Quality of Life in Thalassemia Major Patients in an Iranian District

Introduction: Beta thalassemia major is a chronic inherited disease which leaves a lot of physical and mental effects on the individual and family. The present research was carried out with the objective of a study on quality of life in thalassemia major patients of Boeir-Ahmad. Method: This research is a descriptiveanalytical study which was carried out on 72 thalassemia patients referring to thalassemia center of Boeir-Ahmad. The patient’s quality of life was studied using an Iranian version of short questionnaire with 36 options on quality of life. The social and individual characters of Patient’s were evaluated and reported. Data are reported using descriptive statistics such as frequency, mean and standard deviation and also inferential statistics. Results: The age mean and standard deviation (SD) of participants were 20.5±4.7 years, 65% were single, and 40% with mild to moderate face deformities. The findings in the research showed the quality of life was relatively desirable in 52 % of the patients, undesirable in 19% and desirable in 29%. Also the findings showed that the emotional role is most affected aspect of quality of life. A significant correlation was observed only with facial changes (p<0.001).undesirable and relatively desirable quality of life and also the high prevalence of facial deformities are significant health problems in Boirahmad thalassemia patients. Conclusion: So we suggest applying appropriate educational plans and psychiatric consultations and also better medical services to reduce the complications of the disease in these patients.


Introduction
Beta Thalassemia major is an inherited hypochromic microcytic anemia which results from abnormality in the structure of beta hemoglobin chain.Based on severity of genetic defect, there are three types of beta Thalassemia including beta Thalassemia minor, inter media and major (Fergus, 2002).Each year, about 100,000 children might be born with severe forms of the disease over the world (Bateman & Wisconsin, 2005).It is also the most prevalent genetic disease in Iran and statistics released by The Iranian Foundation for special disease reported 22000 patients suffering from Thalassemia major (Charity foundation for special disease, 2003).Thalassemia prevalence in Iran varies in terms of geographical zones, so that the highest prevalence is on the Caspian Sea shores and the Persian Gulf.Guilan, Mazandaran, Hormozgan, Khuzestan, and Kohgiluyeh Boer Ahmad are Iranian provinces with the highest prevalence rates (Vichinsky et al., 2005).The disease is associated with severe and long-term complications including anemia, splenomegaly and facial and bone deformities (Borgna-Pignatti et al., 2005).
Also concern over premature death may result in anger, despair, isolation and sever tension in these patients (Ismail et al., 2006).These patients have to get lifetime medical treatment that result in numerous social, psychiatric and economic problems for them (Pakbaz et al., 2005).Generally the disease is diagnosed in childhood, and medical care should starts as soon as the diagnosis is confirmed.Unpleasant, frequent and longitudinal treatment processes, beside their complications, result in increasing psychosocial problems in these patients (Vlachaki 2015).
Consequently a consensus conference of international experts has stated that health related quality of life instruments should include physical, social and emotional functioning as well as perceptions of overall quality of life or general life satisfaction (Mohammadi, 2014).Compared with healthy individuals, thalassemia patients have more serious psycho -social problems.Medical treatment should be along with emotional and psycho -social supports to improve quality of life and prevent irreversible complications in these patients (Aydin et al., 1997).
Boirahmad is a province in south-east of Iran and because of prevalent familial marriages; thalassemia is an important health problem in this county.However no data is available on the quality of life in the thalassemic patients living in Boirahmad.The present research was carried out with the objective of study on quality of life in thalassemia major patients referring to thalassemia center of Boirahmad.

Materials and Methods
Seventy two of thalassemia major patients over the 14 years of age, who referred to thalassemia center of Yasouj, participated in the study.Data was collected interviewing and filling in questionnaire.Demographic data, (age, gender, marital status and economic status), regularity in treatment follow up , type and severity of clinical complications, level of education , history of Despheral use, the common transfusion blood product, RH type, history of familiar thalassemia, consanguinity and facial deformities were recorded in a data sheet.Quality of life was assessed using a standardized Iranian questionnaire.In this questionnaire the patient's quality of life is evaluated using 36 questions, in eight sub-scales including physical function, physical role, bodily pain, general health, vitality, social function, emotional role, and mental health (Montazeri et al., 2005).The questions were scored based on a 1 to 5 Lickert scale.
The eight, scales were summed up into two more general domains named mental health and physical health.The physical health domain included physical role, physical pain, physical function and general health and the mental health domain included vitality, emotional role, social function, and mental health.
Quality of life scores were transformed in percent of maximum attainable point and categorized into three groups; group with impaired quality of life, group with relatively desirable and desirable quality of life.Also quality of life scores are reported as mean ± standard deviation of computed percent.Data were analyzed using SPSS version 15.Binomial variables were compared using U. Man Whitney; categorical variables were compared using Kruskal Wallis test.

Results
Socio-economic characteristics of cases are presented in table 1.Results indicate that mean age of the cases was 20.± 4.7 years and 65% of them were female (n=47).Table 2 shows mean and standard deviation of different aspects of patient's quality of life.Findings showed that 19% of cases had impaired quality of life and 52% had relatively desirable and 29% of cases had desirable quality of life.Mean score in eight domains of quality of life is shown in table 3. The highest score in quality of life was related to bodily pain (73.1±19.9),while the lowest score was in role emotion (55.8±33.)domain.Except for face deformities and complication severity, we didn't find any significant relation between quality of life scores and other variables.Findings related to demographic characteristics are shown in Table 4.

Discussion
Painful and prolonged treatments of thalassemia disease, exposes the patients to various type of social, emotional and behavioral problems, which deteriorate patients quality of life.Hadi et al, in their study on Shiraz thalassemia patients showed that thalassemia regardless of its severity affect different aspects of patient's quality of life (Hadi et al., 1990).
Similar findings were reported on quality of life and mental health in thalassemia patients by Khani et al (khani et al., 2009).Mikelli et al also reported lower quality of life in patients with thalassemia major compared with their handicapped peers (Mikelli & Tsiantis, 2004).
Our findings showed that bodily pain was the most affected domain of quality of life in these patients, while the healthiest domain was related to emotional role.Khani et al in their study on over 15 years old thalassemia patients of Mazandaran reported desirable physical health in the patients (khani et al., 2009) But Alavi et al showed physical health as the most problematic domain in 8-18 years old Shahrkord thalassemia patients (alavi et al., 2007).However in a study on over 14 years old thalassemia patients of Tehran, Tabarsi et al, reported mental health as the most affected quality of life domain (Tabarsi et al., 2006).Hadi also reported physical health a main problem in Shiraz thalassemia patients (Hadi et al., 1990).
In this research the status of emotional health was shown to be impaired.It is likely that in addition to physical and functional problems, the thalassemia patients might be faced to other circumstances such as marriage, higher education and getting appropriate job, which cause many mental problems in these patients (Kashani et al., 1989).
In a study by Pour Movahed, it was shown that thalassmia patients have a high anexity compared with their peers age (Pourmovahed et al., 2003).Also Haqpanah reported that thalassemia children have higher psychiatric disorders including anxiety and depression compared with other chronic disabilities (Haghpanah and Nasirabadi, 2013).These problems might ultimately lead to despair and reduced social functions.In this research, general quality of life and physical health of the patients participating in the research did not show a statistically significant association with level of education, marital status, place of residence, family history of thalassemia and economic status, and the only significant association was with facial deformity.
Lack of medical equipment's, distance from medical centers, costs of treatment and diagnosis, are reported factors that causes economic problems in these patients (Karimov and Asadov, 1995).It is reported that in a group of thalassemia patients 35% had high and 49% had moderate economic problems (Tabarsi et al., 2006).
Due to numerous mental and physical problems these patients are not often successful in continuing their education (Dipalma et al., 1998).With higher education and information, these patients can solve their problems easier, and consequently they will be healthier than uneducated patients (Hadi et al., 1990).Self-care training and educating the patients about complications, signs and symptoms of thalassemia would improve their quality of life (Kasper et al., 2005).In this study facial deformity was the most important factor related to physical health and quality of life.Similar findings are reported by other studies (Tsiantis et al., 1996).Khani Also showed 64.9% of the thalassemia patients suffered from mood and mental disorders that were related to thalassemia deformities (khani et al 2009).Generally, physical deformities of thalassemia extensively expose patients to emotional problems during adulthood and can lower quality of life in these patients (Kashani et al., 1989).

Table 1 .
Social and individual characteristics of Thalacemic patients

Table 2 .
Mean and standard deviation of the marks of the quality of life (General and in components of physical and mental health) in thalassemia patients of Boir Ahmad

Table 3 .
Mean and standard deviation of the marks of the eight domains of SF-36 in the thalassemia major patients of Boir-Ahmad

Table 4 .
Marks (status)of general quality of life in thalassemia patients of Boyer Ahmad in terms of individual-social characteristic Because of insufficient sample volume performance of statistical test was not possible.