Orkambi — Cigna

Initial criteria

• Patient has TWO copies of the F508del mutation in the cystic fibrosis transmembrane conductance regulator gene
• Patient has at least one of the following: positive cystic fibrosis newborn screening test OR family history of cystic fibrosis OR clinical presentation consistent with signs and symptoms of cystic fibrosis
• Patient has evidence of abnormal cystic fibrosis transmembrane conductance regulator function as demonstrated by at least one of the following: elevated sweat chloride test OR two cystic fibrosis-causing cystic fibrosis transmembrane conductance regulator mutations OR an abnormal nasal potential difference