The RISE study protocol: resilience impacted by positive stressful events for people with cystic fibrosis

Els van der Heijden, Rutger M van den Bor, Cornelis K van der Ent, Sanne L Nijhof, Sabine E I van der Laan*

*Corresponding author for this work

Research output: Contribution to journalArticleAcademicpeer-review


INTRODUCTION: For people with cystic fibrosis (CF), gaining access to elexacaftor/tezacaftor/ivacaftor (ETI) therapy, a new modulator drug combination, is perceived as a positive life event. ETI leads to a strong improvement of disease symptoms. However, some people with CF experience a deterioration in mental wellbeing after starting ETI therapy. The primary objective of this study is to investigate if and in which direction mental wellbeing of people with CF changes after starting ETI therapy. Our secondary objectives include, among others, investigation of underlying biological and psychosocial factors associated with a change in mental wellbeing of people with CF after starting ETI therapy.

METHODS AND ANALYSIS: The Resilience lmpacted by Positive Stressful Events (RISE) study is a single-arm, observational, prospective longitudinal cohort. It has a timeframe of 60 weeks: 12 weeks before, 12 weeks after, 24 weeks after and 48 weeks after the start of ETI therapy. The primary outcome is mental well-being, measured at each of these four time points. Patients aged ≥12 years at the University Medical Center Utrecht qualifying for ETI therapy based on their CF mutation are eligible. Data will be analysed using a covariance pattern model with a general variance covariance matrix.

ETHICS: The RISE study was classified by the institutional review board as exempt from the Medical Research Involving Human Subjects Act. Informed consent was obtained by both the children (12-16 years) and their caregivers, or only provided by the participants themselves when aged ≥16 years.

Original languageEnglish
Article number00535-2022
Number of pages11
JournalERJ Open Research
Issue number3
Publication statusPublished - 1 May 2023


Dive into the research topics of 'The RISE study protocol: resilience impacted by positive stressful events for people with cystic fibrosis'. Together they form a unique fingerprint.

Cite this