Abstract
This study explored experiences of stigma in 25 adults with xeroderma pigmentosum. Analysis of semi-structured interviews revealed the changing nature of stigma over the lifetime. Bullying occurred in childhood, whereas adults were questioned about both their photoprotection and skin damage, often resulting in internalised feelings of stigma. Resilience and rejection of feelings of stigma increased with age and experiences of stigma differed across cultures. Findings indicate a need to develop social skills training to help people reject feelings of stigma. Future research should explore perspectives of families, friends and formal institutions and their potential to cause or reduce feelings of stigma.
Introduction
Xeroderma pigmentosum (XP) is a rare genetic skin condition in which DNA damage from ultraviolet radiation (UVR) in daylight cannot be effectively repaired leading to pigmentary changes as a result of UVR exposure. XP is a heterogeneous condition in terms of the precise gene mutations involved. In particular around half of people with XP exhibit severe sunburn reactions and the other half have normal sunburn reactions for their skin type (Sethi et al., 2013). There are increased risks of cancer, with an estimated 10,000-fold increase in the incidence of non-melanoma skin cancer and a 2000-fold increase in melanoma skin cancer before the age of 20 years, and approximately 24 per cent of people with XP develop neurodegeneration (Bradford et al., 2011).
People with XP are advised to minimise their exposure to UVR to reduce their chances of developing skin cancer, ocular problems and pigmentary changes. Personal photoprotective measures involve regularly applying sunscreen and covering up with long sleeves, trousers, gloves, glasses and hats. UVR protective face visors and film on windows are also recommended.
This strict photoprotection regimen and the visible changes associated with XP have the potential to result in stigmatisation, based on the notion of unacceptable difference of a person or group of people (Goffman, 1963). Health-related stigma can involve both felt and enacted stigma. Felt health–related stigma is an internalised sense of shame relating to the condition or the fear of negative responses by others. Enacted stigma is the experience of being discriminated against or treated differently by others (Jacoby, 1994; Scambler, 1989). Both can be equally damaging and felt stigma is likely to lead to concealment of the condition to avoid enacted stigma (Scambler, 2009).
A number of potential sources of stigma can be hypothesized for people with XP. Previous studies have identified experiences of stigma in other visible skin conditions, for example, people with psoriasis thought other people perceived them as dirty and unhygienic (Bewley et al., 2014). However, not all people with XP exhibit visible pigmentary changes. For these participants, the distinguishing photoprotection measures required may make their condition more visible and therefore could lead to stigmatisation. XP is a rare condition (around 2.3 per 1,000,000 live births in Western Europe; Kleijer et al., 2008) and so there is little knowledge about it in the general public. This could make disclosure difficult as it would require more explanation, and misunderstandings about the causes and severity could lead to further stigmatisation.
Health-related stigma can have a significant detrimental effect on individuals, both psychosocially and clinically. Stigma has been linked with reduced self-esteem in conditions such as chronic pain (Holloway et al., 2007). Stigma can also lead to rational non-adherence whereby people consider the costs of following stigmatising treatment regimens and choose not to adhere. Berger et al. (2011) found some people with chronic obstructive pulmonary disease (COPD) used their inhaler less in public because of fears of stigmatisation. Adherence to photoprotection measures is crucial in XP. For those who do not have neurodegeneration, a normal life expectancy is possible as long as photoprotection measures are used. Without any photoprotection, life expectancy is severely reduced.
To date, there is no published research examining stigma in XP nor any published qualitative study of adults with XP. The aim of this study is to explore the range of experiences of stigma in adults with XP and the strategies they use to reduce stigma and increase normalisation.
Method
Participants and recruitment
Participants were recruited through the National XP Specialist Service at St Thomas’ Hospital, London. Participants were considered eligible if they were aged 16 years or over, had a laboratory proven diagnosis of XP (reduced unscheduled DNA repair), and had adequate English to participate. Those who had neurodegeneration or had previously requested not to be approached for participation in research were excluded. Children and those with neurodegeneration were the focus of separate studies. A total of 38 clinic attenders were identified as satisfying eligibility criteria by a consultant dermatologist and contacted by the research nurse using letters, phone calls and talking to patients in clinic. Of these, six declined to participate (four males, two females) with reasons of lack of time and not wanting to think about XP and seven could not be contacted. In total, 25 interviews were therefore undertaken. Participants were not offered any incentives for taking part.
Procedure
Interviews took place in participants’ homes in a private room, with the exception of one phone interview for logistical reasons. Two researchers carried out the one-to-one interviews. Neither had XP but one has an occasional stutter that she disclosed. The interviewers were accompanied by the research nurse who obtained informed consent, but was not present for the interview.
Interviews were based on a topic guide designed for a larger study with the aim of exploring experiences of XP and adherence to photoprotection. Initial questions focussing on the participant’s personal story were used to build a rapport, followed by questions about psychosocial meanings of XP, photoprotection and perceptions of risk (Supplementary Table 1). The topic guide directed the conversation but allowed flexibility with interviews led by participants’ experiences and responses. Interviewers also prompted and probed as necessary to gain a clearer and more detailed understanding. Following the interview, field notes documented a description of the participant and reflections on the process and content of the interview. The study received a favourable ethical opinion from Camden and Kings Cross Research Ethics Committee 15/LO/1395.
Analysis
Interviews were audio-recorded with permission. They were transcribed verbatim, checked for accuracy and entered into NVivo 10 software. All transcripts were read by three team members. A coding framework was developed on the basis of team discussions and was added to and refined as new codes arose. Initially, duplicate coding of the transcript data was undertaken. Following this, one person (R.A.) completed the coding with the other two discussing and checking samples of coding on an ongoing basis. Analysis involved both retrieval of coded segments and rereading individual transcripts to identify patterns and relationships for individuals and to compare across cases.
Findings
Characteristics of participants
The 25 participants were aged 16–63 years (mean = 37.64 years, standard deviation (SD) = 5.53). In total, 17 were males and 8 females, reflecting the preponderance of males in the adult clinic caseload (26 males and 17 females). Nine were European (mainly white British), eight of Pakistani ethnicity and eight mainly of Middle Eastern, Indian or Bangladeshi background, reflecting the relatively high prevalence of XP in Pakistan and the Middle East (Fassihi, 2013).
Childhood experiences of stigma
Photoprotection began in childhood for 16 of the 25 participants. Ten had been formally diagnosed with XP as childrenand a further six were not diagnosed until adulthood but experienced severe sunburn requiring photoprotection from an early age. These children knew they needed to protect themselves from sunlight but not why this was the case for them and not for others. Rather than describing specific worries about XP, accounts of childhood indicated that a need for protection from UVR led to a general feeling of being different to their friends. Despite having close friendships, the things they did to protect from UVR had isolated them and also drew unwanted attention. Participant 6 who burned from the age of 3 years described his experience:
Everybody would go outside and the teacher would say you have to stay behind and put cream on. She used to put cream on me, the teacher did. That’s one thing that I remember. The kids would look at me like what’s going on there? (Participant 6, male, 20 years)
Bullying from other children at school was common. People were teased about their freckles and sunscreen, and one participant described having his hat stolen and kicked around. People also reported receiving comments and stares from strangers, often adults, which added to feelings of difference. Participant 12 described how he was affected by strangers’ negative reactions tothe UVR protective visor he wore as a child:
Sometimes I did feel like crying and stuff like that, but other times I just put my head down and walked on. (Participant 12, male, 21 years)
Impact of diagnosis
Childhood experiences of stigmatisation and feelings of difference became easier to cope with following increased age and being given an official XP diagnosis of XP. As participants understood their condition more clearly, they found it easier to handle questions and could explain why their skin was different. Participant 15 recalled,
I called it everything: allergy to the sun. Crazy names came out, like anything. Just something to call it, it really puts you in a group and makes it easier. When people ask you what’s that mean, you say go Google it. Couldn’t do that before, right? (Participant 15, male, 34 years)
While receiving the diagnosis did not remove all stigma, it made it easier to manage and helped participants feel less different within themselves by providing a sense of group identity. Participant 4, diagnosed at 31 years, was initially reluctant to get tested feeling he was already protecting effectively, but described how eventually visiting the XP clinic changed the way he saw himself:
I was just gobsmacked in a sense where, when I was at [the XP clinic], I’d seen other patients there as well and obviously they’ve got different skin to myself. I thought I was, myself, my sister, and my cousin were the only one probably that has it. I didn’t really think of anyone else in the world […] I didn’t feel different. (Participant 4, male, 36 years)
For the nine participants, who did not have signs of XP as children, issues of stigma did not arise until their diagnosis as adults. They were happy to have had a normal childhood without being separated from peers or experiencing bullying with the diagnosis bringing the potential for stigmatisation that they had not experienced before. Participant 9 explained,
The fact that I had to take care of myself so intensely made me feel like a sick person, which I hadn’t felt before. (Participant 9, male, 38 years)
However, not all participants diagnosed as adults felt this way with the five diagnosed over the age of 40 years being less impacted. This group was more confident in how they saw themselves and learning they had XP did not damage their self-concept. One participant who was diagnosed with XP in her forties described how she found it easier to adapt to photoprotection:
Because older, you’re kind of stuck in your ways all these times. As I said, I don’t feel, besides I just want to slow down a bit, I don’t think or feel differently. (Participant 25, female, 55 years)
Adulthood
Experiences of stigma
During adulthood, overt negative reactions were less frequent, but comments and questioning about skin damage and photoprotection continued reflecting the continuing requirement to engage in photoprotection which made the condition more visible and highlighted participants’ difference. The four participants who currently wore a visor spoke about people who take the mickey a bit and experiences of comments, pointing and staring. Remarks and mocking about photoprotection measures other than the visor were rare, but there were numerous accounts of participants being asked why they were wearing thick clothes in summer. Three participants (one Caucasian and two Asian males) were stopped by police, who questioned why they were wearing hoodies in summer.
In addition to distinctive photoprotection, increasingly visible skin damage from UVR exposure and surgery scars further distinguished participants. These concerns occurred across all ethnicities, but the negative social meanings and impact of skin damage were a particular issue for Asian participants. Freckles are rare in Asian skin and drew more attention. As adults, although non-Asian participants had concerns about visible skin damage, they rarely experienced enacted stigma. However, Asian participants described numerous experiences of direct comments and questioning about skin damage from strangers, friends and family, making them feel different from others in the Asian community. Participant 6, a young Pakistani man, avoided spending time with other Asian people because of this and described the negative interactions he had encountered:
All I would hear is ah, are your freckles ever going to go away? How the hell are you, a brown guy with freckles? They don’t understand that. They associate freckles with Caucasian people. They, I guess they try to make me feel like an outsider. (Participant 6, male, 20 years)
Three participants reported that people assumed they would want to get rid of their freckles and made suggestions of remedies to lighten their skin. This was frustrating because it implied blame, as if they would do something about it if they wanted to:
People used to say oh, there’s this cream. If you keep wearing it, they will fade. Your freckles would fade, and I used to get angry with that, thinking what’s wrong with me having freckles? I don’t want them to fade and how can freckles fade? […] I’m not just being weird and I’m not looking after myself; it’s because I have a condition. (Participant 2, female, 35 years)
Rejecting feelings of stigma
Despite these common experiences, 13 of the 25 participants were categorised as ‘resilient’ in the face of comments and questions. Although degrees of resilience may have varied, these were participants who may have experienced negative effects of stigma but had learned or were learning to reject this and saw this as the other person’s problem, not theirs. Within the interviews, what other people might think about their XP or photoprotection was therefore not as central to their descriptions of experiences of XP as it was for others. Some also felt stronger from overcoming the challenges of XP, and one participant described his uniqueness as a positive:
I don’t have a problem with XP. I have little or no stigma about it, because actually it’s interesting. I’m an interesting patient. It’s almost reversed, almost pride. (Participant 20, male, 55 years)
These participants compared themselves to others and saw that everyone is different and so could normalise XP. Some had little visible difference from XP making this normalisation easier, whereas for others support from family and friends helped them to adapt and make positive social comparisons. One participant described how a particularly severe operation helped her accept that she will always look different and that people have to just accept that.
Concerns about difference were less salient in the eight participants aged over 45 years. These participants were more confident and so were less likely to worry about what other people thought. Those who were diagnosed young had worried about what others might think or say in the past, but their perspective had changed, and they could now reject feelings of stigma. Participant 21 frequently spoke about feeling sorry for younger people with XP. He was asked whether he worried about what other people thought about him:
It’s a wrong question, because I’m 62. If you ask somebody, like 25, 20, it would be a different answer. It’s never bothered me. Maybe it has when I was young. (Participant 21, male, 62 years)
While adherence to photoprotection measures was not always high in these participants, they had decided on a level of protection they were comfortable with and so did not worry about other people’s reactions; whereas many younger participants were reluctant to act differently and draw attention to themselves. One 55-year old described how others’ opinions about his photoprotection did not concern him:
I don’t feel any anxiety about who I’m with or what I’m doing […] If I’m with somebody and they want to walk on the sunny side of the street I’ll say no, I don’t want to. I want to walk on that side. So I don’t think it affects my, I don’t think my behaviour is affected by other people. (Participant 20, male, 55 years)
There were also five younger participants who managed to reject feelings of stigma. They had dealt with this since childhood and over time changed the way they responded. Participant 5 was teased about her freckles throughout childhood but described how she stopped worrying about these comments:
I used to think oh, why did I have freckles? If I didn’t have them, it would’ve been better off because no one would’ve said anything to me. But as I grew up and I started to think so what, I’ve got freckles. Why should I listen to other people? They’re just making me feel bad about me. (Participant 5, female, 26 yrs)
However, others who were also bullied as children were less successful in rejecting feelings of stigma. In particular, five men aged 21–37 years reacted angrily to those who asked questions or made negative comments and sometimes confronted them. This indicated that they did still care about and were affected by what people thought or said. These participants were conflicted about how to respond and this often depended on their mood at the time. Participant 12 would quietly ignore looks and comments from others as a child, but as a young man he became angrier. He explained the problems of responding in this way:
I just look back, straight, whatever. Like what are you looking at or something. Or if you want to look, come on, let’s look then. […] In that moment it helps, but then you just think through it after, I’m being looked at differently. Sometimes that just brings out the side in yourself that you don’t really, it’s not really you. (Participant 12, male, 21 years)
Felt stigma
The 12 participants who did not successfully reject negative responses from others were more likely to internalise stigma and felt different even in the absence of comments from others. Comparisons with others often led to the feeling that they were not ‘normal’. This was not always a result of worries about what other people thought but rather a self-stigma in the belief that they were fundamentally different because of XP. Participant 7 described how XP could dominate his thoughts, and he would question why he could not be like everyone else:
Why have I got XP and then why am I suffering from this and why can’t I be normal, why can’t I go out. All of these questions, which are normal questions if you, I clearly mind anyway. Because it’s not normal to suffer from XP. (Participant 7, male, 27 years)
Accounts of feeling different in adulthood were common and related to both skin damage and photoprotection. A total of 10 participants described persistent unwelcome feelings of difference because of visible skin damage. Their main concern was that this damage would mark them out as different and affect the way people saw them. Participants described questioning themselves during social interactions and assuming that people were looking at their skin. When having surgery, the resulting scars meant having another thing to hide which was more worrying than the pain and discomfort. One participant was determined to keep her condition from her work colleagues to avoid being treated differently and so having facial surgery was particularly difficult for her:
My shoulder, they’ve done that; that was fine. They did my leg, that was fine. Not fine-fine, but in comparison to having it on my face because again, on your face people can see it. (Participant 24, female, 28 years)
For some, it was frustrating to see skin changes, but this did not have a significant effect on their confidence. However, for others, appearance dominated their self-concept and reduced their confidence. Participant 2 was bullied for her freckling as a child and described how the feeling of needing to blend in and not being good enough continued into adulthood. She explained how her visible difference skin damage affected how she saw herself:
I just want to be normal. I know I keep saying normal, and I am normal, but like I said when you see other women, and you just think why can’t I? You know, blemishes. Why am I so different? I look odd. I just look odd. (Participant 2, female, 35 years)
Participants also spoke about feeling different because of their photoprotection. They worried that they could not participate with others outside, and when they did they had to act weird by reapplying sunscreen or dodging the sun. This was a particular issue for younger participants who were more likely to be forming new relationships and avoiding time outside during daylight hours limited these opportunities. Participant 12 did not experience enacted stigma at university, but described how XP makes him feel different to other students:
Everyone’s always socialising and everything like that. So today, sunny days they’ll always be out on campus and everything and just relaxing in the sun. So obviously I can’t do that. So it’s just about fitting in. (Participant 12, male, 21 years)
Support and stigma-free spaces
Positive responses from those close to participants often reduced felt stigma for those who did not reject negative responses from others and created safe, stigma-free environments. Family and friends were described as providing emotional support but more importance was placed on their role in normalising XP by understanding and incorporating photoprotection into normal life. For example, participant 22 was highly adherent to photoprotection, planning all of his activities around this and also described how his friends would do the same, without making it feel like an imposition:
They didn’t even think a second thing about it. It’s like we don’t go out until it’s sunset. That’s it. Or do you want to come here? Things they are organising, like dinners for their birthdays. It’s like right, we’ll go out at 9:00 and then you can. It’s not like oh, then you can come. It’s like we’ll go at this time. They don’t need to say why or… I think that’s just good friends. (Participant 22, male, 26 years)
Another source of normalisation was formal institutions such as schools, workplaces and hospitals. For example, having window film installed at schools and workplaces meant UVR protection did not need to be worn inside. However, four participants described unsupportive workplaces. Some felt that they had missed out on employment opportunities because of their XP, and employers did not always act on requests for adaptations to reduce UVR exposure. Participant 18 was reluctant to ask for time off work for appointments relating to XP:
They’re just money-making strategies coming through and whatnot. They really don’t care. And even when you tell them you’ve got to drop to a hospital appointment, they’re like ah. (Participant 18, 31 years)
The XP clinic was an important formal resource providing both emotional support and practical solutions such as cards to help explain XP to others and tinted sunscreen that is less noticeable than standard sunscreen as it blends in more easily. Participant 2 described feeling like an outsider throughout her interview, but explained that she did not feel this way at the XP clinic:
There are other people, so you’re not, you are different, but you’re not so different. So it brings, it felt nice, and obviously the XP team, they obviously deal with XP so you don’t feel ashamed, different. You don’t feel that I can’t say this and I mustn’t say that. (Participant 2, female, 35 years)
Managing stigma and normalisation
Concealing visible difference
The participants described wanting to reduce visible skin damage and fears about future skin changes. The use of clothing, make-up or specialist camouflage to cover skin damage was described by seven participants and improved confidence in social interactions. However, four of these participants had internalised stigma and for them concealing their difference was a necessity. They would not go out without make-up or camouflage because of worries about their appearance and so these routines added to the everyday burden of XP.
As well as covering skin damage, all participants were keen to minimise the visible difference of photoprotection. This sometimes meant utilising practical adaptations like tinted sunscreen, but often led to reductions in protection. For some participants, who struggled to reject feelings of stigma, the use of this strategy was inconsistent due to uncertainty about how much protection to use. Photoprotection can be stigmatising, but it allows people with XP to participate with others without worrying about UVR exposure and can help prevent visible skin damage. There was therefore a conflict between avoiding visible damage and the associated stigma, avoiding attention from wearing photoprotection and increasing participation with others. Participant 22 who wore a visor which regularly received negative attention described conflicting concerns:
It’s just looking different. Then at the back of it, so knock-on effect, you’re worried about the skin changes it’ll make if you don’t wear all that. Then there’s particularly what women will think of you. (Participant 22, male, 26 years)
Managing disclosure
Differing approaches to disclosure of the XP diagnosis were found within the sample. Some described limiting stigma by avoiding disclosure wherever possible. Their close family knew and some told employers if they felt it was necessary, but they did not discuss it openly. They argued that they don’t really think it’s any of their business, and that people would not understand. Not telling others meant they would be treated the same as everyone else, but required effort to conceal their XP. Another smaller group was more open, but struggled to decide who to tell and how much information to give.
These approaches were mostly adopted by participants who had internalised stigma, although three participants who rejected feelings of stigma were also selective in their disclosure. They did not hide their XP, but brushed off questions with simplistic explanations of being ‘allergic’ or ‘sensitive’ to sun unless they felt close to the person.
There was a group of 12 other participants who were open about their XP. These participants saw openness as the best strategy to reduce potential stigma, as they believed that if people understood more about XP they would not judge the way they look or the steps they take to protect themselves. One participant explained that his friends knowing about his XP means they do not question his behaviour:
Because most of my friends know about my skin condition, they just brush it off like yeah, that’s fine. As long as you’re safe, we’re happy to chill out and let you put [suncream] on. (Participant 19, male, 18 years)
Some were proactive in telling people about XP to increase awareness, but mostly these participants did not give much thought to whether to disclose their XP. While they did not specifically tell people, they were happy to discuss it if asked. This was generally not only a successful approach which had the benefit of not drawing unnecessary attention to themselves but also avoided the burden of hiding their condition.
Discussion
This is the first study to explore experiences of stigma among people with XP and showed feelings of unacceptable difference to be widespread. Most studies of stigma focus on a single point in time, whereas this study identified changes in experiences of stigma from childhood, through diagnosis and adulthood, and compared responses to stigma across younger and older participants.
Participants’ accounts revealed that the nature of stigmatisation changed significantly over their lifetime. Childhood was the time of the most direct enacted stigma including accounts of bullying. In adulthood, it was mostly questioning rather than direct comments, and felt stigma relating to concerns about being different was more salient. Previous studies interviewing children with other visible conditions have also found these types of childhood-specific stigma issues (Bogart, 2015). In addition to this, our study also accessed pre-diagnosis accounts of stigma in childhood and explored how this changed following diagnosis. For those who had experienced adverse reactions to UVR and needed to photoprotect from childhood, a diagnosis of XP reduced stigma by giving an explanation for their difference and a group identity. Conversely, for those diagnosed under the age of 40 years who had not previously experienced adverse impacts of XP, diagnosis generally brought the potential for a stigma they had not previously experienced. Early diagnosis and support at this time are therefore key to both reducing skin cancers and also to providing formal assistance to young adults, families and schools to help them understand XP and create safe, stigma-free environments.
As adults, distinctive skin lesions and appearances associated with XP combined with the unique photoprotection measures required resulted in multiple experiences of stigma. While visible skin changes are the main source of stigma in most skin conditions, our findings showed that in XP, photoprotection measures were often the principle signs of difference to themselves and others. This often led to ‘rational’ non-adherence, consistent with previous research in chronic hand eczema where patients were embarrassed to use protective measures such as gloves in public (Mollerup et al., 2013). Compared to non-adherence in other skin conditions, the consequences can be much more severe in XP due to the high skin cancer risk. Additionally, non-adherence to photoprotection can conflict with other attempts to reduce stigma using photoprotection to increase UVR-safe participation with others and prevent further visible skin changes. The relationship between photoprotection adherence and stigma is therefore complex and varied within this heterogeneous group.
The qualitative approach of this study gave insights into current experiences of middle-aged and older people with XP and identified how increased age reduced stigma self-consciousness, adding further depth to previous quantitative findings of reductions in stigma with age in conditions such as HIV (Emlet et al., 2015). While participants of different ages in our sample rejected stigma, this was more common in those over 45 years old who also appeared more comfortable with their level of photoprotection and confident in their self-concept. These participants were more likely to engage in positive reappraisal of their situations, a coping strategy which has been found to support resilience and positive adjustment in people with visible differences (Egan et al., 2011).
In addition to age differences in experiences of stigma, this study revealed significant ethnic differences. Asian participants experienced more enacted stigma than non-Asians and were regularly made to feel like outsiders in their communities because of their freckling. Cultural factors that contribute to stigma were also found in a study interviewing South Asian women with vitiligo (Thompson et al., 2010). This study adds to this literature, and by exploring cultural differences within the wider context of stigma, also provides insights into experiences of stigma that are common across different cultures.
We interviewed over half of adults without neurodegeneration on the register of the only XP specialist centre in the United Kingdom, suggesting a strong basis for research and practice recommendations within the United Kingdom. The strength of the study was its flexible, qualitative approach which allowed cultural differences within the United Kingdom that emerged during interviews to be explored. However, there may be cross-cultural differences in experiences of stigma within the international XP population with practices of covering up with clothing and societal preferences for lighter skin tones affecting patients’ and the public’s perceptions of both visible skin changes and steps taken to protect from UVR. There is therefore a need for further international research to explore experiences of stigma across cultures.
A further limitation was that accounts of stigma in childhood depended on participants’ recollections which may not be entirely accurate. This approach had the benefit of accessing accounts of changing experiences of stigma across the lifetime; however, specifically interviewing children with XP about their current experiences would add valuable insights to this narrative.
Our findings suggest a need to develop interventions to address stigma in order to aid adjustment in XP. Aiming this at young adults may be particularly valuable as this appears to be a key time when people with XP begin to either internalise or reject stigma. Moss (2005) found that a high perceived severity of a disfiguring condition on appearance was a predictor of poor adjustment. He suggested that this may in part be due to a lack of social skills causing further negative reactions from others which are then internalised. Social skills training could therefore be beneficial to help people cope with enacted stigma, increase resilience and aid with disclosing their condition to others. Our findings support previous research which showed that whilst disclosing a medical condition can risk increased stigma, often it is a positive experience and openness can increase access to social support (Kent, 2000; Sayles et al., 2007). An online programme consisting of social skills training and exposure-based techniques reduced appearance concerns, anxiety and depression in people with visible differences (Bessell et al., 2012). Given the geographical spread of patients, these sorts of online resources would be appropriate for use in XP.
Participants’ accounts highlighted the potential for family, friends and formal institutions to cause feelings of stigma as well as their role in reducing stigma by normalising XP and the associated photoprotection. This suggests that these groups should also be the focus of interventions. Research aimed at gaining the perspective of potential sources of stigma and support could inform interventions and campaigns to educate people and reduce stigma, rather than putting the emphasis wholly on individuals to reject stigma.
While reducing instances of enacted stigma and helping people reject feelings of stigma should be priorities, it is likely that people with XP will still want to reduce the visibility of their photoprotection. Any efforts to increase adherence must therefore consider its impact on feelings of stigma. People with XP should also be assisted to make informed decisions which balance the level of risk of UVR exposure and the amount of visible photoprotection.
Footnotes
Acknowledgements
The authors are most grateful to the 25 participants who contributed to this study and to Lesley Foster who was instrumental in recruitment of participants and facilitated the interviews. We also thank the specialist XP team at St Thomas’ Hospital and particularly Tanya Henshaw and Sally Turner for helpful discussions. We are grateful to the PPI group for their ongoing involvement and to the programme leads, Robert Sarkany and John Weinman,
Declaration of conflicting interests
The author(s) declared no potential conflicts of interest with respect to the research, authorship and/or publication of this article.
Funding
The author(s) disclosed receipt of the following financial support for the research, authorship, and/or publication of this article: This article presents independent research funded by the National Institute for Health Research (NIHR) under its Programme Grants for Applied Research Scheme (RP-PG-1212-20009). The views expressed are those of the authors and not necessarily those of the NHS, the NIHR or the Department of Health.
References
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