Tentative hope: an exploration of the impact of Cystic Fibrosis transmembrane conductance regulator modulators on people with Cystic Fibrosis and their families.

Edwards, Hannah (2024) Tentative hope: an exploration of the impact of Cystic Fibrosis transmembrane conductance regulator modulators on people with Cystic Fibrosis and their families. Doctoral thesis, University of East Anglia.

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Abstract

Background. Cystic Fibrosis (CF) is a rare, life-limiting genetic condtion, which impacts the functioning of multiple organ systems (Bierlaagh et al., 2021). People with CF (pwCF) experience a range of difficulties, which affect their physical and psychological health (Ernst et al., 2010), as well as that of their families (Daly et al., 2022).

Cystic Fibrosis transmembrane conductance regulator (CFTR) modulator therapies are a new and highly anticipated treatment for CF (Bierlaagh et al., 2021). They have been found to significantly improve the physical health of pwCF and are hoped to improve life-expectancy (Balfour-Lynn & King, 2022). There is, however, little known about the psychological experience of starting these potentially lifechanging medications, for either pwCF or their families. Indeed, early research has intimated possible negative psychological experiences (Talwalkar et al., 2017). It is therefore important to understand the nature of the relationship between CFTR modulators and the psychological experiences of pwCF and their families.

Aims. This thesis aims to understand the impact of CFTR modulators on the psychological wellbeing of pwCF and to gain insight into the experiences of parents whose children have started CFTR modulators.

Methods. A systematic review was conducted on the impact of CFTR modulators on the psychological wellbeing of pwCF. Ten papers were identified and reviewed using narrative synthesis. Interpretative Phenomenological Analysis (IPA) was used to explore parental experiences.

Results. In the systematic review, CFTR modulators were observed to have a slight positive impact on the quality of life of pwCF, but not on levels of anxiety and depression. Several themes emerged in the qualitative paper, illustrating parents’ complex feelings towards the medication and the factors that influenced this including hope, uncertainty, and adjustment.

Conclusion. PwCF and their families have a complex psychological relationship to starting CFTR modulators influenced by nonphysical factors such as hope and uncertainty.

Item Type:	Thesis (Doctoral)
Faculty \ School:	Faculty of Medicine and Health Sciences > Norwich Medical School
Depositing User:	Nicola Veasy
Date Deposited:	07 Nov 2024 14:32
Last Modified:	07 Nov 2024 14:32
URI:	https://ueaeprints.uea.ac.uk/id/eprint/97568
DOI:

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