Young people with cystic fibrosis (CF) are asked to avoid a number of environments associated with increased infection risk, but in practice they need to balance this with competing priorities such as building and sustaining relationships with friends and family. This study explored the process by which young people make these decisions. Mixed methods were used: a vignette study presenting choices around engaging in activities involving a degree of infection risk and a thematic analysis of participant's accounts of their decision making. The eight participants chose to engage in high risk behaviours in 59% of the choices. All participants chose to engage in at least one risky behavior, though this was less likely when the risk was significant. Thematic analysis revealed large areas of misunderstanding and lack of knowledge, leading to some potentially worrying misconceptions about the nature of infections and risk. Young people with CF are not currently making informed decisions around activities that involve increased risk of infection, and there is an urgent need for CF teams to address this in information provision. 1. Introduction Cystic fibrosis (CF) is the most common transmitted genetic disease affecting Caucasians. It is characterised by a defect in the cystic fibrosis transmembrane conductance regulator (CFTR) gene which controls chloride transport in cells. This leads to a buildup of thick and sticky mucous, causing numerous complications, particularly in the respiratory and digestive systems. It also significantly increases susceptibility to respiratory infection, with subsequent lung damage and eventual mortality. CF is life limiting and previously considered a paediatric condition, but advances in treatment have witnessed a gradual increase in life expectancy [1] and a corresponding focus on the impact of the condition on psychosocial functioning in young adulthood. The treatment burden in CF is extremely intrusive, and adherence to CF medication in young people is a topic of understandable interest [2]. Less well studied is adherence to guidelines on reducing risk of infection. Significant risks are posed by infections such as Burkholderia cepacia complex (Bcc) and Pseudomonas aeruginosa (PsA) [3, 4], some strains of which are highly contagious, and for people with CF known to cause exacerbations in respiratory symptoms and increased morbidity. Efforts to reduce infection rates include complete segregation of all patients with CF in clinic to reduce cross infection. Particular infections are associated with particular environments. Known
References
[1]
J. A. Dodge, P. A. Lewis, M. Stanton, and J. Wilsher, “Cystic fibrosis mortality and survival in the UK: 1947–2003,” European Respiratory Journal, vol. 29, no. 3, pp. 522–526, 2007.
[2]
R. S. Bernard and L. L. Cohen, “Increasing adherence to cystic fibrosis treatment: a systematic review of behavioural techniques,” Pediatric Pulmonology, vol. 37, no. 1, pp. 8–16, 2004.
[3]
B. Frederiksen, S. Lanng, C. Koch, and N. Hoiby, “Improved survival in the Danish centre-treated cystic fibrosis patient: results of aggressive treatment,” Pediatric Pulmonology, vol. 21, pp. 153–158, 1996.
[4]
A. Pamukcu, A. Bush, and R. Buchdahl, “Effects of Pseudomonas aeruginosa colonization on lung function and anthropometric variables in children with cystic fibrosis,” Pediatric Pulmonology, vol. 19, no. 1, pp. 10–15, 1995.
[5]
Cystic Fibrosis Trust, Pseudomonas Aeruginosa Infection in People with Cystic Fibrosis, Suggestions for Prevention and Infection Control, Cystic Fibrosis Trust, 2nd edition, 2004.
[6]
L. Saiman and J. Siegel, “Infection control recommendations for patients with cystic fibrosis: microbiology, important pathogens, and infection control practices to prevent patient-to-patient transmission,” Infection Control and Hospital Epidemiology, vol. 24, no. 5, pp. S6–S52, 2003.
[7]
Cystic Fibrosis Trust, The Burkholderia Cepacia Complex, Suggestions for Prevention and Infection Control, Cystic Fibrosis Trust, 2nd edition, 2004.
[8]
Cystic Fibrosis Trust, Methicillin-Resistant Staphylococcus aureus (MRSA), Report of the UK Cystic Fibrosis Trust Infection Control Working Group, 2008.
[9]
L. Wray-Lake, A. C. Crouter, and S. M. McHale, “Developmental patterns in decision-making autonomy across middle childhood and adolescence: European American parents' perspectives,” Child Development, vol. 81, no. 2, pp. 636–651, 2010.
[10]
L. M. Bachmann, A. Mühleisen, A. Bock, G. ter Riet, U. Held, and A. G. H. Kessels, “Vignette studies of medical choice and judgement to study caregivers' medical decision behaviour: systematic review,” BMC Medical Research Methodology, vol. 8, article 50, 2008.
[11]
M. Ryan, “Discrete choice experiments in health care. NICE should consider using them for patient centred evaluations of technologies. Editorial,” British Medical Journal, vol. 328, pp. 360–361, 2004.
[12]
K. Gilhooly and C. Green, “Protocol analysis: theoretical background,” in Handbook of Qualitative Research Methods for Psychology and the Social Sciences, J. T. E. Richardson, Ed., The British Psychological Society, Leicester, UK, 1996.
[13]
V. Braun and V. Clarke, “Using thematic analysis in psychology,” Qualitative Research in Psychology, vol. 3, no. 2, pp. 77–101, 2006.
[14]
M. H. Becker, The Health Belief Model and Personal Health Behaviour, vol. 2 of Health Education Monographs, 1974.
[15]
I. Ajzen, “The theory of planned behavior,” Organizational Behavior and Human Decision Processes, vol. 50, no. 2, pp. 179–211, 1991.
[16]
F. A. McEwan, M. E. Hodson, and N. J. Simmonds, “The prevalence of “risky behaviour” in adults with cystic fibrosis,” Journal of Cystic Fibrosis, vol. 11, no. 1, pp. 56–58, 2011.
