Cognitive Function in Cystic Fibrosis and CFTR Modulator Therapy

Author Info

Judy Allen-Graham Elyssa Williams Catherine Rang Brenda Button Deirdre Edgeworth Felicity Finlayson Toby Winton-Brown Tom Kotsimbos Dominic Keating John Wilson

Corresponding Author

Judy Allen-Graham
Cystic Fibrosis Service, Alfred Health, Melbourne, Australia

A B S T R A C T

Background: Cystic fibrosis is not typically associated with cognitive dysfunction that is easily discernible. Whether having a CFTR mutation has a direct effect on the CNS function is yet to be elucidated, despite widespread expression of the CFTR protein throughout the human nervous system. Methods: We aimed to study the effects of CFTR modulators ivacaftor and lumacaftor/ivacaftor on cognition in two separate CF cohorts. These were ivacaftor, in CF patients with at least one copy of the G551D mutation, and lumacaftor/ivacaftor in homozygous F508del subjects. Using a panel of cognitive testing tools (MOCA, TMT, Cogstate™) targeting various domains that included executive function, memory and attention. Results: The two cohorts improved significantly on CFTR modulator treatment when measured by the MOCA, TMT and by a combined cognitive score. Most prominently, these represent improvements in executive function. Conclusion: Suggested CNS effect of CFTR mutation in CF and the impact of CFTR modulators on this.

Article Info

Article Type

Research Article

Publication history

Received: Mon 06, Jun 2022
Accepted: Mon 20, Jun 2022
Published: Mon 11, Jul 2022

Copyright

© 2023 Judy Allen-Graham. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited. Hosting by Science Repository.
DOI: 10.31487/j.PDR.2022.01.01