Psychosocial burden in thalassemia
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《美国医学杂志》
1 Department of Child Development, Government Home Science College, Chandigarh, India
2 Department of Pediatrics, Post Graduate Institute of Medical Education and Research (PGIMER), Chandigarh, India
Abstract
Objective. To study the psychosocial life aspects of Indian adolescents suffering from transfusion dependent β-thalassemia major. Earlier studies done in Italy, Greece and United Kingdom reported the presence of psychosocial burden associated with the disease. We aimed to determine the presence of disease burden in the psychosocial life aspects of Indian adolescents affected with thalassemia. Methods. Structured interviews were carried out with each of the subjects using a schedule which contained questions relating to perceived burden of thalassemia in the various psychosocial life aspects of affected adolescents. Results. Adverse impact of thalassemia was perceived in the domains of education (70%) and sports (72%). Most thalassemics were not satisfied with their body image. Almost all the study subjects felt that the disease did not affect their family or social relationships. The adolescents were anxious about their future health and education. Majority of the subjects(80%) did not discuss about their disease and its related problems with their friends. They mainly depended on their parents for monetary and emotional support. Conclusion. There is tremendous psychosocial disease burden perceived by the affected adolescents, and hence it is imperative to ameliorate it by promoting a clear understanding of the disease and initiating intervention programs.
Keywords: Thalassemic adolescents; Disease burden; Psychosocial life aspects
Thalassemia, at present, is one of the most challenging hematological disorder with no permanent cure. Patients with β- thalassemia major need regular blood transfusions in order to live. The resulting iron overload also requires chelation therapy. Although optimal medical management has reduced the difficulties faced by thalassemics, the psychosocial problems faced by them are now of primary importance. In developing countries like India, the main cause of death from thalassemia is non-compliance with the treatment due to psychosocial factors.[1]
The psychosocial burden is perceived even more in the adolescence stage when the teenager is confronted with various difficulties like identity formation, developing intimate relationships and entering the working world. Accepting and integrating thalassemia into one's identity becomes a difficult task at this stage.
Thalassemia challenges the individual at the physical, emotional, cognitive levels and disrupts the quality of life.[2] Its frequent and complex treatment might also lead to financial burden for the individual and his/her family. In this communication we bring into focus the observations made in our study designed to evaluate the psychosocial burden in adolescents with transfusion dependent thalassemia.
Material and methods
The present study was conducted in the Thalassemia Transfusion Unit of the Division of Pediatric Hematology - Advanced Pediatric Center, Nehru Hospital, Postgraduate Institute of Medical Education and Research (PGIMER), Chandigarh.
Adolescents who fulfilled the inclusion criteria were enrolled in the study. The criteria were :
(a) established diagnosis of thalassemia; (b) receiving regular red cells transfusion in the Advanced Pediatric Center. (c) age range between 12-20 years.
70 adolescents were found to fulfill the above criteria. From this 50 adolescents (25 males and 25 females) were randomly selected by draw of lots.
The lack of a standardized test tool for assessing the psychosocial burden in Indian adolescents with thalassemia prompted us to develop a questionnaire which would be suitable for use in Indian subjects. The thalassemia profile questionnaire developed by Ratip and Modell[1] was modified and used as an interview schedule. Questions on marriage and employment were omitted and the remaining were reframed to fit the purpose of the study. The questions were discussed, edited and re-edited with the help of the guides. For studying all the aspects objectively and getting in-depth information, both close-ended and open-ended questions were included. Open-ended questions were framed so that the adolescents were free to express themselves. The questions were kept simple, unambiguous, free of cultural bias and suited to the Indian context. The final interview schedule consisted of 55 questions which assessed the psychosocial life aspects of thalassemic adolescents under the 9 sub-heads of education, sports activities, self-image, family interactions, social interactions, anxiety, feelings of difference, ways of coping and support services. (Annexure I )
A pilot study was carried out in a few cases to test the practicality and feasibility of the interview schedule. On its basis, appropriate changes were made in the questions to get clarity of responses. After refining the test tool, structured interviews were carried out with each of the study subjects. The interview lasted for about 1-2 hours. After the data was collected, it was coded and tabulated. Frequencies and percentages were calculated for each item on the interview schedule and the response patterns for male and female adolescents were recorded.
Results
In the domain of education, 35(70%) adolescents expressed that thalassemia had an adverse impact on their scholastic performance. They had to take 1-3 days off from school every month to get their regular red cell transfusions. The affected adolescents complained that their academic performance was average or less because of their disease and its related problems. 62% of the adolescents reported that they had faced no deprecatory remarks from their peers or teachers.
Over 2/3rd of the adolescents were unable to engage in outdoor play at the same level as their peers, because of physical weakness related to their disease. Indulgence in sport activities was therefore limited.
68% of the adolescents were not satisfied with their body image. Almost all of them cited physical appearances as their major reason of disappointment. Stunted growth, skeletal abnormalities and delayed puberty generated a negative self-concept.
In response to the questions pertaining to family interactions, adolescents with thalassemia had no problems in family relationships, either with parents or siblings. They generally discussed their problems and worries with their parents.
Most of the adolescents (80%) reported that thalassemia had not affected their social life. However, on further questioning it was revealed that they preferred the company of a selected group of close friends rather than socializing with a larger group of people. Their frequency of going out was less than once a month.
The adolescents worried about their disease and its complications. On being questioned about their worries, they responded that they were mainly concerned about their future health (56%) and education (34%). Patients belonging to the lower socio-economic strata had additional worries related to the financial ramifications of their therapy.
Their source of strength to cope with the disease was mainly parents and God. Most of the adolescents (82%) did not like discussing their illness and its related problems with their friends as they could not trust them and feared being ridiculed.
In response to being asked about the need for additional financial and medical support, adolescents from the low income group (20%) felt that they were denied the benefits of optimal medical management because of lack of financial resources. The rest of the study cohort felt that they had sufficient funds to ensure appropriate therapy.
Discussion
The presence of a chronic disease like thalassemia places tremendous psychosocial burden on the patient and the family. Improvement in prognosis and modern treatments have greatly increased the life expectancy of thalassemics. The need to ameliorate the associated psychosocial burden thus becomes even more important. The scarcity of data on the psychosocial life aspects of thalassemics limits us from implementing proper intervention strategies. Keeping this in mind, the present study was designed to define the psychosocial burdens faced by thalassemic adolescents in their everyday lives. A structured interview was conducted in a sample of 50 adolescents and the impact of the disease was observed in main domains like education, sports, family life and social life.
In the sphere of education, the findings revealed that thalassemic adolescents perceived an adverse impact of the disease on their scholastic performance. In a study by Ratip et al[4] in United Kingdom with 27 thalassemic subjects, it was observed that 90% of these subjects had to take time off from school because of their medical condition. These findings are in consonance with the observations made by us. In another study of 138 thalassemics, Logitihis et al[3] found that the intelligence quotient of all the thalassemic children was within normal range.
There is scant available information on how thalassemia affects sports activities of adolescents. The study by Ratip et al[4] showed that 86% of the 27 thalassemic patients complained of tiredness and sub-optimal performance in sports. The present study findings are congruent with these.
Chronic illnesses like thalassemia bring along feelings of being different and inferior with consequent loss of self-esteem and increased dependence. Facial characteristics in thalassemia occur as a consequence of the expansion of bones particularly the skull and jaw bones. Anemia and iron overload in these patients often leads to short stature and delayed puberty. Georganda[5] conducted open-ended interviews with five thalassemic adolescents and reported the impact of the disease on their self-image. Most of them feared being rejected and ridiculed by others because of their looks. In our study, we found that 45 adolescents(91%) had similar apprehensions about their physical appearances.
Contrary to the findings by Ratip and Modell[1], who reported lack of communication and withdrawal of thalassemics from their families, the adolescents in our study cohort felt that the disease had little or no impact on their family relationships. In fact, they liked sharing their problems and worries with their parents. The possible reasons for this difference could be that Indian families tend to be more close knit and the adolescents are dependent on their parents till later ages for monetary and emotional support. In a study of Italian adolescents, the investigators[8] reported that thalassemics remain dependent on their parents with few friends from the peer age group.
Anxiety is common among thalassemic adolescents and those with physical abnormalities are especially prone to be anxious. Sherman[6] in a study sample of 23 thalassemic children, found poor psychiatric adaptation with dysphoric moods and low self-esteem in most of them. In another study of 22 thalassemics, Woo et al.[7] reported that 2/3rd of the patients were worried about pain, death and the unknown. These findings are similar to our observations.
It is evident from the findings of our study that besides the clinical burden, thalassemics have to shoulder high psychosocial burden associated with the disease. The disease affects their education and sports life. They are likely to suffer from reduced self-esteem, feelings of difference, poor self-image, being dependent and anxiety over issues such as pain and death.
Conclusion
As thalassemia has an impact on the different psychosocial life aspects of adolescents, it is very essential to provide psychosocial support, including promotion of a clear understanding of the disease. Thalassemia support groups are very important as they not only educate the patients and their families about the disease but also provide patients with an opportunity to meet their peers and participate in social activities.
Psychosocial support may include liaison with teachers or school authorities who should be informed about the disease. The adolescents must be allowed to get involved in normal activities with healthy peers to allow the development of adequate self-image.
The need for psychosocial support is even higher in a developing country like India where the regular and expensive treatment can pose a financial burden as well for the families of the thalassemics. It is imperative to focus our attention on psychosocial life aspects of thala-ssemics and initiate intervention programs with a view to integrate thalassemics into the social mainstream and help them in leading healthy, creative and fulfilling lives.
References
1. Ratip S, Modell B. Psychological and sociological aspects of the thalassaemias. Seminars in Hematology 1996; 33: 53-65.
2. Tsiantis J, Xypolita-Tsantili D, Papadakou-Lagoyianni S. Family reactions and their management in a parents group with b-thalassaemia. Archiv Dis Child 1982; 57: 860-863.
3. Logithis J, Haritos-Fatouros M, Coonstantoulakis M. Intelligence and behavioral paterns in patients with Cooley's anaemia; a study based on 138 consecutive cases. Pediatr 1971; 48: 740-743.
4. Ratip S, Skuse D, Porter J. Psychosocial and clinical burden of thealassaemia intermedia and its implications for prenatal diagnosis. Arch Dis Child 1995; 72: 408-412.
5. Georganda ET. The impact of thalassaemia on body image, self-image and self-esteem. Annals New York Academy of Sciences 1998; 850: 466-472.
6. Sherman M, Koch D, Giardiana P. Thalassaemic children's understanding of illness: A study of cognitive and emotional factors. Annals New York Academy of Sciences 1985; 445: 327-336.
7. Woo R, Giardina P, Hilgartner M. A psychosocial needs assessment of patients with homozygous β -thalassemia. Ann NY Acad Sci 1985; 445: 316-323.
8. Massaglia P, Carpignano M. Psychology of the thalassaemia patient and his family. Thalassaemia today; The Mediterranean experience. Proceedings of the 2nd Mediterranean Meeting, Milano. 1985: 28-30.(Khurana Atika, Katyal Sud)
2 Department of Pediatrics, Post Graduate Institute of Medical Education and Research (PGIMER), Chandigarh, India
Abstract
Objective. To study the psychosocial life aspects of Indian adolescents suffering from transfusion dependent β-thalassemia major. Earlier studies done in Italy, Greece and United Kingdom reported the presence of psychosocial burden associated with the disease. We aimed to determine the presence of disease burden in the psychosocial life aspects of Indian adolescents affected with thalassemia. Methods. Structured interviews were carried out with each of the subjects using a schedule which contained questions relating to perceived burden of thalassemia in the various psychosocial life aspects of affected adolescents. Results. Adverse impact of thalassemia was perceived in the domains of education (70%) and sports (72%). Most thalassemics were not satisfied with their body image. Almost all the study subjects felt that the disease did not affect their family or social relationships. The adolescents were anxious about their future health and education. Majority of the subjects(80%) did not discuss about their disease and its related problems with their friends. They mainly depended on their parents for monetary and emotional support. Conclusion. There is tremendous psychosocial disease burden perceived by the affected adolescents, and hence it is imperative to ameliorate it by promoting a clear understanding of the disease and initiating intervention programs.
Keywords: Thalassemic adolescents; Disease burden; Psychosocial life aspects
Thalassemia, at present, is one of the most challenging hematological disorder with no permanent cure. Patients with β- thalassemia major need regular blood transfusions in order to live. The resulting iron overload also requires chelation therapy. Although optimal medical management has reduced the difficulties faced by thalassemics, the psychosocial problems faced by them are now of primary importance. In developing countries like India, the main cause of death from thalassemia is non-compliance with the treatment due to psychosocial factors.[1]
The psychosocial burden is perceived even more in the adolescence stage when the teenager is confronted with various difficulties like identity formation, developing intimate relationships and entering the working world. Accepting and integrating thalassemia into one's identity becomes a difficult task at this stage.
Thalassemia challenges the individual at the physical, emotional, cognitive levels and disrupts the quality of life.[2] Its frequent and complex treatment might also lead to financial burden for the individual and his/her family. In this communication we bring into focus the observations made in our study designed to evaluate the psychosocial burden in adolescents with transfusion dependent thalassemia.
Material and methods
The present study was conducted in the Thalassemia Transfusion Unit of the Division of Pediatric Hematology - Advanced Pediatric Center, Nehru Hospital, Postgraduate Institute of Medical Education and Research (PGIMER), Chandigarh.
Adolescents who fulfilled the inclusion criteria were enrolled in the study. The criteria were :
(a) established diagnosis of thalassemia; (b) receiving regular red cells transfusion in the Advanced Pediatric Center. (c) age range between 12-20 years.
70 adolescents were found to fulfill the above criteria. From this 50 adolescents (25 males and 25 females) were randomly selected by draw of lots.
The lack of a standardized test tool for assessing the psychosocial burden in Indian adolescents with thalassemia prompted us to develop a questionnaire which would be suitable for use in Indian subjects. The thalassemia profile questionnaire developed by Ratip and Modell[1] was modified and used as an interview schedule. Questions on marriage and employment were omitted and the remaining were reframed to fit the purpose of the study. The questions were discussed, edited and re-edited with the help of the guides. For studying all the aspects objectively and getting in-depth information, both close-ended and open-ended questions were included. Open-ended questions were framed so that the adolescents were free to express themselves. The questions were kept simple, unambiguous, free of cultural bias and suited to the Indian context. The final interview schedule consisted of 55 questions which assessed the psychosocial life aspects of thalassemic adolescents under the 9 sub-heads of education, sports activities, self-image, family interactions, social interactions, anxiety, feelings of difference, ways of coping and support services. (Annexure I )
A pilot study was carried out in a few cases to test the practicality and feasibility of the interview schedule. On its basis, appropriate changes were made in the questions to get clarity of responses. After refining the test tool, structured interviews were carried out with each of the study subjects. The interview lasted for about 1-2 hours. After the data was collected, it was coded and tabulated. Frequencies and percentages were calculated for each item on the interview schedule and the response patterns for male and female adolescents were recorded.
Results
In the domain of education, 35(70%) adolescents expressed that thalassemia had an adverse impact on their scholastic performance. They had to take 1-3 days off from school every month to get their regular red cell transfusions. The affected adolescents complained that their academic performance was average or less because of their disease and its related problems. 62% of the adolescents reported that they had faced no deprecatory remarks from their peers or teachers.
Over 2/3rd of the adolescents were unable to engage in outdoor play at the same level as their peers, because of physical weakness related to their disease. Indulgence in sport activities was therefore limited.
68% of the adolescents were not satisfied with their body image. Almost all of them cited physical appearances as their major reason of disappointment. Stunted growth, skeletal abnormalities and delayed puberty generated a negative self-concept.
In response to the questions pertaining to family interactions, adolescents with thalassemia had no problems in family relationships, either with parents or siblings. They generally discussed their problems and worries with their parents.
Most of the adolescents (80%) reported that thalassemia had not affected their social life. However, on further questioning it was revealed that they preferred the company of a selected group of close friends rather than socializing with a larger group of people. Their frequency of going out was less than once a month.
The adolescents worried about their disease and its complications. On being questioned about their worries, they responded that they were mainly concerned about their future health (56%) and education (34%). Patients belonging to the lower socio-economic strata had additional worries related to the financial ramifications of their therapy.
Their source of strength to cope with the disease was mainly parents and God. Most of the adolescents (82%) did not like discussing their illness and its related problems with their friends as they could not trust them and feared being ridiculed.
In response to being asked about the need for additional financial and medical support, adolescents from the low income group (20%) felt that they were denied the benefits of optimal medical management because of lack of financial resources. The rest of the study cohort felt that they had sufficient funds to ensure appropriate therapy.
Discussion
The presence of a chronic disease like thalassemia places tremendous psychosocial burden on the patient and the family. Improvement in prognosis and modern treatments have greatly increased the life expectancy of thalassemics. The need to ameliorate the associated psychosocial burden thus becomes even more important. The scarcity of data on the psychosocial life aspects of thalassemics limits us from implementing proper intervention strategies. Keeping this in mind, the present study was designed to define the psychosocial burdens faced by thalassemic adolescents in their everyday lives. A structured interview was conducted in a sample of 50 adolescents and the impact of the disease was observed in main domains like education, sports, family life and social life.
In the sphere of education, the findings revealed that thalassemic adolescents perceived an adverse impact of the disease on their scholastic performance. In a study by Ratip et al[4] in United Kingdom with 27 thalassemic subjects, it was observed that 90% of these subjects had to take time off from school because of their medical condition. These findings are in consonance with the observations made by us. In another study of 138 thalassemics, Logitihis et al[3] found that the intelligence quotient of all the thalassemic children was within normal range.
There is scant available information on how thalassemia affects sports activities of adolescents. The study by Ratip et al[4] showed that 86% of the 27 thalassemic patients complained of tiredness and sub-optimal performance in sports. The present study findings are congruent with these.
Chronic illnesses like thalassemia bring along feelings of being different and inferior with consequent loss of self-esteem and increased dependence. Facial characteristics in thalassemia occur as a consequence of the expansion of bones particularly the skull and jaw bones. Anemia and iron overload in these patients often leads to short stature and delayed puberty. Georganda[5] conducted open-ended interviews with five thalassemic adolescents and reported the impact of the disease on their self-image. Most of them feared being rejected and ridiculed by others because of their looks. In our study, we found that 45 adolescents(91%) had similar apprehensions about their physical appearances.
Contrary to the findings by Ratip and Modell[1], who reported lack of communication and withdrawal of thalassemics from their families, the adolescents in our study cohort felt that the disease had little or no impact on their family relationships. In fact, they liked sharing their problems and worries with their parents. The possible reasons for this difference could be that Indian families tend to be more close knit and the adolescents are dependent on their parents till later ages for monetary and emotional support. In a study of Italian adolescents, the investigators[8] reported that thalassemics remain dependent on their parents with few friends from the peer age group.
Anxiety is common among thalassemic adolescents and those with physical abnormalities are especially prone to be anxious. Sherman[6] in a study sample of 23 thalassemic children, found poor psychiatric adaptation with dysphoric moods and low self-esteem in most of them. In another study of 22 thalassemics, Woo et al.[7] reported that 2/3rd of the patients were worried about pain, death and the unknown. These findings are similar to our observations.
It is evident from the findings of our study that besides the clinical burden, thalassemics have to shoulder high psychosocial burden associated with the disease. The disease affects their education and sports life. They are likely to suffer from reduced self-esteem, feelings of difference, poor self-image, being dependent and anxiety over issues such as pain and death.
Conclusion
As thalassemia has an impact on the different psychosocial life aspects of adolescents, it is very essential to provide psychosocial support, including promotion of a clear understanding of the disease. Thalassemia support groups are very important as they not only educate the patients and their families about the disease but also provide patients with an opportunity to meet their peers and participate in social activities.
Psychosocial support may include liaison with teachers or school authorities who should be informed about the disease. The adolescents must be allowed to get involved in normal activities with healthy peers to allow the development of adequate self-image.
The need for psychosocial support is even higher in a developing country like India where the regular and expensive treatment can pose a financial burden as well for the families of the thalassemics. It is imperative to focus our attention on psychosocial life aspects of thala-ssemics and initiate intervention programs with a view to integrate thalassemics into the social mainstream and help them in leading healthy, creative and fulfilling lives.
References
1. Ratip S, Modell B. Psychological and sociological aspects of the thalassaemias. Seminars in Hematology 1996; 33: 53-65.
2. Tsiantis J, Xypolita-Tsantili D, Papadakou-Lagoyianni S. Family reactions and their management in a parents group with b-thalassaemia. Archiv Dis Child 1982; 57: 860-863.
3. Logithis J, Haritos-Fatouros M, Coonstantoulakis M. Intelligence and behavioral paterns in patients with Cooley's anaemia; a study based on 138 consecutive cases. Pediatr 1971; 48: 740-743.
4. Ratip S, Skuse D, Porter J. Psychosocial and clinical burden of thealassaemia intermedia and its implications for prenatal diagnosis. Arch Dis Child 1995; 72: 408-412.
5. Georganda ET. The impact of thalassaemia on body image, self-image and self-esteem. Annals New York Academy of Sciences 1998; 850: 466-472.
6. Sherman M, Koch D, Giardiana P. Thalassaemic children's understanding of illness: A study of cognitive and emotional factors. Annals New York Academy of Sciences 1985; 445: 327-336.
7. Woo R, Giardina P, Hilgartner M. A psychosocial needs assessment of patients with homozygous β -thalassemia. Ann NY Acad Sci 1985; 445: 316-323.
8. Massaglia P, Carpignano M. Psychology of the thalassaemia patient and his family. Thalassaemia today; The Mediterranean experience. Proceedings of the 2nd Mediterranean Meeting, Milano. 1985: 28-30.(Khurana Atika, Katyal Sud)