ADMINISTRATIVE POLICY PROCEDURE Policy #: Subject: PCSK9 INHIBITS (ex: Repatha) Section: Care Management Effective Date: January 1, 2015 Revision Date(s): NA Review Date(s): NA Responsible Parties: Patryce Toye Responsible Department(s): Case Management Regulatory References: Approved: Carol Attia, RN AVP, Care Management Patryce A. Toye, MD Senior Medical Director Purpose: Scope: Policy: It is the purpose of this policy to define the conditions under which MFC Physician Advisors may authorize Proprotein Convertase Subtilisin/Kexin type (PCSK9) Inhibitors for Familial Hypercholesterolemia. All members of MedStar Family Choice, District of Columbia Healthy Families and Alliance. It is the policy of MedStar Family Choice to cover PCSK9 inhibitors for appropriate members. Background: MedStar Family Choice will require prior authorization for PCSK9 inhibitors. Procedure: Requests for PCSK9 inhibitors should be forwarded along with supporting clinical information in accordance with the MedStar Family Choice Prior Authorization Policy.
Indications: PCSK9 Inhibitors are indicated for treatment of adults with heterozygous familial hypercholesterolemia (HeFH) or clinical atherosclerotic cardiovascular disease as an adjunct to diet and maximally tolerated statin therapy under specific conditions as outlined below: PCSK9 Inhibitors are indicated for patients with homozygous familial hypercholesterolemia (HoFH) as an adjunct to diet and other LDL-lowering therapies (e.g., statins, ezetimibe, LDL apheresis) under specific conditions as outlined below. Approval Criteria PCSK9 inhibitors may be approved when the following criteria are met: Homozygous Familial Hypercholesterolemia [evolocumab (Repatha) only] 1. The member is 13 years of age. 2. Evolocumab (Repatha) must be prescribed by or in consultation with a cardiologist or lipid specialist. 3. There must be clinical documentation of one of the following (a or b): a. Genetic confirmation of two mutant alleles at the LDLR, APOB, PCSK9, or LDLRAP1 gene locus b. An untreated LDL-C of > 500 mg/dl (or a treated LDL-C of > 300 mg/dl) with either: i. Cutaneous or tendon xanthoma before age 10 years
ii. Evidence of heterozygous familial hypercholesterolemia in both parents 4. Evolocumab (Repatha) will be used concomitantly with a maximally-tolerated statin unless all statins are contraindicated or not tolerated. Statin intolerance is defined as the following: a. The member had statin-related rhabdomyolysis documented by muscle pain, weakness, acute renal failure and/or creatine kinase levels 10 times the upper limit of normal. b. Patient had muscle symptoms (e.g. muscle weakness, myalgias) and meets both of the criteria below: i. The muscle related symptoms occurred while receiving separate trials of two different statins (documentation required) ii. The muscle related symptoms resolved upon discontinuation of each statin. 5. Evolocumab (Repatha) is not being used concomitantly with lomitapide (Juxtapid), mipomersen (Kynamro), or another PCSK9 inhibitor. Heterozygous Familial Hypercholesterolemia 1. The member is 18 years of age. 2. Evolocumab (Repatha) must be prescribed by or in consultation with a cardiologist or lipid specialist.
3. There must be clinical documentation of one of the following (a, b or c): a. Presence of causal mutation for familial hypercholesterolemia by genetic testing b. Physical signs of familial hypercholesterolemia, such as presence of tendon xanthomas, corneal arcus in a member < 45 years of age, tuberous xanthomas, or xanthelasma c. Clinical diagnosis based on the WHO criteria/dutch Lipid Clinical Network criteria with score > 8 points or the Simon Broome register diagnostic criteria with a criterion for definite familial hypercholesterolemia 4. Documentation of LDL-C 190 mg/dl ( 160 mg/dl if < 20 years of age) prior to initiating lipid-lowering therapy. 5. Treatment with at least two 12-week trials of different high-intensity statins* used concomitantly with ezetimibe (Zetia) has been ineffective**. Adherence to the current statin regimen must be evidenced by consistent pharmacy claims over the past 12 weeks, unless new to the plan. 6. The member will be using the PCSK9 inhibitor concomitantly with a maximally-tolerated statin unless the member is determined to be statin intolerant as defined by the following: a. The member had statin-related rhabdomyolysis documented by muscle pain, weakness, acute renal failure and/or creatine kinase levels 10 times the upper limit of normal. b. Patient had muscle symptoms (e.g. muscle weakness, myalgias) and meets both of the criteria below:
i. The muscle related symptoms occurred while receiving separate trials of two different statins (documentation required) ii. The muscle related symptoms resolved upon discontinuation of each statin. *High Intensity Statins are defined as atorvastatin 40 mg or higher rosuvastatin 20 mg or higher. **Treatment is considered ineffective if it results in a < 50% reduction in LDL-C or an LDL-C 160 mg/dl. In higher risk patients, treatment is considered ineffective if it results in an LDL-C 100 mg/dl. High risk is defined as: clinically evident coronary heart disease (CHD) or other atherosclerotic cardiovascular disease, diabetes, a family history of very early CHD (men < 45 years of age and women < 55 years of age), current smoking, or high lipoprotein (a) 31 mg/dl. Hypercholesterolemia, ASCVD 1. Evolocumab (Repatha) must be prescribed by or in consultation with a cardiologist or lipid specialist. 2. The member ( 18 years of age) has a documented diagnosis of clinical atherosclerotic cardiovascular disease (ASCVD), defined as one of the following: a. Acute coronary syndrome b. History of myocardial infarction c. Stable or unstable angina d. Coronary or other arterial revascularization (stent) e. Stroke f. Transient ischemic attack g. Peripheral arterial disease presumed to be of atherosclerotic origin. 3. Treatment with at least two 12 week trials of different high-intensity statins used concomitantly with ezetimibe (Zetia) has been ineffective (LDL-C 100 mg/dl). Adherence to the current statin regimen must be evidenced by consistent pharmacy claims over the past 12 weeks, unless new to the plan.
4. The member will be using the PCSK9 inhibitor concomitantly with a maximallytolerated statin. 5. Confirmation of patient enrollment in a lipid clinic or disease management program. REAUTHIZATION CRITERIA PCSK9 Inhibitors may be approved for continued therapy after an initial 3 month trial if the following criteria are met: 1. Criteria outlined for initial Prior Authorization has been satisfied. a. HoFH: Documentation of an LDL-C reduction. b. Heterozygous Familial hypercholesterolemia: Documentation of an LDL-C reduction by at least 50% from pre-treatment level or an LDL-C < 160 mg/dl (if pre-treatment LDL-C was > 160 mg/dl). c. Hypercholesterolemia, ASCVD: Documentation that LDL-C < 100 mg/dl or there has been at least a 40% LDL-C reduction from pre-treatment level. d. Hypercholesterolemia, ASCVD: Confirmation of patient enrollment in a lipid clinic or disease management program. DURATION OF AUTHIZATION Initial: If approved, initial coverage will be granted for up to 3 months. Maintenance: If approved (see Reauthorization Criteria above) maintenance coverage will be granted for up to 12 months
REFERENCES 1. Stone, N. J., Robinson, J., Lichtenstein, A. H., et al. 2013 ACC/AHA Guideline on the treatment of blood cholesterol to reduce atherosclerotic cardiovascular risk in adults: A report of the American College of Cardiology/American Heart Association Task Force on practice guidelines. Circulation 2013. Retrieved from: http://circ.ahajournals.org. 2. Goldberg, A. C., Hopkins, P. N., Toth, P. P., et al. Familial hypercholesterolemia: Screening, diagnosis and management of pediatric and adult patients. Clinical guidance from the National Lipid Association Expert Panel on Familial Hypercholesterolemia. J. of Clinical Lipidology 2011 Volume 5, Number 3S. 3. Repatha [prescribing information]. Thousand Oaks, CA: Amgen Inc.; August 2015. 4. DRUGDEX System (Micromedex 2.0). Greenwood Village, CO: Truven Health Analytics; c1974-2013. Accessed 7/27/2015. 5. Cannon CP, Blazing MA, Giugliano RP, et al. Ezetimibe added to statin therapy after acute coronary syndromes. N Engl J Med 2015;372:2387-2397. 6. Clinical Pharmacology [database online]. Tampa, FL: Gold Standard, Inc.: 2015. URL: http://www.clinicalpharmacology.com. Updated periodically.