Mental status changes during elexacaftor/tezacaftor / ivacaftor therapy

J Cyst Fibros. 2022 Mar;21(2):339-343. doi: 10.1016/j.jcf.2021.10.002. Epub 2021 Nov 3.

Abstract

Elexacaftor/tezacaftor/ivacaftor (ELX/TEZ/IVA, Trikafta) is the newest Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) modulator drug approved by the Food and Drug Administration. Post-marketing reports with earlier CFTR modulators suggest these medications can impact mood, and in clinical trials an adverse effect of headache was reported with all currently approved CFTR modulators. However, there are no other documented reports of mental status changes during clinical trials or in post-marketing reports with elexacaftor/tezacaftor/ivacaftor. In this case series, we describe 6 patients who reported "mental fogginess" or other mental status changes shortly after initiation of this drug. The mechanism of this patient-reported side effect is still unclear. All patients noticed a change within the first 3 months of therapy. The management differed in each case, with all four cystic fibrosis (CF) care teams utilizing a patient-centered decision-making approach to address this concern.

Keywords: Cystic fibrosis; Dementia; Drug-Related Side Effects and Adverse Reactions; Elexacaftor; Memory loss; altered mental status.

MeSH terms

  • Affect* / drug effects
  • Aminophenols
  • Benzodioxoles
  • Chloride Channel Agonists
  • Cystic Fibrosis Transmembrane Conductance Regulator* / drug effects
  • Cystic Fibrosis* / drug therapy
  • Cystic Fibrosis* / genetics
  • Drug Combinations
  • Humans
  • Indoles
  • Mental Fatigue
  • Pyrazoles
  • Pyridines
  • Pyrrolidines
  • Quinolones
  • Somatoform Disorders

Substances

  • Aminophenols
  • Benzodioxoles
  • Chloride Channel Agonists
  • Drug Combinations
  • Indoles
  • Pyrazoles
  • Pyridines
  • Pyrrolidines
  • Quinolones
  • tezacaftor
  • Cystic Fibrosis Transmembrane Conductance Regulator
  • ivacaftor
  • elexacaftor

Supplementary concepts

  • long COVID brain fog