Dataset Information

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

ABSTRACT: 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. Statement of contribution 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 representations of XP were associated with avoidance of going outside during daylight hours.

SUBMITTER: Walburn J

PROVIDER: S-EPMC6772157 | biostudies-other | 2019 Sep

REPOSITORIES: biostudies-other

ACCESS DATA

Publications

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

Walburn Jessica J Canfield Martha M Norton Sam S Sainsbury Kirby K Araújo-Soares Vera V Foster Lesley L Berneburg Mark M Sarasin Alain A Morrison-Bowen Natalie N Sniehotta Falko F FF Sarkany Robert R Weinman John J

British journal of health psychology 20190610 3

<h4>Objectives</h4>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.<h4>Design</h4>Cross-sectional survey of adults with XP and caregivers of patients <16 years ...[more]

PMID: 31183946

Similar Datasets

Project description: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. Statement of contribution 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.

Project description:IntroductionPoor adherence to photoprotection for people with xeroderma pigmentosum (XP) can be life-threatening. A randomised controlled trial (RCT) is being conducted to test the efficacy of a personalised adherence intervention (XPAND) to reduce the level of ultraviolet radiation (UVR) reaching the face, by improving photoprotection activities in adults with XP.Methods and analysisA two-armed parallel groups RCT, where we randomised 24 patients with suboptimal adherence to either an intervention group who received XPAND in 2018 or a delayed intervention group who will receive XPAND in 2019. XPAND involves seven sessions, one-to-one with a facilitator, using behaviour change techniques and specially designed materials to target barriers to photoprotection. Following baseline assessment in April 2018 (t0) and intervention, the primary outcome will be measured across 21 consecutive days in June and July 2018 (t1). The primary outcome is the average daily UVR dose to the face (D-to-F), calculated by combining objective UVR exposure at the wrist (measured by a dosimeter) with face photoprotection activities recorded on a daily UVR protection diary. Secondary outcomes include average daily UVR D-to-F across 21 days in August (t2); psychosocial process variables measured by daily questions (t0, t1, t2) and self-report questionnaires (t0, t1, t2, December 2018 (t3)). Intervention cost-utility is assessed by service use and personal cost questionnaires (t0, t3). The delayed intervention control arm participants will complete three further assessments in April 2019 (t4) and June-July 2019 (t5), and December 2019 (t6) with dosimetry and UVR protection diary completed for 21 days at t4 and t5. A process evaluation will be conducted using mixed methods.Ethics and disseminationEthical approval has been received from West London & GTAC REC 17/LO/2110. Results will be disseminated in peer-reviewed journals and at conferences. This study tests a novel intervention, which, if successful, will be integrated into routine care.Trial registration numberNCT03445052; Pre-results.

Project description:Background: Intervention Mapping (IM) is a systematic approach for developing theory-based interventions across a variety of contexts and settings. This paper describes the development of a complex intervention designed to reduce the dose of ultraviolet radiation (UVR) reaching the face of adults with Xeroderma Pigmentosum (XP), by improving photoprotection. XP is a genetic condition that without extreme UVR photoprotection, leads to high risk of developing skin cancer. Methods: The IM protocol of 6 steps was applied, involving comprehensive mixed-methods formative research. Key stakeholders (XP clinical staff and Patient and Public Involvement Panel), were instrumental at every step. Behaviour change methods were informed by the IM taxonomy, therapeutic approaches (e.g. ACT, CBT) and coded according to the taxonomy of behaviour change techniques (version 1). Results: We designed a personalised modular intervention to target psychosocial determinants of photoprotective activities that influence the amount of UVR reaching the face. Content was developed to target determinants of motivation to protect and factors preventing the enactment of behaviours. Participants received personalised content addressing determinants/barriers most relevant to them, as well as core 'behaviour-change' material, considered important for all (e.g. SMART goals). Core and personalised content was delivered via 7 one-to-one sessions with a trained facilitator using a manual and purpose designed materials: Magazine; text messages; sunscreen application video; goal-setting tools (e.g. UVR dial and face protection guide); activity sheets. Novel features included use of ACT-based values to enhance intrinsic motivation, targeting of emotional barriers to photoprotection, addressing appearance concerns and facilitating habit formation. Conclusion: IM was an effective approach for complex intervention design. The structure (e.g. use of matrices) tethered the intervention tightly to theory and evidence-based approaches. The significant amount of time required needs to be considered and may hinder translation of IM into clinical and non-academic settings.

Project description:BackgroundA high level of photoprotection is required by people with xeroderma pigmentosum (XP), a rare skin disease, to reduce skin cancer and other risks. However poor photoprotection is thought to be widespread.PurposeThis study examines the influences on photoprotection behaviours in adults with XP.DesignInductive qualitative study with semistructured interviews. Analysis employed a framework approach.SettingNational sample recruited through a specialist XP centre in London.MethodsSemistructured interviews at patients' homes. All transcripts were coded and themes charted for each participant. Comparisons within and across cases identified common themes and differing motivations and approaches to photoprotection. Credibility of interpretations assessed through patient/carer input and clinic adherence scores.Participants25 adults (17 male, eight female) aged 16-63 years with diagnosed XP attending a specialist centre. 18 lived outside London.ResultsAwareness of risks of ultraviolet radiation (UVR) and photoprotection was high. However, photoprotection behaviours varied according to perceived necessity and concerns. Three behavioural responses were identified: (1) 'dominated' by planning and routines to achieve a high level of photoprotection with significant activity restrictions and psychosocial impacts. (2) 'resistant' to photoprotection with priority given to avoiding an illness identity and enjoying a normal life. (3) Photoprotection' integrated' with an individual's life with little psychosocial impact. These responses were influenced by illness, personal and contextual factors including age, life stage and social support. Only the 'integrated' group achieved an equilibrium between perceived 'necessity' and 'concerns'.ConclusionsThe personal balance between perceived risks of UVR and social/psychological 'concerns' led to differing behavioural responses and contributes to an understanding of adaptation and normalisation in chronic illness. The study will also inform a series of individualised behavioural interventions to reduce measured UVR exposure among people with XP that are potentially applicable to other conditions with high risks of skin cancer.

Project description:OBJECTIVE:To assess the age at menarche and menopause of women with xeroderma pigmentosum, a DNA repair disease with premature aging, in a longitudinal natural history study. METHODS:We conducted a natural history study that reviewed medical records for gynecologic and reproductive health of all female patients with xeroderma pigmentosum aged older than 9 years examined at the National Institutes of Health (NIH). We performed gynecologic and laboratory examinations on a subset of the patients. Women in a second subset, who could not be examined, were interviewed using a questionnaire. Women who were deceased or lost to follow-up formed a third subset. RESULTS:Sixty females with xeroderma pigmentosum aged older than 9 years (median 29 years, range 10-61 years) were evaluated at the NIH from 1971 to 2018. Of these 60, 31 had history, questionnaire, record review, and gynecologic evaluation; 14 had record review and questionnaire interview by telephone; and 15 had only NIH record review. Menarche in females with xeroderma pigmentosum occurred at a median age of 12.0 years (range 9-17 years), which was comparable with the U.S. general population. Among the 18 patients with menopause, the median age at menopause of 29.5 years (range 18-49.5 years) was more than 20 years younger than in the U.S. general population (52.9 years). Premature menopause (before age 40 years) occurred in 14 of the 45 (31%) women aged 18 years or older, and primary ovarian insufficiency was documented in nine of them. There were 32 live births among 21 of the women, five of whom subsequently developed premature menopause. CONCLUSION:Females with xeroderma pigmentosum in our study had a normal age at menarche and were fertile but had increased incidence of premature menopause. Premature menopause, a symptom of premature aging, should be considered for gynecologic and reproductive health as well as implicating DNA repair in maintaining normal ovarian function. CLINICAL TRIAL REGISTRATION:ClinicalTrials.gov, NCT00001813.

Dataset Information

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

Publications

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

Similar Datasets

OmicsDI is part of the ELIXIR infrastructure

Tweets