A cross sectional, observational survey to assess levels and predictors of psychological wellbeing in adults with epidermolysis bullosa

Abstract

Epidermolysis bullosa (EB) describes a cluster of genetically determined skin disorders. Symptoms can be painful, disabling and disfiguring, yet there is little research on the psychological impact of the disease. The study aim was to measure psychological wellbeing in adults with EB; and to examine the association between psychological wellbeing and self efficacy, health locus of control and adjustment to appearance in an observational, cross sectional survey. Questionnaire packs comprising the General Health Questionnaire (GHQ-12), the General Self Efficacy Scale (GSE), the Multidimensional Health Locus of Control Scale (MHLOC), and the Derriford Appearance Scale (DAS-24), were sent to approximately 385 adults with EB. The data were analysed using SPSS. Eighty-seven participants responded. Scores on the GHQ-12 showed non-problematic psychological health in 36% of the sample; levels bordering on clinical disorder in 32.1% and severe psychological distress in 31.9%. No correlations were found between demographic factors (age and sex) or clinical factors (EB type and perceived severity) and psychological wellbeing. Scores on the GSE, the internal locus of control sub-scale of the MHLOC and the DAS-24 showed them to be statistically significant correlates of psychological wellbeing (P<0.001; P<0.018; and P<0.001 respectively). In a regression analysis, adjustment to appearance and self efficacy accounted for 24% of the variation in psychological wellbeing. Adults with EB might be at risk of experiencing poor psychological health. Interventions designed to enhance disease self management, self efficacy and improve body image are likely to be beneficial in this clinical group.

Psychosocial recommendations for the care of children and adults with epidermolysis bullosa and their family: evidence based guidelines

“…A negative body image can be related to poorer psychological well-being. Women and younger people with EB may struggle more than men in this respect [33]. For some the visibility of EB, such as on hands and face, can invite scrutiny from others [34, 37, 46] as can the invisibility of EB for others [37].…”

“…Although there were no intervention-based studies within the literature, the authors recommend that interventions are needed to enable people with EB to have a stronger belief in being able to gain control over aspects of their condition. This can be associated with well-being and improved body image, particularly in children with EB [33]. Having access to knowledge and resources about EB and inviting people with EB to take a collaborative rather than ‘being done to’ approach to their care, can help them to have a greater role in managing their EB.…”

“…We strongly recommend psychosocial support to improve well-beingTo promote self-efficacy and support around body image to aid psychological well-being • Having access to knowledge and resources about EB can help people have a greater role in managing their EB. This self-management can help improve well-being. • Improved self-efficacy and locus of control, as well as support around body-image could help to develop a more positive sense of well-being.For support during transition periods in life (school transitions, transition into adulthood) • Communication and education about EB to improve people’s understanding. • Support from families, EB healthcare professionals and DEBRA.Review of inherited &autoimmune blistering diseasesAdults ( n = 87) RDEB, DEB, EBSChildren 10–14 years old ( n = 11) EBS (autosomal recessive)Young male adults (aged 21–35 years) with RDEB ( n = 5) and EBS ( n = 2)Observational reportC2+1-2+2+44[32][33][34][35][36]iii. We strongly recommend gaining access to psychosocial support for the whole familyPeople diagnosed with EB should be referred for psychosocial support as early as possible in childhood or in adulthood, if the person with EB wishes• To support the family unit.Encourage supportive network for the family, for example: • Education about EB for others • Provide access to DEBRA (or other EB support groups)✓Children ( n = 11/82) EBS; JEB; DDEB; RDEBChildren ( n = 16) JEB Adult ( n = 1) RDEBC2-2-2-4[37][38][39] α iv.…”

Associations of religiosity and spiritual well‐being with appearance concerns after head and neck cancer surgery

“…In the present study, more women had higher levels of appearance concerns than men, as found in a previous study. 29 However, no significant associations between appearance concerns in individuals with other health conditions and sociodemographic variables were identified in previous studies, [28][29][30] suggesting that distress is more related to personal and social life histories.…”

“…26 Scores of appearance concerns measured using the DAS24 scale in the sample of the present study were lower than the average score found in patients with other visible sequelae that were not necessarily in the head and neck region. 19,27,28 It must be taken into account that self-perception of appearance is a subjective concept, which is also based on the perception of the reactions of others, in addition to self-analysis. 8 Thus, the results should consider cultural and social aspects inherent to each society.…”