Open-label study to investigate the effects of Orkambi in CF patients homozygous for the
F508del mutation by functional respiratory imaging. Primary endpoints in this study are the
changes in Specific airway volumes (siVaw) and Specific Airway resistance (siRaw). A total of
20 ORKAMBI-naive patients with Cystic Fibrosis, homozygous for the F508del mutation will be
included in this open label study and will be followed through 3 months of treatment. The
treatment will be started after all assessments are performed at visit 1. After the start of
the treatment some baseline measurements will be repeated throughout the 3-month treatment
period. The patient will be asked to visit the hospital monthly. All study visits should be
scheduled around the same time.