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Alirocumab/Evolocumab for HeFH Special Authorization Form (ABC 60060)

SPECIAL AUTHORIZATION

"Special authorization coverage may be provided for the reduction of Low Density Lipoprotein Cholesterol (LDL-C) if the following clinical criteria and conditions are met:

I) Patient has a definite or probable diagnosis of Heterozygous Familial Hypercholesterolemia (HeFH) using the Simon Broome or Dutch Lipid Network criteria or genetic testing.

AND

II) Patient is unable to reach LDL-C target (i.e., LDL-C < 2.0 mmol/L for secondary prevention or at least a 50% reduction in LDL-C from untreated baseline for primary prevention) despite:

a) Confirmed adherence to high dose statin (e.g., atorvastatin 80 mg or rosuvastatin 40 mg) in combination with ezetimibe for at least 3 months.

OR

b) Confirmed adherence to ezetimibe for at least 3 months.
AND
Patient is unable to tolerate high dose statin, defined as meeting all of the following:

i) Inability to tolerate at least two statins with at least one started at the lowest starting daily dose,
AND
ii) For each statin (two statins in total), dose reduction is attempted for intolerable symptom (myopathy) or biomarker abnormality (creatine kinase (CK) > 5 times the upper limit of normal) resolution rather than discontinuation of statin altogether,
AND

iii) For each statin (two statins in total), intolerable symptoms (myopathy) or abnormal biomarkers (CK > 5 times the upper limit of normal) changes are reversible upon statin discontinuation but reproducible by re-challenge of statins where clinically appropriate,
AND
iv) One of either:
- Other known determinants of intolerable symptoms or abnormal biomarkers have been ruled out,
OR
- Patient developed confirmed and documented rhabdomyolysis.

OR

c) Confirmed adherence to ezetimibe for at least 3 months.
AND
Patient is statin contraindicated, i.e., active liver disease or unexplained persistent elevations of serum transaminases exceeding 3 times the upper limit of normal.

- Initial coverage may be approved for either 140 mg every two weeks or 420 mg every month for a period of 3 months.
- Patients prescribed evolocumab 420 mg every month must use the 420 mg/dose formulation.
- Patients will be limited to receiving a one-month supply of evolocumab per prescription at their pharmacy.

For continued coverage beyond 3 months, the patient must meet the following criteria:
- Patient is adherent to therapy.
- Patient has achieved a reduction in LDL-C of at least 40% from baseline (4-8 weeks after initiation of evolocumab).

Continued coverage may be approved for 140 mg every 2 weeks or 420 mg every month for a period 12 months. Patients prescribed evolocumab 140 mg every 2 weeks are limited to 26 doses per year. Patients prescribed evolocumab 420 mg every month are limited to 12 doses per year.

Ongoing coverage may be considered only if the following criteria are met at the end of each 12-month period:
- Patient is adherent to therapy.
- Patient continues to have a significant reduction in LDL-C (with continuation of evolocumab) of at least 40% from baseline since initiation of PCSK9 inhibitor. LDL-C should be checked periodically with continued treatment with PCSK9 inhibitors (e.g., every 6 months)."

All requests (including renewal requests) for evolocumab for Heterozygous Familial Hypercholesterolemia must be completed using the Alirocumab/Evolocumab for HeFH Special Authorization Request Form (ABC 60060).