Living with primary ciliary dyskinesia: a prospective qualitative study of knowledge sharing, symptom concealment, embarrassment, mistrust, and stigma

Abstract

BackgroundPrimary ciliary dyskinesia (PCD) is a chronic respiratory disease for which there is little psycho-social research and no qualitative studies of individuals living with the condition. A questionnaire-based survey in 2003 found evidence of stigmatisation in some individuals with PCD. Although the questionnaire had face and construct validity, stigmatisation was not cross-validated against interviews. The present study had the twin aims of carrying out a qualitative study of the adult patients living with PCD, and using a structured design to validate the questionnaire measure of stigma.MethodsInterviews were carried out with six pairs of individuals with PCD, matched for sex, situs, and age, one with a high stigma score in 2003 and the other with a low stigma score. Depth-qualitative interviews were conducted by one author to explore themes surrounding the psycho-social impact of PCD using a grounded theory analysis. The interviewer was blind to the stigma scores of participants, and after the qualitative analysis was completed, the interviewer made an assessment of which member of each pair seemed the more stigmatised, after which the code was broken.ResultsInterviews revealed a number of themes, including other people's knowledge of PCD, the sharing of knowledge about PCD, the concealment of symptoms of PCD, embarrassment at symptoms, changes of behaviour in response to PCD, mistrust of medical care, in particular in relation to problems in diagnosis, a mistrust of general practitioners who were seen as poorly informed, and the importance of expert care at tertiary referral centres. Although stigmatisation as such was rarely mentioned directly by respondents, when the interviewer's judgement on level of stigmatisation was correlated with stigma scores from 2003, it was found that the more stigmatised member had been correctly identified in all six pairs (p = .016).ConclusionOur results suggest that some people with PCD feel isolated through mistrust in medicine, and lack of knowledge surrounding PCD. Many responses to PCD can be explained in terms of stigmatisation, and in particular felt stigma. The correlation between questionnaire used several years previously, and the interviewer's judgements of stigmatisation suggest that the stigma questionnaire had both predictive validity and long-term stability. As in other chronic conditions, stigmatisation occurs only in some individuals with PCD, and the present study explores the basis of stigmatisation, and validate the questionnaire as a measure of difference in stigma.

Health-related quality of life and unmet needs in patients with primary ciliary dyskinesia

“…This is expected to increase shared knowledge, decrease mistrust of general practitioner who is generally poorly informed of what is a rare disease, and possibly to facilitate early diagnosis [30]. There is also the need for support from peer groups and volunteers to diminish stigmatisation and to give practical help to patients with PCD and to their families.…”

The Implications of Stigma and Anonymity for Self-Disclosure in Health Blogs

“…However, the item used in this study addresses an important dimension of stigma that is particularly relevant to the context of blogging about health. Both theory (Goffman, 1963;Scambler & Hopkins, 1986) and the results of research (Gray, 2002;Snadden & Brown, 1991;Whalley & McManus, 2006) identify shame and embarrassment as being central to the experience of stigma. Moreover, items addressing shame and embarrassment are pervasive in measures of health-related stigma; at least 10 different measures of stigma specific to particular health conditions (e.g., HIV, epilepsy, etc.)…”

“…In his work on stigma, Goffman (1963, p. 7) notes that "shame becomes a central possibility, arising from the individual's perception of one of his [or her] own attributes as being a defiling thing to posses." Qualitative research also offers evidence that embarrassment is a central element of stigma among people coping with a range of health conditions such as primary ciliary dyskinesia (Whalley & McManus, 2006) and asthma (Snadden Downloaded by [UNAM Ciudad Universitaria] at 11:37 22 December 2014& Brown, 1991, as well as among parents of autistic children (Gray, 2002). Embarrassment and shame concerning one's self-with-illness is a particularly important dimension of stigma to consider.…”

Validation of pediatric health‐related quality of life instruments for primary ciliary dyskinesia (QOL‐PCD)

“…To date, few studies have reported the psychological and social burden of PCD . Qualitative studies have shown the emotional impact of PCD, with prominent themes arising including anger and frustration on account of the constant symptoms, treatment burden, and anxiety about their future health .…”

“…Qualitative studies have shown the emotional impact of PCD, with prominent themes arising including anger and frustration on account of the constant symptoms, treatment burden, and anxiety about their future health . Feelings of embarrassment were attributed to the need to cough, expectorate sputum, or blow nose in social settings . In the absence of disease‐specific questionnaires, researchers have used generic HRQoL measures to investigate the psycho‐social impact of PCD including the Wechsler intelligence scale for children, the child behavior check‐List questionnaire, St George's respiratory questionnaire, Leicester cough questionnaire, and medical outcomes study short form 36 .…”