Policy Number: 1054 Policy History Approve Date: 06/01/2018 Effective Date: 06/01/2018 Preauthorization All Plans Benefit plans vary in coverage and some plans may not provide coverage for certain service(s) listed in this policy. Decisions for authorization are subject to all terms and conditions of the applicable benefit plan, including specific exclusions and limitations as well as applicable state and/or federal laws. Please review the benefit plan descriptions for details. We utilize a combination of MCG and internally developed evidence-based clinical guidelines to support our prior authorization work. All internally developed prior authorization guidelines follow a rigorous process including, but not limited to, review by clinical pharmacist, clinical nurse manager, Chief Medical Officer, independent 3 rd party physician review agency and Health Tradition s Medical Advisory Committee. Prior authorization guidelines are reviewed at least annually, or when there are significant labeling changes made by FDA or peer-reviewed clinical outcomes (via Cochrane or Hayes). INSTRUCTIONS: Please review section I and complete sections II, III, and IV (type or print clearly). Prescribers are asked to submit this form along with supporting clinical documentation to Health Tradition for review. Submission of form does not guarantee approval. Submit preauthorization (PA) by fax to Health Tradition at (608) 781-9654 or by mail to Health Tradition, Correspondence, P.O. Box 21171, Eagan MN 55121. Section I Approval Criteria I. is medically necessary for the treatment of an individual with documented paroxysmal nocturnal hemoglobinuria (PNH) at initiation of therapy when the following criteria are met: A. Flow cytometry demonstrates: i. At least 10% PNH type III red cells OR ii. Greater than 50% of glycosylphosphatidylinositol-anchored proteins (GPI-AP)- deficient polymorphonuclear cells (PMNs) AND B. Individual has been immunized with a meningococcal vaccine at least two weeks prior to administration of the first dose of (unless the clinical record documents that the risks of delaying outweigh the risk of meningococcal infection) AND C. There is no evidence of an active meningococcal infection AND D. Either of the following criteria i or ii are met: i. The individual has: 1. Had at least four transfusions in the last 12 months OR HT MHS 7032 1018
Page 2 ii. 2. At least one transfusion in the past two years for anemia or anemia-related symptoms or personal beliefs that preclude transfusions 3. Hemoglobin is less than or equal to 7 g/dl, or the individual has symptoms of anemia and the hemoglobin is less than or equal to 9 g/dl AND 4. Lactate dehydrogenase (LDH) is 1.5 times the upper limit of normal OR Documented history of a major adverse vascular event (MAVE) from thromboembolism II. is medically necessary in an initial six week trial for the treatment of atypical hemolytic uremic syndrome (ahus) when the following criteria are met: A. The diagnosis of ahus is supported by the absence of Shiga toxin-producing E. coli infection AND B. Thrombotic thrombocytopenic purpura (TTP) has been ruled out (for example, normal ADAMTS 13 activity and no evidence of an ADAMTS 13 inhibitor), or if TTP cannot be ruled out by laboratory and clinical evaluation, a trial of plasma exchange did not result in clinical improvement AND C. Individual has been immunized with a meningococcal vaccine at least two weeks prior to administration of the first dose of (unless the clinical record documents that the risks of delaying outweigh the risk of meningococcal infection) AND D. There is NO evidence of an active meningococcal infection III. Continuation of eculizumab following an initial six week trial for the treatment of ahus is medically necessary when there is clinical improvement after the initial trial (for example, increased platelet count or laboratory evidence of reduced hemolysis) IV. is considered investigational and is not medically necessary when the criteria above are not met and for all other indications, including but not limited to treatment of: A. Antibody mediated rejection in organ transplantation B. Antineutrophil cytoplasmic autoantibody (ANCA) vasculitis C. Antiphospholipid antibody syndrome D. Dense deposit disease or C3 nephropathy E. Hemolysis elevated liver enzymes and low platelets (HELLP) syndrome in preeclampsia F. Hemolytic cold agglutinin disease G. Myasthenia gravis H. Neuromyelitis optica I. Nonexudative (dry) macular degeneration J. Shiga toxin E. coli-related hemolytic uremic syndrome (STEC-HUS) K. Systemic lupus erythematosus (SLE) L. Thrombotic thrombocytopenic purpura (TTP) V. has a black box warning for serious meningococcal infections. Life threatening and fatal meningococcal infections have been reported with the use of Soliris. Individuals should receive the meningococcal vaccine at least two weeks prior to receiving the first dose of Soliris, unless the risks of delaying therapy outweigh the risk of developing a meningococcal infection. The most current Advisory Committee on Immunization Practices (ACIP) recommendations should be complied with in respect to meningococcal vaccination in individuals with complement deficiencies. Soliris is available only through a restricted Risk Evaluation and Mitigation Strategy (REMS) program VI. Discontinuation of may be associated with serious adverse clinical events including life threatening thrombosis Renewal Criteria I. All renewals will be for three months based on continued effectiveness as evidenced in the medical record and in the absence of significant adverse effects subsequent to an initial six week trial
Page 3 References 1. FDA approved product labeling and Lexicomp 2. Hillmen P, Young NS, Schubert J, Brodsky RA, SociéG, Muus P, Röth A, Szer J, Elebute MO, Nakamura R, Browne P, Risitano AM, Hill A, Schrezenmeier H, Fu CL, Maciejewski J, Rollins SA, Mojcik CF, Rother RP, Luzzatto L The complement inhibitor eculizumab in paroxysmal nocturnal hemoglobinuria. N Engl J Med. 2006;355(12): 1233 3. Brodsky RA, Young NS, Antonioli E, Risitano AM, Schrezenmeier H, Schubert J, Gaya A, Coyle L, de Castro C, Fu CL, Maciejewski JP, Bessler M, Kroon HA, Rother RP, Hillmen P Multicenter phase 3 study of the complement inhibitor eculizumab for the treatment of patients with paroxysmal nocturnal hemoglobinuria. Blood. 2008;111(4): 1840 4. Hillmen P, Muus P, Röth A, Elebute MO, Risitano AM, Schrezenmeier H, Szer J, Browne P, Maciejewski JP, Schubert J, Urbano-Ispizua A, de Castro C, SociéG, Brodsky RA Long-term safety and efficacy of sustained eculizumab treatment in patients with paroxysmal nocturnal haemoglobinuria. Br J Haematol. 2013 Jul;162 (1): 62-73. Epub 2013 Apr 25 5. Legendre CM, Licht C, Muus P, Greenbaum LA, Babu S, Bedrosian C, Bingham C, Cohen DJ, Delmas Y, Douglas K, Eitner F, Feldkamp T, Fouque D, Furman RR, Gaber O, Herthelius M, Hourmant M, Karpman D, Lebranchu Y, Mariat C, Menne J, Moulin B, Nürnberger J, Ogawa M, Remuzzi G, Richard T, Sberro-Soussan R, Severino B, Sheerin NS, Trivelli A, Zimmerhackl LB, Goodship T, Loirat C Terminal complement inhibitor eculizumab in atypical hemolytic-uremic syndrome. N Engl J Med. 2013 Jun;368(23): 2169-81 6. Rathbone J, Kaltenthaler E, Richards A, Tappenden P, Bessey A, Cantrell A A systematic review of eculizumab for atypical haemolytic uraemic syndrome (ahus). BMJ Open. 2013;3(11): e003573 7. Licht C, Greenbaum LA, Muus P, Babu S, Bedrosian CL, Cohen DJ, Delmas Y, Douglas K, Furman RR, Gaber OA, Goodship T, Herthelius M, Hourmant M, Legendre CM, Remuzzi G, Sheerin N, Trivelli A, Loirat C Efficacy and safety of eculizumab in atypical hemolytic uremic syndrome from 2-year extensions of phase 2 studies. Kidney Int. 2015 May;87(5): 1061-73. Epub 2015 Feb 04
Page 4 Section II Member Information Name (Last, First, Middle Initial) Date of Birth Health Tradition Subscriber Number Diagnosis 1. MEDICATION 2. STRENGTH 3. DIRECTIONS 4. QUANTITY PER 30 DAYS Specify: 5. DIAGNOSIS: 6. APPROVAL CRITERIA: CHECK ALL BOXES THAT APPLY NOTE: Any areas not filled out are considered not applicable to your patient and MAY AFFECT THE OUTCOME of this request. Patients with a diagnosis of paroxysmal nocturnal hemoglobinuria (PNH): Yes No Does the patient have a documented diagnosis of paroxysmal nocturnal hemoglobinuria (PNH) at initiation of therapy (documentation may include, but is not limited to, chart notes, prescription claims records, prescription receipts, and laboratory data)? Yes No Has flow cytometry demonstrated ONE of the following (please indicate): At least 10% PNH type III red cells Greater than 50% of glycosylphosphatidylinositol anchored proteins (GPI AP) deficient polymorphonuclear cells (PMNs) Yes No Has the patient been immunized with a meningococcal vaccine at least two weeks prior to administration of the first dose of? If No: Yes No Do the clinical records document that the risks of delaying outweigh Yes No the risk meningococcal infection? Is there evidence of an active meningococcal infection? Has patient had at least four transfusions in the last 12 months? OR at least one transfusion in the past two years for anemia or anemia related symptoms or personal beliefs that preclude transfusions? Yes No Is the patient s hemoglobin less than or equal to 7 g/dl, or does the patient have symptoms of anemia and the hemoglobin is less than or equal to 9 g/dl? Yes No Is the patient's lactate dehydrogenase (LDH) 1.5 times the upper limit of normal? Yes No Does the patient have a documented history of a major adverse vascular event (MAVE) from thromboembolism? Patients with a diagnosis of atypical hemolytic uremic syndrome (ahus) (requests for initial therapy): Yes No Does the patient have a diagnosis of atypical hemolytic uremic syndrome (ahus)? Yes No Is the diagnosis of ahus supported by the absence of Shiga toxin producing E. coli infection?
Page 5 Yes No Has thrombotic thrombocytopenic purpura (TTP) been ruled out (for example, normal ADAMTS 13 activity and no evidence of an ADAMTS 13 inhibitor), or if TTP cannot be ruled out by laboratory and clinical evaluation, a trial of plasma exchange did not result in clinical improvement? Yes No Has the patient been immunized with a meningococcal vaccine at least two weeks prior to administration of the first dose of? If No: Yes No Do the clinical records document that the risks of delaying outweigh the risk meningococcal infection? Yes No Is there evidence of an active meningococcal infection? Patients with a diagnosis of atypical hemolytic uremic syndrome (ahus) (requests for continued therapy): Yes No Did the patient have clinical improvement after the initial trial (for example, increased platelet count or laboratory evidence of reduced hemolysis)? Note: Discontinuation of may be associated with serious adverse clinical events including life threatening thrombosis
Page 6 Section III Physician Information Physician Name National Provider Identification (NPI) Number Clinic Name Address (Street, City, State, Zip Code) Physician Telephone Number Physician Fax Number Physician Signature Date Section IV For Health Tradition Use Only Plan: Copay Tiers: Date of Receipt: Remarks: Approve Deny More information is needed Incomplete form Submission and review of this form does not guarantee approval of this request. Unless approved, payment of the brand-name medication will be limited to the amount Health Tradition would have paid for the generic equivalent. Insureds and providers will be informed of the decision in writing within approximately 30 days. Fax to: Pharmacy Services (608) 781-9654 Mail to: Health Tradition Correspondence PO Box 21171 Eagan MN 55121