Impact of SCOPE Program on Health-Related Quality of Life and Health Status of Children With Thalassemia: A Quasi-Experimental Study.

Background: Iron chelation, blood transfusions, and complication management are typical hospital requirements for children with beta-thalassemia major. This affects their health-related quality of life (HRQoL). The purpose of this study was to evaluate how the Supportive and Coping strategies, Ongoing Assessment, Prevention of Complications, and Empowerment (SCOPE) Program impacted the HRQoL and overall health of children with thalassemia. Method: The study employed a quasi-experimental pretest-posttest control group with a sequential follow-up design. A nonprobability purposive sampling technique was used to include 80 children with beta-thalassemia major in the sample, ranging in age from 6 to 18. Data were gathered using a Demographic Profile, PedsQLTM Version 4.0, and a Clinical Profile. The children in the intervention group received the SCOPE Program over the course of 6 months. The data collection included a pretest and a posttest with four follow-up evaluations. Results: During the pretest, children with thalassemia had a very low HRQoL. However, the final assessment after the intervention showed a significant difference in the mean scores between the two groups in the areas of physical functioning (p = .001), emotional functioning (p = .0001), social functioning (p = .001), and school functioning (p = .001). Growth indicators also demonstrated a notable improvement in the intervention group of children. Discussion: The SCOPE Program may be a thorough and efficient intervention for enhancing the general health of children with thalassemia. It can be used as a cooperative, well-organized, family-focused care strategy. Further study with a larger sample size is suggested.

[Health status and quality of life in ß-thalassemia adults in Marseille, France]. / État de santé et qualité de vie des patients ß-thalassémiques adultes à Marseille, France.

INTRODUCTION: The life expectancy of ß-thalassemia patients has increased over the last 20 years. In this study, we evaluated the current health status and quality of life of these patients managed in a reference center in Marseille. METHODS: This is a single-center, descriptive study conducted between June and August 2019 in patients over 18 years of age with ß-thalassemia major or intermedia. Clinical and paraclinical data were collected retrospectively and the SF-36 health survey questionnaire was proposed to each patient. RESULTS: 43 of 64 selected patients were included and divided into 2 groups: 35 patients with transfusion-dependent ß-thalassemia and 8 patients with non-transfusion-dependent ß-thalassemia. Liver iron overload is the most frequent complication, present in 80% of transfusion-dependent and 62.5% of non-transfusion-dependent patients. Cardiac iron overload is present only in the transfusion dependent ß-thalassemia group (20%). Hypogonadotropic hypogonadism remains the most common endocrine disorder (41.9%) followed by osteoporosis (37.2%). Among the 31 patients who completed the SF-36 questionnaire, physical and mental quality of life scores were lowered in transfusion dependent (respectively 42.7 and 46.8) as in non-transfusion-dependent patients (respectively 43.8 and 28.9). CONCLUSION: Despite an improvement in medical care, our patients with ß-thalassemia show an alteration in their quality of life that will need to be characterized in the entire French cohort.

Health-Related Quality-of-Life Impacts Associated with Transfusion-Dependent ß-Thalassemia in the USA and UK: A Qualitative Assessment.

BACKGROUND: Individuals living with transfusion-dependent ß-thalassemia (TDT) experience reduced health-related quality of life due to fatigue and chronic pain, which cause disruptions to daily life. Currently, limited qualitative data exist that describe these impacts. OBJECTIVE: This study aimed to examine the ways in which symptoms and current treatments of TDT impact health-related quality of life, to holistically describe the humanistic burden of TDT, and to identify the unmet needs of individuals living with TDT. METHODS: Adults (aged ≥ 18 years) with TDT and caregivers of adolescents (aged 12‒17 years) with TDT participated in semi-structured one-on-one virtual interviews and focus group discussions. Interviews were conducted in the USA and UK and lasted approximately 60 minutes. After transcription, the interviews were analyzed thematically using a framework approach. RESULTS: A total of ten interviews/focus group discussions (six interviews and four focus group discussions) were conducted with 14 adults with TDT and two caregivers of adolescents with TDT. A framework analysis revealed five themes describing health-related quality of life (negative impacts on daily activities, social life, family life, work and education, and psychological well-being) and three themes describing the lived experience of TDT (impact of red blood cell transfusions and iron chelation therapy, treatment, and stigma). Physical, psychological, and treatment-related factors contributed to negative impacts on daily activities, social and family life, and work and education. Concerns about reduced lifespan, relationships and family planning, and financial independence were detrimental to participants' mental well-being. Participants reported having high resilience to the many physical and psychological challenges of living with TDT. A lack of TDT-specific knowledge among healthcare professionals, particularly regarding chronic pain associated with the disease, left some participants feeling ignored or undermined. Additionally, many participants experienced stigma and were reluctant to disclose their disease to others. CONCLUSIONS: Individuals living with TDT experience substantial negative impacts on health-related quality of life that disrupt their daily lives, disruptions that are intensified by inadequate healthcare interactions, demanding treatment schedules, and stigma. Our study highlights the unmet needs of individuals living with TDT, especially for alternative treatments that reduce or eliminate the need for red blood cell transfusions and iron chelation therapy.

Cascade screening for beta-thalassemia in Pakistan: development, feasibility and acceptability of a decision support intervention for relatives.

The government-funded 'Punjab Thalassaemia Prevention Project' (PTPP) in Pakistan includes cascade screening for biological relatives of children with beta-Thalassaemia Major (ß-TM). However, there is low uptake of cascade screening. This paper presents the (i) development of a paper-based 'decision support intervention for relatives' (DeSIRe) to enable PTPP Field Officers to facilitate informed decision making about carrier testing, and (ii) assessment of the feasibility and acceptability of the DeSIRe. The intervention was developed using the International Patient Decision Aids Standards quality criteria and Ottawa Decision Support Framework. Twelve focus groups were conducted (September and October 2020) to explore the views of healthcare professionals (HCPs) and relatives of children with ß-TM, in six cities. The focus groups were attended by 117 participants (60 HCPs and 57 relatives). Thematic analysis showed that the DeSIRe was considered acceptable for supporting relatives to make informed decisions about cascade screening, and potentially feasible for use in clinical practice. Suggestions for changing some words, the structure and adding information about how carrier testing relates to consanguineous marriages will enable further development of the DeSIRe. Participants generally welcomed the DeSIRe; however, they highlighted the perceived need to use more directive language, hence showed a cultural preference for directive genetic counselling. The findings highlight challenges for researchers using western theories, frameworks, policies and clinical guidelines to develop decision support interventions for implementation more globally. Future research is needed to evaluate the use of the DeSIRe in routine practice and whether it enables relatives to make informed decisions.

Socio-religious Prognosticators of Psychosocial Burden of Beta Thalassemia Major.

The study investigates the socio-religious factors in the propagation of genetically inherited disease of Beta thalassemia. The disorder which reportedly has a significant protraction through repeated cousin marriages results in the social maladjustment of the parents of the sick children due to constant depression, anxiety, and weak social interaction and may lead to social isolation as well. This research aims to find out the significant effect of socio-religious trends on psychosocial burden of beta thalassemia major among cousin and non-cousin couples in the province of Punjab in Pakistan. It takes a sample of 932 parents of sick children, among whom 735 were married with cousins and 197 with non-cousins, for data collection. The findings reveal that inadequate knowledge of the disease, insufficient or misdirected social support, stigmatization, and marriage breakups caused by the disease, superstitions, and misinterpretations of religion and the subsequent practices accordingly as significant predictors of psychosocial burden of beta thalassemia major among non-cousins and cousin couples. Additionally, it also finds patriarchy as only significant predictors of outcome variable among cousin couples.

Development of the Thalassaemia Adult Life Index (ThALI).

BACKGROUND: Beta Thalassaemia Major (ßTM) is a chronic genetic illness whereby the challenges faced by patients exposes them to increased risk of psychosocial issues. Despite this, a disease-specific tool to measure the impact of this illness on adult patients has yet to be developed. METHODS: In collaboration with ßTM adult patients, this study aimed to develop a comprehensive, disease-specific, easy to use psychometrically sound tool to measure the impact of chelation and transfusion dependent ßTM in a cross-cultural patient group in England.The Thalassaemia Life Index (ThALI) was developed in two stages - item generation and pre-testing and item reduction - in collaboration with service users. Recruited adult patients shaped the design of the instrument including its statements and subscales. Standard item reduction techniques were used to develop the instrument. RESULTS: The final version of the ThALI encompasses 35 statements and five sub-scales - general physical health, coping, body image, appearance and confidence, social relationships and autonomy. This endorses the multidimensionality of quality of life (QoL). The factor structure of the ThALI is highly stable and its internal consistency is high (alpha = 0.87 for the overall scale; 0.83-0.94 for its subscales). The ThALI has sound scaling assumptions, acceptability and score variability. Content validity was confirmed by experts and service user interviewees. The loadings for the items retained were adequate and the item discriminant validity sound. CONCLUSIONS: The ThALI covers the impact of ßTM in adult patients. Preliminary testing shows its multidimensionality to be reliable and valid. The national authentication of the tool with patients treated in Centres of Excellence will aim to provide further evidence regarding the ThALI's psychometric properties. Once authenticated, the ThALI may be utilised in research and in clinical settings to assess the effects of new therapies and/or interventions from the patients' perspective to inform practice and/or to identify areas of concern.

Severity of heart failure and health-related quality of life in beta-thalassemia patients: a cross-sectional study.

Cardiovascular complications account for a substantial increase in morbidity and mortality in beta-thalassemia patients. Many patients have structural heart disease, and some of them present with symptomatic heart failure (HF). Quality of life (QOL) of beta-thalassemia patients is lower than that of the general population. The aim of our study was to explore the relationship between HF stages and QOL in beta-thalassemia patients. Seventy-three consecutive adult beta-thalassemia patients took part in this cross-sectional study. Stages of HF, classified with increasing severity as A, B, and C, were determined based on ACC/AHA guidelines. QOL was assessed using the SF-36 questionnaire. Fifteen patients had stage C HF, twenty-eight had stage B HF, and the remaining were considered stage A patients, as beta thalassemia is a predisposing factor for HF. All QOL domains except for bodily pain were significantly lower in stage C patients than in stage A patients. Stage C patients had significantly lower QOL scores for physical functioning, role physical, and social functioning domains than stage B patients. Stage B patients' QOL differed from stage A patients only in the vitality domain. In the multiple regression analysis which took several demographic and clinical factors into account, stage of HF was the most important factor associated with QOL, and negatively and significantly related to five QOL domains, namely physical functioning, role physical, general health, social functioning, and vitality. In conclusion, QOL is negatively affected by the severity of heart failure in beta-thalassemia patients.

Health state utilities associated with treatment for transfusion-dependent ß-thalassemia.

OBJECTIVES: Transfusion-dependent ß-thalassemia (TDT) is a genetic disease that affects production of red blood cells. Conventional treatment involves regular red blood cell transfusions and iron chelation, which has a substantial impact on quality of life. While potentially curative, allogeneic hematopoietic stem cell transplantation (allo-HSCT) is associated with risk of complications, including graft-versus-host disease (GvHD). Gene addition therapy, a novel treatment approach, involves autologous transplantation of the patient's own genetically modified hematopoietic stem cells. The purpose of this study was to estimate utilities associated with treatment approaches for TDT. METHODS: General population respondents in England valued eight health state vignettes (developed with clinician, patient, and parent input) in time trade-off interviews. RESULTS: A total of 207 participants completed interviews (49.8% female; mean age = 43.2 years). Mean (SD) utilities for the pre-transplant health states were 0.73 (0.25) with oral chelation and 0.63 (0.32) with subcutaneous chelation. Mean utilities for the transplant year were 0.62 (0.35) for gene addition therapy, 0.47 (0.39) for allo-HSCT, and 0.39 (0.39) for allo-HSCT with acute GvHD. Post-transplant utilities were 0.93 (0.15) for transfusion independent, 0.75 (0.25) for 60% transfusion reduction, and 0.51 (0.38) for chronic GvHD. Acute and chronic GvHD were associated with significant disutility (acute = - 0.09, p < 0.0001; chronic = - 0.42, p < 0.0001). CONCLUSIONS: Utilities followed expected patterns, with logical differences between treatment options for TDT and substantially greater utility for transfusion independence than for ongoing treatment involving transfusion and chelation. These utilities may be useful in cost-utility models estimating the value of treatments for TDT.

Marriage patterns in Sri Lanka and the prevalence of parental consanguinity in patients with ß-thalassaemia: a cross-sectional descriptive analysis.

Consanguineous marriages potentially play an important role in the transmission of ß-thalassaemia in many communities. This study aimed to determine the rate and socio-demographic associations of consanguineous marriages and to assess the influence on the prevalence of ß-thalassaemia in Sri Lanka. Three marriage registrars from each district of Sri Lanka were randomly selected to prospectively collect data on all couples who registered their marriage during a 6-month period starting 1st July 2009. Separately, the parents of patients with ß-thalassaemia were interviewed to identify consanguinity. A total of 5255 marriages were recorded from 22 districts. The average age at marriage was 27.3 (±6.1) years for males and 24.1 (±5.7) years for females. A majority (71%) of marriages were 'love' marriages, except in the Moor community where 84% were 'arranged' marriages. Overall, the national consanguinity rate was 7.4%. It was significantly higher among ethnic Tamils (22.4%) compared with Sinhalese (3.8%) and Moors (3.2%) (p < 0.001). Consanguinity rates were also higher in 'arranged' as opposed to 'love' marriages (11.7% vs 5.6%, p < 0.001). In patients with ß-thalassaemia, the overall consanguinity rate was 14.5%; it was highest among Tamils (44%) and lowest among Sinhalese (12%). Parental consanguinity among patients with ß-thalassaemia was double the national average. Although consanguinity is not the major factor in the transmission of the disease in the country, emphasis should be given to this significant practice when conducting ß-thalassaemia prevention and awareness campaigns, especially in high-prevalence communities.

Health related quality of life among children with transfusion dependent ß-thalassaemia major and haemoglobin E ß-thalassaemia in Sri Lanka: a case control study.

BACKGROUND: Thalassaemia is a chronic disease without an effective cure in a majority. The clinical management has improved considerably during recent years; however, minimal attempts are made to up lift the quality of life among patients, especially in developing countries. Here we aim to describe and compare and to determine factors associated with health related quality of life among patients with transfusion dependent ß-thalassaemia major and haemoglobin E ß-thalassemia in Sri Lanka. METHODS: A case control study was conducted in the three largest thalassaemia centres of Sri Lanka. All patients with transfusion dependent ß-thalassaemia (ß-thalassaemia major and haemoglobin E ß-thalassaemia) aged 5-18 years were recruited as cases whilst a randomly selected group of children without chronic diseases were recruited as controls. Socio-demographic and clinical data were collected using an interviewer-administered questionnaire and health related quality of life was measured using the validated Paediatric Quality of Life Inventory Version 4.0. RESULTS: Two hundred and seventy one patients with transfusion dependent ß-thalassaemia (male-49.1%; mean age- 10.9 ± 3.6 years) and 254 controls (male-47.2%; mean age- 10.4 ± 3.5 years) were recruited. Mean health-related quality of life scores were significantly lower in patients compared to controls (72.9 vs. 91.5, p < 0.001). Of the patients, 224 (84%) had ß-thalassaemia major and 43 (16%) had haemoglobin E ß-thalassaemia. Quality of life scores in psychological health (p < 0.05), emotional functioning (p < 0.05) and social functioning (p < 0.05) were significantly lower in patients with haemoglobin E ß-thalassaemia compared to ß-thalassaemia major. Splenectomy (p < 0.05), short stature (p < 0.05), under nutrition (p < 0.05) and longer hospital stays (p < 0.05) were significantly associated with lower quality of life scores. CONCLUSIONS: Despite improvements in management, the quality of life among patients with ß-thalassaemia still remains low. This is more pronounced in the subset of patients with haemoglobin E ß-thalassaemia. Splenectomy, short stature, undernutrition and longer hospital stays were significantly associated with poor quality of life. It is timely, even in developing countries, to direct emphasis and to take appropriate steps to improve standards of living and quality of life of patients with ß-thalassaemia.

[Psychosocial and academic consequences of beta-thalassemia major in Tunisia]. / Retentissement psychosocial et scolaire de la bêta-thalassémie majeure en Tunisie.

In Tunisia, beta-thalassemia major is a real public health problem. A study carried out of patients affected shows that for them, this chronic haemoglobinopathy is a disability hampering their physical activities, their social integration, their academic results and their emotional life.

Health-related quality of life (HRQoL) in beta-thalassemia major (ß-TM) patients assessed by 36-item short form health survey (SF-36): a meta-analysis.

PURPOSE: The main purpose of this meta-analysis was to evaluate the impact of beta-thalassemia major (BTM) on the health-related quality of life as assessed by the medical outcomes short-form-36 questionnaire (SF-36). METHODS: A systematic literature search was performed on Cochrane library, Web of Science, Scopus, Science Direct, ProQues, Medline/PubMed, Scholar Google until March 17, 2017 to obtain eligible studies. A fixed effect model was applied to summarize the scores of each domain. The radar chart was used to compare the scores of BTM patients with other health conditions. Spearman's correlation analysis and meta-regression were used to explore the related factors. RESULTS: 26 studies were included in this study, which were all reliable to summarize the scores of the SF36. Pooled mean scores of the physical health domains ranged from 52.74 to 74.5, with the GH and PF domains being the lowest and the highest, respectively. Further, the pooled mean scores of the mental health domains varied between 59.6 and 71.11, with the (MH-VT) and SF domains being the maximum and the minimum, respectively. Patients with BTM had a substantially compromised HRQoL in comparison with the general population. CONCLUSION: BTM could adversely affect the HRQoL of patients. Measuring HRQoL should be considered as an essential part of the overall assessment of health status of BTM patients, which would provide valuable clues for improving the management of disease and making decisions on the treatment.

The burden of psychiatric illnesses in adult patients with beta-thalassemia: a 5-year nationwide inpatient evaluation in the United States.

Beta-thalassemia (ß-thalassemia) is a frequently inherited monogenic disorder worldwide with severe physical and mental health comorbidities. The aim of this study is to evaluate the burden of psychiatric disorders in adult patients hospitalized with ß-thalassemia in the United States (US). We utilized the discharge data from the National Inpatient Sample (NIS) from 2010 through 2014 to identify hospitalized ß-thalassemia patients who are 18 years and older. We assessed categorical and continuous variables using chi-square test and Student's t test respectively. The burden of psychiatric disorders was assessed using descriptive statistics and represented in terms of frequency (n) and percentage (%). A p value threshold of < 0.05 was considered clinically significant. All statistical analyses were performed using SPSS version 22.0 (IBM Corp., Armonk, NY, USA). Of the 10,046 ß-thalassemia patients studied, 24.4% (n = 2448) reported psychiatric disorders. Mood disorders (15.2%; inclusive of 10.3% depression), followed by anxiety (9.4%), and substance abuse disorder (6.5%; 4.3% drug abuse, 2.2% alcohol abuse) were most prevalent. Comorbid mental illnesses increased the length of stay by 44.6% and total costs by 23.4%. All-cause mortality rate was reported at 1.9% in overall ß-thalassemia patients. This study highlights higher rates of psychiatric disorders such as depression and anxiety and correlated increases in length of stay and cost in hospitalized ß-thalassemia patients. To improve medical outcomes and curtail unnecessary health-care costs, implementation of an integrated care model can be considered.

Psychosocial Issues in Children With Thalassemia: From Identification to a Model for Management in a Developing Country.

INTRODUCTION: Children with chronic disorders like ß thalassemia major (TM) and their care givers are known to face various psychosocial problems. This study used screening tests to detect these psychosocial issues so that prompt referral for counseling is possible. METHODS: A semistructured demographic questionnaire, Pediatric Symptom Checklist (PSC-17) and the Strengths and Difficulties Questionnaire (SDQ) were administered to 30 children with TM, on regular treatment at a tertiary care hospital. The same questionnaires were administered to age-matched and sex-matched controls. Parents were administered the General Health Questionnaire (GHQ). Children with TM scored higher on both PSC-17 (9.93 vs. 4.87; P<0.001) and SDQ (14.9 vs. 10.9; P=0.008). The parents of children with TM scored higher on the GHQ (10.3 vs. 8.0; P=0.027). RESULTS: Psychosocial morbidity was higher in children with TM and their care-givers. Screening tools help detect children and care givers in need of formal counseling. CONCLUSIONS: The study makes a case for management of these issues by the pediatrician in tandem with mental health professionals. Some strategies that can be used in managing these children and their families are presented. In addition, issues in management with particular focus on a developing country context are highlighted and discussed.

Factors affecting quality of life in children and adolescents with thalassemia in Iraqi Kurdistan.

OBJECTIVES: To assess the health related quality of life (HRQoL) in Iraqi Kurd children and adolescents with thalassemia, and identify the factors that affect it. METHODS:  In the period between May and June 2018, 100 thalassemic patients and 100 healthy subjects between the ages of 6-18 years were enrolled. The patients included 73 with thalassemia major (TM) and 27 with intermedia (TI). Patients were clinically re-evaluated, and the pediatric quality of life inventory (PedsQL) 4.0 was administered by both child and parent reports. Results: The mean HRQoL score of thalassemic patients was significantly lower than that of healthy subjects, with lowest scores in physical functioning. Furthermore, the mean HRQoL of TM was significantly lower than that of TI subgroup. Significantly lower mean HRQoL scores were seen in those taking ≥6 transfusions/year, with hepatitis C infection, with illiterate parents, and those on oral iron chelation. Pearson correlation revealed that HRQoL was negatively associated with age, frequency of transfusions, and serum ferritin, but positively correlated with age at starting transfusion and age at diagnosis. Only age and serum ferritin remained significant by multivariate analysis. CONCLUSION:  This study shows that among Iraqi Kurds with thalassemia, the disease has a significant negative impact on quality of life, with age and serum ferritin being identified as independent predictors.  Psychosocial, educational, and patient-centered management programs may be needed to improve HRQoL in this disease.

Health-related quality of life in pediatric and adolescent patients with transfusion-dependent ß-thalassemia in upper Egypt (single center study).

BACKGROUND: Thalassemia is a major health problem that disturbs the lifestyle of the affected patient. The aim of this work is to detect the impact of thalassemia on the quality of life regarding physical, social, emotional, psychological scored assessment. METHODS: A case-control survey was conducted in Minia University children's hospital on 64 patients recruiting pediatric hematology outpatient clinic from July 2014 to February 2017. PedsQL™ 4.0 Generic Core Scale (Arabic version) was used to assess HRQOL in 64 thalassemia patients between 8 and 18 years of ages. Other related clinical data of the involved patients were collected from the pediatric hematology records. RESULTS: Mean physical, emotional, social, school performance, psychological and total scores (- 36.9 ± 20.9, 49.4 ± 17, 47.2 ± 21.3, 38.5 ± 15.5, 45.3 ± 13.8, 47.9 ± 38.8 respectively) were significantly decreased compared with control (p = 0.001 for all). The younger age group had better scores regarding social, emotional, psychological and total scores compared to older ones (p = 0.01, 0.03, 0.01 and 0.009 respectively). Older age of starting transfusion was statistically significant protecting factor from poor physical QOL in thalassemia patients (OR = 0.96, p = 0.03). The presence of hepatomegaly was a statistically significant predictor for poor physical QOL (OR = 8.5, p = 0.02). Household income was the statistically significant predictor for poor emotional QOL (OR = 5.03, p = 0.04). High serum ferritin was the statistically significant predictor for poor social QOL (OR = 1.1, CI 95%=, p = 0.04). Regarding poor psychological QOL (OR = 0.94, p = 0.01) and total QOL (OR = 0.94, p = 0.01) scores, older age of starting transfusion was the statistically significant protecting factor. CONCLUSION: Scheduled programs giving psychosocial help and a network connecting between the patients, school officials, thalassemia caregivers and the physician is required especially in developing countries where the health services are not integrated with social organizations. Special school services for thalassemia patients are required to deal with the repeated absence and anemia induced low mental performance of thalassemia children.

Quality of life among adolescents aged 14 to 18 years with Beta-Thalassemia Major (TM) in Qatar.

BACKGROUND: Thalassemia  is a heterogeneous group of inherited disorders of hemoglobin synthesis. It is a common disease in Mediterranean, Southeast Asia, Indian subcontinent, and Middle East countries, including Qatar. PURPOSE: The aim of this study was to assess the quality of life (QOL) among patients aged 14 to 18 years with thalassemia major (TM) in Qatar and correlates their QOL with bio-demographic data of the patients compared to healthy controls. MATERIALS AND METHODS: This cross-sectional study measured the QOL in adolescents with thalassemia major who were attending ambulatory units in a tertiary hospital in Qatar. Forty children and adolescents with TM and 40 healthy participants were enrolled in the study. Forty-two (52.5%) participants were males and 38 (47.5%) females. Data were collected utilizing PedsQLTM 4.0 generic core scale and were analyzed using the appropriate statistical method. RESULTS: Children with TM had significantly lower and more variable overall quality of life score (69.1 ± 16.8) compared to healthy matched children (77 ± 12.8), (p <0.001). Both groups were not different from the physical, emotional, and social domains. Thalassemic  adolescents had also a significantly lower school performance. CONCLUSIONS: TM adversely affects the QOL of children and adolescents and this necessitates applying more efforts to help them improve and achieve a desirable quality of life. Patients with TM need more attention in schools that can be accomplished by implementing a special program for their management that needs a mutual collaboration between Ministry of Public Health (MoPH) and Ministry of Education (MoE) in Qatar.

Decisional conflict and regret: shared decision-making about pregnancy affected by ß-thalassemia major in Southeast of Iran.

To study the effect of shared decision-making (SDM) on decisional conflict (DC) scores immediately after consultation and to assess the decisional regret (DR) scores in the first 3 months following women's decision regarding termination of pregnancy. This quasi-experimental study was conducted during August 3rd-February 20th, 2016. We included 80 women whose fetuses were diagnosed with ß-thalassemia major (ß-TM) through chorionic villi sampling and were referred to the only prenatal diagnosis center at Ali-Asghar Hospital, Zahedan, Iran. While the control group went through the routine procedures, the intervention group received a 90-min counseling session based on SDM. The demographic characteristics form and DC scale were filled out immediately after the consultation session. After 3 months, the women were contacted via telephone call to collect data on their level of DR. The mean DC score was significantly (P = < 0.0025) lower in the intervention group (8.47 ± 4.63) compared with the control group (44.10 ± 14.5). Moreover, the mean score of DR was significantly (P = 0.004) lower in the intervention group (9.37 ± 15.44) compared with the control group (24.37 ± 23.42). SDM consultation can help women experience significantly lower levels of DC and DR.