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

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.

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.

scholarly journals Dermoscopy of topical steroid-dependent or damaged face: A cross-sectional study

2021 ◽  
Vol 0 ◽  
pp. 1-7
Author(s):  
Sheenam Sethi ◽  
Payal Chauhan ◽  
Rashmi Jindal ◽  
Yashwant Singh Bisht
Keyword(s):  
Clinical Symptoms ◽  
Low Cost ◽  
Topical Steroid ◽  
Cross Sectional Study ◽  
Clinical Findings ◽  
Cross Sectional ◽  
Female Ratio ◽  
Exact Test ◽  
Steroid Dependent ◽  
Number Of Patients

Background: Topical corticosteroid (TCS) abuse is rampant and results in steroid addiction labeled as topical steroid-dependent or damaged face (TSDF). Indian market is replete with triple combination creams containing TCS sold as over-the-counter products at low cost, luring people to use them without prescription. The resultant damage if detected late is irreversible and difficult to treat. Dermoscopy can help in the early identification of features of TSDF at a preclinical stage resulting in better prognosis. However, the literature on the same is limited. Aims: This study is undertaken to characterize dermoscopic features of TSDF and to correlate them with potency and duration of application of the TCS. Methods: One hundred and thirty-two patients aged 18 years or above, with clinical symptoms and signs suggestive of TSDF and with history of application of TCS on the face for a period of more than one month, were enrolled in the study. Their demographic details, clinical features, and dermoscopy findings were recorded using a predesigned structured format. Comparison of dermoscopic findings with clinical examination, gender, potency of TCS, and duration of TCS use was done using Chi-square test, Fisher’s exact test, and one-tailed Z-test. Results: Mean age of the patients was 31.7 ± 8.1 years. Male to female ratio was 2:9. Sixty-nine (52.3%) patients abused TCS for more than one year. Clinical findings noted in the patients were erythema (81.1%), hyperpigmentation (80.3%), and hypertrichosis (68.2%). The most common dermoscopy findings seen were brown globules (96.2%), red diffuse areas (92.4%), vessels (87.1%), white structureless areas (86.4%), and hypertrichosis (80.3%). Red diffuse areas, vessels, brown globules, white structureless areas, and white hair were observed in a statistically higher proportion of cases dermoscopically. Y-shaped vessels and brown globules were seen in significantly higher number of patients, using TCS for more than three months and in those continuing it beyond six months, polygonal vessels were predominant. Limitations: Lack of histopathological correlation is the limitation of our study. Furthermore, brown globules seen in 96.2% patients of TSDF on dermoscopy may have been over-estimated and not always signify TSDF; instead, it could represent melasma for which patient applied TCS. Conclusion: Dermoscopy in TSDF can help dermatologists in a multitude of ways from confirming the diagnosis to differentiating from other causes of red face and predicting the approximate duration of TCS abuse.

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 Knowledge and Awareness of Parents Toward Thalassemia

2021 ◽  
Vol 20 (1) ◽  
pp. 12-15
Author(s):  
Tanuka Barua ◽  
Dazy Barua ◽  
Dhananjoy Das ◽  
Rupam Talukdar ◽  
Razia Sultana ◽  
...  
Keyword(s):  
Prenatal Diagnosis ◽  
Disease Burden ◽  
Cross Sectional Study ◽  
Carrier Status ◽  
Therapeutic Abortion ◽  
Blood Disorders ◽  
Cross Sectional ◽  
Number Of Patients ◽  
Premarital Screening ◽  
Awareness Level

Background: Thalassemias are the most common heritable blood disorders that represents a major public concern. Poor awareness and lack of knowledge lead to increase number of carrier that is a silent reservoir of the disease. To observe the knowledge and awareness level of parents of thalassemic children about the disease. Materials and methods: This descriptive cross-sectional study was conducted in the Thalassemia ward of Chattogram Maa Shishu-O-General Hospital, Chattogram from July 2013 to June, 2014. Parents of 70 thalassemia patients aged 2-18 years interviewed with a formulated questionnaire based on knowledge status and awareness level of parents towards thalassemia. Data were analyzed by both manually and by SPSS-18. Results: Majority of patients were from rural background (54.3%). Only 8.6% parents were consanguineous parents and majority of them completed only secondary education. 44.3% resolved it as inherited disorder. 52.9% resolved thalassemia cannot be cured. Only 24.3% regarded bone marrow transplantation as a measure of cure. More than half (55.7%) did not know how to prevent thalassemia. Only 37.1% knew about prenatal diagnosis. Carrier status of both father and mother were unknown in majority of patients (80%) and screening of sibs was not done at all in a significant number of patients (51.5%). Only 34.3% wanted to do prenatal diagnosis after conception and 65.7% parents were ready to accept therapeutic abortion if fetus would be diagnosed as thalassemia by prenatal diagnosis. Conclusion: Knowledge level and awareness of parents of thalassemic child regarding the disease is unsatisfactory. To reduce disease burden an awareness program regarding the disease and its prevention covering premarital screening, acceptance of prenatal diagnosis and therapeutic abortion is essential. Chatt Maa Shi Hosp Med Coll J; Vol.20 (1); January 2021; Page 12-15

scholarly journals Earlier detection of glomerular dysfunction in β-thalassemia major patients

2020 ◽  
Vol 10 (1) ◽  
Author(s):  
Waseem F. Al Tameemi ◽  
Zainab M. J. Altawry
Keyword(s):  
Serum Creatinine ◽  
Iron Overload ◽  
Cystatin C ◽  
Chelation Therapy ◽  
Iron Chelation ◽  
Therapeutic Index ◽  
Thalassemia Major ◽  
Cross Sectional Study ◽  
Cross Sectional ◽  
Thalassemia Major Patient

Chronic transfusions program in β-thalassemia patients will inevitably lead to iron overload with a significant morbidity and mortality. Glomerular filtration rate (GFR) is progressively declined in relation to iron overload as well as chronic anemia. Objective is to define levels of Cystatin C in transfusion dependent β-thalassemia major patients as a sensitive marker for detection of earlier glomerular dysfunction in addition to understand the effect of iron overload, chelating therapy and hepatitis infection. A cross sectional study conducted at Al-Basrah Hemoglobinopathy Centre for the period from September 2017 to January 2018 to enroll 75 β-thalassemia major patients. Data collected included duration of the disease, total transfusion requirement, details of chelation therapy and its therapeutic index. In addition to blood urea, serum creatinine and Cystatin C with estimated GFR (eGFR). The mean Cystatin C was 1.075 mg/L where 66.6% of patients had abnormal renal function which is higher proportion than those with renal (42.6%) detected according to serum creatinine level Cystatin C was significantly higher in patients who received desferrioxamine as compared to those received deferasirox (P=0.007), in accordance with GFR which is significantly higher in patients receiving the latter chelation therapy (P=0.009). A significant inverse relationship between Cystatin C, and GFR, while positive relationship between ferritin and Cystatin C (P=0.0001, 0.001 respectively). Cyctatin C is better for detection and monitoring of glomerular dysfunction in B thalassemia major patient which is already not uncommon complications for the disease and iron chelation therapy.

Iron Chelation Therapy in CAPD: A New and Effective Treatment of Iron Overload Disease in Esrd Patients

1983 ◽  
Vol 3 (2) ◽  
pp. 99-101 ◽  
Author(s):  
Glen H Stanbaugh ◽  
A. W, Holmes Diane Gillit ◽  
George W. Reichel ◽  
Mark Stranz
Keyword(s):  
Peritoneal Dialysis ◽  
Iron Overload ◽  
End Stage Renal Disease ◽  
Chelation Therapy ◽  
Iron Chelation ◽  
Iron Chelation Therapy ◽  
Peritoneal Dialysate ◽  
Stage Renal Disease ◽  
End Stage ◽  
Esrd Patients

A patient with end-stage renal disease on CAPD, and with massive iron overload is reported. This patient had evidence of myocardial and hepatic damage probably as a result of iron overload. Treatment with desferoxamine resulted in removal of iron in the peritoneal dialysate. On the basis of preliminary studies in this patient it would appear that removal of iron by peritoneal dialysis in conjunction with chelation therapy is safe and effective. This finding should have wide-ranging signficance for patients with ESRD.

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