Original article
Psychosocial adjustment in siblings of young people with Duchenne muscular dystrophy

https://doi.org/10.1016/j.ejpn.2009.09.011Get rights and content

Abstract

Duchenne muscular dystrophy (DMD) is a progressive, impairing, life-limiting disorder of childhood. Little is known about how siblings adapt to this. The aim of this study is to document psychosocial adjustment in siblings of patients with DMD. Healthy siblings (11–18 years old) of young people with DMD attending a specialist paediatric centre and their parent/main carer took part. Parents, siblings and teachers completed a battery of questionnaires: (i) to assess psychiatric risk the Strengths and Difficulties Questionnaire (SDQ), General Health Questionnaire (GHQ), Hospital Anxiety and Depression Scale (HADS); (ii) to measure general wellbeing: SF-36; (iii) to document DMD illness disability: Functional Disability Inventory (FDI); (iv) to assess family function and life stresses for the unaffected sibling: Family Assessment Device (FAD), Family Burden Interview Schedule and Life Events Checklist. Forty six/77 eligible siblings (24 females/22 males); (mean age 14 years (SD 2.3)) took part. Although their mean psychological functioning and wellbeing questionnaire scores were comparable to normative data, there was a trend for more siblings scoring at high-risk for psychological (mainly emotional) problems. Weak/moderate associations with psychological symptoms in siblings varied according to informant and included the following factors: closeness in age to the affected sibling; older sibling age; extent of wheelchair use, burden of illness on family interactions, and siblings reporting high impact of illness on their lives. Psychological symptoms were also associated with less sibling involvement in patient care, with broader psychosocial and family disadvantage and with life stresses. Siblings have an increased risk for emotional problems, which appears influenced by specific illness factors.

Introduction

Duchenne muscular dystrophy (DMD) is a progressive neuromuscular disorder affecting males leading to increased muscular weakness and disability and a shortened life span. Children typically experience the initial symptoms in the first 5 years of life. The diagnosis, progressive weakness, and loss of ambulation by age 12 can cause practical and emotional demands on families who may face continual deterioration without significant periods of stabilisation. High levels of stress have been documented in parents,1, 2 specially at times of more obvious health deterioration likely to re-awaken feelings of grief experienced when the original diagnosis was made.3

The psychological adjustment of the unaffected siblings of young people with DMD has been documented only to a limited extent. The literature on siblings of children with other paediatric conditions suggests that they are at slightly increased risk of developing psychiatric problems, especially emotional disorders such as anxiety and depression.4, 5, 6 It is unclear whether sibling age and gender influence adjustment, but some reports suggest increased psychological symptoms in female siblings, more specifically in older sisters of affected males.7 There are also suggestions that conditions requiring daily treatment may be associated with more negative effects,5 perhaps due to the differential treatment of siblings by parents, and/or the excessive domestic responsibilities or caretaking roles placed upon unaffected siblings.8

Nevertheless, the literature has also documented positive outcomes in unaffected siblings and families of children with chronic health conditions thought to be derived from the experience of living with illness. These include improved family cohesion, empathy, and personal growth, with unaffected siblings described as particularly responsible, caring, warm, and independent.6, 9

One study addressed the psychological adjustment of unaffected siblings of young people with one of the neuromuscular disorders, spinal muscular atrophy (SMA), and reported an increased risk for emotional and behavioural disorders in siblings when compared to healthy controls as well as the siblings affected with SMA, suggesting that siblings carry a greater burden within the family.10 It may be expected that adverse effects will be more marked in families with DMD because, unlike SMA type III and less so type II, DMD has a relentless tendency to deteriorate and in one out of three sufferers also affects intellectual and cognitive functions making comparisons with DMD on psychological impact on siblings less meaningful. Nevertheless some SMA types – such as SMA type I and SMA with respiratory distress – can progress even more rapidly and have a significantly reduced life expectancy.

The present study aims to document the psychosocial adjustment of unaffected siblings of young people with DMD, and compare wellbeing and psychiatric adjustment to general population norms. It also aims to examine the associations between the unaffected sibling's psychosocial adjustment and both illness related and other contextual factors. We hypothesized that problems in psychiatric adjustment would be increased in unaffected siblings and related to illness related factors such as high levels of involvement with the affected sibling.

Section snippets

Participants

Patients with DMD attending the Dubowitz Neuromuscular Centre – one of the two major centres for care of paediatric neuromuscular patients in the UK – between September 2006 and July 2007, with unaffected siblings aged 11–18 years were identified from clinic notes, databases, and discussions with clinicians and members of the multidisciplinary team. The centre is the only specialist resource in the area, and children attending are therefore likely to be representative of the generality of

Results

The unaffected siblings taking part in the study (n = 46) were comparable to the non-participating unaffected siblings (n = 31) in age distribution, but significantly more non-participants were female (28/31 vs 24/46: χ2(1) = 8.0, p = .004). The age range was 11–18 years. There was no difference in wheelchair use among affected siblings (as a proxy measure of illness severity), nor in family gene-carrier status (where this was known).

Discussion

Even though a third of the siblings of young people with DMD reported that the illness exerted an impact on their lives, and substantial impact on family life was noted by half the families, the unaffected siblings only had a slightly higher risk for emotional difficulties compared to the general population norms. The emotional and psychological adjustment of unaffected siblings showed associations with a measure of illness progression, and with other factors on which the illness might have a

Conclusion

In spite of the high practical and emotional demands placed upon families by DMD our results suggest that the majority of unaffected siblings are psychologically well adjusted, with only a modest increased risk for emotional symptoms. It will therefore be of interest to study additional potentially protective mechanisms generated by the experience of illness in a sibling. The study identifies both general psychosocial and illness related factors that may increase the risk of emotional problems

Acknowledgements

The financial support of a Muscular Dystrophy Campaign Centre Grant to the Dubowitz Neuromuscular Centre is gratefully acknowledged.

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