Psychosocial adjustment of thalassaemic children's siblings

Journal of Psychosomatic Research, Vol. 39, No. 7, pp. 911-919, 1995 Copyright © 1995 Elsevier Science Inc. Printed in Great Britain. All rights reserved. 0022-3999/95 $29.00 + 0.00

Pergamon 0022-3999(95)00061-M

PSYCHOSOCIAL ADJUSTMENT OF THALASSAEMIC CHILDREN'S SIBLINGS S P Y R I D O U L A L A B R O P O U L O U and S T A V R O U L A B E R A T I S (Received 29 July 1994; accepted 29 March 1995)

Abstract- I]-Thalassaemiais a chronic disease causing serious symptoms to the patients and considerable burden to their families. The psychopathology and psychosocial adjustment of 71 siblings of thalassaemic patients and 71 matched control subjects were investigated. Siblings of thalassaemic patients were found to have significantly more psychiatric disorders than the control subjects (p<0.05). The frequency of psychopathology among the patients' siblings, when compared to the controls, was significantly greater after the age of 10 years (p<0.01), with no difference between the two groups before the age of 10. Siblings of thalassaemic patients also scored significantly lower on sports and nonsports activities and on social functioning in terms of number of friends and number of contacts with them. These findings show that siblings of thalassaemic patients are at risk for psychiatricsymptomatologyand for impaired psychosocialfunctioning. Keywords: Psychopathology of thalassaemic siblings, Psychosocial functioning of thalassaemic siblings, 13-thalassaemia, Thalassaemic patients' siblings.

INTRODUCTION L o n g - t e r m illness in a child has a serious impact on the functioning o f the entire family, draining its resources. However, only a few systematic studies have been reported concerning the psychological and social adjustment o f the siblings o f paediatric patients with chronic disease. Some show that siblings score more highly on indices o f p s y c h o p a t h o l o g y t h a n n o r m a l controls [1, 2], whereas others have not f o u n d any abnormalities [3-5]. The reports o f a b n o r m a l p s y c h o p a t h o l o g y refer to a negative b o d y image [6], anxiety [2, 6, 7], depression [7], somatizing [7], and inadequate handling o f aggression [2, 4, 7, 8]. In addition, difficulties have been observed in the social functioning [8, 9] and in school p e r f o r m a n c e [9l. Certain sibling age groups have been considered especially psychologically vulnerable. Thus, siblings between the ages o f 3 and 6 years o f children with haematology, cardiology, and plastic surgery problems and male siblings between the ages o f 7 and 13 years o f patients with blood disorders have been reported to show more psychopathology t h a n siblings o f other age subgroups [8]. A n investigation o f siblings o f children with nephrotic s y n d r o m e f o u n d that the pre-adolescent and adolescent age groups were the most vulnerable [9].

Department of Psychiatry, University of Patras School of Medicine, General University Hospital, Patras, Greece. Address correspondence to: Dr. Stavroula Beratis, Department of Psychiatry, University of Patras School of Medicine, P.O. Box 1045, 26 1 10 Patras, Greece. 911

912

s. LABROPOULOUand S. BERATIS

There are also findings concerning the birth order and the sex of siblings of chronically ill children. Breslau [4], who examined siblings o f children with cystic fibrosis, cerebral palsy, myelodysplasia, and multiple handicaps, found that younger brothers had a greater psychological impairment than younger sisters, whereas the reverse was true for older siblings. An investigation of siblings o f male children with diabetes, with pervasive developmental disorders, or with other chronic illnesses showed that samesex siblings may be at higher risk than opposite-sex pairs [5]. Results of studies exploring the relationship between the type o f disease and the effect on the sick child's sibling are conflicting. Breslau found no association between the type o f illness and the level of disability with the degree o f psychopathology [4]. On the other hand, illness-specific reactions have been described in siblings of children with autistic disorder and with diabetes [5], whereas siblings of children with Crohn's disease have been reported as having more psychological problems than siblings of children with ulcerative colitis [7]. In summary, while several studies show that siblings o f chronically ill children may have greater psychological difficulties than control subjects, the severity of psychopathology is variable, as it appears that certain areas o f functioning may be impaired, whereas others remain intact. It is apparent that factors, such as age, sex, birth order of the child, and type o f chronic illness may interact and increase the risk for psychological disturbances. The complexity in conducting research on the adjustment o f children who have a brother or a sister with a chronic illness and the need for further investigation of this topic have been underlined by a number of authors [2, 8, 10-12]. 13-thalassaemia is a genetic disorder transmitted according to the Mendelian type o f inheritance. Patients with 13-thalassaemia major present with severe chronic anaemia, growth retardation, hepatosplenomegaly, skeletal abnormalities, and dysmorphic facial characteristics. Treatment requires frequent blood transfusions and subcutaneous infusions o f deferoxamine, administered overnight, 5 to 7 days a week. All these factors impose a considerable burden on the families and might be expected to affect the psychosocial status o f the patients' siblings. The present study investigated whether or not siblings of children with 13-thalassaemia differ from control subjects in (1) the nature o f psychopathology, (2) the social functioning, or (3) the psychosocial adjustment according to age, sex, and birth order. METHOD Subjects The study involved71 non-thalassaemic siblings (Group A) of 59 patients with 13-thalassaemia followed in the Thalassaemia Unit of the Department of Paediatrics of the University of Patras Medical School, at the University General Hospital of Patras, Greece. Children of gypsy descent were excluded from the study because the members of these families had a different cultural background from the familymembers of the other thalassaemic children. Of the siblings of the thalassaemic patients, 32 were boys and 39 were girls. The mean age +SD of all male and female children in Group A was 12.1± 3.6 years (range 5.5 to 19 years). Control subjects (Group B) were patients admitted to the paediatric wards because of an acute illness of short duration. They were children matched for age, sex, schoolgrade attendance, place of residence (rural or urban), socioeconomic status, and parental educational level. Children with a chronic or a psychosomaticdisorder or siblings of such patients were excluded from the control group. The mean age ±SD of the children in Group B was 12.4+_3.8 years (range 5.3 to 19 years). The greatest age deviation in each pair of thalassaemic patient's sibling and control subject was 8 months.

Procedures A semistructured interview of the parents of the two groups covered the family'splace of residence; the

Siblings of thalassaemic children

913

number of children in the family; and the parental marital status, educational level, and occupation, together with child's personal history, school performance according to the marks obtained, school grade, and psychopathology, which was classified according to the diagnostic criteria of the Diagnostic and Statistical Manual III-R (DSM-III-R) of the American Psychiatric Association [13]. In addition, the children were interviewed using a semistructured interview reported in an earlier paper [14]. Psychiatric disorders were diagnosed using the method reported by Rey et al. [15]. The two investigators made independent DSM-III-R diagnoses using the information obtained from the interviews. The second investigator did not examine the parents or the children, but reviewed the data from the interviews being blind as to their assignment in Group A or B. Cases in which there was disagreement as to the diagnosis were re-evaluated by the same two investigators, and a joint decision was made according to the DSM-III-R. The unweighted kappa for interrater agreement between the two diagnosticians was 0.81 (z = 7.18, p<0.001). A version [14] of the Child Behavior Checklist (CBCL) designed to be filled out by parents [16l was used for the evaluation of the children's social competence; it included an Activities scale and a Social scale. Activities were assessed by exploring the sports the child liked to participate in, the mean score for participation and skill in sports, the nonsports activities, the mean score of participation and skill in activities according to their age, the jobs or chores the child had, and the quality of these jobs. Social competence was evaluated by assessing the number of close friends the child had, the number of contacts with friends, the behavior with brothers, sisters, other children and parents, and the child's ability to play and work by himself or herself. Although the CBCL is designed to be filled out by the parents, for the purpose of this study, the parents were asked about these items by the interviewing psychiatrist, who filled out the answers. The expression "behavior with brothers, sisters, other children, and parents" refers to the ability of the child to get along with his or her brothers and sisters as well as other children and to the way he or she behaves with his or her parents, as compared to other children of the same age. The scores used for this evaluation were 0 to 2, a high score indicating better adjustment for the child regarding activities and sociability. The CBCL was modified to the extent that the item referring to "organisations to which the child belongs" was omitted, since Greek children do not usually belong to formal clubs, teams, or groups. Problems of behavior were assessed by using 120 questions from the behavior profile of the CBCL. A three-step response scale (0, 1, 2) was used, a lower score indicating better adjustment. The questions were administered orally to the parents at the end of the interview. Because Greek children differ in their cultural status from the American children on whom the test was standardized, we compared the scores of the siblings of the thalassaemic patients not to those of the normative sample of children included in the CBCL, but to the scores of the matched control group of normal children derived from the same population.

Statistical analysis The data were analyzed by applying the chi-square test, one-factor analysis of variance, and the Mann-Whitney U test, where appropriate. The interrater agreement was calculated using the kappa statistic [17]. Values are expressed as means ±SD.

RESULTS

Psychopathology Totalsample. Of the 71 siblings of patients with 13-thalassaemia studied, 29 had one psychiatric disorder and one had two disorders, whereas of the 71 control subjects investigated 17 had one psychiatric disorder and one had two. The greater number of children with psychiatric disorders in Group A reached statistical significance when compared to Group B (Table I). No particular psychiatric diagnosis was especially prevalent in Group A when compared to Group B. However, a greater occurrence of oppositional defiant disorder in the siblings of the thalassaemic patients just failed to reach the level of significance.

Subgroups. When the siblings of the thalassaemic patients and the control subjects were compared in the subgroups- 5 to 10 years and 10 to 19 years - it was found that 15°70 of the siblings of the thalassaemic patients younger than 10 years old and 2207o of the corresponding control subjects had one or more psychiatric disorders. In the age group 10 to 19 years, 61070 and 3707o had one or more psychiatric disorders, respectively (Table II). No association was observed between the presence of the characteristic dysmorphic

S. LABROPOULOU and S. BERATIS

914

Table l . - N u m b e r of psychiatric disorders in siblings of thalassaemic patients (Group A) and control subjects (Group B) Psychiatric Disorders Overanxious disorder Attention deficit-hyperactivity disorder Oppositional defiant disorder Functional enuresis Stereotypic habit disorder Avoidant disorder Separation anxiety disorder Psychologiacl factors affecting physical condition Gender identity disorder Undifferentiated attention deficit disorder Social phobia Stuttering Undifferentiated somatoform disorder Other specified family circumstances Total

Group A

Group B

7 2 7* 3 2 2 1 2 1 1 2 0 0 0 30"*

5 1 2* 3 0 2 2 1 0 0 0 1 1 1 19"*

*X 2 = 2.97, d f = 1, p = 0.08. **X 2 = 3.77, df = l , p = 0.05.

facies of the thalassaemic patients, resulting from inadequate blood transfusions, and the existence of psychiatric disorders in their siblings.

Family and school status Regarding family status, in the group of siblings of the thalassaemic patients the parents were married in 69 cases and separated in one. In addition, there was a widowed parent. All parents of the 71 control subjects were married. In Group A, the school performance was good in 34 children, moderate in 20, and bad in 5. In Group B, the performance was good in 44, moderate in 10, and bad in 5. The difference is not significant (X2=4.62, df=2, p=0.1). Seven children in Group A and 3 in Group B dropped out of school (X2=1.72, dr=l, p=0.19).

Activities profile Total sample. There was no statistically significant difference in the overall activities profile between the subjects in Group A and those in Group I3. The score was 7.40 + 1.87, and 7.82 + 1.52, respectively (F= 2.12, p = 0.15). However, a significant

Table l I . - P s y c h i a t r i c disorders in the age groups 5 to 10 and 10 to 19 years of thalassaemic patients' siblings and control subjects Siblings of thalassaemics

Control subjects

Significance

Age group (years)

PD

N

PD

N

X~

p

5-10 10-19

3 27

17 24

4 15

14 38

0.33 6.55

0.57 0.01

PD = psychiatric disorder. N = normal.

Siblings of thalassaemic children

915

difference was present between Groups A and B in both the sports and the nonsports activities as well as in participation and skill in sports and in nonsports activities (Table III). There was no significant difference between Groups A and B in the number of jobs or chores the children had and in the way they carried out these jobs. Except for the items referring to jobs or chores, the overall scores of the Activities scale, of Groups A and B were 5.09 + 1.55 and 6.00+_0.98, respectively. This difference is highly significant (F= 17.54, p=0.0001). Subgroups. Furthermore, the boys and girls of Group B obtained significantly higher scores in the number of sports and nonsports activities when compared to the boys and girls of Group A. In addition, the girls of Group B exceeded the girls of Group A in both participation and skill in sports as well as in nonsports activities (Table III). For siblings of the opposite sex from the thalassaemic patients, the score for participation in jobs or chores was 1.54+_0.75, whereas in the same-sex siblings it was 1.16+_0.68 (F= 5.19, p=0.02). Also, the siblings of the opposite sex from the thalassaemic patients showed significantly higher scores in their ability to play and work by themselves than the siblings of the same sex (1.06 +_0.43 and 0.84 _ 0.44, respectively; F = 4.50, p = 0.04).

Social profile Table IV lists the items of the Social Scale in which significant differences in scores were found between children in Group A and those in Group B. The total score on the social profile of the siblings of the thalassaemic patients was significantly lower than that of the control subjects. Further analysis of each of the items of the Social scale demonstrated that the two groups differed in the number of close friends and Table I I I . - C B C L items of the activity scale with significant differences between siblings of thalassaemic patients (Group A) and control subjects (Group B) Mean score + SD Comparison G r o u p A vs Group B Group A vs G r o u p B G r o u p A vs Group B G r o u p A vs Group B G r o u p A M vs G r o u p B M G r o u p A M vs G r o u p B M G r o u p A F vs G r o u p B F G r o u p A F vs G r o u p B F G r o u p A F vs G r o u p B F Group A F vs G r o u p B F

M = male. F = female.

Significance

Item

Group A

Group B

F

p

Number of sports Participation and skill in sports Number of nonsports activities Participation and skill in nonsports activities Number of sports Number of nonsports activities Number of sports Participation and skill in sports Number of nonsports activities Participation and skill in nonsports activities

1.48 + 0.77 1.28 + 0.57

1.83 _+ 0.47 1.63 + 0.35

10.68 4.35

0.001 0.04

1.11 +_ 0.37

1.50 +_ 0.52

10.77

0.001

1.00 +_ 0.27

1.17 ___ 0.33

10.84

0.001

1.63 + 0.66 1.69 _+ 0.64

1.94 _+ 0.35 1.94 +_ 0.25

5.58 4.20

0.02 0.04

1.36 +_ 0.84 0.80 +_ 0.39

1.74 + 0.55 1.02 + 0.45

5.71 5.11

0.02 0.03

1.72 + 0.60

1.97 + 0.16

6.55

0.01

0.96 + 0.19

1.16 + 0.36

9.68

0.003

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S. LABROPOULOUand S. BERATIS

the number of contacts with friends. In these aspects the siblings of the thalassaemic patients, both boys and girls, scored significantlylower than the control subjects. There was no significant difference in any of the items of the Activities or the Social scales between the older and the younger siblings of the thalassaemic patients.

Behavioral profile Comparison of the scores of the subjects of Group A with those of Group B on the 120 questions of the Behavior Profile of the CBCL demonstrated a significantly greater score only in the internalizing item "uncommunicative" for boys 6 to 11 years (Z-- - 2.11, p = 0 . 0 2 ) and the externalizing item "delinquent" for girls older than 12 years (z=-1.66, p=0.05).

DISCUSSION

The results show that the siblings of thalassaemic patients are more prone to psychopathology than siblings of normal children. Similar findings have been reported for siblings of patients with other chronic illnesses, such as spina bifida, diabetes, juvenile rheumatoid arthritis, gastrointestinal disorders, congenital heart disease, and leukaemia [1, 2, 8]. The significantly greater frequency, as compared with controls, of psychiatric disorders in the siblings of thalassaemic patients aged 10 years or older, but not in the younger siblings, indicates a greater vulnerability for psychiatric symptomatology after the age of 10. It suggests that the process of adolescence makes the older age group especially susceptible to the stressful effect of having a thalassaemic sibling. The 67°?o risk that each phenotypically normal sibling is a carrier, could also have contributed to the increased psychopathology of the older siblings. However, this factor should not have a major impact because programmes for routine detection of the carrier state and prenatal diagnosis have greatly reduced the birth of thalassaemic children. It is noteworthy that approximately 10% of the siblings of the thalassaemic patients were found to have oppositional defiant disorder, as compared to less than 3 °7o in the control group. In previous studies we reported an increased frequency of psychiatric disorders in patients with fl-thalassaemia, particularly of oppositional defiant disorder, which was diagnosed in 23°?o [14] and associated with inadequate compliance of thalassaemic patients with iron chelation therapy [18]. These observations suggest a trend for the siblings of thalassaemic children to develop a psychopathology similar to that of the affected children. The occurrence of psychosocial consequences for siblings is mediated by individual characteristics and environmental agents. No association was observed between the presence of the characteristic dysmorphic facies in some thalassaemic children and sibling psychiatric disorder. This indicates that disfigurement does not have a significant effect. Although similar findings have been reported in the literature [5], some other authors have pointed to a "stigmatizing" effect of visible characteristics of an illness and the impact it has on siblings' body image and behaviour [8]. We think that no general conclusion can be reached, because other factors may intervene- factors related to the nature of the disease and the community's attitude toward it and the affected child. Six to 11-year-old male siblings of the thalassaemic children obtained a greater score

917

Siblings of thalassaemic children

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s. LABROPOULOU and S. BERATIS

on the internalizing u n c o m m u n i c a t i v e item, whereas girls older than 12 obtained a greater score on the externalizing delinquent item. This might indicate an interaction o f the impact o f the sibling's chronic illness with other factors, such as age and sex, in the development o f the p h e n o m e n o l o g y o f the maladaptive behavior. The finding that the patients' siblings o f opposite-sex showed a better adjustment in some aspects o f their activity and social profile than did the same-sex siblings again indicates gender differences. We might expect that siblings o f the opposite sex run a lower risk t h a n same sex siblings in identifying with the passive, helpless behavior o f the thalassaemic child and that they react by b e c o m i n g more active and responsible. Some aspects o f the siblings' social functioning were clearly impaired. The impairment in social a d a p t a t i o n resulted from having a small n u m b e r o f close friends they maintained and a limited n u m b e r o f contacts. Reduction o f sports and nonsports activities has not previously been reported in siblings o f patients with any chronic illness. However, impaired social activity, manifested as social withdrawal, has been reported in siblings o f children with paediatric disorders o f haematology, cardiology, and those requiring plastic surgery [8]. In a n o t h e r study other investigators have reported increased social competence in siblings o f children with diabetes and pervasive developmental disorders [5]. The findings o f this study indicate the existence o f significantly greater psychopatholo g y in the siblings o f thalassaemic patients, particularly those over the age o f 10 years. In addition, these children show adverse effects o n various domains o f psychosocial functioning, particularly an impairment in some o f their activities and sociability. REFERENCES 1. TEW B, LAURENCE KM. Mothers, brothers and sisters of patients with spina bifida. Dev Med Child Neurol 1973; 15: (suppl 29): 69-76. 2. TRITT SG, ESSES LM. Psychosocial adaptation of siblings of children with chronic medical illnesses. A m J Orthopsychiatry 1988; 58:211-220. 3. GAYTON WF, FR1EDMAN SB, TAVORMINA JF, TUCKER F. Children with cystic fibrosis: 1. Psychological test findings of patients, siblings and parents. Pediatrics 1977; 59: 888-894. 4. BRESLAU N. Siblings of disabled children: Birth order and age-spacing effects. J A b n o r m ChildPsychol 1982; 10: 85-96. 5. FERRARI M. Chronic illness: Psychosocial effects on siblings-I. Chronically ill boys. J ChildPsychol Psychiatry 1984; 25: 459-476. 6. CAIRNS NU, CLARK GM, SMITH SD, LANSKY SB. Adaptation of siblings to childhood malignancy. J Pediatr 1979; 95: 484-487. 7. WOOD B, BOYLE JT, WATKINS JB, et al. Sibling psychological status and style as related to the disease of their chronically ill brothers and sisters: Implications for models of biopsychosocial interaction. J Dev Behav Pediatr 1988; 9: 66-72. 8. LAVIGNE JV, RYAN M. Psychologic adjustment of siblings of children with chronic illness. Pediatrics 1979; 63: 616-627, 9. VANCE JC, FAZAN LE, SATTERWH1TE B, PLESS IB. Effects of nephrotic syndrome on the family: a controlled study. Pediatrics 1980; 65: 948-955. 10. DROTAR D, CRAWFORD P. Psychological adaptation of siblings of chronically ill children: Research and practice implications. J Dev Behav Pediatr 1985; 6: 355-362. 11. LOBATO D, FAUST D, SPIRITO A. Examining the effects of chronic disease and disability on children's sibling relationships. J Pediatr Psychol 1988; 13: 389-407. 12. LOBATO D, BARBOUR L, HALL LJ, MILLER CT. Psychosocial characteristics of preschool siblings of handicapped and nonhandicapped children, J A b n o r m Child Psychol 1987; 15: 329-338. 13. AMERICAN PSYCHIATRIC ASSOCIATION. Diagnostic and Statistical Manual o f Mental Disorders, Third Edn revised (DSM-III-R). Washington, DC: American Psychiatric Association, 1987. 14. BERATIS S. Psychosocial status in pre-adolescent children with ~-thalassaemia. J Psychosom Res 1993; 37: 271-279.

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15. REY JM, PLAPP JM, STEWART GW. Reliability of psychiatric diagnosis in referred adolescents. J Child Psychol Psychiatry 1989; 311: 879-888. 16. ACHENBACH TM, EDELBROCK C. Manual f o r the Child Behavior Checklist and Revised Behavior Profile. Burlington, VT: Department of Psychiatry, University of Vermont, 1983. 17. FLEISS JL. Statistical Methods f o r Rates and Proportions. New York: Wiley, 1981. 18. BERATIS S. Noncompliance with iron chelation therapy in patients with 15-thalassaemia. J Psychosore Res 1989; 33: 739-745.