Highlights
- •Being deemed not eligible for ETI shapes PwCF experiences and future expectations.
- •Disappointment was reported by all participants and appears to play a pivotal role in their experience.
- •Participants disclose mixed emotions: concerns for their health and happiness to see other people thrive.
- •Psychosocial support for PwCF not eligible for ETI should be a central aspect of care.
Abstract
Background
Elexacaftor-tezacaftor-ivacaftor (ETI) represents a significant step forward in cystic
fibrosis (CF) care and could change the course of CF lung disease and quality of life
for many people with CF (PwCF). However, several PwCF cannot benefit from these modulators
because their rare mutations are not eligible for treatment. This study aimed to investigate
the lived experiences of PwCF who are not eligible for ETI.
Methods
Data were collected through semi-structured interviews with 13 individuals with CF
that were not eligible for ETI. Thematic analysis was used to identify the key themes
of their experiences.
Results
Two main themes and six subthemes were identified. The first main theme (being deemed
ineligible for ETI) had four subthemes (disappointment, information, happiness, and
concerns). The second main theme (coping with a life without ETI) had two subthemes
(lack of hope and continued hope).
Conclusions
PwCF who are not eligible for ETI experience intense disappointment and conflicting
emotions that can influence their decision-making linked to diminishing/renewal hope.
Integrated care, including mental health monitoring programs, should be provided to
these patients to aid them in overcoming their disappointment and to improve their
coping.
Keywords
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Article info
Publication history
Published online: December 20, 2022
Accepted:
December 16,
2022
Received in revised form:
December 15,
2022
Received:
August 22,
2022
Publication stage
In Press Corrected ProofIdentification
Copyright
© 2022 European Cystic Fibrosis Society. Published by Elsevier B.V. All rights reserved.
