The FDA approved Orkambi for people with CF ages 12 and older who have two copies of the F508del mutation.  Nearly half of individuals with CF have two copies of this mutation and could eventually benefit from the therapy.  Orkambi improves lung function and significantly reduces the rate of pulmonary exacerbations, which can lead to frequent hospitalizations and accelerated lung disease. Orkambi combines lumacaftor and ivacaftor to treat the underlying cause of CF in a two-step approach. Lumacaftor helps move the defective CFTR protein to its proper place at the cell surface. Ivacaftor increases the activity of the protein once it is there, supporting the flow of salt and fluids, which helps thin the thick mucus that builds up in the lungs of people with CF.   For more information, visit