Psychosocial profiles of children with achondroplasia in terms of their short stature-related stress: a nationwide survey in Japan

Authors

  • Naoko Nishimura MS, RN,

    Doctoral Student, Corresponding author
    1. School of Health Sciences, Tottori University Faculty of Medicine, Yonago, Japan
    • Correspondence: Naoko Nishimura, Doctoral Student, School of Health Sciences, Tottori University Faculty of Medicine, 86 Nishi-cho, Yonago 683-8503, Japan. Telephone: +81 859 38 6317.

      E-mail: nawoko1@med.tottori-u.ac.jp

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  • Keiichi Hanaki MD, PhD

    1. School of Health Sciences, Tottori University Faculty of Medicine, Yonago, Japan
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Abstract

Aims and objectives

To assess psychosocial profiles of children with achondroplasia using a nationwide survey.

Background

Achondroplasia, showing short stature and disproportionately short limbs, causes physical inconvenience such as difficulty in reaching high objects. It is, however, still controversial whether the condition is associated with psychological problems, especially in childhood.

Design

A cross-sectional descriptive design was employed.

Methods

To evaluate psychosocial profiles and adaptation processes in children with achondroplasia, we developed an inventory of scales based on the psychological stress model of which conceptual framework was comprised of stressor, coping process, coping resource and adaptation outcome domains. Participants were recruited nationwide through the largest advocacy support group for achondroplasia in Japan. Of the 130 group members, 73 X-ray-diagnosed patients, aged 8–18 years, completed the inventory of questionnaires to be analysed.

Results

As for the stressor domain, patients experienced short stature-related unpleasant experiences more frequently (z-score: +1·3 in average, +3·9 in physical inconvenience). Nevertheless, these experiences had little effect on the coping process (threat appraisal: −0·2, control appraisal: +0·1) and the adaptation outcome (stress response: +0·3, self-concept: 0·0). Interestingly, self-efficacy in the coping resource domain was noticeably increased (+3·1) and was strongly correlated with most variables in the coping process and in adaptation outcome domains.

Conclusions

Although the children with achondroplasia experienced more short stature-related stressors, there was no evidence of any psychosocial maladaptation. This finding suggests that coping process as well as coping resources such as self-efficacy could be important targets for promoting psychological adjustment in children with achondroplasia.

Relevance to clinical practice

To help children with achondroplasia adapt socially, nurses and other healthcare providers should routinely assess their psychological adaptation process, especially cognitive appraisal and self-efficacy.

Ancillary