Hyftor (sirolimus) — Blue Cross Blue Shield of Kansas

Initial criteria

• The patient has a diagnosis of tuberous sclerosis complex (TSC) confirmed by ONE of the following: (A) The patient has two major features OR one major and two minor features of TSC clinical diagnostic criteria (Major features: hypomelanotic macules ≥3, at least 5 mm diameter; angiofibroma ≥3 or fibrous cephalic plaque; ungual fibromas ≥2; shagreen patch; multiple retinal hamartomas; multiple cortical tubers and/or radial migration lines; subependymal nodule ≥2; subependymal giant cell astrocytoma; cardiac rhabdomyoma; lymphangiomyomatosis (LAM); angiomyolipomas ≥2; note that a combination of LAM and angiomyolipomas without other features does not meet definite diagnosis) OR (Minor features: confetti skin lesions; dental enamel pits ≥3; intraoral fibromas ≥2; retinal achromic patch; multiple renal cysts; nonrenal hamartomas; sclerotic bone lesions) OR (B) The patient has a pathogenic variant in the TSC1 or TSC2 gene confirmed by genetic testing
• The patient has three or more facial angiofibromas
• If the patient has an FDA-labeled indication, then ONE of the following: (A) The patient’s age is within FDA labeling for the requested indication (age ≥6 years) OR (B) There is support for using the requested agent for the patient’s age for the requested indication
• The prescriber is a specialist in the area of the patient’s diagnosis (e.g., dermatologist, geneticist) or the prescriber has consulted with a specialist in the area of the patient’s diagnosis
• The patient does NOT have any FDA labeled contraindications to the requested agent

Reauthorization criteria

• The patient has been previously approved for the requested agent through the plan’s Prior Authorization process
• The patient has had clinical benefit with the requested agent
• The prescriber is a specialist in the area of the patient’s diagnosis (e.g., dermatologist, geneticist) or the prescriber has consulted with a specialist in the area of the patient’s diagnosis
• The patient does NOT have any FDA labeled contraindications to the requested agent

Approval duration

initial 12 weeks; reauth 12 months