Medicina (Kaunas) 2004; 40(7) 663 VISUOMENĖS SVEIKATA Psychological adjustment of children with congenital hypothyroidism and phenylketonuria as related to parental psychological adjustment Roma Jusienė, Vaidutis Kučinskas1 Department of Psychology, Faculty of Social Sciences, Law University of Lithuania, 1 Department of Human and Medical Genetics, Faculty of Medicine, Vilnius University, Lithuania Key words: congenital hypothyroidism, phenylketonuria, psychological adjustment. Summary. Phenylketonuria and congenital hypothyroidism are the inherited metabolic diseases that can be diagnosed and successfully treated early from birth. Nevertheless, children with phenylketonuria and congenital hypothyroidism are found to be in the risk for psychological maladjustment. Parental adjustment – as significant condition for child’s psychological adjustment – and related factors are explored in this study. Parents of 63 children with congenital hypothyroidism and phenylketonuria (age 2 to 14 years) answered the Child Behavior Checklist, Coping Strategies Questionnaire and the questionnaire on reactions to child’s disease, relations with a sick child, with the spouse and other people. Severity of the disease and child’s age are considered as well. Results of the study show that parental emotional (maladaptive) coping and indulgence of a sick child account for the higher rates of internalizing problems of children with phenylketonuria and congenital hypothyroidism. In addition, the higher rates of children’s psychological problems are related to parental feelings of guilt as a reaction to child’s disease. Introduction Due to modern technologies of biomedicine many chronic pediatric conditions have become treatable and their impact on the individual’s physical health is controlled as much as possible. Thus, another question gained importance: how chronic conditions influence the patients’ mental health, their psychological adjustment? Many psychological studies conclude that children with different chronic diseases are at higher risk for child and adolescent psychopathology (1–6). More precisely, children with chronic diseases have less behavioral and emotional disturbances, if compared to clinical sample, but the number of emotional problems is almost as twice as in general population (4, 6). Hence, it is necessary to identify the highest risk group of sick children, and to find risk and protective factors to prevent maladjustment and to help those who encounter problems (5). Mass neonatal screening recently prosecuted in Lithuania allows early identification of two inherited diseases: phenylketonuria (since 1975) and congenital hypothyroidism (since the second half of 1993). Phenylketonuria (PKU) is an inherited metabolic disorder, which is caused by the absence or inactivity of phenylalanine hydroxylase, the enzyme that converts the amino acid phenylalanine (Phe) to tyrosine (7). Accumulation of phenylalanine and its metabolites in organism is particularly harmful for the development of central nervous system (8). A lifelong low protein dietary treatment is recommended for all patients with PKU early from birth to avoid mental retardation and other somatic and neurological symptoms. Congenital hypothyroidism (CH) is an inherited metabolic disorder, which is characterized by the scarcity of thyroid hormones starting in a fetus during pregnancy and continuing after birth (9). Thyroid hormones are vital for the growth of the whole organism, particularly for the development of central nervous system. The lifelong treatment with L-thyroxin is recommended early from birth to prevent retardation of mental and physical development. Regular blood controls of thyroid hormones (in the case of CH) and phenylanine (in the case of PKU) are performed monthly during the first year of life and every three months later on to evaluate effecti- Correspondence to V. Kučinskas, Medical Genetics Center, Santariškių 2, 08406 Vilnius, Lithuania E-mail: [email protected] 664 Roma Jusienė, Vaidutis Kučinskas veness of the treatment. Physical and mental development of these children is monitored. Children early and continuously treated for PKU and CH usually have normal intellectual abilities, thus slightly lower than that of normal peers and siblings (10). Some neuropsychological deficiencies, which manifest themselves as worse academic abilities or learning difficulties, are also observed. The relation between intelligence and severity of PKU and CH are documented by several researchers (10). The studies of psychological adjustment of children affected by these diseases reveal that they have more internalizing problems, which tend to increase with the sick children’s age (11–13). There is no agreement whether psychological adjustment of children with PKU can be related to the severity of illness or elevated Phe levels in patient’s blood (12). In addition, the studies of children with PKU reveal that stress evoked by the treatment requirements of the illness, psychological impact of the illness on the entire family and other psychological reasons have a determining significance on children’s psychological adjustment (14). Our previous studies reveal that Lithuanian children with treated PKU have lower intellectual quotient (IQ) than healthy controls, and higher rates for emotional and behavioral problems, especially in school age (15). Higher Phe levels were found to correlate with children’s IQ but not with the psychological problems. However, the lower IQ is related to the higher rates of psychological problems (15). Emotional and behavioral problems of children with CH can be related to severity of the disease (11). Psychological adjustment of children with CH as well as psychological related variables have not been studied in Lithuania and other countries thoroughly. Parental psychological adjustment can be essential factor of chronically ill child’s psychological adjustment. As it is asserted in many studies of children with disabilities and chronic conditions, child’s psychological adjustment reflects parental psychological adjustment (1, 4, 16). Parental psychological adjustment can influence children’s psychological adjustment indirectly through efficacy of the treatment that is affecting as an organic factor, as the parents of very young chronically ill children are mostly responsible for the treatment compliance. Parental psychological adjustment, that is coping abilities, emotional state and attitudes toward a sick child and the illness, can also directly influence children’s psychological adjustment through relations with a child. Parental overprotection and indulgence as well as the absence of feedback about inappropriate behavior in the case of child’s chronic conditions can determine the lack of social skills (3). Furthermore, lack of social skills can manifest itself as other emotional and behavioral problems (17). On the other hand, the care of a child with chronic conditions increases probability of parental maladjustment (18). In addition to the usual strain with a newborn child, parents of children with disabilities or other chronic conditions have to deal with additional demands, as for example, the grievance and disappointment of having given birth to a sick child, and the management of the special treatment requirements. The aim of the study The aim of the present study is to find out the variables related to the psychological maladjustment of children treated for phenylketonuria and congenital hypothyroidism and of their parents. The following main hypothesis have been formulated in this study: Parental psychological maladjustment can predict children’s psychological maladjustment: i. e. parental coping styles and reactions to a child’s illness and the quality of relations with a child is related to child’s emotional and behavioral problems. Methods Participants of the study are 63 children (ages 2– 14 years) with early and continuously treated PKU and CH and their parents. These children are patients at Medical Genetics Center of Vilnius University Hospital “Santariškių klinikos”. Forty-six (22 girls, 24 boys, ages 2–14 years) of them are children with phenylketonuria. Seventeen sick children are children with congenital hypothyroidism (9 girls, 8 boys; 2–6 years old, because the screening program for CH was started in late 1993 in Lithuania). The inclusion criteria for participation in the study were parental agreement, the absence of mental retardation in the child, and treatment started early and continued without interruption. Measures. Psychological adjustment of children with PKU and CH was assessed by their parents’ ratings on Child Behavior Checklist (19, 20). CBCL 4/18 for assessment of children aged 4–18 years and CBCL 2/3 for assessment of children aged 2–3 years were used in our study. CBCL 4/18 measures emotional and behavioral problems on eight syndrome scales: withdrawn, somatic complaints, anxious/depressed (these three comprise internalizing problems), delinquent and aggressive behavior (these two comprise externalizing problems), social problems, thought Medicina (Kaunas) 2004; 40(7) Psychological adjustment of children with congenital hypothyroidism problems and attention problems. Total problems are the score derived from all eight scales. CBCL 2/3 measures emotional and behavioral problems on six syndrome scales: withdrawn, somatic complaints, anxious/ depressed (these three comprise internalizing problems), destructive and aggressive behavior (these two comprise externalizing problems) and sleep problems. Total problems are the score derived from all six scales. The Coping Strategies Questionnaire (CSQ) (21) was used for assessment of parental everyday stress coping styles. CSQ measures four coping styles: rational, detachment (these two comprise adaptive coping), emotional and avoidance (these two comprise maladaptive coping). The structured questionnaire was used to assess the parental reactions to child’s disease and relations in the family, social relations and relations with a sick child as affected/changed by child’s disease. Parents answered “yes” or “no” to the statements such as: “When I learned about child’s disease, I felt confused”, “I blamed myself”, “I often argue with the child”, “I make connivance to a sick child”, etc. The severity of illness of the children with PKU and CH was assessed by ratings of the medical geneticians in Medical Genetics Center. The severity of PKU was assessed according the biochemical phenotype and genotype; the severity of CH was assessed according the functionality of thyroid tissue. Results Severity of the illnesses The comparison of children’s with CH behavior problems according severity of the illness is complicated as only 4 cases are diagnosed as severe form of CH and 12 cases – as moderate and 1 case as mild form of CH. Those with severe CH have more total, externalizing and particularly internalizing problems than the children with moderate and mild form of CH, though differences do not reach significant level (as compared with Mann-Whitney U test, Z=1.934, 665 p=0.053, for internalizing problems). The comparison of CBCL scores according severity of the illness is complicated in PKU-affected children as in the case of CH. 40 children with PKU are diagnosed with severe form of disease and only 5 as moderate and 1 as mild. Results revealed the nonsignificant tendency for severity of illness to relate to internalizing problems. The latter may also be related to age, because mean age of children with different severity of illness differs significantly. Parental coping strategies as related to children’s behavioral problems Coping strategies of parents with PKU and CH children do not differ significantly between these two groups. Correlation analysis of coping strategies as related to CBCL scores reveal significant relations. As Table 1 shows parental total maladaptive coping and especially emotional coping are related to children’s internalizing and total problems. Parents’ with PKU and CH children response to the child’s disease Most of parents (95%) accepted the medical diagnosis believing that the disease can be treated and controlled. Nevertheless, they have other emotional reactions as well. For 68% of parents it took long time to get use to thought about child’s disease; 95% felt confused and puzzled, because of uncertainty about the future, and 77% stated they were afraid because had no knowledge about the illness. One third of parents (32%) blamed themselves and felt guilty, and 42% felt anger toward themselves / others / God. Responses to child’s illness do not differ significantly while comparing responses of parents of children with PKU and CH. As for example 25% parents who have a child with CH blamed themselves and 35% parents of PKU-affected child did. Feelings of anger were indicated by 27% of parents with CH child and by 44% of parents with PKU child (these differences are not significant as compared with Pearson’s Chi-square). Table 1. Correlations between parental coping strategies and CBCL scores Parental coping strategies Total maladaptive coping Emotional coping Avoidant coping Total adaptive coping * p<0.05; ** p<0.01 Medicina (Kaunas) 2004; 40(7) Internalizing problems Externalizing problems Total problems 0.325* 0.382** 0.146 –0.075 0.154 0.236 0.022 0.076 0.275* 0.356** 0.094 –0.034 Roma Jusienė, Vaidutis Kučinskas 666 The comparisons of CBCL scores with MannWhitney U test according to the parental reactions to child’s disease do show several significant differences (Table 2). Parents who blamed themselves also rated their sick children as having more internalizing and total problems. Parental feelings of anger are also related to higher CBCL scores, though these differences are not significant. The results also show that parental feelings of guilt and anger are related to their emotional and total maladaptive coping (Table 2). As compared with MannWhitney U test parents who felt anger also tend to use more emotional stress coping strategies (Z=2.783, p<0.01) and maladaptive coping (Z=2.449, p<0.05), than those who did not feel anger. Parents who felt guilt use more emotional coping strategies (Z=2.063, p<0.05) than those who did not feel guilt. There where no significant differences found in groups of parents who blamed / felt anger according their education, children’s age and severity of illness. Parental relations with a sick child and with a spouse More than half of parents (53%) are indulging their sick child. Thirty percent of parents answered they do not require discipline from a sick child and 20% parents often have quarrels with a sick child. Parents of children with PKU and CH differ according their relations with a child. Parents of children with PKU are more often indulging their child (Pearson c2=4.560, p<0.05), more of them do not require discipline (Pearson c2=5.640, p<0.05) and do quarrel with a sick child (Pearson c2=5.312, p<0.05). Two latter peculiarities of the relations are also related to a child’s age: parents require no discipline and quarrel with the older children. The between groups comparison of CBCL scores according the parental connivance to a sick child with Mann-Whitney U test reveal several significant differences. Children with PKU and CH who are indulged by their parents have more internalizing and total problems than those who are not indulged (Table 2). The mean children’s age does not differ significantly between two groups. Results of analysis of the association between child-parent relations and parental responses to illness show that indulgence significantly relates to feelings of guilt (Pearson c2=8.744, p<0.01) and anger (Pearson c2=4.659, p<0.05, Fig. 1). Parents who indulge their child also tend to use significantly more emotional (Mann-Whitney U test’s Z=2.642, p<0.01) and maladaptive (Z=2.518, p<0.05) coping (Table 2). Fifty-seven percent of parents upbringing children with PKU and CH indicated that the child’s disease consolidated/strengthened the relations with a spouse; 53% said the relations did not change; 14% of parents said the child’s disease caused the conflicts with a Table 2. Comparison of CBCL scores and coping styles between parents who indulge and do not indulge a sick child and who feel guilty and angry and do not feel guilty and angry Mean (SD) Problems and coping styles Indulging a child “Yes” N=33 “No” N=28 Total problems 42.3 (16.3) 29.9 (15.2) Internalizing problems 15.3 (7.1) Externalizing problems Mean (SD) p value Feeling guilty “Yes” N=20 “No” N=41 p<0.01 40.9 (19.5) 35.6 (15.3) 9.07 (5.3) p<0.01 14.6 (8.1) 15 (6.7) 13.6 (7.3) ns Total maladaptive coping 23 (6.6) 19.7 (4) Emotional coping 8.9 (3.4) Avoidant coping Total adaptive coping Mean (SD) p value Feeling angry p value “Yes” N=26 “No” N=36 p<0.05 39.6 (19.7) 34.3 (14) ns 11.4 (6.3) p<0.05 13.9 (7.8) 11.6 (6.4) ns 15.9 (8.5) 13.6 (6.1) ns 14.9 (7.8) 13.7 (6.4) ns p<0.05 22.3 (5) 21.1 (6.2) ns 23.8 (5.2) 19.7 (5.7) p<0.05 6.7 (3) p<0.01 9.2 (2.8) 7.3 (3.5) p<0.05 9.4 (2.7) 6.7 (3.5) p<0.01 14.1 (4.9) 13 (2.6) ns 13.1 (3.4) 13.8 (4.3) ns 14.4 (4) 13 (3.9) ns 23.7 (5.8) 23.5 (7.2) ns 24.2 (4.5) 23.3 (7.2) ns 23.7 (6.1) 23.7 (6.6) ns Medicina (Kaunas) 2004; 40(7) Psychological adjustment of children with congenital hypothyroidism 667 100% 4 80% 8 24 20 60% not indulging Not indulging 40% 18 Indulging indulging aa child child 16 17 15 No feelings of guilt No feelings of anger 20% 0% Felt guilty Felt angry Fig. 1. The number and proportion of parents indulging a child and feeling guilty and angry spouse and 5% divorced as a result. The comparison of CBCL scores according the marital relations of parents show the tendency that incidence of conflicts and divorce relates to higher rates of children’s total, internalizing and externalizing problems, though the differences are not significant. Results of analysis of relations with a spouse show significant relation with parental education: high-educated parents indicated more negative affects of child’s disease on the family. Other difficulties of families with children with PKU and CH Parents of children with PKU indicated negative influence of child’s disease on social relations and parental employment, but did not parents of children with CH. Eleven percent of mothers of children with PKU have changed the job and 20% have quited the job because of child’s disease. Twenty-nine percent of parents of children with PKU indicated that they go out (from home) less after the child with PKU was born. Nonparametric Spearman correlations reveal that negative effect on parental social relations and employment relates to parental response to child’s disease (rho=0.436, p<0.01) and to relations with a sick child (rho=0.488, p<0.01). Analysis of the results also shows significant correlations between financial embarrassment (as indicated by parents) and negative influence of disease on social relations and/or employment and with parental requirement for psychological and social help. Analysis of interaction between psychological adjustment of children with PKU and CH and of their parents Multiple regression analysis was applied to explore the variables predicting the emotional and behavioral Medicina (Kaunas) 2004; 40(7) problems of children with PKU and CH. Results show, that children’s total problems can be predicted by parental emotional coping and indulgence (Model’s F=7.383, p<0.01; coefficient of determination R2=0.20, Table 3). Parental emotional coping and indulgence account also for 28% of variance of the children with PKU and CH internalizing problems (Model’s F=11.054, p<0.001; Table 4). Table 3. Results of multiple reggression analysis predicting children’s total problems Predictor Indulging Emotional coping Total maladaptive coping Feeling guilty Feeling angry b t p 0.287 0.261 0.182 2.285 2.076 1.445 0.026 0.042 0.154 0.052 0.069 0.395 0.546 0.694 0.587 Note. Multiple R=0.447, R2=0.20. Table 4. Results of multiple reggression analysis predicting children’s internalizing problems Predictor Indulging Emotional coping Total maladaptive coping Feeling guilty Feeling angry b t p 0.354 0.292 0.226 2.968 2.453 1.889 0.004 0.017 0.064 0.0677 0.060 0.550 0.476 0.584 0.636 Note. Multiple R=0.529, R2=0.28. Roma Jusienė, Vaidutis Kučinskas 668 Parental emotional coping Parental feelings of guilt and / or anger Indulgence of a sick child Children’s psychological maladjustment (emotional and behavioral problems) Fig. 2. Summary of relations between children’s with PKU and CH psychological maladjustment and parental emotional coping, feelings of guilt/anger and indulgence Results of regression analysis and previous correlation and comparative analysis between parental and children’s psychological maladjustment are summarized in Figure 2. Discussion The results of the study confirm hypothesis that parental psychological maladjustment predicts the psychological maladjustment of children with congenital hypothyroidism and phenylketonuria. Parents who tend to use more emotion-oriented coping in everyday stress situations and who indulge their sick children also rate them as having more psychological problems. The relationship between parental and children’s psychological adjustment is documented in studies of depressed families and families with disabled children as well (1, 16, 22, 23). There are also considerations that parenting style and ability to solve problems are related to children’s with phenylketonuria psychological adjustment (12). Parental feelings of guilt and anger, though relate to the adjustment problems of children with PKU and CH in our study, do not have a predictive value. Thus, we assume that children’s psychological adjustment or, more precisely, mental and physical health, also may have influence on parental responses and relations with a sick child. As for example when a child has more emotional and behavioral problems (as conditioned by severe form of illness or other organic and intrapersonal factors), his parents may blame themselves and to overindulge the child. Additionally, there is a possibility that poorly ad- justed parents may be prone to detect more psychological problems in their children (5, 24, 25). The evaluations of children’s behavior problems obtained from other informants (teachers, psychologists, older children themselves) could help answer this question in future studies. However even keeping in mind the limitations of the present study we can conclude that parental adjustment – their reactions to child’s disease, coping abilities, interpersonal relations and social environments – is of utmost importance to consider when dealing with child’s inherited disorders. On the one hand, children learn to adjust and cope from their parents (26). On the other hand, depressed parental mood can restrict their ability to satisfy children’s social and emotional needs (22). Moreover, parental distress and depression can negatively affect adherence to treatment requirements. The indulgence of the sick child thus implies not only inadequate parental-child relationship, which can disturb development of child’s social relations, but also implies “making excuses” from the low protein diet in the case of PKU. Increase of the concentration of phenylalanine in child’s organism, as a result, may directly influence child’s mental health, that is cognitive and emotional functioning. According to our results, social environments interact with parental-child relationships and parental psychological adjustment. Additional requirements of PKU versus CH treatment indicate also more negative impact on social environments. Thus, the social supports are essential for families of children with pheMedicina (Kaunas) 2004; 40(7) Psychological adjustment of children with congenital hypothyroidism nylketonuria and other diseases with additional treatment requirements. Finally, we suggest that children with phenylketonuria, congenital hypothyroidism and other inherited or chronic conditions can be protected from psychopathology by collaborative efforts of medical staff, psychologists and social workers and, most importantly, parents and children themselves. First, it is necessary to assure that parents and children would be active participants in creating their own psychological well being; secondly, that they always would have a possibility to get professional help. Conclusions Emotional and total maladaptive stress coping strategies of parents, who have children with phenylketonuria and congenital hypothyroidism, are related to children’s total and internalizing problems. Parents who felt guilty as a response to child’s disease and who indulged a sick child also rate their children with phenylketonuria and congenital hypothyroidism as 669 having more internalizing and total behavioral problems. Parental feelings of guilt and anger and parental indulgence of a sick child are related to parental emotional and total maladaptive coping. Parental reactions to a child’s illness and interactions with a child are related to the influence of the illness on parental social relations and/or employment. The influence of phenylketonuria on social relations and/or employment is more negative than that of congenital hypothyroidism. Parental psychological adjustment, their ability to react to a child’s illness properly, which manifests itself through interactions with a sick child, and the use of adaptive coping patterns are important factors of a good psychological adjustment of a chronically ill child. Acknowledgements The authors thank the parents of children with phenylketonuria and congenital hypothyroidism and Loreta Cimbalistienė, M.D., Ph.D. and Dalia Juščienė, M.D., Ph.D for cooperation in this study. Įgimta hipotiroze ir fenilketonurija sergančių vaikų psichologinių problemų sąsajos su tėvų psichologiniu prisitaikymu Roma Jusienė, Vaidutis Kučinskas1 Lietuvos teisės universiteto Socialinio darbo fakulteto Psichologijos katedra 1 Vilniaus universiteto Medicinos fakulteto Žmogaus ir medicininės genetikos katedra Raktažodžiai: fenilketonurija, įgimta hipotirozė, psichologinis prisitaikymas. Santrauka. Anksti pradėtas ir tinkamas paveldimų medžiagų apykaitos ligų fenilketonurijos ir įgimtos hipotirozės gydymas sąlygoja normalią sergančių vaikų psichinę ir fizinę raidą. Tačiau, kai kurių tyrimų duomenimis, net ir gydant šias ligas, jos kaip ir kitos lėtinės ligos gali lemti vaiko emocinius ar elgesio sutrikimus, kitus šeimos psichologinius sunkumus. Viena svarbiausių sergančio vaiko psichologinio prisitaikymo prielaidų yra tėvų psichologinis prisitaikymas, jų reagavimas į vaikui diagnozuotą ligą, streso įveikimo būdai, santykiai su sergančiu vaiku, santykiai šeimoje bei su kitais žmonėmis. Šio tyrimo tikslas – išanalizuoti, kaip tėvų psichologinio prisitaikymo ypatybės gali būti susijusios su vaikų, sergančių fenilketonurija ir įgimta hipotiroze, sunkesniu psichologiniu prisitaikymu. Tyrime dalyvavo 63 vaikai, sergantys paveldimomis medžiagų apykaitos ligomis ir dėl šių ligų gydomi (2– 14 metų; 46 iš jų serga fenilketonurija, 17 – įgimta hipotiroze), bei jų tėvai. Vaikų psichologiniam prisitaikymui įvertinti naudojome Vaikų elgesio klausimyną, kurį pildė tėvai (CBCL 2/3 ir CBCL 4/18, Achenbach, 1991). Tėvų streso įveikimo būdams vertinti naudojome Įveikimo strategijų klausimyną (Elklit, 1996). Tėvai taip pat pildė klausimyną, kur buvo klausiama apie reagavimą į vaiko ligą, ligos įtaką santykiams su vaiku, sutuoktiniu, socialiniams santykiams ir užimtumui. Tyrimo duomenimis, tėvų emocionalūs ir apskritai neadaptyvūs streso įveikimo būdai bei tėvų kaltės jausmas, nuolaidžiavimas sergančiam vaikui yra susiję su fenilketonurija ir įgimta hipotiroze sergančių vaikų elgesio bei emocijų sunkumais. Įvertinus tėvų emocinius streso įveikimo būdus ir nuolaidžiavimą sergančiam vaikui, galima prognozuoti vaikų emocinius sunkumus. Tėvų kaltės jausmas ir pyktis, neadaptyvūs streso įveikimo būdai bei nuolaidžiavimas sergančiam vaikui yra labai susiję. Manome, jog labiausiai sergančio vaiko psichologinį prisitaikymą veikia tėvų ir sergančio vaiko tarpusavio santykiai. Adresas susirašinėjimui: V. Kučinskas, Medicininės genetikos centras, Santariškių 2, 08406 Vilnius El. paštas: [email protected] Medicina (Kaunas) 2004; 40(7) 670 Roma Jusienė, Vaidutis Kučinskas References 1. Eiser C. 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