ECFS best practice guidelines: the 2018 revision

Developments in managing CF continue to drive dramatic improvements in survival. As newborn screening rolls-out across Europe, CF centres are increasingly caring for cohorts of patients who have minimal lung disease on diagnosis. With the introduction of mutation-specific therapies and the prospect of truly personalised medicine, patients have the potential to enjoy good quality of life in adulthood with ever-increasing life expectancy. The landmark Standards of Care published in 2005 set out what high quality CF care is and how it can be delivered throughout Europe. This underwent a fundamental re-write in 2014, resulting in three documents; center framework, quality management and best practice guidelines. This document is a revision of the latter, updating standards for best practice in key aspects of CF care, in the context of a fast-moving and dynamic field. In continuing to give a broad overview of the standards expected for newborn screening, diagnosis, preventative treatment of lung disease, nutrition, complications, transplant/end of life care and psychological support, this consensus on best practice is expected to prove useful to clinical teams both in countries where CF care is developing and those with established CF centres. The document is an ECFS product and endorsed by the CF Network in ERN LUNG and CF Europe

Positive and Negative Structures and Processes Underlying Academic Performance: A Chained Mediation Model

This study proposed and tested a comprehensive, chained mediation model of university students’ academic performance. The hypothesized model included adaptive-positive and maladaptive-negative submodels. The structures and processes in the adaptive-positive submodel were hypothesized to facilitate students’ academic performance, whereas the structures and processes in the maladaptive-negative submodel were hypothesized to undermine it. A sample of 373 university students completed a set of questionnaires measuring their approaches to studying, positive and negative affect, evaluation anxiety, use of creative cognition, motivational orientations, and adaptive and maladaptive metacognitions. Participants’ end-of-semester and prior semester academic performance was retrieved from the university registry. A structural equation model explained 90 % of the variance in students’ future academic performance, supported all but one hypothesized intermediate paths, and revealed that only positive affect in studying and prior academic performance predict directly future academic performance. The theoretical and practical implications of these findings are outlined

International well-being index : the Austrian version

The International Well-being Index (IWI) measures both personal and national well-being. It comprises two subscales: the Personal Well-being Index (PWI) and the National Well-being Index (NWI). The aim of this paper is to test the psychometric properties (validity and reliability) of the translated scale in Austria. Convergent validity is assessed using the Scales of Psychological Well-Being, the Satisfaction with Life Scale and the Positive and Negative Affect Scale. In addition, a Visual&ndash;Analog Scales capturing &ldquo;satisfaction with life as a whole&rdquo; was applied. The participants were 581 students of the Medical University Innsbruck (female: 47.7%; age: 23.2 &plusmn; 3.7). Internal consistency (Cronbach&rsquo;s &alpha;) of the IWI was for both scales &gt; .70 (PWI: .85; NWI: .83). The exploratory factor analysis of the IWI identified a 2-factor-structure identical with the two scales of the IWI explaining 54.2% of the variance. The convergent validity hypotheses were confirmed, construct validity was partly confirmed for the PWI being a deconstruction of a first factor called &ldquo;satisfaction with life&rdquo; (38.1% explained variance). Happy participants scored higher on the PWI (84.3 &plusmn; 7.9 vs. 68.7 &plusmn; 13.7; p &lt; .001) and NWI (64.3 &plusmn; 15.8 vs. 57.9 &plusmn; 15.1; p &lt; .001) scores than unhappy participants. It is concluded that the Austrian version of the IWI is a reliable and valid instrument to assess personal and national well-being. Further studies including a representative sample should be carried out on a recurring basis to use the IWI as an indicator for social science research in Austria.<br /

The experience of men and women with cystic fibrosis who have become a parent: a qualitative study

To explore the experiences of men and women with cystic fibrosis in becoming parents.As lifespan for people with cystic fibrosis increases, and reproductive technology advances, having a child of their own becomes a possibility.This study utilised a phenomenological framework.Seven Australian adults with cystic fibrosis were invited to describe their experiences of becoming parents in the context of a semi-structured interview. Analysis of the data involved highlighting recurrent phrases and isolating emergent themes.Two overarching themes characterised the participants' experience: Counting the cost, as they recalled Concentric communication and Pathways to pregnancy; and Living the dream, as they cast a retrospective view over this, their major achievement, in light of their Reaction: a dream comes true, Coping: a question of balance, Conjecture: the future redefined and Confidence: recalibrating.While advances in cystic fibrosis care and reproductive technology have increased the possibility of individuals with cystic fibrosis becoming parents, the passage to becoming a parent is a complex process.These findings can inform health professionals to support the adaptive work necessary for families that include members with cystic fibrosis. A contemporary understanding of this phenomenon is necessary for facilitating clinically relevant communication. This article is protected by copyright. All rights reserved