(248) Self-reported adherence with concomitant medications by adults with cystic fibrosis after starting elexacaftor/tezacaftor/ivacaftor therapy

Background: Adherence to medications plays an important role in maintaining lung health in individuals with CF and is important for long term morbidity and mortality. Recommended treatment regimens include many medications across different classes and routes which can be complicated and time consuming for adults with CF. [1] Adherence to highly effective cystic fibrosis transmembrane conductance regulator (CFTR) modulators is generally high, but adherence may be lower for other treatments such as inhaled antibiotics, dornase alfa, hypertonic saline, pancreatic enzyme replacement therapy, airway clearance techniques, and vitamins. [2] Highly effective CFTR modulator medications, such as elexacaftor/tezacaftor/ivacaftor (ETI), have dramatically improved respiratory health as well as the perception of quality of life (QOL) for many people. With this improvement of QOL, the concern is that individuals with CF will feel so well that they decide to stop taking medications that still may be necessary for maintaining their health.

Methods: An IRB-approved Redcap survey was developed and emailed to adults with CF followed at the Leroy W. Matthews CF Center. Inclusion criteria included individuals age 18 years or older who reported that they were currently taking ETI. Non-English speaking and pediatric individuals were excluded. Results of the survey were anonymous.

Results: Surveys were emailed to 185 individuals. Thirty-one survey responses were received, and 7 individuals were excluded due to responding that they were not currently taking ETI. There were 11 (46%) individuals who reported complete adherence to their medications, and 13 (54%) individuals who reported having self-decreased or self-discontinued at least one medication. Individuals reported choosing to stop medications (n=6) or decrease frequency of medications (n=10) on their own without discussing with their CF physician first. The medications most frequently reported as being self-adjusted were inhaled antibiotics (decreased n=4, stopped n=4) and mucolytics (decreased n=6, stopped n=2), followed by bronchodilators (decreased n=3), ibuprofen (stopped n=1), pancreatic enzymes (decreased n=1), and montelukast (decreased n=1).

Conclusions: Our survey showed that many individuals decided to stop medications on their own after starting ETI. It is important to encourage honest conversations to ensure that providers are able to get a true picture of overall medication adherence. More research is needed to determine the impact on short and long term health outcomes in the setting of improved QOL and self-discontinuation of concomitant medications while taking highly effective CFTR modulator therapies.

Acknowledgements:

References: 1. Mogayzel PJ Jr, Naureckas ET, Robinson KA, et al. Pulmonary Clinical Practice Guidelines Committee. Cystic fibrosis pulmonary guidelines. Chronic medications for maintenance of lung health. Am J Respir Crit Care Med. 2013 Apr 1;187(7):680-9. doi: 10.1164/rccm.201207-1160oe.

2. Narayanan S, Mainz JG, Gala S, Tabori H, Grossoehme D. Adherence to therapies in cystic fibrosis: a targeted literature review. Expert Rev Respir Med. 2017 Feb;11(2):129-145. doi: 10.1080/17476348.2017.1280399.