Guidelines for the Standard Monitoring of Patients With Thalassemia

Tubman, Venée N.; Fung, Ellen B.; Vogiatzi, Maria; Thompson, Alexis A.; Rogers, Zora R.; Neufeld, Ellis J.; Kwiatkowski, Janet L.

doi:10.1097/mph.0000000000000307

Cited by 72 publications

(60 citation statements)

References 65 publications

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“…Similar to other chronic diseases, thalassemia, as a chronic disease, imposes serious physical, socio-mental, congenital and economic effects to the patients and their families (8). Physical problems such as chronic anemia, bone deformation, growth change, short height and delayed physical maturing on the one hand and unpleasant as well as long and repetitive therapies on the other hand affect different life aspects of the patients (9, 10).…”

Section: Introductionmentioning

confidence: 99%

Effects of Home-Care Training on the Self-Efficacy of Patients with Beta Thalassemia Major

Moghadam¹,

Nourisancho²,

Shahnazi³

et al. 2016

Mater Sociomed

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Background:The self-efficacy of thalassemia patients is an important factor in creating behavioral changes in such patients. Home-care training reduces hospitalization duration as well as relevant costs and improves disease outcomes. This study was designed to assess the effect of home-care training on the self-efficacy of patients with beta thalassemia major.Methodology:This was a quasi-experimental, case-control, before and after intervention study conducted on 136 thalassemia cases from January 2014 to October 2015. Data was collected through Shere general self-efficacy questionnaire (SGSES). Home-care trainings were provided in the form of training courses with respect to the training needs of thalassemia major cases. Two (2) months after the end of training courses, SGSES questionnaire was filled again and the obtained data was analyzed by SPSS 21 as well as descriptive-inferential statistics (significance level=P≤0.05).Results:The results of this study revealed that the mean self-efficacy score of control group was 48.69±6.82 before intervention which increased to 46.69±6.81 after intervention. The mean self-efficacy score of case group was 44.58±5.23 before intervention which increased to 49.5±6.66 after intervention. The rise of self-efficacy score, after intervention, was significantly higher in the case group compared with the control group (P≤.001).Conclusion:According to results, home-care training can develop self-efficacy in thalassemia patients. In home-care training procedure patients play an active role. By providing home-care trainings, therefore, an effective step should be taken to promote the self-efficacy of the patients and to decrease associated problems.

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Section: Introductionmentioning

confidence: 99%

Effects of Home-Care Training on the Self-Efficacy of Patients with Beta Thalassemia Major

Moghadam¹,

Nourisancho²,

Shahnazi³

et al. 2016

Mater Sociomed

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“…Nevertheless, the survival of TM patients has greatly improved over the last few decades as illustrated by large registries and observational cohorts (Brittenham et al, 1994;Calleja et al, 1998;Borgna-Pignatti et al, 2004a;Cunningham et al, 2004;Roudbari et al, 2008;Maggio et al, 2009a;Telfer, 2009;Marsella et al, 2010;Ladis et al, 2011;Kwiatkowski et al, 2012;Rajaeefard et al, 2015;Tubman et al, 2015;Zamani et al, 2015). Cunningham et al (2004), on behalf of the Thalassaemia Clinical Research Network (TCRN), reported such improvements and associated them with the dramatic advances in management of TM patients over the past 40 years.…”

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confidence: 99%

The era of comparable life expectancy between thalassaemia major and intermedia: Is it time to revisit the major‐intermedia dichotomy?

et al. 2016

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SummaryIn the last few decades, the life expectancy of regularly transfused b-thalassaemia major (TM) patients has dramatically improved following the introduction of safe transfusion practices, iron chelation therapy, aggressive treatment of infections and improved management of cardiac complications. How such changes, especially those attributed to the introduction of iron chelation therapy, improved the survival of TM patients to approach those with b-thalassaemia intermedia (TI) remains unknown. Three hundred and seventy-nine patients with TM (n = 284, dead 40) and TI (n = 95, dead 13) were followed retrospectively since birth until 30 June 2015 or death. Kaplan-Meir curves showed statistically significant differences in TM and TI survival (P < 0Á0001) before the introduction of iron chelation in 1965, which were no longer apparent after that date (P = 0Á086), reducing the Hazard Ratio of death in TM compared to TI from 6Á8 [95% confidence interval (CI) 2Á6-17Á5] before 1965 to 2Á8 (95% CI 0Á8-9Á2). These findings suggest that, in the era of iron chelation therapy and improved survival for TM, the major-intermedia dichotomy needs to be revisited alongside future directions in general management and prevention for both conditions.

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“…In transfusion‐dependent patients, iron overload develops after 1–2 years of regular transfusions, but hemosiderosis is also present in nontransfusion‐dependent thalassemia patients, likely because anemia and ineffective erythropoiesis cause suppression of hepcidin and increased iron absorption . Iron toxicity from hemosiderosis seems to increase reactive oxygen species (ROS) in bone marrow, worsening ineffective erythropoiesis by apoptosis induction.…”

Section: Discussionmentioning

confidence: 99%

Ringed sideroblasts in β‐thalassemia

Cattivelli

Campagna

Schmitz-Abe

et al. 2016

Pediatric Blood & Cancer

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Symptomatic β-thalassemia is one of the globally most common inherited disorders. The initial clinical presentation is variable. Whereas common hematological analyses are typically sufficient to diagnose the disease, sometimes the diagnosis can be more challenging. We describe a series of patients with β-thalassemia whose diagnosis was delayed, required bone marrow examination in one affected member of each family, and revealed ringed sideroblasts, highlighting the association of this morphological finding with these disorders. Thus, in the absence of characteristic congenital sideroblastic mutations or causes of acquired sideroblastic anemia, the presence of ringed sideroblasts should raise the suspicion of β-thalassemia.

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Guidelines for the Standard Monitoring of Patients With Thalassemia

Cited by 72 publications

References 65 publications

Effects of Home-Care Training on the Self-Efficacy of Patients with Beta Thalassemia Major

Effects of Home-Care Training on the Self-Efficacy of Patients with Beta Thalassemia Major

The era of comparable life expectancy between thalassaemia major and intermedia: Is it time to revisit the major‐intermedia dichotomy?

Ringed sideroblasts in β‐thalassemia

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