Orkambi - Lumacaftor with Ivacaftor

Lumacaftor increases the number of proteins that each the cell surface. Ivacaftor is a cystic fibrosis transmembrane conductance regulator (CFTR) protein potentiator that increases chloride transport in the abnormal CFTR protein.

By mouth using granules
Child 2–5 years (body-weight up to 14 kg): 100/125 mg every 12 hours.
Child 2–5 years (body-weight 14 kg and above): 150/188 mg every 12 hours.

By mouth using tablets
Child 6–11 years: 200/250 mg every 12 hours.
Child 12–17 years: 400/250 mg every 12 hours.
Adult: 400/250 mg every 12 hours – specialist use only.