Dose adjustments of Elexacaftor/Tezacaftor/Ivacaftor in response to mental health side effects in adults with cystic fibrosis

•A minority of adults with CF on ELX/TEZ/IVA experience deterioration in mental health.•Every effort should be made to continue ELX/TEZ/IVA while minimising side effects.•Dose adjustment may improvement mental health AEs in some individuals with CF.•Sweat chloride concentration may prove useful as a...

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Veröffentlicht in:Journal of cystic fibrosis 2022-11, Vol.21 (6), p.1061-1065
Hauptverfasser: Spoletini, G., Gillgrass, L., Pollard, K., Shaw, N., Williams, E., Etherington, C., Clifton, I.J., Peckham, D.G.
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Sprache:eng
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Zusammenfassung:•A minority of adults with CF on ELX/TEZ/IVA experience deterioration in mental health.•Every effort should be made to continue ELX/TEZ/IVA while minimising side effects.•Dose adjustment may improvement mental health AEs in some individuals with CF.•Sweat chloride concentration may prove useful as a surrogate marker of CFTR function. Deterioration in mental health has been reported in a minority of individuals with cystic fibrosis starting elexacafor/tezacaftor/ivacaftor (ELX/TEZ/IVA). We report our experience of using sweat chloride and markers of clinical stability to titrate dose reduction with the aim of minimising adverse events and maintaining clinical stability. Adults (n = 266) prescribed ELX/TEZ/IVA, were included. Adverse events, sweat chloride, lung function and clinical data were collected. Nineteen (7.1%) individuals reported anxiety, low mood, insomnia and “brain fog” with reduced attention and concentration span. Thirteen underwent dose reduction with sweat chloride remained normal (
ISSN:1569-1993
1873-5010
DOI:10.1016/j.jcf.2022.05.001