Date Published: April 12, 2019
Publisher: Public Library of Science
Author(s): Ruth H. Keogh, Diana Bilton, Rebecca Cosgriff, Dominic Kavanagh, Oliver Rayner, Philip M. Sedgwick, Stefan Wölfl.
Cystic fibrosis (CF) is the one of the most common inherited diseases. It affects around 10,000 people in the UK, and the median survival age is 47. Recent developments making use of longitudinal patient registry data are producing more detailed and relevant information about predicted life expectancy in CF based on current age and clinical measurements. The objective of this study was toconduct an online survey of adults with CF living in the UK using a web-based questionnaire to investigate: (i) if and how they access information on life expectancy; (ii) what they use it for; (iii) if they want more personalised information on life expectancy or the time until other milestones. The survey was advertised through the Cystic Fibrosis Trust using social media. There were 85 respondents, covering men (39%) and women (61%) aged 16–65. 75% had received information on life expectancy either from their CF care team (34%) or other sources (71%), the most common being the Cystic Fibrosis Trust website and research literature. Most people who received information found it to be beneficial and reported using it in a variety of ways, including to plan strategies for maintaining as best health as possible and to psychologically manage current health status. 82% of respondents were interested in more personalised information about their life expectancy, and participants also noted interest in other outcomes, including time to needing transplant or reaching a low level of lung function. Themes arising in text responses included the importance of good communication of information, the difficulty of relating general information to one’s own circumstances, and a desire for increased information on factors that impact on survival in CF. As an outcome from this work, research is underway to establish how information on life expectancy can be presented to people with CF in an accessible way.
Life expectancy for people with cystic fibrosis (CF) has increased considerably over recent decades due to improved treatments and care [1,2,3]. The estimated median survival age for babies born today with CF in the UK is 47 . With increased life expectancy, it has become important to be able to predict prognosis. Numerous factors are associated with prognosis, including genotype, sex and clinical measurements such as pulmonary function. Several tools for prediction of survival in CF have been developed: for summaries see Buzetti et al , McCarthy et al , MacNeill . Data from national patient registries provide the potential to develop prediction tools using longitudinal information from large samples with long-term follow-up and such tools have been developed using CF patient registry data in the US , Canada  and France . Recent work using UK CF patient registry data has provided more detailed information on life expectancy for people with CF in the UK [11,12], including through a model providing ‘personalised’ predictions which take into account longitudinally collected clinical data available in the registry .
The aim of this survey was to investigate if and how adults with CF access information about life expectancy, how they use it, and whether they are interested in more personalised information. The following messages emerged: