Psychological wellbeing post Elexacaftor-Tezacaftor-Ivacaftor (ETI) treatment in cystic fibrosis

Cystic fibrosis transmembrane conductance regulator (CFTR) modulator therapies have brought about significant physical health improvements however, numerous post-marketing observations have suggested a potential linkage between ETI therapy and deleterious mental health outcomes, proposing an important area of study. Fourteen adult participants (males: 6; age range: 22 - 45 years; average FEV1: 82.71%) who had experienced a change in psychological wellbeing post-modulator therapy were recruited via social media to participate in a semi-structured interview. Following a deterioration in mental health two participants were on a reduced dose of ETI therapy and three participants had stopped ETI therapy. Alongside the manifest benefits of ETI therapy there are several new challenges a small but significant minority within the Cystic Fibrosis (CF) population are experiencing. Participants discussed a debilitating deterioration in psychological wellbeing post-ETI therapy including new and/or worsening depression, anxiety, suicidality, hallucinations, impaired memory, and hormonal changes. Changes in mental health were attributed to the challenge of acclimatising to a change in identity by some people, while others considered changes to be direct side effects of ETI-therapy. Being able to provide safe and effective care for physical and psychological wellbeing is critical in CF care. Alongside the physical health benefits associated with ETI therapy, there may be serious psychological implications for a minority of individuals with CF which require proactive and effective psychological support.