Orkambi (lumacaftor/ivacaftor tablets and oral granules – Vertex) — Cigna

Initial criteria

• Patient age ≥ 1 year AND
• Patient has TWO copies of the F508del mutation in the cystic fibrosis transmembrane conductance regulator gene AND
• Patient meets at least ONE of the following (i, ii, or iii): i. Positive cystic fibrosis newborn screening test; OR ii. Family history of cystic fibrosis; OR iii. Clinical presentation consistent with signs and symptoms of cystic fibrosis (examples include meconium ileus, sino-pulmonary symptoms such as persistent cough, wheezing, pulmonary function tests consistent with obstructive airway disease, excess sputum production, bronchiectasis, sinusitis, failure to thrive, pancreatic insufficiency) AND
• Patient has evidence of abnormal cystic fibrosis transmembrane conductance regulator function as demonstrated by at least ONE of the following (i, ii, or iii): i. Elevated sweat chloride test; OR ii. Two cystic fibrosis-causing cystic fibrosis transmembrane conductance regulator mutations; OR iii. Abnormal nasal potential difference AND
• Medication is prescribed by or in consultation with a pulmonologist or a physician who specializes in the treatment of cystic fibrosis

Approval duration

1 year