The relationship between quality of life and coping strategies of children with EB and their parents

Petra J Mauritz,José C Duipmans,Mariët Hagedoorn,Marieke Bolling

doi:10.1186/s13023-021-01702-x

Abstract

BackgroundEpidermolysis bullosa (EB) is a group of rare genetic skin disorders that primarily manifest as blisters and erosions following mild mechanical trauma. Despite the crucial role of the parents of children with EB in managing the disease, studies focusing on the parent–child relationship remain a gap in the literature. To address this gap, the current quantitative study, involving 55 children with all types of EB and 48 parents, assessed the relationship between their quality of life and coping strategies. Quality of life was measured with the Pediatric Quality of Life Inventory and TNO-AZL Questionnaire for Adult’s Health- related Quality of Life, and coping strategies were assessed with the Coping with a Disease Questionnaire. The majority of the analyses were descriptive and the results were interpreted qualitatively because of the small sample size.ResultsOverall, the quality of life of children with EB and that of their parents was somewhat lower compared with the quality of life of healthy children and adults. Children with EB who more frequently used emotional reactions and cognitive-palliative strategies to cope with the disease demonstrated lower levels of emotional and social functioning, while children who showed more acceptance and distancing showed higher levels of functioning on all domains. Parents who frequently demonstrated emotional reactions reported lower levels of social functioning and experienced more depressive emotions and anger. Parents who used more avoidance showed higher levels of positive emotions. Within parent–child dyads, acceptance, cognitive-palliative strategies and distancing were positively related. Children’s emotional and social functioning were negatively associated with their parents’ depressive emotions. Parents’ acceptance was linked to higher physical functioning in children, whereas children’s avoidance was linked to a lower level of anger in parents.ConclusionChildren who are able to accept the disease or distance themselves from it appear to be better off in contrast to those who tend to engage in the cognitive-palliative strategies and expressing emotional reactions. Parents seem to be better off when they are able to use avoidance in contrast to those who tend to show emotional reactions. Further research is needed to substantiate these findings.

Highlights

Epidermolysis bullosa (EB) is a group of rare genetic skin disorders that primarily manifest as blisters and erosions following mild mechanical trauma
EB is categorized into four main types according to cleavage levels within the skin: EB simplex (EBS) with intra-epidermal blister formation; junctional EB (JEB), Mauritz et al Orphanet J Rare Dis (2021) 16:53 entailing skin cleavage at the level of the lamina lucida; dystrophic EB, in which there is blistering at the level of the sublamina densa; and Kindler syndrome, which may exhibit varying levels of blister formation [1]
Dystrophic epidermolysis bullosa (DEB) varies from a mild dominant form, entailing lesions confined to the hands, knees, and elbows to a severe recessive form characterized by generalized blistering, scar formation with pseudosyndactyly, esophageal strictures, and the development of aggressive squamous cell carcinoma with reduced life expectancy

Summary

Introduction

Epidermolysis bullosa (EB) is a group of rare genetic skin disorders that primarily manifest as blisters and erosions following mild mechanical trauma. Despite the crucial role of the parents of children with EB in managing the disease, studies focusing on the parent–child relationship remain a gap in the literature To address this gap, the current quantitative study, involving 55 children with all types of EB and 48 parents, assessed the relationship between their quality of life and coping strategies. DEB varies from a mild dominant form, entailing lesions confined to the hands, knees, and elbows to a severe recessive form characterized by generalized blistering, scar formation with pseudosyndactyly, esophageal strictures, and the development of aggressive squamous cell carcinoma with reduced life expectancy. There is currently no cure for EB, and treatment focuses on symptom relief and wound prevention [8]

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