An investigation of the predictors of photoprotection and UVR dose to the face in patients with XP: a protocol using observational mixed methods

British J Health Psychol

Canfield

Norton

et al. 2019

Self Cite

Objectives Xeroderma pigmentosum (XP) is an extremely rare genetic disorder (approximately 100 known cases in the United Kingdom), where DNA damage caused by ultraviolet radiation in daylight cannot be repaired. Adherence to photoprotection is essential to prevent skin cancer. We investigated psychological correlates of photoprotection in the XP population of Western Europe and the United States. Design Cross‐sectional survey of adults with XP and caregivers of patients <16 years and those with cognitive impairment in the United Kingdom, Germany, the United States, and France (n = 156). Methods Photoprotection activities to protect the face and body when outdoors; avoidance of going outside during daylight hours; intention; self‐efficacy; and social support were assessed using measures developed for this study. Participants answered questions about their illness representations of XP (BIPQ); beliefs about photoprotection (BMQ); automaticity (i.e., without conscious effort) (SRBAI); clinical and demographic characteristics. Ordinal logistic regressions determined factors associated with photoprotection. Results One third did not achieve optimal face photoprotection. After controlling for demographic and clinical factors, modifiable correlates of higher photoprotection included greater perceived control of XP, stronger beliefs in necessity and effectiveness of photoprotection, and higher intention. Avoidance of going outside was associated with greater photoprotection concerns, more serious illness consequences, and higher XP‐related distress. Greater automaticity and higher self‐efficacy were associated with better protection across all outcomes. Conclusions Approximately half of all known cases across three European countries participated. Identified modifiable predictors of photoprotection may be targeted by interventions to reduce the incidence of skin cancers in the immediate future, when a treatment breakthrough is unlikely. What is already known on this subject? Adherence to photoprotection in other populations at elevated risk from skin cancer is poor; however, the level in XP is unknown. Research across chronic conditions shows that adherence to treatment and lifestyle recommendations are influenced by illness perceptions, self‐efficacy, and treatment beliefs. Studies on photoprotection conducted with the general population have found that perceived risk, perceptions of ultraviolet radiation (UVR) protection, self‐efficacy for the behaviour, and automaticity (behaviours that are enacted with little conscious awareness) are related to better photoprotection. What does this study add? This is the first international survey to examine adherence and its correlates in people with XP (an under‐researched group at very high risk of fatal skin cancer). Adherence varies and at least one third have potential for improvement. Perceptions about XP, photoprotection beliefs, self‐efficacy, intention, and automaticity were associated with photoprotection of the face and body when outdoors. Negative emotional re...

Section: Methodsmentioning

confidence: 97%

Psychological correlates of adherence to photoprotection in a rare disease: International survey of people with Xeroderma Pigmentosum

British J Health Psychol

Canfield

Norton

et al. 2019

Self Cite

“…Sixty six patients diagnosed with XP (approximately 65% of total XP population in the UK) were recruited from the national XP specialist service at Guy's & St Thomas' NHS Foundation Trust. All were recruited as part of a larger mixed‐methods study of photoprotection in people with XP . Individuals of any age were eligible to participate if they had a confirmed diagnosis of XP.…”

Section: Methodsmentioning

confidence: 99%

“…For this study, only the 21 days matching the period where the UVR dosimeter was worn was used. Detailed information about the diary can be found elsewhere …”

Section: Methodsmentioning

confidence: 99%

“…This can be achieved by reducing time spent outside, particularly during the middle of the day when UVR levels are highest, and when outside by applying sunscreen and lip sunblock, wearing a UVR protective face visor and photoprotective clothing (eg, long trousers, long sleeves, broad brimmed hat and gloves). Prior to our recent programme of research, no studies have explored the use of photoprotection in this population, and little has been known about rates of adherence.…”

Section: Introductionmentioning

confidence: 99%

See 1 more Smart Citation

Facial photoprotection in xeroderma pigmentosum (XP) patients: Validation of a new self‐reported questionnaire of adherence

Canfield

Norton

Photoderm Photoimm Photomed

et al. 2019

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Background/Purpose Adherence to photoprotection is the only way to prevent skin cancers and eye disease in xeroderma pigmentosum (XP). No validated self‐report questionnaire exists for assessing adherence to photoprotection practices in individuals with XP. We sought to validate a self‐reported measure of adherence to face photoprotection in this population. Methods Sixty six XP patients recruited from the patient list of the XP specialist service in London, UK, completed a questionnaire of adherence to specific photoprotection behaviours. We measured objective ultraviolet radiation (UVR) exposure to the face continuously for 21 days with a wristworn UVR electronic dosimeter combined with a daily photoprotection diary. Reliability and convergent validity of the questionnaire were tested in relation to overall UVR exposure, UVR dose to the face, daily photoprotection activities, other self‐reported photoprotection practices and clinical ratings of patient's protection. Results Internal consistency of the questionnaire was satisfactory. Questionnaire total scores were concordant with objective UVR exposure and UVR dose to the face. However, not all participants who reported good/excellent face photoprotection on the questionnaire recorded high levels of photoprotection in the daily diary. Correlations between the questionnaire and other practices and the clinical rating ranged from small to large in size. There was no correlation between the level of face photoprotection and self‐reported avoidance of going outside. Conclusions Our questionnaire was reliable and had good convergent validity with other indicators of photoprotection. This questionnaire could assist clinicians to detect low levels of adherence, and the methodology used to develop validated questionnaires for other photosensitive conditions.

“…Psychological models employed to understand adherence, such as the Common-Sense Model of illness (Leventhal, Meyer, & Nerenz, 1980) and the Health Action Process Approach (Schwarzer, 2008), acknowledge the importance of social influence but the processes involved have not been examined. As part of a programme of mixed-methods research to improve adherence to photoprotection amongst people with Xeroderma Pigmentosum (XP; see Walburn et al, 2017), a very rare genetic condition, we identified gaps in the understanding of the influence of social processes on adherence. As part of a programme of mixed-methods research to improve adherence to photoprotection amongst people with Xeroderma Pigmentosum (XP; see Walburn et al, 2017), a very rare genetic condition, we identified gaps in the understanding of the influence of social processes on adherence.…”

Section: Statement Of Contributionmentioning

confidence: 99%

Forms, interactions, and responses to social support: A qualitative study of support and adherence to photoprotection amongst patients with Xeroderma Pigmentosum

British J Health Psychol

Anderson

Morgan

2019

Self Cite

Objectives Social support influences adherence to treatment in chronic illness, but there is uncertainty about its facilitators and constraints. This study explored the forms, processes, and responses associated with mobilization of informal support across three life contexts amongst patients with Xeroderma Pigmentosum (XP), a condition requiring rigorous photoprotection to reduce cancer risks. Design Qualitative interview study. Methods A total of 25 adults with XP participated in semi‐structured interviews conducted face to face. An inductive thematic analysis was applied using a framework approach. Results Practical support, involving both assistance with recommended photoprotection and adjusting daily activities to reduce exposure, was the key form of support provided by family and friends. However, responses to this support differed with two groups identified based on the relative priority given to photoprotection in daily life and processes of disclosure. For ‘positive responders’, support aligned with their own priorities to photoprotect, conveyed feelings of being cared‐for and was facilitated by talking openly. In contrast, for ‘negative responders’ support conflicted with their priority of living ‘normally’ and their limited disclosure hindered receipt of helpful support in personal, clinic, and work interactions. Fears of workplace stigma also reduced disclosure amongst participants open in other contexts. Conclusions Practical support conveyed psychosocial support with positive effects on adherence. This suggests the traditional separation into practical and emotional support is overly simplistic, with measures potentially missing important aspects. Interactional processes contribute to the effects of support, which can be addressed by targeting disclosure, stigma, and other barriers at individual and organizational levels. What is already known on this subject? Social support can be both a facilitator and a hindrance to treatment adherence. Practical support is identified as the most important form of support in the context of adherence. The processes of support underpinning its relationship to adherence are unclear. What does this study add? Variations in the provision and impacts of support are influenced by participants’ disclosure and attitudes to photoprotection, with two key groups comprising ‘positive responders’ and ‘negative responders’. The influence of emotional support on adherence may be underestimated through neglect of the ways in which practical support often conveys feelings of being valued and cared‐for. Barriers to mobilizing effective adherence support extends across life spheres, with fears of stigma and discrimination in work settings highlighting the need to intervene at individual and organizational levels.