scholarly journals Comparative Effects of Three Iron Chelation Therapies on the Quality of Life of Greek Patients with Homozygous Transfusion-Dependent Beta-Thalassemia

2012 ◽  
Vol 2012 ◽  
pp. 1-8 ◽  
Author(s):  
Vasilis Goulas ◽  
Alexandra Kourakli-Symeonidis ◽  
Charalambos Camoutsis
Keyword(s):  
Quality Of Life ◽  
Iron Chelation ◽  
Self Esteem ◽  
Physical Component Summary Score ◽  
Summary Score ◽  
Beta Thalassemia ◽  
Adherence To Treatment ◽  
Treatment Rate ◽  
Treatment Benefits

This prospective study assessed the quality of life of patients with homozygous transfusion-dependent beta-thalassemia in Greece receiving three different iron chelation treatments. Patients enrolled were receiving one of the following chelation therapies: deferoxamine (n=21), deferasirox (n=75), or deferoxamine in combination with deferiprone (n=39). The three groups were compared in terms of their quality of life, satisfaction and adherence to treatment, control of their health, and self-esteem through the completion of five questionnaires. A higher percentage of patients receiving deferoxamine felt that their treatment negatively influenced their body and skin appearance and limited their ability to work, attend school, and perform daily tasks (P=0.0066). The adherence to treatment rate and self-esteem were the lowest in the deferoxamine group (P<0.05). The deferoxamine group also had the lowest physical component summary score in the SF-36 questionnaire (P=0.014). This study suggests that the quality of life of beta-thalassemia patients receiving chelation therapy is dependent on the type of iron chelation treatment they receive. The study provides insight into important factors associated with the quality of life of these patients, which are essential for developing a more suitable clinical support team and counseling in order to maximize the treatment benefits for these patients in daily clinical practice.

scholarly journals Comparative Effects of Iron Chelators on the Transfusion-Dependent Beta-Thalassemia Patients

2018 ◽  
Vol 26 ◽  
pp. 14-19
Author(s):  
Muhammad Mahbub Ul Alam ◽  
M Alauddin ◽  
M Jollilur Rahman ◽  
Rawshan Akhter ◽  
Chinmoy Kanti Das
Keyword(s):  
Quality Of Life ◽  
Iron Chelation ◽  
Self Esteem ◽  
Physical Component Summary Score ◽  
Summary Score ◽  
Beta Thalassemia ◽  
Adherence To Treatment ◽  
Treatment Rate ◽  
Group I

This prospective study assessed the quality of life of patients with transfusion-dependent beta-thalassemia receiving three different iron chelation treatments. Patients enrolled were receiving one of the following chelation therapies: Group-I: deferoxamine (n=21), Group-II: deferasirox (n=75) and Group-III: deferoxamine in combination with deferiprone (n=39). The three groups were compared in terms of their quality of life, satisfaction and adherence to treatment, control of their health, and self-esteem through the completion of five questionnaires. A higher percentage of patients receiving deferoxamine felt that their treatment negatively influenced their body and skin appearance and limited their ability to work, attend school, and perform daily tasks (P=.0.0066). The adherence to treatment rate and self-esteem were the lowest in the deferoxamine group (P<0.05). The deferoxamine group also had the lowest physical component summary score in the SF-36 questionnaire (P=0.014). This study suggests that the quality of life of beta-thalassemia patients receiving chelation therapy is dependent on the type of iron chelation treatment they receive. The study provides insight into important factors associated with the quality of life of these patients, which are essential for developing a more suitable clinical support team and counseling in order to maximize the treatment benefits for these patients in daily clinical practice.TAJ 2013; 26: 14-19

scholarly journals CURING HEMOGLOBINOPATHIES: CHALLENGES AND ADVANCES OF CONVENTIONAL AND NEW GENE THERAPY APPROACHES.

2019 ◽  
Vol 11 (1) ◽  
pp. e2019067 ◽  
Author(s):  
Irene Motta ◽  
Valentina Ghiaccio ◽  
Andrea Cosentino ◽  
Laura Breda
Keyword(s):  
Quality Of Life ◽  
Health Care ◽  
Gene Therapy ◽  
Bone Marrow ◽  
Iron Chelation ◽  
Beta Thalassemia ◽  
Allogenic Bone ◽  
Number Of Patients ◽  
Monogenic Diseases

Inherited hemoglobin disorders, including beta-thalassemia (BT) and sickle-cell disease (SCD) are the most common monogenic diseases worldwide, with a global carrier frequency of over 5%. With migration they are becoming more common worldwide, making their management and care an increasing concern for health care systems. BT is characterized by an imbalance in the α/β-globin chain ratio, ineffective erythropoiesis, chronic hemolytic anemia, and compensatory haemopoietic expansion. Globally, there are over 25,000 births each year with transfusion-dependent thalassemia (TDT). The current available treatment for TDT is lifelong transfusions and iron chelation therapy or allogenic bone marrow as curative option. SCD affects 300 million people worldwide  and severely impacts the quality of life of patients, who experience unpredictable, recurrent acute and chronic severe pain, stroke, infections, pulmonary disease, kidney disease, retinopathy, and other complications. While survival has been dramatically extended, quality of life is markedly reduced by disease- and treatment-associated morbidity. The development of safe, tissue specific and efficient vectors, and efficient gene editing technologies have led to the development of several gene therapy trials for BT and SCD. Yet, the complexity of the approach presents its hurdles. Fundamental factors at play include the requirement for myeloablation on a patient with a benign disease, the age of the patient and consequent bone marrow microenvironment. A successful path from proof-of-concept studies to commercialization must render gene therapy a sustainable and accessible approach for a large number of patients. Furthermore, the cost of these therapies is a considerable challenge for the health care system. While new promising therapeutic options are emerging and many others are on the pipeline5, gene therapy can potentially cure patients. We herein provide an overview of the most recent potentially curative therapies for hemoglobinopathies and a summary of the challenges that these approaches entail.

Quality of Life and Satisfaction with Iron Chelation Therapy in Patients with Beta Thalassemia Major: Results from the ITHACA Study.

Blood ◽  
2006 ◽  
Vol 108 (11) ◽  
pp. 3344-3344 ◽  
Author(s):  
Lorenzo Giovanni Mantovani ◽  
Luciana Scalone ◽  
Simona Ravera ◽  
Diana Rofail ◽  
Maria Domenica Cappellini ◽  
...  
Keyword(s):  
Quality Of Life ◽  
Side Effects ◽  
Iron Chelation ◽  
Clinical Effectiveness ◽  
Thalassemia Major ◽  
Beta Thalassemia ◽  
Perceived Effectiveness ◽  
Old Patients ◽  
Sf 36

Abstract Background Patients with beta Thalassemia Major (TM) require life-long blood transfusions, which often cause iron overload that may increase patients’ morbidity and mortality. Iron Chelation Treatment (ICT), aimed to reduce iron overload, is based on 8–12 hour infusions of Deferoxamine (DFO) for 5–7 days/week, and/or Deferiprone (L1) orally administered. Current ICT can be related to low satisfaction, low compliance, and potentially negative consequences on clinical effectiveness, patients’ wellbeing and on healthcare costs. Aims: To investigate the Health-Related Quality-of-Life (HRQoL) of TM patients and their satisfaction with ICT. Methods: The Italian-THAlassemia-Cost-&-Outcomes-Assessment (ITHACA) was a naturalistic multicentre study conducted to evaluate costs, HRQoL, compliance and treatment satisfaction in TM patients undergoing ICT for at least 3 years, enrolled at Italian Thalassemia Care Centers. HRQoL was measured in >14 years old patients with 2 generic instruments: EQ-5D; Short Form-36 (SF-36). To measure satisfaction >12 years old patients received a 28-item instrument consisting of 4 domains: ‘perceived effectiveness’, ‘acceptance’, ‘burden’, and ‘side effects’. Each domain scored from from 1 (very dissatisfied) to 5 (very satisfied). Results Based on 126 patients: median age 29.4 years (12.3–48.5), 49.6% male. At enrolment 48.0% were using DFO, 33.6% L1, 18.4% were treated with DFO+L1. 86.5% of patients had at least one TM-related complication, 13.5% changed treatment regimen at least once in a median of 11.6 months before enrolment. With EQ-5D profile patients reported moderate problems with ‘mobility’ (9.1%), ‘self care’ (0.8%), ‘usual activities’ (23.5%), moderate or severe ‘pain/discomfort’ (60.5%) and ‘anxiety/depression’ (39.5%). Mean EQ-5D-Visual Analogue Scale was 73.0 (30–100). The SF-36 Physical Component Summary mean(SD) score was 47.7(8.4), while the mean score estimated in the Italian general population comparable for age and sex is 53.3; the Mental Component Summary mean(SD) score in TM patients was 45.1(8.8), while the Italian general population mean score was 47.7. Mean satisfaction scores were 4.29 (perceived effectiveness), 3.37 (acceptance), 3.87 (burden), and 3.57 (side effects). Simple linear regression analysis showed that satisfaction with burden (r2=12.6%, p<0.0001), side effects (r2=12.3%, p<0.0001) and acceptance(r2=11.3%, p=0.0001), are positively associated with the adherence item ‘never thinking about stopping medication’ (p<0.0001 in both cases). Conclusions: TM patients on ICT have impaired levels of physical and mental HRQoL. Therapies improving patients’ satisfaction and compliance to ICT may have positive consequences not only on clinical effectiveness but also on overall patients’ well-being.

Impact of Age, Sex, Physical Function, Health-related Quality of Life, and Treatment with Adalimumab on Work Status and Work Productivity of Patients with Ankylosing Spondylitis

2009 ◽  
Vol 37 (2) ◽  
pp. 385-392 ◽  
Author(s):  
WALTER P. MAKSYMOWYCH ◽  
KATHERINE L. GOOCH ◽  
ROBERT L. WONG ◽  
HARTMUT KUPPER ◽  
DÉSIRÉE VAN DER HEIJDE
Keyword(s):  
Quality Of Life ◽  
Ankylosing Spondylitis ◽  
Physical Component Summary ◽  
Work Productivity ◽  
Physical Component Summary Score ◽  
Summary Score ◽  
Activity Impairment ◽  
Factors Associated ◽  
Sf 36

Objective. To determine factors associated with work in patients with ankylosing spondylitis (AS).Methods. Three hundred fifteen patients with AS were enrolled in a 24-week, randomized controlled study of adalimumab with a longterm, open-label, adalimumab extension phase. Patient-reported outcome (PRO) measures included the Medical Outcome Study Short Form 36 Health Survey (SF-36), AS Quality of Life Questionnaire (ASQOL), Health Utilities Index Mark 3 (HUI-3), and Work Productivity and Activity Impairment-Specific Health Problem Questionnaire (WPAI-SHP). Multivariate logistic regression was used to analyze differences between working and nonworking patients. The relationships between PRO and WPAI-SHP scores were assessed using Pearson correlation coefficients. Multivariate modeling was applied to determine factors associated with productivity while at work. WPAI-SHP was assessed through 3 years of adalimumab exposure.Results. Younger age (p = 0.002) and male sex (p < 0.001) were significantly and independently associated with working patients with AS. The SF-36 Physical Component Summary score (p < 0.001), ASQOL score (p < 0.001), HUI-3 scores (p < 0.001), and both patient’s global assessment of disease activity (p < 0.001) and nocturnal pain (p < 0.001) scores were independently associated with working status. Work absenteeism due to AS was weakly correlated with all PRO scores. WPAI-SHP components of work presenteeism (lack of productivity at work), activity impairment, and overall work productivity loss due to AS were moderately correlated with quality of life as measured by the ASQOL, the SF-36 Physical Component Summary score, and the SF-36 Bodily Pain domain. Linear multivariate analyses indicated that work presenteeism was significantly associated with pain, functioning, and disease activity. Longterm adalimumab treatment was associated with sustained improvements in WPAI-SHP scores.Conclusions. Quality of life and the physical consequences associated with AS have a direct relationship with a patient’s ability to work. Adalimumab sustains improvements in work outcomes in patients with AS.

scholarly journals Quality of Life and Poor Oral Health: A Comparison of Postmenopausal Women

2016 ◽  
Author(s):  
Kristin A. Williams ◽  
Hebba Shamia ◽  
Christine DeBaz ◽  
Leena Palomo
Keyword(s):  
Quality Of Life ◽  
Oral Health ◽  
Postmenopausal Women ◽  
Self Esteem ◽  
Summary Score ◽  
T Test ◽  
Occupational Performance

Interrelationships between traditional dental variables are becoming more evident in far reaching aspects of life such as psychosocial interaction, self-esteem, overall health and even occupational performance. This study compares quality of life (QoL) in postmenopausal women (PMW) with poor oral health (POH) with QoL in PMW with good oral health. 200 randomly recruited PMW received a dental evaluation and completed Utian Quality of Life Survey. The participants were divided into POH and healthy groups based on dental exam. Mean scores were calculated for each QoL item, domain and the overall summary score. For each of the four parameters for periodontitis diagnosis, periodontitis patients’ QoL outcomes were compared to those of healthy patients using T-test with threshold of significance at p

THE HEALTH-RELATED QUALITY OF LIFE IN PATIENTS WITH ANKYLOSING SPONDYLITIS ASSESSED BY SF-36

2019 ◽  
pp. 63-67
Author(s):  
Hoang Thanh Van Nguyen ◽  
Thi Thuy Lien Vo
Keyword(s):  
Quality Of Life ◽  
Ankylosing Spondylitis ◽  
Mental Component Summary ◽  
Physical Component Summary Score ◽  
Summary Score ◽  
Health Related Quality ◽  
Sf 36 ◽  
Related Quality ◽  
Health Related

Background: Ankylosing spondylitis is a chronic arthritis primarily affecting the sacroiliac joints and spine, progressively progressing decrease or loss of spine mobility, severely influences the quality of life. The SF-36 questionnaire is useful in assessing quality of life, monitoring clinical outcomes and effectiveness of treament of ankylosing spondylitis. This study aims to assess the quality of life in patients with ankylosing spondylitis and investigate its correlation with clinical factors. Method: In this cross-sectional study, included 34 ankylosing spondylitis patients who were diagnosed according to the modified New York 1984 criteria. The health status was assessed by using the SF-36 questionnaire. Results: The patients with ankylosing spondylitis had average quality of life (82.4%) and low (17.6%). The mean physical component summary score, mental component summary and SF-36 score was: 36.48 ± 17.89, 48.79 ± 18.49 and 42.66 ± 17.52. There was significant correlation with disease activity, the body mass index and no correlation with age at diagnosis, duration of disease. Conclusions: The patients with ankylosing spondylitis is had significantly low SF-36 scores in all domains. The physical component summary score was more affected than the mental component summary score. Key words: Ankylosing spondylitis, the health-related quality of life, the SF-36 questionnaire

Determinants of quality of life in patients with skull base chordoma

2014 ◽  
Vol 120 (2) ◽  
pp. 528-537 ◽  
Author(s):  
Roberto Jose Diaz ◽  
Nicole Maggacis ◽  
Shudong Zhang ◽  
Michael D. Cusimano
Keyword(s):  
Quality Of Life ◽  
Skull Base ◽  
Mental Component Summary Score ◽  
Pain Medication ◽  
Physical Component Summary Score ◽  
Summary Score ◽  
Sf 36 ◽  
The Mean ◽  
Skull Base Chordomas

Object Skull base chordomas can be managed by surgical intervention and adjuvant radiotherapy. As survival for this disease increases, identification of determinants of quality of life becomes an important focus for guiding comprehensive patient care. In this study the authors sought to measure functional outcome and quality of life in patients with skull base chordomas and to identify determinants of quality of life in these patients. Methods The authors carried out an internet-based cross-sectional survey, collecting detailed data for 83 individual patients. Demographic and clinical variables were evaluated. Functional outcomes were determined by Karnofsky Performance Scale (KPS) and Glasgow Outcome Scale Extended (GOSE), quality of life was measured using the 36-Item Short Form Health Survey (SF-36), and depression was assessed using Patient Health Questions–9 (PHQ-9) instrument. Caregiver burden was assessed using the Zarit Burden Interview (ZBI). Univariate and multivariate analysis was performed to identify determinants of the physical and mental components of the SF-36. Results Patients with skull base chordomas who have undergone surgery and/or radiation treatment had a median KPS score of 90 (range 10–100, IQR 10) and a median GOSE score of 8 (range 2–8, IQR 3). The mean SF-36 Physical Component Summary score (± SD) was 43.6 ± 11.8, the mean Mental Component Summary score was 44.2 ±12.6, and both were significantly lower than norms for the general US population (p < 0.001). The median PHQ-9 score was 5 (range 0–27, IQR 8). A PHQ-9 score of 10 or greater, indicating moderate to severe depression, was observed in 29% of patients. The median ZBI score was 12 (range 0–27, IQR 11), indicating a low burden. Neurological deficit, use of pain medication, and requirement for corticosteroids were found to be associated with worse SF-36 Physical Component Summary score, while higher levels of depression (higher PHQ-9 score) correlated with worse SF-36 Mental Component Summary score. Conclusions Patients with skull base chordomas have a lower quality of life than the general US population. The most significant determinants of quality of life in the posttreatment phase in this patient population were neurological deficits (sensory deficit and bowel/bladder dysfunction), pain medication use, corticosteroid use, and levels of depression as scored by PHQ-9.

Mental Health Status and Health-Related Quality of Life among Systemic Lupus Erythematosus (SLE) Patients in Thailand: A Multi-Site Study

2020 ◽  
Vol 103 (11) ◽  
pp. 1185-1193
Keyword(s):  
Quality Of Life ◽  
Mental Health ◽  
Health Status ◽  
Lupus Erythematosus ◽  
Self Esteem ◽  
Mental Health Status ◽  
Health Related Quality ◽  
Related Quality ◽  
Health Related

Background: The systemic lupus erythematosus (SLE) patients oftentimes suffer from both physical and psychosocial challenges that may lead to low health-related quality of life (HRQoL). However, limited research has been done in this area. Objective: To examined mental health status and HRQoL among SLE patients in Thailand. Materials and Methods: The present study was a cross-sectional study conducted at the rheumatology clinic of four major hospitals in Thailand. The paper-based questionnaire consisted of demographic, health history such as depression, anxiety, stress Scale (DASS-21), and the Rosenberg self-esteem scale (RSE), and the disease-specific Lupus Quality of Life scale (LupusQoL). Depending on the variable’s level of measurement such as categorical or continuous, Spearman’s Rho or Pearson’s product moment correlation coefficients were used to explore the relationships among the variables. Hierarchical multiple regression was used to identify the predictors of LupusQoL. Results: Among the 387 participants, many might have experienced depression, anxiety, and stress (30%, 51%, and 29%, respectively). Self-esteem among the participants was good (31.8 out of 40). All eight domains of LupusQoL were affected with intimate relationship domain being impacted the most. The overall LupusQoL was significantly associated with the number of prescribed medications (r=–0.23), depression (r=–0.70), anxiety (r=–0.58), stress (r=–0.67), and self-esteem (r=0.59), p<0.001. Significant predictors of the overall LupusQoL were mental health status (depression, anxiety, and stress) and self-esteem, F (3, 81)=43.10, p<0.001, adjusted R²=0.60. Conclusion: SLE patients should be holistically assessed in both physical and psychological aspects. In addition to proper medical treatments, healthcare providers should use a multidisciplinary team approach to resolve the patients’ psychosocial issues, which in turn, may increase the patients’ quality of life. Self-care education may be necessary to help the patients manage the condition and decrease the number of medications. Keywords: Mental health, Quality of life, SLE, Thailand

Sign in / Sign up

Export Citation Format

Share Document