Challenges and proposed solutions for formative research to inform systematic intervention development in rare and unstudied conditions: The case example of Xeroderma Pigmentosum

Sainsbury, Kirby; Walburn, Jessica; Araujo‐Soares, Vera; Weinman, John

doi:10.1111/bjhp.12287

Cited by 11 publications

(24 citation statements)

References 13 publications

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“…The sample size limited the possibility to analyse photoprotection differences between countries. Given the challenges of recruiting participants in rare disease research (Kwakkenbos et al ., ; Sainsbury, Walburn, Araujo‐Soares, & Weinman, ), we had to extend the data collection period beyond the summer months (May to December 2016) and seasonal differences might have had an influence on some responses. Despite these limitations, this is the only survey that has collected internationally comparable quantitative data in people diagnosed with XP.…”

Section: Discussionmentioning

confidence: 99%

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

Walburn

Canfield

Norton

et al. 2019

British J Health Psychol

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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...

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Section: Discussionmentioning

confidence: 99%

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

Walburn

Canfield

Norton

et al. 2019

British J Health Psychol

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“…Recruitment of sample sizes required for full psychometric assessment with typical levels of precision are simply not feasible. Although a larger sample size is not possible, we have adapted our analytical approach to assess validity against a range of other tools, which provides confidence that the measure is valid and reliable.…”

Section: Discussionmentioning

confidence: 99%

“…However, there have only been a few attempts to validate these self‐report questionnaires of photoprotection against objective measures of UVR exposure . A key issue for the use of these questionnaires in populations that require rigorous photoprotection, such as XP, is that they are unlikely to be sensitive to the detection of small but important differences in photoprotection practices, particularly in clinically important areas such as the face . The only existing tool for use in XP patients is clinician‐reported and has not been psychometrically validated .…”

Section: Introductionmentioning

confidence: 99%

“…11,14,18,19 A key issue for the use of these questionnaires in populations that require rigorous photoprotection, such as XP, is that they are unlikely to be sensitive to the detection of small but important differences in photoprotection practices, particularly in clinically important areas such as the face. 20,21 The only existing tool for use in XP patients is clinician-reported and has not been psychometrically validated. 22 As such, there is a need to develop and validate a new self-report questionnaire for people with XP.…”

Section: Introductionmentioning

confidence: 99%

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

Canfield

Norton

Walburn

et al. 2019

Photoderm Photoimm Photomed

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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.

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“…ideally wearing a UVR protective face visor, trousers, long sleeved top, and gloves). Potentially due to the rare nature of XP, few studies have explored the use of photoprotection in this population, and little is known about rates of photoprotection adherence or the key drivers of adherence to photoprotection (Walburn et al, 2017).…”

Section: Introductionmentioning

confidence: 99%

Adherence to face photoprotection behaviours in in Xeroderma Pigmentosum (XP) patients: initial validation of a new measure

Canfield¹,

Norton²,

Walburn³

et al. 2018

Preprint

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Background: Few studies have examined photoprotection practices in individuals with rare genetic diseases, such as Xeroderma Pigmentosum (XP), where photoprotection is an important aspect of managing the condition. Currently no validated self-report tool exists for assessing adherence. Aims: We sought to validate a self-reported measure of adherence to face photoprotection behaviours in individuals diagnosed with XP. Methods: 66 XP patients recruited from the patient list of the XP specialist service in London, UK, completed a survey including self-reported adherence to specific photoprotection behaviours and wore a wrist-worn UVR electronic dosimeter for 21 days while also completing a daily UVR photoprotection diary. Internal reliability and convergent validity of the measure was explored in relation to self-reported UVR photoprotection practices, clinical variables, and objective measures of UVR protection. Results: Internal consistency of the self-reported scale was satisfactory. Correlations between the adherence to photoprotection measure with other photoprotection practices and clinical variables ranged from small to large in size. There was general agreement between the self-reported measure and diary and dosimeter measures as the mean scores for levels of adherence to photoprotection increased with average day protection. However, there were outliers specifically at the higher ends of average day protection and poor agreement between levels of adherence to the face photoprotection and the amount of UVR dose reaching the face. Conclusions: Findings showed that our measure may provide a standardised and internally reliable self-reported assessment of UVR protection. However, given the potential low sensitivity to detect high adherence, we encourage future studies to combine objective and subjective reports to increase the confidence in the accuracy of the measure of adherence to face photoprotection.

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Challenges and proposed solutions for formative research to inform systematic intervention development in rare and unstudied conditions: The case example of Xeroderma Pigmentosum

Cited by 11 publications

References 13 publications

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

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

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

Adherence to face photoprotection behaviours in in Xeroderma Pigmentosum (XP) patients: initial validation of a new measure

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