Psychosocial problems, coping strategies, and the need for information of parents of children with Prader–Willi syndrome and Angelman syndrome

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Abstract

The aim of the present study was to identify the psychosocial problems of parents of a child with Prader–Willi syndrome or a child with Angelman syndrome. In addition, the strategies these parents apply to cope with these problems as well as their need for information are described. To assess these topics, parents filled in a self-report questionnaire. Both parent groups were found to have a high need for information, high feelings of loss of control, relatively high depressive feelings, particularly in mothers in both syndrome groups. Differences due to the type of syndrome were found on the fear factor. Parents of a child with Angelman syndrome had greater feelings of fear for the negative consequences for themselves, whereas parents of Prader–Willi children were more concerned about the consequences for the child. In general, coping strategies were not found to be different between the parent groups of children who had either type of syndrome.

Introduction

Parents of a child with a mental handicap are frequently confronted with various kinds of psychosocial and practical problems that sometimes cause high levels of parental distress. These include emotional problems such as fear, depression and worry, reduced satisfaction in their marriage, practical and financial problems, problems related to educational tasks, as well as reduced levels of self-esteem and self-confidence [1], [2], [3]. Understanding the specific nature and determinants of these problems as well as the ways parents cope with problems is highly relevant for the development of family interventions and strategies for prevention, education and support. An approach to understanding how parents cope with the education and care of a handicapped child can be based on the transactional theory on coping of Lazarus and Folkman [4], [5]. According to the stress-coping theory of Lazarus and Folkman [4], the extent to which having a child with a mental handicap is experienced as stressful will depend firstly on the parents’ appraisal of the situation of their child and the expected consequences for the family and the family members well-being (primary appraisal). Subsequently, parents will appraise the resources (external and intrapersonal) they have available to cope with the situation, the effectiveness of the different resources and the expected outcomes after implementation of these resources (secondary appraisal). Parents will appraise the efficacy of external resources such as available services, the special education facilities for their child, or opportunities for work. They will also appraise their personal resources and skills in handling the stress for themselves and their family. Based on this appraisal process, parents will apply specific problem-focused and emotion-focused strategies in coping with the situation and with their emotions. According to Lazarus and Folkman [4] coping is seen as a dynamic interaction process between a person and the situation, in which that person constantly tries to keep a balance between his or her own resources and the requirements of the situation.

The appraisal of the situation depends on characteristics of the handicapped child and on characteristics of the parents and family. Characteristics of the child that appear to be clearly related to a higher level of stress in their parents are poor physical health, difficult temperament, low social responsiveness, behavioral problems, limited social skills of the child, and more unusual care taking demands [6], [3].

With regard to parental characteristics, research by Schilling and associates [7] indicates that mothers and fathers differ in their perception of their handicapped child’s needs and in their own sense of responsibility for addressing those needs. Thus, they may differ in the way they deal with the daily stresses of caring for their child. Other characteristics that have been mentioned as possibly having an influence on experiencing the situation as stressful are age of the parent, social class, income and personality [3]. Also, characteristics of the family that have been mentioned as possibly having an impact on the amount of stress that parents may experience are the life-cycle stage of the family, number of children, age of handicapped child in relation to the others, family integration prior to the birth of the handicapped child, and relations of the handicapped child with the other children [8], [3].

The present study focuses on the psychosocial problems of parents with a child with Prader–Willi syndrome or a child with Angelman syndrome, on the strategies these parents apply to cope with these problems, and on their need for information.

Prader–Willi syndrome and Angelman syndrome are rare genetic disorders caused by the loss of function of imprinted genes on the proximal long arm of chromosome 15 (15q11–13) [9]. The estimated prevalence is around 1 in 20,000 for each syndrome. Other studies mention prevalence rates between 1 in 10,000 and 1 in 40,000 live births [10], [11]. Both syndromes occur across all ethnic groups.

Prader–Willi syndrome was first described by Prader, Labhart and Willi in 1956 [12]. The syndrome is characterized by severe neonatal hypotonia, feeding difficulties, delayed developmental milestones and a typical physical phenotype including a typical dysmorphic facial appearance with triangular mouth and almond-shaped eyes, short stature, small hands and feet, genital underdevelopment and delayed secondary sexual characteristics [13], [14]. There is an increased prevalence of scoliosis and strabismus. In the first months of life severe muscle hypotonia and a failure to thrive are the most prominent features. After a few years the most noticeable characteristic is a developing hyperphagia leading to life-threatening obesity if not controlled. Many of the manifestations are related to functional hypothalamic deficiency [15].

Prader–Willi syndrome is said to have a characteristic psychological and behavioural phenotype. Some of the symptoms of Prader–Willi such as insatiable appetite, outbursts of rage, stubbornness, and difficulty in dealing effectively with changes in their daily routines become increasingly apparent and severe with age [13], [16], [17]. Skin picking and abnormal sleep patterns have been reported in most of the affected individuals as well as difficulties in social relationships with peers and reduced physical activity.

Angelman syndrome is, like the Prader–Willi syndrome, a complex multiple-anomaly syndrome, which is clinically distinct from Prader–Willi syndrome. Angelman syndrome, which was first described by Angelman in 1965 [18], is characterized by severe or profound learning difficulties and lack of speech, delayed motor development, inappropriate bouts of laughter, an ataxic gait, and jerky voluntary movements. Furthermore, there is the typical facial appearance with a long face and prominent jaw, a wide mouth with widely spaced teeth, thin upper lip, an underdeveloped mid-face and deep-set eyes, and a flat occiput. Among the frequent clinical characteristics are microcephaly, epileptic seizures and an abnormal characteristic EEG pattern with large-amplitude slow-spike waves [19], [20], [21].

Because of the typical psychological and behavioral characteristics of a child with Prader–Willi syndrome or a child with Angelman syndrome it is expected that these characteristics will have a specific impact on the parents’ interpretation of their situation, resulting in a particular pattern of psychosocial problems and in particular strategies for coping with these problems. In addition to an analysis of the psychosocial problems of parents, this study also focuses on analyzing the parents’ need for information. Information available to parents or information received from professionals or from other sources may highly influence the extent to which parents perceive their situation as stressful. Use of information also plays an important role in certain coping strategies, and providing information may enhance the use of specific coping strategies preferred by parents in coping with their problems.

Section snippets

Participants and procedure

To assess the impact of having a child with Prader–Willi syndrome or a child with Angelman syndrome on the psychological functioning of parents, a self-report questionnaire was sent to all families who were members of the Dutch Prader–Willi/Angelman Parent Association and who either had a child with Prader–Willi syndrome (46 families) or a child with Angelman syndrome (31 families) between 0 and 12 years of age. Families living abroad were excluded from the study. One family was excluded later

Results

In Table 2, Table 3, Table 4, Table 5, Table 6, Table 7, Table 8 the scores on the individual items of each scale measuring psychosocial problems of parents of both children with Prader–Willi syndrome and children with Angelman syndrome are presented. In Table 9 psychosocial problems of parents with a child with Prader–Willi syndrome and parents of a child with Angelman syndrome are compared. For each of the syndromes, psychosocial problems of fathers and mothers are compared in Table 10.

Discussion

The results indicate a great need for information for parents with respect to many different aspects that may impact on the development and future prospects of their child.

Parents particularly have a great need for information about the possible consequences of the syndrome for their child, about how their child may develop in the future, and how education might contribute to this development. As compared to the parents with Prader–Willi syndrome the need for information of parents with a child

Acknowledgements

We thank the John William Joseph Ackermans and Wife Lenore Kirschweg Ackermans Memorial to the City of Maastricht, the Netherlands, for the support for this project. We thank the parents for providing us with information about their needs for information, coping strategies and psychosocial problems.

References (26)

  • R.F. Schilling et al.

    Coping with a handicapped child: differences between mothers and fathers

    Soc Sci Med

    (1985)
  • J.F.A. Pruyn

    Coping with stress in cancer patients

    Patient Educ Couns

    (1983)
  • S. Zeitlin et al.

    The coping with stress model: a counselling approach for families with a handicapped child

    J Couns Dev

    (1987)
  • L.L. Dyson

    Families of young children with handicaps: parental stress and family functioning

    Am J Ment Ret

    (1991)
  • H.W. van Berkum

    Stress bij ouders van een verstandelijk gehandicapt kind: evaluatie van thuiszorg (Stress in parents with a child with a mental handicap: an evaluation of home care)

    (1992)
  • R.S. Lazarus et al.

    Stress, appraisal and coping

    (1984)
  • B.A. Beresford

    Resources and strategies: how parents cope with the care of a disabled child

    J Child Psychol Psychiatry Allied Discip

    (1994)
  • P.J. Beckman

    Influence of selected child characteristics on stress in families of handicapped infants

    Am J Ment Def

    (1983)
  • E.A. Byrne et al.

    The effects of mentally handicapped children on families: a conceptual view

    J Child Psychol Psychiatry Allied Discip

    (1985)
  • C.C. Glenn et al.

    Genomic imprinting: potential function and mechanisms revealed by the Prader–Willi and Angelman syndromes

    Mol Hum Reprod

    (1997)
  • L. Burd et al.

    Prevalence study of Prader–Willi syndrome in North Dakota

    Am J Med Genet

    (1990)
  • A. Åkefeldt et al.

    Prader–Willi syndrome in a Swedish rural county: epidemiological aspects

    Dev Med Child Neurol

    (1991)
  • A. Prader et al.

    Ein Syndrom von Adipositas, Kleinwuchs, Kryptorchismus und Oligophrenie nach myatonieartigem Zustand im Neugeborenenalter

    Schweiz Med Wochenschr

    (1956)
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