IPD2.08 Dose modification of lumacaftor/ivacaftor and the immediate effects on lung function in cystic fibrosis patients with advanced lung disease and 12-month outcomes in this cohort

Abstract

Objectives: LUM/IVA therapy improves outcomes in patients with CF homozygous for F508del, however outcome data beyond 24-weeks in patients with percent predicted FEV1 (ppFEV1)<40 are lacking. This cohort reports an increased incidence of respiratory-related adverse events with treatment initiation. We have previously reported observations of immediate (within 2 hrs) FEV1 declines in patients with a ppFEV1<40 initiated on LUM/IVA (400/250 mg) therapy. Half-dose LUM/IVA (200/ 125 mg) at initiation may be associated with reduced adverse events. This observational study compares the immediate changes in FEV1 in patients with ppFEV1<40 initiated on full or half-dose LUM/IVA and reports efficacy data in this cohort at 12-months.