Exploring the lived experiences of individuals with cystic fibrosis after the implementation of elexacaftor/tezacaftor/ivacaftor for treatment.

BACKGROUND

CFTR modulators are a class of medications prescribed to people with cystic fibrosis (pwCF) who have certain genetic mutations. In 2019, elexacaftor/tezacaftor/ivacaftor (ETI) was approved by the FDA and led to significant health improvements for pwCF. Despite ETI showing significant health improvements compared to prior modulators, little is known about how ETI impacts the lived experience of pwCF.

OBJECTIVES

This study explored how the implementation of ETI in cystic fibrosis treatment has impacted the lived experience of pwCF and what additional resources healthcare providers should be offering to their patients.

DESIGN

We conducted a qualitative study that recruited pwCF from an adult CF center who were over the age of 18 and had been on ETI for at least a year.

METHODS

We conducted and recorded semi-structured interviews with participants over the Zoom platform. Dedoose was used to code the data and themes were constructed using inductive coding rooted in grounded theory.

RESULTS

Four themes were constructed from the data in this study: (1) "transformation in health" refers to the change in physical health the participants have noticed since starting ETI, (2) "future planning and opportunities" highlights new opportunities becoming available and a new desire to plan for the future, (3) "impact on relationships" describes how participants felt their relationships with their partner, family, and care teams, were impacted by ETI, (4)"new worries and struggles" describes new struggles and concerns that participants have faced.

CONCLUSION

The data from this study provide evidence that ETI has not only impacted pwCF on a physical level, but may also have an impact on relationships, mental health, and future planning. It also highlights the need for additional resources that CF care teams should be providing to their patients.