Judy  Allen-Graham,Elyssa  Williams,Catherine  Rang,Brenda  Button,Deirdre  Edgeworth,Felicity  Finlayson,Toby  Winton-Brown,Tom  Kotsimbos,Dominic  Keating,John  Wilson,
Cognitive Function in Cystic Fibrosis and CFTR Modulator Therapy
Psychological Disorders and Research
2022
2674-2470
http://dx.doi.org/10.31487/j.PDR.2022.01.01
https://www.sciencerepository.org/cognitive-function-in-cystic_PDR-2022-1-101 
Abstract: 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.Keywords: CFTR modulator, cystic fibrosis, cognitive function