ABSTRACT
In the present study, the aim was to explore the quality of life of thalassaemia major (TM) patients according to age, sex, school performance, severity and complications of the disease. A cross-sectional descriptive study was conducted. Quality of life was evaluated by 4 questionnaires; demographic characteristics, the short form 36 health survey (SF-36), Persian version of symptoms checklist-90-revised (SCL-90-R) and life satisfaction index (LSI). 687 (41.08%) of the eligible patients with TM completed the questionnaires. With the SF-36 questionnaire, 329 (47.9%) participants had an excellent level of physical functioning. With the SCL-90-R questionnaire, 446 (64.9%) participants had a global severity index of > 0.7, considered psychiatric patients, 141 (20.5%) patients had GSI between 0.4 and 0.7, considered suspected psychiatric patients and 100 (14.6%) participants had GSI of < 0.4, considered non-psychiatric patients. The mean score of LSI questionnaire was 20.50 (SD 5.95), range 2-37. Beta-TM patients are at risk of psychiatric symptoms and need appropriate psychiatric counselling.
Subject(s)
Health Status , Mental Health , Quality of Life , beta-Thalassemia/epidemiology , beta-Thalassemia/rehabilitation , Adolescent , Adult , Comorbidity , Cross-Sectional Studies , Educational Status , Employment , Female , Health Surveys , Humans , Iran/epidemiology , Male , Mental Disorders/epidemiology , Middle Aged , beta-Thalassemia/psychologyABSTRACT
This study explores the rate of psychosocial dysfunction in affected and unaffected children from families with haemophilia or beta-thalassaemia, as part of a cross-sectional, multicentre study into the resilience of 115 families with blood disorders. Sociodemographic and developmental data were collected from the parents using a standardized and semi-structured interview format, and medical data were obtained from the clinician. The children's social functioning over the year prior to the assessment was assessed with The Social Adjustment Scale adapted for school-aged children. Children with beta-thalassaemia showed significantly higher rates of social dysfunction than their unaffected siblings or children with haemophilia and their siblings. Older children showed significantly higher social dysfunction at school. The high rate of social dysfunction in children with beta-thalassaemia compared with unaffected siblings is likely to have a basis in the negative experiences associated with their medical problems. In contrast, the therapeutic advances in haemophilia allows boys to lead an almost normal life. Overall, the rates of social dysfunction in families with both these disorders proved commoner than reported in population surveys, but with the unavailability of local population controls, caution needs to be exercised in the interpretation of this finding.
Subject(s)
Blood Coagulation Disorders/psychology , Social Adjustment , Adolescent , Age Factors , Blood Coagulation Disorders/rehabilitation , Child , Cross-Sectional Studies , Family Health , Female , Hemophilia A/psychology , Hemophilia A/rehabilitation , Humans , Leisure Activities/psychology , Male , Psychometrics , Schools , Sex Factors , beta-Thalassemia/psychology , beta-Thalassemia/rehabilitationABSTRACT
The thalassaemias are a heterogeneous group of inherited haemoglobin disorders. With modern treatment, most patients with severe disease survive to adult life. If they have no complications, and their general health is good, then the condition should have little impact on their fitness for work, though exposure to agents that are toxic or suppress the bone marrow should be avoided. Most patients with thalassaemia have minor disease and are asymptomatic. Their condition generally has no effects on their fitness to work. Pre-employment screening for thalassaemia is not justified.
