDT incidence is now low following a successful national screening program since the early 1990s, there are still about 100 TDT 8 and more NTDT patients in Singapore. Our institution is one of Singapore's tertiary adult thalassemia centers with over 100 thalassemia patients on active follow-up.…”

Section: Introductionmentioning

confidence: 99%

Non‐transfusion dependent thalassemia is independently associated with higher alloimmunization risk than transfusion dependent thalassemia and would benefit the most from extended red cell antigen‐matching

Ang

Lim

et al. 2021

Transfusion

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Background: Alloimmunization prevalence is conventionally used to identify RBCs alloimmunization risk factors among thalassemia patients, but it may be confounded by differences in transfusion exposure especially between nontransfusion dependent thalassemia (NTDT) and transfusion dependent thalassemia (TDT) patients. To better identify thalassemia patients with high alloimmunization risks, we used cumulative incidence of first alloimmunization as a function of RBCs transfused to compare alloimmunization risks between TDT and NTDT and to evaluate other risk factors. We also proposed practical strategies to prevent alloimmunization in thalassemia.Study Design and Methods: Adult TDT and NTDT patients who had received ≥2 transfusions and no alloimmunization before their first transfusion were included. Alloimmunization was defined as the development of clinically significant alloantibodies. We estimated the first alloimmunization incidence from transfusion by Kaplan-Meier analysis with the horizontal axis expressed as cumulative non-antigen-matched RBC units transfused. We compared this incidence between TDT and NTDT, and analyzed for other alloimmunization risk factors and the alloantibody specificities/frequencies. Results: The alloimmunization prevalence was similar between TDT and NTDT (27% vs. 30% respectively, p = .726). However, for the same transfusion exposure, NTDT had higher alloimmunization incidence than TDT (hazard ratio 8.59, 95% confidence interval [2.25-32.74], p = .002), independent of age at first transfusion and last follow-up, gender, and splenectomy. Anti-E, anti-c, anti-Mi a , and anti-Jk a were most frequent.Discussion: NTDT has the highest alloimmunization risk and would benefit the most from extended RBC antigen-matching, especially C, c, E, and e. Other blood group antigen-matching should be guided by the patient/donor disparities and alloantibody frequencies in different populations.

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“…The published articles were of worldwide origins and were conducted in countries including the United Kingdom, Italy, the United States, Canada, Australia, Singapore, Malaysia, Thailand, Iran, Egypt, Syria, and India. Most of the studies were cross-sectional studies (N=16, 80% ) [11][12][13][14][15][16][17][18][19][20][21][22][23][24][25][26], followed by cohort studies (N=3, 15%) [27][28][29], and only one randomised controlled trial [30]. The majority of the studies were single-centred (N=13, 65%) and about 30% of the studies were conducted in multiple settings [12,17,24,25,27,30], while the remaining study did not mention the setting [11] A variety of medication measures was used across the studies.…”

Section: Study Characteristicsmentioning

confidence: 99%

“…Most of the studies were cross-sectional studies (N=16, 80% ) [11][12][13][14][15][16][17][18][19][20][21][22][23][24][25][26], followed by cohort studies (N=3, 15%) [27][28][29], and only one randomised controlled trial [30]. The majority of the studies were single-centred (N=13, 65%) and about 30% of the studies were conducted in multiple settings [12,17,24,25,27,30], while the remaining study did not mention the setting [11] A variety of medication measures was used across the studies. Most of the studies (N= 7, 35%) examined the rate of compliance using the frequency of ICT's administration [11,12,14,16,18,19,23], followed by studies that evaluated patients' compliance status using vial or pill count (N=5, 25%) [13,[27][28][29][30].…”

Section: Study Characteristicsmentioning

confidence: 99%

“…Most of the studies (N= 7, 35%) examined the rate of compliance using the frequency of ICT's administration [11,12,14,16,18,19,23], followed by studies that evaluated patients' compliance status using vial or pill count (N=5, 25%) [13,[27][28][29][30]. Several studies (N = 3, 15%) used the Likert Scale [17,21,26], while some studies (N = 3, 15%) measured compliance using self-reported questionnaires such as Morisky Medication Adherence Scale (MMAS-8) [22], the Medication Compliance Questionnaire (MCQ) [24], and standardised questionnaire (not mentioned in the study) [25].…”

Section: Study Characteristicsmentioning

confidence: 99%

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The Impact of Chelation Compliance in Health Outcome and Health-Related Quality of Life in Thalassaemia Patients: A Systematic Review

Jin

Ain

et al. 2022

Preprint

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Understanding the consequences of poor chelation compliance is crucial given the enormous burden of post-transfusional iron overload complications. We systematically reviewed iron-chelation therapy (ICT) compliance, and the relationship between compliance with health outcome and health-related quality of life (HRQoL) in thalassaemia patients. Several reviewers performed systematic search strategy of literature through PubMed, Scopus, and EBSCOhost. The preferred reporting items of systematic reviews and meta-analyses (PRISMA) guidelines were followed. Of 4917 studies, 20 publications were included. The ICT compliance rate ranges from 20.93% to 75.3%. It also varied per agent, ranging from 48.84-85.1% for desferioxamine, 87.2-92.2% for deferiprone and 90-100% for deferasirox. Majority of studies (N=10/11, 90.91%) demonstrated significantly negative correlation between compliance and serum ferritin, while numerous studies revealed poor ICT compliance linked with increased risk of liver disease (N=4/7, 57.14%) and cardiac disease (N=6/8, 75%), endocrinologic morbidity (N=4/5, 90%), and lower HRQoL (N=4/6, 66.67%). Inadequate compliance with ICT therapy is common. Higher compliance is correlated with the lower serum ferritin, lower risk of complications, and higher HRQoL. These findings should be interpreted with caution given the few numbers of evidence.

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Clinical and health-related quality of life outcomes of transfusion-dependent thalassaemia patients in Singapore

Cited by 11 publications

References 13 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

Non‐transfusion dependent thalassemia is independently associated with higher alloimmunization risk than transfusion dependent thalassemia and would benefit the most from extended red cell antigen‐matching

The Impact of Chelation Compliance in Health Outcome and Health-Related Quality of Life in Thalassaemia Patients: A Systematic Review

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