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…
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.