- Email: [email protected]
Psychosocial burden of β-thalassaemia major in Antalya, south Turkey
Social Science & Medicine 56 (2003) 815–819
Psychosocial burden of b-thalassaemia major in Antalya, south Turkey Duran Canatana, Siret Ratipb,*, Saniye Kaptanc, Ruya . Cosand b
a Department of Paediatric Haematology, Antalya State Hospital, P.O. Box 624, 07001 Antalya, Turkey Department of Haematology, Marmara University Hospital, Tophanelioglu Caddesi, Altunizade, 81190 Istanbul, Turkey c Thalassaemia Centre, Antalya State Hospital, P.O. Box 624, 07001 Antalya, Turkey d Antalya State Hospital, P.O. Box 624, 07001 Antalya, Turkey
Abstract b-thalassaemia is a recessively inherited blood disorder characterised by chronic anaemia. It requires monthly blood transfusions and regular iron chelation. Thousands of affected children are born annually and the magnitude of the problem is most severe in developing countries. Ninety-nine children and 32 adults with thalassaemia major, and 112 parents of patients were interviewed in Antalya, south Turkey, using specifically designed questionnaires to evaluate psychosocial burden. The education of most of the thalassaemic children of school age (60%) was affected, mainly due to having to attend hospital for investigation and transfusions. A high level of parental anxiety (82%) was reported. Nearly half of the families (47%) had employment and financial problems as a result of thalassaemia, yet there was a low level of marital breakdown (1.8%). A substantial majority (93%) of the parental couples would have chosen to terminate an affected pregnancy if they had known that the foetus had thalassaemia major. The results reflect the need for a national policy for public education and screening of thalassaemia in Turkey in order to offer prenatal diagnosis for all families at risk of homozygous thalassaemia. r 2002 Elsevier Science Ltd. All rights reserved. Keywords: Turkey; b-thalassaemia major; Psychosocial burden; Developing country; Prenatal diagnosis
Background b-thalassaemia is a recessively inherited blood disorder characterised by chronic anaemia, resulting from deficient synthesis of b-chains of haemoglobin. Thalassaemia major requires monthly transfusions and regular infusions for life of the iron chelating agent desferrioxamine (Modell & Berdoukas, 1984). The condition requires regular medical supervision as distressing clinical problems can occur, including growth retardation, sexual dysfunction, heart failure, diabetes mellitus secondary to iron overload, liver disease due to hepatitis B or C secondary to blood transfusions as well as HIV infection, and facial and bony deformities due to the anaemia itself. *Corresponding author. Tel.: +90-216-326-5415; fax: +90216-326-6240. E-mail address: [email protected] (S. Ratip).
Thalassaemias are common in Mediterranean countries, the Middle East, Africa and South East Asia. The migration of populations has extended the thalassaemias to other European countries, North and South American countries and Australia. Thousands of affected children are born annually and many live long enough to require treatment (Weatherall, 1998). The magnitude of the problem is most severe in developing countries and it is essential to make the thalassaemia problem more visible to governments and international health agencies involved in health care in these countries. The average prevalence of the b-thalassaemia carrier state in Turkey is 2.1%, but there is regional variability (Arcasoy, 1992). South Turkey has the highest level with 11.7% in Antalya district (Akar et al., 1998). In Turkey, as in other developing countries, medical management for thalassaemia patients has understandably taken precedence over psychosocial problems, which received little attention in the past, leading to a lack of knowledge
0277-9536/03/$ - see front matter r 2002 Elsevier Science Ltd. All rights reserved. PII: S 0 2 7 7 - 9 5 3 6 ( 0 2 ) 0 0 0 8 0 - 1
816
D. Canatan et al. / Social Science & Medicine 56 (2003) 815–819
about this issue. However, psychosocial problems are of primary importance to patients; despite improvements in survival, the burdensome nature of the treatment often results in patients giving up the iron chelation treatment partially or completely. Psychosocial problems for parents are also important, not only because they cause distress, but because adequate support, especially for younger thalassaemics, is best achieved by supporting parents and increasing their confidence in coping with the illness (Ratip & Modell, 1996). The aim of this study was to analyse the psychosocial burden of thalassaemia major from a developing country’s perspective in a major thalassaemia centre in Antalya, south Turkey.
Methods The study was undertaken at the thalassaemia centre of Antalya State Hospital, which cares for 183 thalassaemic children of 163 families. One hundred and fifty-one of these were paediatric and 32 were adult patients. Five parents of 6 affected children refused to participate in the study. Forty-six families with 46 paediatric children, whose attendance at the unit was less than 1 yr, were not approached. Thirteen of these 46 families had a recent diagnosis and so would not yet have experienced the chronic nature of the illness. Children (all aged o5 yr) of the other 33 families were recent referrals from health centres in villages within the Antalya district. They were not approached as they had attended the unit on only a few occasions and were not yet familiar enough with the medical staff to be interviewed. Therefore, the final sample size was 99 children and 32 adult patients (total number 131) and 112 parents of patients. Sixty-five patients were male and 66 female. They were provided with written information about the interview and their consent was obtained. The paediatric patients were aged 1–15 yr (mean 7.0; SD 4.6 yr), and adult patients were aged 16–27 yr (mean 19.3; SD 3.3 yr): eight families had 2 affected children, one family had 3 affected children and another family had 4 affected children. Out of the 32 adult patients, 2 were married and one had a child. Patients and parents were interviewed using a structured questionnaire either in the clinic or at home, depending on their choice. Preexisting questionnaire measures of psychosocial adjustment are either inappropriate for this chronic inherited disease, or too cumbersome for use in a clinical setting (Rutter, 1967; Rutter, Tizard, & Whitmore, 1970; Goldberg & Hillier, 1979; Deasy-Spinetta, Spinetta, & Oxman, 1988; Achenbach, 1992). Consequently, a questionnaire designed to measure the psychosocial burden of the thalassaemias (Ratip, 1996), and used in previous studies (Ratip et al., 1995; Ratip & Modell, 1996; Klein et al., 1998) was
translated into Turkish and used here. (Copies may be obtained from the author.) Briefly, the questions were designed to be simple, non-judgmental and acceptable to all cultures. The main topics included are shown with the results. Three versions are available: (A) a parentreport form for patients under 16 yr of age; (B) a selfreport form for adult patients; and (C) a form for parents to report their own situation. Eleven variables were created, each based on responses to one or more questions bearing on that topic. For example, items concerning anxiety were: Do you worry about thalassaemia (not at all, a little, sometimes, a lot, all the time)? Why? What is your biggest worry? Answers for this and other variables were then coded by the interviewer as none or unaffected (0) to severe (3), given a specific operational definition of each code. The adult patients were interviewed on their own. The parent-report form for paediatric patients was used to interview parents in the presence of their child. (In this culture, parents expect to be answerable for children under 16 yr.) Parents were interviewed together using the parent form, and the consensual answers recorded. The interviewers (SK/RC) wrote the answers verbatim during the interview, which lasted approximately 1 h/ person (range 45 min–1.5 h). At the end of the interview each response was graded for degree of impairment (0=unaffected, 1=mild, 2=moderate, 3=severe). We ensured reliability of grading by having two of us (DC/ SR) to rate a proportion of interviews.
Results Results of psychosocial burden for adult and paediatric patients and their parents are presented in Table 1. Statistical analysis revealed a weak positive correlation of 0.23 (95% confidence interval 0.03–0.41) between paediatric patients’ and their parents’ psychosocial burden scores.
Discussion Currently, there are 1983 registered patients with homozygous thalassaemia in Turkey, nearly one-third (36%) of whom are resident in the southern provinces. Patients with homozygous thalassaemia in Antalya district constitute 45% of the patients in south Turkey (Akar et al., 1998), and Antalya State Hospital cares for nearly one-quarter (26%) of these patients. The sample is therefore representative. The study is the largest in the literature analysing psychosocial problems of patients with thalassaemia major and their parents in a developing country. The attitude to prenatal diagnosis in this study is similar to parents, mainly of Mediterranean descent, of adult
D. Canatan et al. / Social Science & Medicine 56 (2003) 815–819
817
Table 1 Frequencies (%) of thalassaemia patients and their parents with moderate or severe psychosocial burden Aspect of life
Adult patients (n ¼ 32) Freq. (%)
Paediatric patients (n ¼ 99) Freq. (%)
Parents (n ¼ 112) Freq. (%)
Educationa Time off schoola Sportb Anxiety Family interactions Social isolation Social life/interactions Feelings of difference Stigmatisation Social integration Self image Denial Confusion Feelings of guilt Finances Family size Prenatal diagnosis if it had been available
15 (47) 15 (47) 22 (69) 27 (84) 2 (6) 6 (19) 8 (25) 16 (50) 6 (19) 15 (47) 21 (67) 8 (25) NA NA See social integration NA NA
28 (60) 30 (64) 26 (29) 31 (31) 3 (3) 12 (12) 20 (20) 24 (24) 2 (2) NA NA NA NA NA NA NA NA
NA NA NA 92 (82) 7 (6) 29 (26) 29 (26) NA NA NA NA 8 (9) 16 (14) 28 (25) 53 (47) 54 (48) 104 (93)
a b
Education and time off school explored for 47 paediatric patients as others at preschool stage. Sports activity explored for 91 paediatric patients as others are too young for assessment. NA=not applicable/not assessed.
homozygous thalassaemia patients in United Kingdom. Lower rates of uptake for prenatal diagnosis have been reported for Lebanese and Bangladeshi families (Der Kaloustian, Khudr, Firzli, & Dabbous, 1987; Ratip & Modell, 1996). Education of the paediatric patients in this study is more severely affected in comparison with reports from developed countries (Table 2). The reason is likely to be the lack of clinic and transfusion services outside of school hours. These have now been organised in response to these findings. Paediatric patients in this study also suffered from anxiety, few sports activities, and feeling different. In a study of paediatric thalassaemics from Izmir, on the west coast of Turkey, Aydin, Yaprak, Akarsu, Okten, and Ulgen (1997) reported a low self-image and anxiety, providing evidence that psychosocial dysfunction of the paediatric thalassaemics is also present in other geographic regions of Turkey. Most adult thalassaemics in this study had an extremely high psychosocial burden in comparison with reports from developed countries (Table 2). Their problems may partly reflect their earlier experiences with the disease at a time when there was little access to medical treatment for thalassaemia in this region of Turkey. Because, the psychosocial burden upon thalassaemia patients is known to be influenced by the severity of the disease (Ratip et al., 1995), provision for modern medical treatment should still be the priority in developing countries.
A large majority (82%) of the parents worried about thalassaemia. High levels of anxiety have also been a consistent feature of the studies of psychosocial dysfunction in parents of thalassaemia patients in developed countries (Table 2), and occur even in the milder syndrome thalassaemia intermedia (Ratip et al., 1995). The Antalya and Lebanese studies also provide evidence that the level of confusion about the illness among parents is an important issue in developing countries. The level of financial hardship for families in the Antalya study may appear surprisingly high, considering that iron chelation and blood transfusion expenses in Turkey are covered by the state. Currently, the compulsory minimum wage in Turkey is 122 million Turkish Liras (TL) (equivalent to 81 US dollars) and the official limit of poverty stands at 250 million TL (166 US dollars). Therefore, many people with incomes below the poverty line may find that minor additional costs such as travel expenses strain their finances. Implications 1. A national policy for thalassaemia screening is urgently required in Turkey as a substantial majority (93%) of the parent couples would have chosen to terminate an affected pregnancy if they had known the foetus had thalassaemia major. This could entail
818
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Table 2 Psychosocial studies on the thalassaemias Study
Country
N
Results
Canatan et al. (the present study)
Turkey
131
Di Palma et al. (1998)
Italy
109
Klein et al. (1998)
Canada
24
Aydin et al. (1997) Ratip and Modell (1996)
Turkey United Kingdom
25 27 TM
Adults: anxiety 84%, feelings of difference 50%, education affected 47%, social integration affected 47%, social activities reduced 25%, denial 25% Children: education affected 60%, family adjustment affected 3%, social interactions affected 20%, feelings of difference 24%, anxiety 31% Parents: employment affected 47%, anxiety 82%, confusion 14%, severe family adjustment problems 6%, social isolation 26%, marital breakdown in 1.8%, prenatal uptake would have been chosen if it had been available by 93% A high psychosocial burden for the patients weakly correlates with a high psychosocial burden for the parents Adults: social adjustment, self-esteem, selfdescription and family relationships not affected A high psychosocial burden for the patients correlates with a high psychosocial burden for the parents Children: low self-image, increased anxiety Adults (TM): anxiety 77%, feelings of difference 24%, education affected 29%, social integration affected 67%, social activities reduced 24%, denial 19%. Parents (TM): employment affected 31%, anxiety 88%, confusion 19%, family adjustment problems 19%, social isolation 19%, no marital breakdown. Prenatal uptake would have been chosen if it had been available by 100% for TM (mainly Mediterrnean), 81% for TI (mainly Mediterranean) and 56% for EB (Bangladeshi) parents. A high psychosocial burden for the patients correlates with a high psychosocial burden for the parents Parents: marital breakdown 7% for Greek and 5% for Italian parents. No marital breakdown for Cypriots and parents in United Kingdom Children: education unaffected, family and social adjustment problems 23% Adults: 20% of the Greek patients received no more than elementary education, feelings of difference 25% Children: anxiety common in patients with facial abnormalities Parents: anxiety common (descriptive) Parents: confusion 48%, prenatal uptake would have been chosen if it had been available by 75% Children: education not affected, feelings of difference Parents: guilt and denial (descriptive) Children: low self-esteem 23%, poor psychiatric adaptation with dysmorphic moods Adults: anxiety 66%, feelings of difference 54%, social activities reduced 82%
27TI
14 EB
Tsiantis et al. (1996)
Greece, Italy, United Kingdom & Cyprus
188
Beratis (1993)
Greece
57
Politis et al. (1990)
Greece & Italy
283
Tsiantis (1990)
Greece
40
Der Kaloustian et al. (1987)
Lebanon
54
Massaglia and Carpignano (1985) Sherman et al. (1985)
Italy
280
United States
23
Woo et al. (1985)
United States
22
N=patient number, TM=thalassaemia major, TI=thalassaemia intermedia, EB=HbE/b thalassaemia.
D. Canatan et al. / Social Science & Medicine 56 (2003) 815–819
national premarital screening, as implemented in other countries with a high prevalence of thalassaemia, such as Cyprus and Sardinia. 2. The presence of major psychosocial burden for both the patients and their parents illustrates the need for psychosocial as well as medical aid for these families. Therefore, the multidisciplinary care team should include mental health professionals. 3. Informing parents about the illness should be a priority.
References Achenbach, T. M. (1992). Manual for the child behaviour checklist: 2/3 1992 profile. Burlington, VT: University of Vermont Department of Psychiatry. Akar, N., Agaoglu, L., Altay, C - ., Aydinok, Y., Basak, N., & Canatan, D., et al. (1998). Turkish national hemoglobinopathy register-1998. 26th Turkish National Haematology Congress. Ankara (pp. 171–173). Arcasoy, A. (1992). Hemoglobinopathies in Turkey. Hematology Reviews and Communications, 6, 667. Aydin, B., Yaprak, I., Akarsu, D., Okten, N., & Ulgen, M. (1997). Psychosocial aspects and psychiatric disorders in children with thalassaemia major. Acta Paediatrica Japonica, 39, 354–357. Beratis, S. (1993). Psychosocial status in pre-adolescent children with—thalassaemia. Journal of Psychosomatic Research, 37, 271–279. Deasy-Spinetta, P., Spinetta, J. J., & Oxman, J. B. (1988). The relationship between learning deficits a social adaptation in children with leukaemia. Journal of Psychology and Oncology, 6(3/4), 109–121. Der Kaloustian, V. M., Khudr, A., Firzli, S., & Dabbous, I. (1987). Psychosocial and economic profile of a sample of families with thalassaemic children in Lebanon. Journal of Medical Genetics, 24, 772–777. Di Palma, A., Vullo, C., Zani, B., & Facchini, A. (1998). Psychosocial integration of adolescents and young adults with thalassaemia major. Annals of New York Academy of Sciences, 850, 355–360. Goldberg, D. P., & Hillier, V. F. (1979). A scaled version of the general health questionnaire. Psychological Medicine, 9, 139–145.
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Klein, S., Sen, A., Rusby, J., Ratip, S., Modell, B., & Olivieri, N. F. (1998). The psychosocial burden of Cooley’s anemia in affected children and their parents. Annals of New York Academy of Sciences, 850, 512–513. Massaglia, P., & Carpignano, M. (1985). Psychology of the thalassaemia patient and his family. Thalassaemia today: The Mediterranean experience. Proceedings of the second Mediterranean Meeting, Milano (pp. 28–30). Modell, B., & Berdoukas, V. (1984). The clinical approach to thalassaemia. New York and London: Grune and Stratton. Politis, C., Di Palma, A., Fisfis, M., Giasanti, A., Richardson, S. C., Vullo, C., & Masera, G. (1990). Social integration of the older thalassaemic patient. Archives of Disease in Childhood, 65, 984–986. Ratip, S. (1996). Clinical, genetic and psychosocial aspects of the b-thalassaemias. MD Thesis, University of London. Ratip, S., & Modell, B. (1996). Psychological and sociological aspects of the thalassaemias. Seminars in Haematology, 33, 53–65. Ratip, S., Skuse, D., Porter, J., Wonke, B., Yardumian, A., & Modell, B. (1995). Psychosocial and clinical burden of thalassaemia intermedia and its implications for prenatal diagnosis. Archives of Disease in Childhood, 72, 408–412. Rutter, M. (1967). A children’s behaviour questionnaire for completion by teachers: Preliminary findings. Journal of Child Psychology and Psychiatry, 8, 1–11. Rutter, M., Tizard, J., & Whitmore, K. (1970). Appendix 6: A children’s behaviour questionnaire for completion by parents. Education, health and behaviour (pp. 412–421). London: Longmans. Sherman, M., Koch, D., Giardina, P., Hymowitz, P., Siegel, P., & Shapiro, T. (1985). Thalassaemic children’s understanding of illness: A study of cognitive and emotional factors. Annals of New York Academy of Sciences, 445, 327–336. Tsiantis, J. (1990). Family reactions and relationships in thalassaemia. Annals of New York Academy of Sciences, 612, 451–461. Tsiantis, J., Dragonas, T., & Richardson, C., et al. (1996). Psychosocial problems and adjustment of children with thalassaemia and their families. European Child and Adolescent Psychiatry, 5, 193–203. Weatherall, D. J. (1998). Thalassaemia in the next millenium. Annals of New York Academy of Sciences, 850, 1–9. Woo, R., Giardina, P., & Hilgartner, M. (1985). A psychosocial needs assessment of patients with homozygous b-thalassaemia. Annals of New York Academy of Sciences, 445, 316–323.
Psychosocial burden of b-thalassaemia major in Antalya, south Turkey Duran Canatana, Siret Ratipb,*, Saniye Kaptanc, Ruya . Cosand b
a Department of Paediatric Haematology, Antalya State Hospital, P.O. Box 624, 07001 Antalya, Turkey Department of Haematology, Marmara University Hospital, Tophanelioglu Caddesi, Altunizade, 81190 Istanbul, Turkey c Thalassaemia Centre, Antalya State Hospital, P.O. Box 624, 07001 Antalya, Turkey d Antalya State Hospital, P.O. Box 624, 07001 Antalya, Turkey
Abstract b-thalassaemia is a recessively inherited blood disorder characterised by chronic anaemia. It requires monthly blood transfusions and regular iron chelation. Thousands of affected children are born annually and the magnitude of the problem is most severe in developing countries. Ninety-nine children and 32 adults with thalassaemia major, and 112 parents of patients were interviewed in Antalya, south Turkey, using specifically designed questionnaires to evaluate psychosocial burden. The education of most of the thalassaemic children of school age (60%) was affected, mainly due to having to attend hospital for investigation and transfusions. A high level of parental anxiety (82%) was reported. Nearly half of the families (47%) had employment and financial problems as a result of thalassaemia, yet there was a low level of marital breakdown (1.8%). A substantial majority (93%) of the parental couples would have chosen to terminate an affected pregnancy if they had known that the foetus had thalassaemia major. The results reflect the need for a national policy for public education and screening of thalassaemia in Turkey in order to offer prenatal diagnosis for all families at risk of homozygous thalassaemia. r 2002 Elsevier Science Ltd. All rights reserved. Keywords: Turkey; b-thalassaemia major; Psychosocial burden; Developing country; Prenatal diagnosis
Background b-thalassaemia is a recessively inherited blood disorder characterised by chronic anaemia, resulting from deficient synthesis of b-chains of haemoglobin. Thalassaemia major requires monthly transfusions and regular infusions for life of the iron chelating agent desferrioxamine (Modell & Berdoukas, 1984). The condition requires regular medical supervision as distressing clinical problems can occur, including growth retardation, sexual dysfunction, heart failure, diabetes mellitus secondary to iron overload, liver disease due to hepatitis B or C secondary to blood transfusions as well as HIV infection, and facial and bony deformities due to the anaemia itself. *Corresponding author. Tel.: +90-216-326-5415; fax: +90216-326-6240. E-mail address: [email protected] (S. Ratip).
Thalassaemias are common in Mediterranean countries, the Middle East, Africa and South East Asia. The migration of populations has extended the thalassaemias to other European countries, North and South American countries and Australia. Thousands of affected children are born annually and many live long enough to require treatment (Weatherall, 1998). The magnitude of the problem is most severe in developing countries and it is essential to make the thalassaemia problem more visible to governments and international health agencies involved in health care in these countries. The average prevalence of the b-thalassaemia carrier state in Turkey is 2.1%, but there is regional variability (Arcasoy, 1992). South Turkey has the highest level with 11.7% in Antalya district (Akar et al., 1998). In Turkey, as in other developing countries, medical management for thalassaemia patients has understandably taken precedence over psychosocial problems, which received little attention in the past, leading to a lack of knowledge
0277-9536/03/$ - see front matter r 2002 Elsevier Science Ltd. All rights reserved. PII: S 0 2 7 7 - 9 5 3 6 ( 0 2 ) 0 0 0 8 0 - 1
816
D. Canatan et al. / Social Science & Medicine 56 (2003) 815–819
about this issue. However, psychosocial problems are of primary importance to patients; despite improvements in survival, the burdensome nature of the treatment often results in patients giving up the iron chelation treatment partially or completely. Psychosocial problems for parents are also important, not only because they cause distress, but because adequate support, especially for younger thalassaemics, is best achieved by supporting parents and increasing their confidence in coping with the illness (Ratip & Modell, 1996). The aim of this study was to analyse the psychosocial burden of thalassaemia major from a developing country’s perspective in a major thalassaemia centre in Antalya, south Turkey.
Methods The study was undertaken at the thalassaemia centre of Antalya State Hospital, which cares for 183 thalassaemic children of 163 families. One hundred and fifty-one of these were paediatric and 32 were adult patients. Five parents of 6 affected children refused to participate in the study. Forty-six families with 46 paediatric children, whose attendance at the unit was less than 1 yr, were not approached. Thirteen of these 46 families had a recent diagnosis and so would not yet have experienced the chronic nature of the illness. Children (all aged o5 yr) of the other 33 families were recent referrals from health centres in villages within the Antalya district. They were not approached as they had attended the unit on only a few occasions and were not yet familiar enough with the medical staff to be interviewed. Therefore, the final sample size was 99 children and 32 adult patients (total number 131) and 112 parents of patients. Sixty-five patients were male and 66 female. They were provided with written information about the interview and their consent was obtained. The paediatric patients were aged 1–15 yr (mean 7.0; SD 4.6 yr), and adult patients were aged 16–27 yr (mean 19.3; SD 3.3 yr): eight families had 2 affected children, one family had 3 affected children and another family had 4 affected children. Out of the 32 adult patients, 2 were married and one had a child. Patients and parents were interviewed using a structured questionnaire either in the clinic or at home, depending on their choice. Preexisting questionnaire measures of psychosocial adjustment are either inappropriate for this chronic inherited disease, or too cumbersome for use in a clinical setting (Rutter, 1967; Rutter, Tizard, & Whitmore, 1970; Goldberg & Hillier, 1979; Deasy-Spinetta, Spinetta, & Oxman, 1988; Achenbach, 1992). Consequently, a questionnaire designed to measure the psychosocial burden of the thalassaemias (Ratip, 1996), and used in previous studies (Ratip et al., 1995; Ratip & Modell, 1996; Klein et al., 1998) was
translated into Turkish and used here. (Copies may be obtained from the author.) Briefly, the questions were designed to be simple, non-judgmental and acceptable to all cultures. The main topics included are shown with the results. Three versions are available: (A) a parentreport form for patients under 16 yr of age; (B) a selfreport form for adult patients; and (C) a form for parents to report their own situation. Eleven variables were created, each based on responses to one or more questions bearing on that topic. For example, items concerning anxiety were: Do you worry about thalassaemia (not at all, a little, sometimes, a lot, all the time)? Why? What is your biggest worry? Answers for this and other variables were then coded by the interviewer as none or unaffected (0) to severe (3), given a specific operational definition of each code. The adult patients were interviewed on their own. The parent-report form for paediatric patients was used to interview parents in the presence of their child. (In this culture, parents expect to be answerable for children under 16 yr.) Parents were interviewed together using the parent form, and the consensual answers recorded. The interviewers (SK/RC) wrote the answers verbatim during the interview, which lasted approximately 1 h/ person (range 45 min–1.5 h). At the end of the interview each response was graded for degree of impairment (0=unaffected, 1=mild, 2=moderate, 3=severe). We ensured reliability of grading by having two of us (DC/ SR) to rate a proportion of interviews.
Results Results of psychosocial burden for adult and paediatric patients and their parents are presented in Table 1. Statistical analysis revealed a weak positive correlation of 0.23 (95% confidence interval 0.03–0.41) between paediatric patients’ and their parents’ psychosocial burden scores.
Discussion Currently, there are 1983 registered patients with homozygous thalassaemia in Turkey, nearly one-third (36%) of whom are resident in the southern provinces. Patients with homozygous thalassaemia in Antalya district constitute 45% of the patients in south Turkey (Akar et al., 1998), and Antalya State Hospital cares for nearly one-quarter (26%) of these patients. The sample is therefore representative. The study is the largest in the literature analysing psychosocial problems of patients with thalassaemia major and their parents in a developing country. The attitude to prenatal diagnosis in this study is similar to parents, mainly of Mediterranean descent, of adult
D. Canatan et al. / Social Science & Medicine 56 (2003) 815–819
817
Table 1 Frequencies (%) of thalassaemia patients and their parents with moderate or severe psychosocial burden Aspect of life
Adult patients (n ¼ 32) Freq. (%)
Paediatric patients (n ¼ 99) Freq. (%)
Parents (n ¼ 112) Freq. (%)
Educationa Time off schoola Sportb Anxiety Family interactions Social isolation Social life/interactions Feelings of difference Stigmatisation Social integration Self image Denial Confusion Feelings of guilt Finances Family size Prenatal diagnosis if it had been available
15 (47) 15 (47) 22 (69) 27 (84) 2 (6) 6 (19) 8 (25) 16 (50) 6 (19) 15 (47) 21 (67) 8 (25) NA NA See social integration NA NA
28 (60) 30 (64) 26 (29) 31 (31) 3 (3) 12 (12) 20 (20) 24 (24) 2 (2) NA NA NA NA NA NA NA NA
NA NA NA 92 (82) 7 (6) 29 (26) 29 (26) NA NA NA NA 8 (9) 16 (14) 28 (25) 53 (47) 54 (48) 104 (93)
a b
Education and time off school explored for 47 paediatric patients as others at preschool stage. Sports activity explored for 91 paediatric patients as others are too young for assessment. NA=not applicable/not assessed.
homozygous thalassaemia patients in United Kingdom. Lower rates of uptake for prenatal diagnosis have been reported for Lebanese and Bangladeshi families (Der Kaloustian, Khudr, Firzli, & Dabbous, 1987; Ratip & Modell, 1996). Education of the paediatric patients in this study is more severely affected in comparison with reports from developed countries (Table 2). The reason is likely to be the lack of clinic and transfusion services outside of school hours. These have now been organised in response to these findings. Paediatric patients in this study also suffered from anxiety, few sports activities, and feeling different. In a study of paediatric thalassaemics from Izmir, on the west coast of Turkey, Aydin, Yaprak, Akarsu, Okten, and Ulgen (1997) reported a low self-image and anxiety, providing evidence that psychosocial dysfunction of the paediatric thalassaemics is also present in other geographic regions of Turkey. Most adult thalassaemics in this study had an extremely high psychosocial burden in comparison with reports from developed countries (Table 2). Their problems may partly reflect their earlier experiences with the disease at a time when there was little access to medical treatment for thalassaemia in this region of Turkey. Because, the psychosocial burden upon thalassaemia patients is known to be influenced by the severity of the disease (Ratip et al., 1995), provision for modern medical treatment should still be the priority in developing countries.
A large majority (82%) of the parents worried about thalassaemia. High levels of anxiety have also been a consistent feature of the studies of psychosocial dysfunction in parents of thalassaemia patients in developed countries (Table 2), and occur even in the milder syndrome thalassaemia intermedia (Ratip et al., 1995). The Antalya and Lebanese studies also provide evidence that the level of confusion about the illness among parents is an important issue in developing countries. The level of financial hardship for families in the Antalya study may appear surprisingly high, considering that iron chelation and blood transfusion expenses in Turkey are covered by the state. Currently, the compulsory minimum wage in Turkey is 122 million Turkish Liras (TL) (equivalent to 81 US dollars) and the official limit of poverty stands at 250 million TL (166 US dollars). Therefore, many people with incomes below the poverty line may find that minor additional costs such as travel expenses strain their finances. Implications 1. A national policy for thalassaemia screening is urgently required in Turkey as a substantial majority (93%) of the parent couples would have chosen to terminate an affected pregnancy if they had known the foetus had thalassaemia major. This could entail
818
D. Canatan et al. / Social Science & Medicine 56 (2003) 815–819
Table 2 Psychosocial studies on the thalassaemias Study
Country
N
Results
Canatan et al. (the present study)
Turkey
131
Di Palma et al. (1998)
Italy
109
Klein et al. (1998)
Canada
24
Aydin et al. (1997) Ratip and Modell (1996)
Turkey United Kingdom
25 27 TM
Adults: anxiety 84%, feelings of difference 50%, education affected 47%, social integration affected 47%, social activities reduced 25%, denial 25% Children: education affected 60%, family adjustment affected 3%, social interactions affected 20%, feelings of difference 24%, anxiety 31% Parents: employment affected 47%, anxiety 82%, confusion 14%, severe family adjustment problems 6%, social isolation 26%, marital breakdown in 1.8%, prenatal uptake would have been chosen if it had been available by 93% A high psychosocial burden for the patients weakly correlates with a high psychosocial burden for the parents Adults: social adjustment, self-esteem, selfdescription and family relationships not affected A high psychosocial burden for the patients correlates with a high psychosocial burden for the parents Children: low self-image, increased anxiety Adults (TM): anxiety 77%, feelings of difference 24%, education affected 29%, social integration affected 67%, social activities reduced 24%, denial 19%. Parents (TM): employment affected 31%, anxiety 88%, confusion 19%, family adjustment problems 19%, social isolation 19%, no marital breakdown. Prenatal uptake would have been chosen if it had been available by 100% for TM (mainly Mediterrnean), 81% for TI (mainly Mediterranean) and 56% for EB (Bangladeshi) parents. A high psychosocial burden for the patients correlates with a high psychosocial burden for the parents Parents: marital breakdown 7% for Greek and 5% for Italian parents. No marital breakdown for Cypriots and parents in United Kingdom Children: education unaffected, family and social adjustment problems 23% Adults: 20% of the Greek patients received no more than elementary education, feelings of difference 25% Children: anxiety common in patients with facial abnormalities Parents: anxiety common (descriptive) Parents: confusion 48%, prenatal uptake would have been chosen if it had been available by 75% Children: education not affected, feelings of difference Parents: guilt and denial (descriptive) Children: low self-esteem 23%, poor psychiatric adaptation with dysmorphic moods Adults: anxiety 66%, feelings of difference 54%, social activities reduced 82%
27TI
14 EB
Tsiantis et al. (1996)
Greece, Italy, United Kingdom & Cyprus
188
Beratis (1993)
Greece
57
Politis et al. (1990)
Greece & Italy
283
Tsiantis (1990)
Greece
40
Der Kaloustian et al. (1987)
Lebanon
54
Massaglia and Carpignano (1985) Sherman et al. (1985)
Italy
280
United States
23
Woo et al. (1985)
United States
22
N=patient number, TM=thalassaemia major, TI=thalassaemia intermedia, EB=HbE/b thalassaemia.
D. Canatan et al. / Social Science & Medicine 56 (2003) 815–819
national premarital screening, as implemented in other countries with a high prevalence of thalassaemia, such as Cyprus and Sardinia. 2. The presence of major psychosocial burden for both the patients and their parents illustrates the need for psychosocial as well as medical aid for these families. Therefore, the multidisciplinary care team should include mental health professionals. 3. Informing parents about the illness should be a priority.
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