Results from an online survey of adults with cystic fibrosis: Accessing and using life expectancy information

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.

囊性纤维化（Cystic fibrosis, CF）是最常见的遗传性疾病之一。英国约有1万人受其累及，中位生存年龄为47岁。近期有研究借助纵向患者登记数据（longitudinal patient registry data），基于患者当前年龄与临床检测指标，生成了更为详尽且贴合临床实际的CF预期寿命预测相关信息。本研究旨在通过网络问卷，对英国境内的成年CF患者开展线上调查，以探究以下三方面内容：（i）患者是否以及如何获取预期寿命相关信息；（ii）他们使用此类信息的具体用途；（iii）是否希望获得更多个性化的预期寿命信息，或是距离其他健康里程碑的时长。本调查通过囊性纤维化信托基金会（Cystic Fibrosis Trust）依托社交媒体进行宣传推广，共回收有效问卷85份，受访者涵盖39%的男性与61%的女性，年龄区间为16至65岁。75%的受访者曾从CF照护团队（占比34%）或其他渠道（占比71%）获取过预期寿命相关信息，其中最常见的获取渠道为囊性纤维化信托基金会官网与研究文献。多数获取过此类信息的受访者认为其具有实用价值，并表示会通过多种方式加以利用，包括制定维持最佳健康状态的策略，以及从心理层面调适自身健康状况。82%的受访者对更多个性化的预期寿命信息抱有兴趣，参与者同时表现出对其他结局指标的关注，例如需接受肺移植的时间或达到低肺功能水平的时长。文本回复中提炼出的核心主题包括：信息有效沟通的重要性、将通用信息与自身具体情况相结合的难度，以及希望获取更多影响CF患者生存的相关因素信息。作为本研究的一项产出成果，目前已有相关研究正在开展，旨在探索如何以易于理解的方式向CF患者呈现预期寿命相关信息。