Lung transplant perspectives in the era of elexacaftor/tezacaftor/ivacaftor (ETI): Navigating clinical uncertainty and ETI limbo among people with cystic fibrosis, a qualitative analysis.
Abstract
Background
Improved health among people with cystic fibrosis (PwCF) taking highly effective modulator therapies (HEMTs), particularly elexacaftor/tezacaftor/ivacaftor (ETI), has altered the trajectory toward lung transplant (LTx). PwCF now face greater uncertainty about clinical outcomes and LTx timing.
Objectives
To explore the experiences and perceptions of PwCF regarding LTx in the context of new clinical uncertainty introduced by HEMTs, particularly ETI.
Methods
This qualitative study was part of a larger trial evaluating an online educational tool ("Take on Transplant"). Participants were adults with CF, FEV1 <50% predicted. qualitative exit interviews were conducted, and data were analyzed using content analysis followed by thematic analysis, achieving data saturation.
Results
Among 40 participant interviews, 2 thematic domains emerged. "New Clinical Uncertainty" highlighted ETI's impact, often described as a "game changer" or "miracle," leading to improvements in FEV1, symptoms, and quality of life, delaying LTx discussions for most. Participants commonly engaged in "hedging" about LTx timing, expressing cautious optimism while acknowledging that sudden illness could alter their trajectories. Some experienced "ETI limbo" - being too healthy for LTx yet characterized by anxiety about LTx timing and dissatisfaction with their health and quality of life despite ETI. "Navigating Clinical Uncertainty" identified participants' reliance on FEV1 and other health markers in assessing LTx urgency, high trust in clinicians' knowledge, and appreciation for ongoing clinical conversations about LTx.
Conclusions
Clinicians must acknowledge evolving patient perspectives, particularly ETI limbo, and foster ongoing, transparent shared decision-making integrating patient values with clinical data, addressing prognostic uncertainty in this new era of care.