| 527 | For the treatment of Heterozygous Familial Hypercholesterolemia (HeFH) in patients 18 years of age or older who meet the following criteria:
- Definite or probable diagnosis of HeFH using the Simon Broome or Dutch Lipid Network criteria or genetic testing;
AND
- Unable to reach Low Density Lipoprotein Cholesterol (LDL-C) target (i.e., LDL-C less than 2.0 mmol/L for secondary prevention) or at least a 50% reduction in LDL-C from untreated baseline despite:
A. Confirmed adherence to high dose statin (e.g., atorvastatin 80mg or rosuvastatin 40mg) in combination with ezetimibe for at least a total of 3 months;
OR
B. Confirmed adherence to ezetimibe for at least a total of 3 months and inability to tolerate high dose statin defined as:
(i). Inability to tolerate at least 2 statins with at least one started at the lowest starting dose; AND
(ii). For each statin (two statins in total), dose reduction is attempted for intolerable symptom (myopathy) or biomarker abnormality (creatine kinase (CK) greater than 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 biomarker (creatine kinase (CK) greater than 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 the following:
I) Other known determinants of intolerable symptoms or abnormal biomarkers have been ruled out; OR
II) Patient developed confirmed and documented rhabdomyolysis; OR
III) Patient is statin contraindicated i.e. active liver disease, unexplained persistent elevations of serum transaminases exceeding 3 times the upper limit of normal
Treatment with Repatha should be discontinued if the patient does not meet all of the following:
1. Patient is adherent to therapy.
2. Patient has achieved a reduction in LDL-C of at least 40% from baseline (4-8 weeks after initiation of Repatha).
3. Patient continues to have a significant reduction in LDL-C (with continuation of Repatha) 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).
Patients prescribed Repatha 140mg every two weeks are limited to 26 prefilled syringes (PFS) per year. Patients prescribed Repatha 420mg every month must use the automated mini doser (AMD) and are limited to 12 AMD per year.
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