Application of Self-Efficacy Theory in Adherence to Iron Chelation Therapy: A Single-Center Cross-Sectional Study

Blood ◽  
2011 ◽  
Vol 118 (21) ◽  
pp. 5284-5284
Author(s):  
Kevin H.M. Kuo ◽  
Richard Ward
Keyword(s):  
Family Support ◽  
Self Efficacy ◽  
Chelation Therapy ◽  
Care Center ◽  
Insurance Coverage ◽  
Iron Chelation ◽  
Comprehensive Care ◽  
Iron Chelation Therapy ◽  
Cross Sectional ◽  
Significant Difference

Abstract 5284 Introduction: Poor adherence to iron chelation therapy (ICT) in beta-Thalassemia Major (TM) is associated with increased risk of cardiac complications and endocrinopathies, and lower survival, with substantial cost to the patient and the health care system. Canada is unique in that several predictors of non-adherence (Financial barriers to medical care, cost of medication and inadequate follow-up) are minimized due to the presence of universal health care, governmental subsidies for medications for patients with chronic disease, and the availability of comprehensive care center for most of the thalassemia patients in the country. Also, the availability of Deferiprone (DFP) via compassionate release program since July 2004 provides an alternative to patients intolerant or having suboptimal response to Deferoxamine (DFO) or Deferasirox (DFX). We hypothesize that the absence of these barriers improve adherence in the Canadian thalassemic population. We also explored self-efficacy as a concept of adherence behavior in our patient population, defined as “individuals' personal beliefs regarding their capabilities to carry out a specific task to achieve a desired outcome” (Bandura, 1989). Methods: A cross-sectional survey was conducted in June and July 2011 at a regional comprehensive care center for transfusion-dependent thalassemia patients. We assessed the age, sex, education, employment status, insurance coverage, types and dosage of ICT, self-reported level of adherence, and side effects. We adapted the Medication Adherence Self-Efficacy Scale (MASES) to assess self-efficacy (Ogedegbe, 2003). Results: Survey return rate was 45% (46/103), with each type of ICT proportionally represented (P = 0.6401). Eight surveys were discarded due to incompletion and 38 were analyzed. Thirty-two patients were on single agent ICT (6 on DFO, 23 on DFX, 3 on DFP) and 6 patients were on combination treatment (1 on DFO+DFX; 3 on DFO+DFP; 2 on DFX+DFP). Median duration of iron chelation was more than 10 years. All patients had either government (n = 10) or workplace (n = 28) coverage. Twenty-three patients (61%) were self-described as completely adherent and 15 were not completely adherent. Mean level of adherence is 90% (SD 16%), similar to those reported in the literature (Trachtenberg et al., 2011), with no significant difference between the different types of ICT (P = 0.1085). Half of the non-adherent patients (8/15, 53%) miss 1 prescribed day of medication per week. There was no significant difference between adherent and non-adherent patients in age (P = 0.1484), sex (P = 0.3764), type of insurance coverage (P = 4752), family support (P = 0.7190), type of ICT (P = 0.0611), participation and satisfaction with the Exjade Patient Support Program (P = 1.000 and 0.3012 respectively), duration of chelation (P = 0.3951), rate of side effects (P = 0.4167), or feelings of depression (P = 0.4780). There was a trend towards differences in education level (P = 0.0565) and a higher proportion of professionals in the non-adherent group. The mean self-efficacy score of patients self-described as completely adherent was significantly higher than the non-completely adherent group (2.66 vs 1.93, P<0.0001). Discussion: In this self-reported survey of patients on ICT in a Canadian regional comprehensive care center, age, presence of family support, and feelings of depression were not found to be a significant predictor of poor adherence, unlike previous studies. This could be because previous studies only examined certain types of ICTs whereas the present study examined all forms of chelation. Small sample sizes of patients on DFO and DFP is the main limitation of the study. This is also the first known application of self-efficacy theory in explaining adherence to ICT. Further studies are required to examine the internal consistency and test-retest reliability of MASES in evaluating self-efficacy in adherence to ICT. Disclosures: Kuo: Novartis Canada: Research Funding. Off Label Use: Deferiprone is an unlicensed drug in Canada and USA. It is an oral iron chelator.

Socio-Economic Impact of Infused Iron Chelation Therapy in France: ISOSFER Study Results.

Blood ◽  
2006 ◽  
Vol 108 (11) ◽  
pp. 3354-3354 ◽  
Author(s):  
Catherine Brun-Strang ◽  
Dora Bachir ◽  
Mariane De Montalembert ◽  
Isabelle Thuret
Keyword(s):  
Adverse Events ◽  
Chelation Therapy ◽  
Ferritin Level ◽  
Iron Chelation ◽  
Organ Damage ◽  
Total Direct Cost ◽  
Iron Chelation Therapy ◽  
Cross Sectional ◽  
Study Results ◽  
The Cost

Abstract Background: Patients suffering from β-thalassemia (TM), sickle cell disease (SCD), and myelodysplastic syndromes (MDS) undergoing chronic blood transfusions are at risk for iron overload which, if not treated by iron chelation therapy (ICT), can cause serious organ damage and reduce life expectancy. Deferoxamine (DFO) is the standard of care for the depletion of excess body iron. It has to be infused for 8–10 hours, 5–7 times a week. Although the clinical need for ICT is clearly established, less is known about the economic burden of DFO treatment. Aim: To estimate the total annual costs of DFO ICT in treatment centers in France. Methods: A cross-sectional study with a prospective recruitment. Among 278 consecutive patients receiving regular transfusions for TM, SCD or MDS who consulted between October 2005 and February 2006 in 24 French centers, 161 were on ICT. 124 patients were treated with DFO alone for more than 1 year. Among them, 67 aged 14 years or more agreed to participate. Resources used were collected through patient and physician questionnaires. Unit costs (2004/2005 €) were applied according to French economic guidelines. Results: DFO was administered via subcutaneous (sc) infusion for 70% of patients, mainly nightly and with a mean duration of 10 hours. Other ways of administering DFO included intravenous (iv) infusion (15%), sc bolus (9%) and combined sc and iv treatment (5%). Patient characteristics are summarized in the table below. TM (n=24) SCD (n=17) MDS (n=26) *Cardiac, liver and endocrine diseases, lens opacities, osteoporosis Median age (min-max), years 30 (15–70) 32 (14–57) 69 (45–85) Sex, M/F 11/13 6/11 14/12 Organ dysfunction potentially related to hemosiderosis* (%) 75 47 54 Ferritin level (median), ng/mL 1049 2653 2627 DFO nb/week (mean) 3.7 4.5 4 Dose (mean) 40 17 43 For all patients, the estimated mean weighted annual cost of infusions is 16009 € (SD ± 13867). Costs are similar for the three diseases. ICT delivery equipment (infusion set and pump) and nursing administration, drug cost, DFO adverse events monitoring, periodic exams and treatment of infused ICT-related adverse events represent respectively 56.5%, 38.5%, 0.3%, 3.7% and 0.9% of total direct cost. The estimated annual mean cost of the drug alone was 6160 € (SD ± 4145). Average cost for DFO adverse events management is low at 151.5€ (SD ± 1224), essentially due to one patient complication. Costs of periodic exams are also low due to the fact that exams are not strictly performed annually as recommended. These estimates of the total annual costs of DFO ICT are likely to be underestimating the overall cost of DFO therapy because treatment costs of the clinical consequences of poor adherence to DFO and lost productivity were not collected in the study. Conclusions: ISOSFER demonstrated that total direct costs of ICT are substantial and well exceed the cost of DFO alone. The cost of DFO administration constitutes a significant portion of the total cost of iron chelation (54%). These data are comparable to other analyses published from US (43% of the total costs, n=155) and Swiss (45%, n=17) databases.

Cardiovascular Complications Of Thalassemia Patients In The Iron Chelation Therapy Era

Blood ◽  
2013 ◽  
Vol 122 (21) ◽  
pp. 5602-5602
Author(s):  
Adisak Tantiworawit ◽  
Suebsakul Tapanya ◽  
Arintaya Phrommitikul ◽  
Lalita Norasetthada ◽  
Chatree Chai-adisaksopha ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Cardiac Function ◽  
Serum Ferritin ◽  
Chelation Therapy ◽  
Conflicts Of Interest ◽  
Ferritin Level ◽  
Iron Chelation ◽  
Cardiac Complications ◽  
Iron Chelation Therapy ◽  
Cross Sectional

Abstract Background Cardiac complications are the most common cause of death in thalassemia, accounting for up to 71% in the past. Iron chelation therapy is given to patients with iron overload to prevent these complications. The cost effectiveness of iron chelation therapy was arguable. This study aims to evaluate the prevalence of cardiac complication and the correlation between risk factors in iron chelation therapy era. Method This is a cross sectional study from June 2011- May 2012. All thalassemia aged > 15 years old were enrolled. Clinical data and hemoglobin typing were reviewed. Echocardiography and CMR T2*, a technique represent cardiac iron deposition used to evaluate cardiac function, were used to evaluate cardiac complications. Results Ninety one patients were enrolled, 63.7% were females, median age of 31 years (16-75). There was 49.5 % homozygous β thalassemia, 31.9% β thalassemia/Hb E disease, 18.7% Hb H disease. Half of patients were transfusion dependent and 63.7% underwent splenectomy. Eighty four percent of patients received iron chelation therapy but few of them got their preferable choice in adequate dosage. Patients with serum ferritin levels more than 1,000 ng/ml. received deferoxamine, deferiprone or deferasirox. Even with the iron chelation therapy, mean serum ferritin level was still high at 3,820 ng/ml for the whole group. CMR T2* was more sensitive in detecting cardiac function. The CMR T2* showed shorter signal (≤ 20 msec) in 11.1%. Only 8.2% had impaired ejection fraction <55% by echocardiography. The CMR T2*  ≤ 20 msec was significant correlated with higher maximum ferritin 5,739.14 ng/ml compared to 3,614 ng/ml (p=0.001). Pulmonary hypertension was found 7 patients (12.7%) and 71.42% had underwent splenectomy. Conclusion From our study, the CMR T2* is the sensitive method for detecting cardiomyopathy and highly correlated with serum ferritin levels. Splenectomy remains the major risk factor for pulmonary hypertension. The incidence of cardiac complications has decreased with iron chelation therapy for maintaining acceptable serum ferritin levels but the problem with cardiomyopathy and pulmonary hypertension still exist. Early detection, more sensitive implementation and aggressive iron chelation therapy are necessary to prevent these complications. The majority of the patients in Thailand which are under universal health-care coverage scheme could not get access to more effective and expensive iron chelator. Regular and adequate chelation plays a major role in the prevention of cardiac complications and the achievement of better quality of life. Disclosures: No relevant conflicts of interest to declare.

scholarly journals Some aspects of thyroid dysfunction in thalassemia major patients with severe iron overload

2011 ◽  
Vol 51 (2) ◽  
pp. 66
Author(s):  
Cynthia Rindang ◽  
Jose R. L. Batubara ◽  
Pustika Amalia ◽  
Hindra Satari
Keyword(s):  
Iron Overload ◽  
Chelation Therapy ◽  
Elevated Serum ◽  
Iron Chelation ◽  
Thalassemia Major ◽  
Primary Hypothyroidism ◽  
Iron Chelation Therapy ◽  
Free T4 ◽  
Cross Sectional ◽  
Female Ratio

Background Severe iron overload due to recurrent transfusions for chronic anemia and inadequate iron chelation therapy in thalassemia major patients result in various complications, including hypothyroidism. Currently, there has been no data on the prevalence of hypothyroidism in thalassemia major patients at the Thalassemia Centers, Department of Child Health, CiptoMangunkusumo Hospital (DCH CMH).Objective To study the prevalence of primary hypothyroidism in thalassemia major patients in the Thalassemia Center, DCH MCH.Methods We performed a cross-sectional, descriptive study. All thalassemia major subjects aged O􀁬18 years with severe iron overload underwent thyroid functionexamination. Primary hypothyroidism was defined as either normal (compensated) or decreased (decompensated) free T4 (FT4) levels, along with elevated sensitive thyroid􀁬stimulatinghonnone (TSH)levels. Results 179 subjects enrolled this study Mth male: female ratio of 1: 1.6. The prevalence of primary hypothyroidism in thalassemia majorpatients Mth severe iron overloadws26.8% (48/179). Of those 48,45 had compensated hypothyroidism and 3 had decompensated hypothyroidism, 25.1% and 1.7% of the total subjects, respectively. Compensated hypothyroidism was observed in 17 subjects aged ≤1O years and in 28 subjects aged> 10 years. All 3 decompensated hypothyroidism cases were> 10 years of age. No relationship was found between the occurrence of primary hypothyroidism and mean pre-tr811sfusion Hb levels (P=0.481, OR 1.30; 95% CI 0.63 to 2.68), elevated serum ferritin levels (P=0.74, OR 0.89; 95% CI 0.46 to 1.75), and compliance to iron chelation therapy (P=0.570, OR 0.76; 95% CI 035 to 1.65). Based on multivariate analysis, only age of <10 year-old (P=O.029, OR 0.469; 95% CI 0.23 to 0.93) was significantly associated Mth primary hypJthyroidism. Further analysis using receiver operator curve (ROC) technique found that age of 8.5 year-old was the cutoff value to predict the risk of hypothyroidism. Conclusion The prevalence of primary hypothyroidism in our study is high. The occurrence of hypothyroidism is associated with age.

scholarly journals Time to Start Delivering Iron Chelation Therapy in Newly Diagnosed Severe β-Thalassemia

2020 ◽  
Vol 2020 ◽  
pp. 1-6
Author(s):  
Susi Susanah ◽  
Ponpon S. Idjradinata ◽  
Nur M. Sari ◽  
Lulu E. Rakhmilla ◽  
Yunia Sribudiani ◽  
...  
Keyword(s):  
Iron Overload ◽  
Chelation Therapy ◽  
Iron Status ◽  
Transferrin Saturation ◽  
Iron Chelation ◽  
Normal Diet ◽  
Iron Chelation Therapy ◽  
Newly Diagnosed ◽  
Hepcidin Level ◽  
Significant Difference

Background. Iron overload is still a major complication of severe β-thalassemia. Indication to start iron chelation therapy is based on serum ferritin (SF) or transferrin saturation (TS) level or the amount of transfusion. The goal of this study is to analyse the pattern of iron status, the amount of transfusion regarding the time to start iron chelator, and serum hepcidin levels in newly diagnosed severe β-thalassemia. Methods. A prospective cohort study was performed at Hasan Sadikin General Hospital on newly diagnosed severe β-thalassemia patients. Subjects had not received any blood transfusion with normal liver function test, CRP, and IL-6 levels who consumed normal diet according to age. The SF and TS levels indicate iron status, while hepcidin level indicates iron regulator status. Main indicator to start iron chelation therapy when SF level ≥1.000 ng/mL, TS level ≥70%, or after receiving transfusion at least 10 times. Statistical analysis used Mann–Whitney and Spearman. Results. Forty-two newly severe β-thalassemia, 30 (71.4%), were diagnosed before 1 year old, mean 9.9 ± 6.4 months, range 2–24 months. Range amount of transfusion until SF level reached ≥1,000 ng/mL were 4-12 times, mean 7 ± 2 times. Mean SF and TS level at diagnosis were 365.6 ± 194.9   ng / mL and 67.3 ± 22.5 % , while hepcidin level was normal, mean 242.6 ± 58   ng / mL . 36/42 patients have reached SF >1000 ng/mL with amount of transfusion less than 10 times. There was no significant difference of SF, TS, and hepcidin levels when SF >1000 ng/mL in the group with amount of transfusion 7–12 and less than 7 ( p = 0.454 , p = 0.084 , p = 0.765 ), respectively. A significant positive correlation between SF and amount of transfusion was observed ( p < 0.001 ; r = 0.781 ). Conclusion. Iron overload in severe β-thalassemia patients might occur earlier even before they received 10 times transfusion. Hepcidin serum level tends to increase when iron overload just started.

scholarly journals Overall Survival in Acquired Pure Red Cell Aplasia in Adults Following Immunosuppressive Therapy: Preliminary Results from the Nationwide Cohort Study (PRCA2016)

Blood ◽  
2018 ◽  
Vol 132 (Supplement 1) ◽  
pp. 2593-2593
Author(s):  
Naohito Fujishima ◽  
Makoto Hirokawa ◽  
Kenichi Sawada ◽  
Shinji Nakao ◽  
Yuji Yonemura ◽  
...  
Keyword(s):  
Cohort Study ◽  
Survival Time ◽  
Immunosuppressive Therapy ◽  
Research Funding ◽  
Chelation Therapy ◽  
Pharmaceutical Research ◽  
Iron Chelation ◽  
Iron Chelation Therapy ◽  
Significant Difference ◽  
Lgl Leukemia

Abstract Background. Idiopathic PRCA and secondary PRCA not responding to the treatment of the underlying diseases are generally treated by immunosuppressive therapy. Because of the rarity of this disease, there are few reports regarding the long-term outcome following immunosuppression. We previously conducted the nationwide study for chronic PRCA in Japan that had been diagnosed between 1990 and 2006 across 109 institutions, and collected the data on a total of 185 patients (PRCA2004/2006 study). This study revealed that poor response to induction therapy and relapse of anemia were associated with death. There was no significant difference in survival between idiopathic and secondary PRCA in the PRCA2004/2006 study cohort. Objective. In order to identify the adverse risk factors for survival in acquired PRCA following immunosuppression in a prospective way, we have conducted another nationwide cohort study for acquired PRCA in adults (PRCA2016 study). The primary endpoint is the overall survival (OS) and the secondary endpoints include efficacy of immunosuppression, causes of treatment failure and survival times with iron-chelation therapy. Methods. This is designed as a prospective longitudinal observational study. Between 2006 and 2014, 554 PRCA patients were registered in the hematological disorder registry managed by the Japanese Society of Hematology. We sent the first questionnaires to the physicians asking for collaboration with this study and the responses showed the potential size of cohort was 181 patients (Fig. 1). We then sent the second questionnaires to collect data regarding underlying disease, laboratory findings including CBC and leukocyte differentials, results of bone marrow examination, immunological and cytogenetics, efficacy of immunosuppression, iron-chelation therapy in refractory cases and outcome. The review boards of participating institutions and the ethical committee of the JSH approved this study and informed consent was obtained from the patients. Results. As of June 16, 2018, 103 patients were registered in this study. Fifty-two patients were classified as having idiopathic PRCA and 51 patients as having secondary PRCA, including 16 thymoma- and 5 large granular lymphocyte (LGL) leukemia-associated PRCA. Seventy-one patients were treated with immunosuppressive agents. The response rates of cyclosporine, predonisolone, cyclophosphamide, azathioprine and combination with more than one immunosuppressants were 84% (43/51), 89% (8/9), 100% (2/2), 0% (0/1), 60% (9/15), respectively (Fig. 2). The Kaplan-Meyer estimates of the survival revealed that the estimated median survival time in idiopathic PRCA was 6,430 days and that in secondary PRCA has not yet been reached (Fig. 3). Survival time was not significantly different between the two subtypes of PRCA. Twenty-two deaths were reported and the major causes of death were infection and heart failure. Discussion/Conclusion. The most common subtypes of chronic PRCA in Japan were idiopathic, thymoma-associated and LGL leukemia-associated PRCA and this result was consistent with our observation in the PRCA2004/2006 study. Cyclosporine and prednisolone were frequently chosen for the induction therapy resulting in favorable responses. Preferential use of these two agents might reflect the physician's concern about the toxicity of the cytotoxic drugs. The median survival time of idiopathic PRCA was quite similar to the results in the previous cohort study. No significant difference in survival between idiopathic and secondary PRCA has been confirmed by the present study. These two cohort studies clearly indicate that novel diagnostic and predictive biomarkers should be developed to segregate the good and poor responders to immunosuppressive therapy in PRCA. Disclosures Nakao: Novartis: Honoraria; Kyowa Hakko Kirin Co., Ltd.: Honoraria; Alexion Pharmaceuticals, Inc.: Consultancy, Honoraria. Yonemura:Alexion Pharma: Honoraria, Research Funding. Matsuda:GlaxoSmithKline K.K.: Honoraria; Novartis Pharma K. K.: Honoraria; Chugai Pharmaceutical Co, Ltd.: Honoraria; Kyowa Hakko Kirin Co, Ltd.: Honoraria; Sumitomo Dainippon Pharma Co., Ltd.: Honoraria; Nippon Shinyaku Co., Ltd.: Honoraria; Celgene Corporation: Honoraria; Alexion Pharmaceuticals, Inc.: Honoraria; Sanofi K.K.: Honoraria; Beckman Coulter K.K.: Honoraria. Kurokawa:Otsuka Pharmaceutical: Research Funding; Astellas Pharma: Research Funding; MSD: Honoraria, Research Funding; Pfizer: Research Funding; Eizai: Research Funding; Ono Pharmaceutical: Honoraria, Research Funding; Sumitomo Dainippon Pharma: Research Funding; Teijin Pharma: Research Funding; Nippon Sinyaku: Honoraria, Research Funding; Takeda Pharmaceutical: Research Funding; Chugai Pharmaceutical: Research Funding; Kyowa Hakko Kirin: Honoraria, Research Funding. Arai:Novartis: Research Funding. Mitani:Kyowa Hakko Kirin Co., Ltd.: Consultancy, Research Funding, Speakers Bureau; Bristol-Myesr Squibb: Research Funding, Speakers Bureau; Celgene: Speakers Bureau; Chugai: Research Funding; Astellas: Research Funding; Sumitomo Dainippon: Research Funding; Novartis: Research Funding; Toyama Chemical: Research Funding.

Changes of the Expressions of the Genes Involved in Iron Metabolism by the Iron Chelation Therapy in the Iron Overloaded Mouse Model.

Blood ◽  
2008 ◽  
Vol 112 (11) ◽  
pp. 1854-1854
Author(s):  
Katsuya Ikuta ◽  
Yoshihiro Torimoto ◽  
Takaaki Hosoki ◽  
Junko Jimbo ◽  
Motohiro Shindo ◽  
...  
Keyword(s):  
Iron Metabolism ◽  
Iron Uptake ◽  
Chelation Therapy ◽  
Iron Chelation ◽  
Iron Chelator ◽  
Treated Group ◽  
The Body ◽  
Iron Chelation Therapy ◽  
Qrt Pcr ◽  
Significant Difference

Abstract [Introduction and aim] Iron chelation therapy has been applied for iron overload. Desferrioxamine (DFO) must have been the most used iron chelator in the world. DFO has been thought to chelate iron from the liver and reticuloendotherial systems in that iron is accumulated, and accelerate iron excretion from the body. On the other hand, many new molecules involved in iron metabolism have been discovered in these 10 years, and the understanding of the molecular mechanisms of iron metabolism has progressed. However, the changes of the genes involved in iron metabolism in iron chelating therapy have not been fully investigated. Therefore, the aim of this study is to clarify the responses of the body to the iron chelation therapy and the mechanisms of iron removal by iron chelator DFO. We investigated the changes of genes involved in iron metabolism in the important organs, such as the liver and the gastrointestinal tract, at the early stage of iron chelation therapy by treating the iron overloaded mouse with DFO. [Methods] Iron overloaded mouse model was made by giving iron dextran interperitonealy (the iron-loaded group). DFO was given for the iron overloaded mice regularly by the interperitoneal (The iron-loaded + DFO treated group). The iron chelation by DFO had continued for 4 weeks. Mice without any treatment were participated as the control. After 4 weeks, mice were sacrificed, and the liver, small intestine, heart, and blood were collected from all mice. Total RNA was then purified, and quantitative RT-PCR (qRT-PCR) was performed for genes involved in iron metabolism. The genes investigated in this study were HFE, transferrin receptor 1 (TfR1), transferrin receptor 2 (TfR2), hepcidin-1 (HAMP1), hepcidin-2 (HAMP2), ferroportin 1 (FPN1), divalent metal transporter 1 (DMT1), duodenal cytochrome b (Dcytb), hephasetin and ferritin. Protein isolated from collected organ was applied for western blot. [Results] There was no significant difference in the physique, the weight of the liver and spleen, hemoglobin, serum iron concentration among all groups of mice. The serum ferritin was remarkably increased in iron-loaded group, and there was no decline of serum ferritin in the iron-loaded + DFO treated group. Although the expressions of HFE, TfR2, HAMP1, HAMP2 in the liver didn’t show significant difference among each of the groups by qRT-PCR, the ferritin mRNA and FPN1 mRNA were decreased significantly in the iron-loaded + DFO treated group compared to the iron-loaded group. In the duodenum, there was no difference in the expressions of DMT1, Dcytb, hephasetin, but the expressions of ferritin mRNA and FPN1 mRNA were significantly decreased in the iron-loaded + DFO treated group. [Discussion and conclusions] In addition to removal of iron from the organ that iron is accumulated, our data showed that iron chelation therapy had the effect on the expressions of the genes involved in iron metabolism. The decreases of ferritin mRNA at both of the liver and the duodenum might imply that intracellular free iron was chelated by DFO. Concerning the decline of FPN1 mRNA, there might be a possibility that intracellular iron depletion by DFO cause the inhibition of translation of FPN1 mRNA via iron responsive element which exists in 5’-untranslated region of FPN1 mRNA. FPN1 in duodenum functions for iron transfer from the enterocyte to blood, so that the decrease of body iron likely increase the expression of FPN1 in the duodenum because body would like to increase iron uptake from duodenum. However, our result indicated that iron chelation therapy did not upregulate FPN1 mRNA expression. Our data rather showed that FPN1 is downregurated, and this indicated that iron chelation would lead to decreased iron uptake from the duodenum, and this would be preferable effect of DFO in the view of iron chelation therapy for iron overload.

scholarly journals Barriers to Adherence to Iron Chelation Therapy in Thalassemia Patients

2021 ◽  
Vol 20 (2) ◽  
pp. 45-49
Author(s):  
Tanuka Barua ◽  
Golam Mohammed Tayab Ali ◽  
Rana Chowdhury ◽  
Dhananjoy Das ◽  
Showrov Barua Chowdhury ◽  
...  
Keyword(s):  
Chelation Therapy ◽  
Low Cost ◽  
Iron Chelation ◽  
Cross Sectional Study ◽  
Iron Chelation Therapy ◽  
Blood Disorders ◽  
Financial Problem ◽  
Cross Sectional ◽  
Number Of Patients ◽  
Chelating Therapy

Background: Thalassemias are the most common inheritable blood disorders requiring regular blood transfusions and iron chelating therapy. Non-adherence to iron chelation therapy increases complications and is a problem in treating thalassemia. To assess the reasons of non-adherence to iron chelating drug in treating thalassemia. Materials and methods: This descriptive cross-sectional study was carried out in the thalassemia ward of Chattogram Maa Shishu-O-General Hospital, Chattogram from July, 2013 to June, 2014. 70 thalassemia patients aged 2-18 years previously treated with iron chelating drugs were included. Parents were interviewed according to a formulated questionnaire based on discontinuation of iron chelating drugs and its reasons. Data were analyzed by both manually and by SPSS-18. Results: About 48.6% patients needed blood transfusion >10 units/year and 62.9% patients were prescribed with iron chelating drugs. Near about half patients (47.7%) did not continued iron chelating therapy till full prescribed period. Deferiprone (31.8%) and combination of deferipronc & desferrioxamine (31.8%) was the most commonly prescribed drug. Deferiprone is the drug to which most of the patients (70%) were adherent and a good number of patients (65%) discontinued desferrioxamine. Financial problem (100%) was the only reason for discontinuation of oral chelator. In case of parenteral chelator, besides finanacial problem (38.5%), time consuming natures (38.5%), need of hospital admission (23%) are the other causes for non-adherence to iron chelation therapy. Conclusion: Financial problem is the main cause of non-adherence to iron chelation therapy. Iron chelating drugs should be available at low cost. Chatt Maa Shi Hosp Med Coll J; Vol.20 (2); July 2021; Page 45-49

Clinical-Reported Outcomes Of Deferoxamine Versus Deferasirox In The Treatment Of Homozygous BetaThalassemia

2018 ◽  
Vol 9 (SPL1) ◽  
Author(s):  
Zeina A Munim Al-Thanoon ◽  
Zeina A Munim Al-Thanoon ◽  
Mustafa Basil ◽  
Nasih A Al-Kazzaz
Keyword(s):  
Serum Ferritin ◽  
Iron Chelation ◽  
Iron Chelator ◽  
Beta Thalassemia ◽  
Control Group ◽  
Current Standard ◽  
Cross Sectional ◽  
Significant Difference ◽  
Multiple Blood ◽  
Group 2

Iron chelation therapy with deferoxamine (DFO),the current standard for the treatment of iron overload in patients with betathalassemia,requires regular subcutaneous or intravenous infusions. This can lead to reduced quality of life and poor adherence,resulting in increased morbidity and mortality in iron-overloaded patients with beta-thalassemia. Deferasirox (DFX) is an orally administered iron chelator that has been approved for use in many countries. The requirement of an effective,well tolerated iron chelator with a less demanding mode of administration has led to the development of deferasirox. The present study was aimed to compare the satisfaction and compliance with deferoxamine versus deferasirox (Exjade®),a novel oral iron chelator in patients with transfusion - dependent beta- thalassemia. A cross-sectional,single-center investigation study was carried out in the Thalassemia Center of Ibn-Atheer Teaching Hospital in Nineveh province,Iraq. One hundred and eight thalassemic patients aged between 2- 20 years old having received multiple blood transfusions and a serum ferritin greater than 1500 ng/ml. Patients were randomised into two groups. Group 1 received deferoxamine at a dose of 20-50mg/kg/day and group 2 received deferasirox at the dose of 10-30 mg/kg/day. Another 56 apparently healthy volunteers were used as a control group. The assessment of chelation was done during the period between November 2013 and February 2014 by measurement of serum ferritin. Satisfaction and compliance was assessed by using a special questionnaire prepared by the researcher. Out of the 108 thalassemic patients enrolled there was no discontinuation in treatment with the two drugs under study. The serum ferritin did not change significantly in any of the chelation groups. In comparison with the patients who were treated with DFO,those receiving DFX reported a significantly higher rate of compliance and satisfaction (P < 0.05). However,no significant difference was observed between the two groups regarding their satisfaction (P > 0.05).Compliance with deferasirox (50 %) was more than that with deferoxamine (20 %). Satisfaction with deferoxamine was significantly lower than deferasirox (p= 0.00).

Sign in / Sign up

Export Citation Format

Share Document