The psychosocial impact of CFTR modulator therapies: A qualitative meta-synthesis, and A qualitative exploration of the experiences of adults with Cystic Fibrosis unable to benefit from Triple Combination Therapy

Mwelwa, Mulongwe 2024. The psychosocial impact of CFTR modulator therapies: A qualitative meta-synthesis, and A qualitative exploration of the experiences of adults with Cystic Fibrosis unable to benefit from Triple Combination Therapy. ClinPsy Thesis, Cardiff University. Item availability restricted.

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Abstract

Cystic Fibrosis (CF) is a life limiting genetic condition characterised by the accumulation of mucus in various parts of the body, mainly impacting the lungs. Historically, treatment options for people with CF were focused solely on managing symptoms. However, the past decade has seen the development of Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) modulator therapies, which address the underlying cause of CF. Since 2012, four CFTR modulators have been introduced for use. The most recent, a triple combination therapy of Elexacaftor, Tezacaftor and Ivacaftor (ETI) released in 2019 in the USA and 2020 in the UK, targets the mutation present in most people with CF, making it beneficial for approximately 90% of the CF population. The current thesis consists of two separate papers. The systematic review aimed to integrate the existing qualitative studies on the psychosocial experiences of people with CF receiving any of the four approved CFTR modulator therapies. A systematic review protocol was developed to search for relevant qualitative studies across five databases. Following guidelines for conducting systematic reviews, nine studies were found that met the inclusion criteria and were quality assessed. Thematic synthesis was used to integrate the findings, resulting in five analytical themes and eight descriptive subthemes. The first theme ‘living a life less defined by CF’, highlighted how CFTR modulator therapies have transformed the lives of people with CF. These treatments have enabled them to envision a future without the previous limitations of CF, allowing for new opportunities due to better health and longer life expectancy. The second theme ‘double edged sword of CFTR modulator therapies’, captured both the positive and negative impact of these treatments, with some participants discontinuing modulator therapies due to side effects. The third theme, ‘complex emotional landscape’ explored the mixed emotions of feeling happy and relief for oneself while experiencing sadness for those without access to CFTR modulators. It also addressed feelings of grief, loss, survivors’ guilt, anxiety, and uncertainty about the long-term efficacy of modulator therapies. The fourth theme, ‘navigating changes in identity’, described the process of adapting to a new sense of self beyond being defined solely by CF. Some participants experienced a loss of identity previously defined by CF, while others embraced a ‘normal identity’ following improved health status. The final theme, ‘CF care in the CFTR modulator therapy era’, included the challenges and opportunities presented by these new treatments. Concerns about reproductive health, weight management, and CF-related lifestyle factors were highlighted, along with a call for increased psychological support. The second part of the thesis aimed to explore the experiences of adults with CF who cannot benefit from ETI due to genetic factors. Research on this topic is limited, as most emerging literature has focused on the experiences of those who can benefit from CFTR modulator therapies. Seven adults participated, with interviews lasting between 35 and 70 minutes, and were conducted through MS Teams. The methodological approach used was Interpretative Phenomenological Analysis (IPA) as it is well suited for exploring how participants make sense of their experiences. In IPA, the researcher interprets how participants make sense of their experiences, and so, the researcher kept a reflective journal and attempted to reduce the risk of any personal biases influencing data interpretation. Four main themes with six subthemes emerged from the analysis. The first theme, ‘feeling forgotten’, highlighted the challenges of being in a minority group and feeling overlooked or left behind. The second theme, ‘conflicted emotions’, explored the emotional impact of comparing one’s situation with those who can benefit from ETI, and whilst feeling happy for others who can benefit from ETI, they experienced disappointment for themselves. The third theme, ‘fragility of hope’, illustrated how many of the participants’ were initially hopeful before the release of ETI, then lost hope upon realising that they could not benefit from it. Despite this, some found a renewed sense of hope for future treatments, while others remained cautiously hopeful, concerned that their declining health might prevent them from benefitting from new drugs. The final theme, ‘remaining on the old CF trajectory’, detailed the ongoing challenges of living with CF, highlighting the significance of ETI and the coping strategies participants use to navigate these difficulties. Both the systematic review and the empirical study contribute to our understanding of the diverse experiences within the CF community in this new era of CFTR modulators. They highlight the positive impact of CFTR modulator therapies on the lives of many people with CF, while also drawing attention to the unique struggles of both those who can and cannot access these medications. Both the review and empirical study offer novel unique insights which have implications for clinical practice, service development and future research. Ultimately, this thesis highlights the need for a holistic approach to CF care that addresses both medical and psychosocial aspects, ensuring that all individuals with CF receive the support they need to navigate their unique journeys.

Item Type:	Thesis (DClinPsy)
Date Type:	Completion
Status:	Unpublished
Schools:	Psychology
Subjects:	B Philosophy. Psychology. Religion > BF Psychology
Date of First Compliant Deposit:	16 September 2024
Last Modified:	24 Sep 2024 09:05
URI:	https://orca.cardiff.ac.uk/id/eprint/172122

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