Doptelet (avatrombopag)

PHARMACY POLICY STATEMENT Kentucky Medicaid DRUG NAME Doptelet (avatrombopag) BILLING CODE Must use valid NDC code BENEFIT TYPE Pharmacy SITE OF SERVICE ALLOWED Home COVERAGE REQUIREMENTS Prior Authorization Required (Preferred Product) Alternative preferred products include Promacta QUANTITY LIMIT see Dosage allowed below LIST OF DIAGNOSES CONSIDERED NOT MEDICALLY NECESSARY Click Here Doptelet (avatrombopag) is a preferred product and will only be con sidered for coverage under the pharmacy benefit when the following criteria are met: Members must be clinically diagnosed with one of the following disease states and meet their individual criteria as stated. IMMUNE THROMBOCYTOPENIC PURPURA (ITP) For initial authorization: 1. Member is 1 8 year of age or older; AND 2. Member has a documented diagnosis of chronic immune (idiopathic) thrombocytopenic purpura (ITP) with an insufficient response to a previous treatment ; AND 3. Medication must be prescribed by or in consultation with a hematologist; AND Member has ONE of the following conditions: a) Current platelet count is

Boniva (ibandronate) injection

PHARMACY POLICY STATEMENT Kentucky Medicaid DRUG NAME Boniva (ibandronate) injection BILLING CODE J1740 BENEFIT TYPE Medical SITE OF SERVICE ALLOWED Office/Outpatient hospital COVERAGE REQUIREMENTS Prior Authorization Required for injectable product only (no Prior Authorization needed for oral product ) Alternative preferred products include zoledronic acid QUANTITY LIMIT see Dosage allowed below LIST OF DIAGNOSES CONSIDERED NOT MEDICALLY NECESSARY Click Here Boniva (ibandronate) injection is a non-preferred product and will only be considered for coverage under the medical benefit when the following criteria are met: Members must be clinically diagnosed with one of the following disease states and meet their individual criteria as stated. OSTEOPOROSIS For initial authorization: 1. Medication is intended to be used for t reatment of osteoporosis in postmenopausal women with high risk for fracture; 2. Members osteoporosis evidenced by one of the following: a) Bone mineral density (BMD) T-score 2.5 or below in the lumbar spine, femoral neck, total, and/or 33% (one-third) radius ; b) Low-trauma spine or hip fracture (regardless of BMD ); c) Osteopenia or low bone mass (T-score between 1 and 2.5) with a fragility fracture of proximal humerus, pelvis, or possibly distal forearm ; d) Osteopenia or low bone mass and high FRAX fracture probability (a 10-year probability for major osteoporotic fr acture is 20% or the 10-year probability of hip fracture is 3% ); AND 3. Member does not have ANY of the following: a) Uncorrected hypocalcemia; b) Dental disease; c) History of receiving Xgeva within the past 6 months; AND 4. Member was instructed to take calcium 1,000 mg daily and at least 400 IU of vitamin Ddaily; AND 5. Documentation of m embers inability to t ake oral bisphosphonate therapies (i.e., alendronate and/or ibandronate) required as evidenced by one or more of the following: a) Esophogeal dysmotility or var ices; b) Member is unable to stand or sit upright for 30-60 minutes; c) Presence of anatomic or functional esophageal abnormalities that might delay tablet transit (e.g., achalasia, stricture, or dysmotility); d) Presence of documented or potential GI malabsorption (e.g., gastric bypass procedures, celiac disease, Crohns disease, infiltrative disorders, etc.); e) Member has experienced intolerance to or treatment failure of one or more bisphosphonate medications; f) Member has a history of non-adherence to oral bisphosphonate medications ; AND 6. Member has had a documented trial and inadequate response to zoledronic acid. 7. Dosage allowed: IV: 3 mg every 3 months. Note: IV form of the drug is only indicated for treatment (not prevention) of osteoporosis in postmenopausal women . If member meets all the requirements listed above, the medication will be approved for 12 months For reauthorization: 1. Member meets all initial criteria; AND 2. Chart notes have been provided that show the member has shown an increase in bone mineral density. If member meets all the reauthorization requirements above, the medication will be approved for an additional 12 months. CareSource considers Boniva ( ibandronate) not medically necessary for the treatment of the following disease states based on a lack of robust clinical controlled trials showing superior efficacy compared to currently available treatments: Bone metastases from solid tumors Giant Cell Tumor of Bone Multiple Myeloma Pagets disease Bone loss (for nonmetastatic prostate cancer or for breast cancer) DATE ACTION/DESCRIPTION 07/29/2019 New policy for Boniva injection created. References: 1. Bonia [prescribing information]. South San Francisco, CA: Genentech, Inc.; April, 2019. 2. Camacho PM, Petak SM, Binkley N, et al. American Association of Clinical Endocrinologists and American College of Endocrinology clinical practice guidelines for the diagnosis and treatment of postmenopausal osteoporosis 2016. Endocr Pract. 2016;22(Suppl 4). Doi: 10.4158/ EP161435.GL. 3. Tu KN, Lie JD, Wan CKV, et al. Osteoporosis: A Review of Treatment Options . P &T. 2018 Feb; 43(2): 92 104. 4. Porter JL, Varacallo M. Osteoporosis.StatPearls Publishing LLC . Bookshelf ID: NBK441901, PMID: 28722930. Available at: https://www.researchgate.net/profile/Matthew_Varacallo/publication/329717790_Osteoporosis/links/5c17f5314585157ac1ca042b/Osteoporosis.pdf?origin=publication_detail. Effective date: 09/26/2019 Revised date: 07/29/2019 Appendix. Risk Factors for Fracture: 1. Prior fracture ; 2. Age 65; 3. Low body weight (

Benlysta (belimumab)

PHARMACY POLICY STATEMENT Kentucky Medicaid DRUG NAME Benlysta (belimumab) BILLING CODE For medical-J0490 For Rx-must use valid NDC BENEFIT TYPE Medical or Pharmacy SITE OF SERVICE ALLOWED Office/Home COVERAGE REQUIREMENTS Prior Authorization Required (Non-Preferred Product) QUANTITY LIMIT see Dosage allowed below LIST OF DIAGNOSES CONSIDERED NOT MEDICALLY NECESSARY Click Here Benlysta (belimumab) is a non-preferred product and will only be con sidered for coverage under the medical or pharmacy benefit when the following criteria are met: Members must be clinically diagnosed with one of the following disease states and meet their individual criteria as stated. SYSTEMIC LUPUS ERYTHEMATOSUS For initial authorization: 1. Member is 5 years of age or older ; AND 2. Medication must be prescribed by a rheumatologist ; AND 3. Member must have active disease with SELENA-SLEDAI score of 6 or greater (documented in chart notes) prior to initiating Benlysta; AND 4. Member is autoantibody-positive with chart notes documentation of anti-nuclear antibody (ANA) titer 1:80 and/or anti-double-stranded DNA (anti-dsDNA) 30 I U/mL; AND 5. Member meets ALL of the following: a) Member requires daily use of oral corticosteroids, unless contraindicated, or previously ineffective or not tolerated ; b) Member has tried and failed to respond to treatment with at least two of the following: chloroquine, hydroxychloroquine, methotrexate, azathioprine, cyclophosphamide, or mycophenolate mofetil for at least 30 days ; c) Member is not currently on intravenously administered cyclophosphamide or another biologic agent . 6. Dosage allowed: Intravenously (for adult and pediatric members ) 10 mg/kg at 2 week intervals for first 3 doses and at 4 week intervals thereafter . Subcutaneously (only for adult members) 200 mg once weekly. If member meets all the requirements listed above, the medication will be approved for 6 months. For reauthorization: 1. Member must be in compliance with all other initial criteria; AND 2. Member has SELENA-SLEDAI score improvement documented in chart notes; AND 3. Chart notes have been provided that show the member has shown improvement of signs and symptoms of disease. If member meets all the reauthorization requirements above, the medication will be approved for an additional 12 months. CareSource considers Benlysta (belimumab) not medically necessary for the treatment of the following disease states based on a lack of robust clinical controlled trials showing superior efficacy compared to currently available treatments: Severe active lupus nephritis Severe active central nervous system lupus DATE ACTION/DESCRIPTION 10/18/2017 New policy for Benlysta created. Length of approval was increased, system involvement limitations were removed and improvement of SELENA-SLEDAI score was added in reauthorization. 07/28/2019 Age coverage expanded from adult population (18 years old and older) to pediatric population of 5 years old and older. References: 1. Benlysta [package insert ]. Rockville, MD: Human d enome Sciences, Inc.; April I 2019. 2. FDA Briefing Document for the Arthritis Advisory Committee Meeting: Benlysta/Belimumab. November 16, 2010. Available at: http://www.fda.gov/downloads/advisorycommittees/committeesmeetingmaterials/drugs/arthritisdrugsadvisorycommittee/ucm233579.pdf. 3. Furie R, Petri M, Zamani O, et al. A phase III, randomized, placebo-controlled study of belimumab, a monoclonal antibody that inhibits Blymphocyte stimulator, in patients with systemic lupus erythematosus. Arthritis Rheum. 2011; 63 (12): 3918 30. 4. Navarra SV, Guzman RM, Gallacher AE, et al. Efficacy and safety of belimumab in patients with active systemic lupus erythematosus: a randomized, placebo-controlled, phase 3 trial. Lancet. 2011; 26 (377): 721 31. 5. Wallace DJ, Sohl W, Furie RA, et al, A phase II, randomized, double-blind, placebo-controlled, dose-ranging study of belimumab in patients with active systemic lupus erythematosus. Arthritis Rheum. 2009; 61 (9): 1168 78. 6. American College of Rheumatology Ad Hoc Committee on Systemic Lupus Erythematosus Guidelines. Guidelines for referral and management of systemic lupus erythematosus in adults. Arthritis Rheum. 1999; 42 (9): 1785 1796. 7. Gold Standard, Inc. Benlysta. Clinical Pharmacology [database online]. Available at: http://www.clinicalpharmacology.com. 8. American College of Rheumatology. Belimumab for systemic lupus erythematosus. March 15, 2011. 9. Bertsias G, Ioannidis JP, Boletis J, et al. EULAR recommendations for the management of systemic lupus erythematosus. Report of a Task Force of the EULAR Standing Committee for International Clinical Studies Including Therapeutics. Ann Rheum Dis. 2008; 67 (2): 195 205. 10. Belimumab. Lexi-Drugs Online [database on internet]. Hudson, OH: Lexi-Comp, Inc.; 2007. Available from: http://online.lexi.com 11. American College of Rheumatology Ad Hoc Committee on Systemic Lupus Erythematosus Response Criteria. The American College of Rheumatology response criteria for systemic lupus erythematosus clinical trials: measures of overall disease activity. Arthritis Rheum. 2004; 50 (11): 3418 26. 12. Petri M. Disease activity assessment in SLE: do we have the right instruments? Ann Rheum Dis. 2007; 66 (suppl III):iii61 iii64. 13. ClinicalTrials.gov. Identifier: NCT01649765. Pediatric Lupus Trial of Belimumab Plus Background Standard Therapy (PLUTO) . Available at: https://clinicaltrials.gov/ct2/show/NCT01649765?term=01649765&rank=1. Effective date: 09/26/2019 Revised date: 07/28/2019

Antihemophilic Agents

PHARMACY POLICY STATEMENT Kentucky Medicaid DRUG NAME Antihemophilic agents: Advate, Adynovate, Afstyla, Alphanate and Alphanate/VWF Complex/Human , AlphaNine SD , Alprolix , Bebulin and Bebulin VH , BeneFIX , Coagadex, Corifact, Eloctate, Esperoct, Factor VIII SD (Human), Feiba, Feiba NF, and Feiba VH Immuno, Fibryga, Helixate and Helixate FS, Hemlibra, Hemofil M, Humate-P and Humate-PHuman, Idelvion, Ixinity, Jivi, Kcentra, Koate, Koate-DVI, and Koate-HP, Kogenate, Kogenate FS, and Kogenate FS Bio-Set, Kovaltry, Monoclate-P, Mononine, Novoeight, NovoSeven and NovoSeven RT, Nuwiq, Obizur , Profilnine and Profilnine SD , Rebinyn, Recombinate, RiaSTAP, Rixubis , Tretten , Vonvendi , Wilate , Xynt ha and Xyntha Solofuse BILLING CODE J7170-Hemlibra; J7192-Advate, Helixate, Kogenate, Recombinate; J7190-Hemofil M , Koate , Monoclate-P ; J7193-Alphanate, Mononine; J7194-Bebulin, Profilnine ; J7195-BeneFIX , Ixinity ; J7175-Coagadex ; J7177-Fibryga; J7178-RiaSTAP; J7179-Vonvendi; J7180-Corifact; J7181-Tretten; J7182-Novoeight; J7183-Wilate; J7185-Xyntha; J7186-Alphanate; J7187-Humate-P; J7188-Obizur; J7189-NovoSeven; J7198-Feiba; J7200-Rixubis; J7201-Alpolix; J7202-Idelvion; J7205-Elo ctate; J7207-Adynovate; J7209-Nuwiq; J7210-Afstyla; J7211-Kovaltry; J3590-Kcentra; J7199-Jivi; J7203 and J7199-Rebinyn; J7199-Esperoct BENEFIT TYPE Medical SITE OF SERVICE ALLOWED Office/Home COVERAGE REQUIREMENTS Prior Authorization Required QUANTITY LIMIT see package insert for each individual drug LIST OF DIAGNOSES CONSIDERED NOT MEDICALLY NECESSARY Click Here All antihemophilic agents will only be considered for coverage under the medical benefit when the following criteria are met: Members must be clinically diagnosed with one of the following disease states and meet their individual criteria as stated. HEMOPHILIA For initial authorization: 1. Member has diagnosis of Hemophilia A or Hemophilia B ; AND 2. Members weight in kilograms, measured within the last 180 days, is documented on medication prior authorization request. 3. Dosage allowed: Per package insert of individual drug . Notes: Documented diagnosis must be confirmed by portions of the individuals medical record which need to be supplied with prior authorization request. These medical records may include, but are not limited to test reports, chart notes from providers office, or hospital admission notes. Refer to the product package insert for dosing, administration and safety guidelines. If member meets all the requirements listed above, the medication will be approved for 6 months. For reauthorization: 1. Members updated measurement of weight in kilograms is documented on medicat ion prior authorization request ; AND 2. Chart notes have been provided that show the member has shown improvement of signs and symptoms of disease. If member meets all the reauthorization requirements above, the medication will be approved for an additional 6 months. CareSource considers antihemophilic agents not medically necessary for the treatment of the diseases that are not listed in this docum ent. DATE ACTION/DESCRIPTION 12/15/2016 Policy issued. 06/12/2018 Policy placed in a new format. Initial authorization length increased to 6 months. 10/05/2018 New drug Jivi added to the list of antihemophilic agents. 08/06/2019 New drug Esperoct added to the list of antihemophilic agents. References: 1. National Institutes of Health. National Heart, lung, and Blood Institute. What is Hemophilia? Available at: https://www.nhlbi.nih.gov/health-topics/hemophilia. 2. Advate [package insert]. Westlake Village, CA : Baxalta US Inc ; Nov 2016. 3. Adynovate [package insert].Westlake Village, CA: Baxalta US Inc; March 2017. 4. Afstyla [package insert] . Kankakee, IL: CSL Behring LLC; Sept 2017. 5. Alphanat e [package insert]. Los Angeles, CA : Grifols Biologicals Inc. ; June 2014. 6. Alphanin e SD [package insert] . Los Angeles, CA: Grifols Biologicals Inc.; March 2017. 7. Alprolix [package insert]. Cambridge, MA :Biogen Inc.; November 2017. 8. Bebulin VH [package insert]. Westlake Village, CA: Baxalta US Inc; July 2012 . 9. Benefix [package insert] . Philadelphia, PA : Wyeth Pharmaceuticals Inc. ; June 2017. 10. Coagadex [package insert]. Durham, NC : Bio Products Laboratory USA, Inc. ; No date. 11. Corifact [package insert]. Kankakee, IL: CSL Behring LLC; Sept 2017. 12. Eloctate [package insert] . Waltham, MA:Bioverativ Therapeutics Inc. ; Dec 2017. 13. Feiba [package insert] . Westlake Village, CA: Baxter Healthcare Corporation.; Nov 2013. 14. Feiba NF [package insert ]. Westlake Village, CA: Baxter Healthcare Corporation.; Feb 2011. 15. Feiba VH [package insert] . Westlake Village, CA: Baxter Healthcare Corporation.; Apr 2005 16. Helixate FS [package insert] . Kankakee, IL: CSL Behring LLC.; May 2014. 17. Hemlibra [package insert]. South San Francisco, CA: Genentech, Inc.; Nov 2017 18. Hemofil M[package insert] . Westlake Village, CA: Baxter Healthcare Corporation.; April 2012. 19. Humate-P[package insert] . Kankakee, IL: CSL Behring LLC.; Aug 2013. 20. Idelvion [package insert] . Kan kakee, IL: CSL Behring LLC.; Mar ch 2016. 21. Ixinity [package insert]. Berwyn, PA : Aptevo BioTherapeutics LLC ; April 2018. 22. Kcentra [package insert] . Kankakee, IL: CSL Behring LLC.; Dec 2013. 23. Koate-DVI [package insert]. Los Angeles, CA: Grifols Biologicals Inc.; Aug 2012. 24. Kogenate FS [package insert]. Tarrytown, NY: Bayer Healthcare; May 2014. 25. Kovaltry [package insert]. Whippany, NJ:Bayer HealthCare LLC ; March 2016. 26. Monoclate-P[package insert] Kankakee, IL: ZLB Behring LLC.; Aug 200427. Mononine [package insert] . Kankakee, IL: CSL Behring LLC.; Feb 2013. 28. Novoeight [package insert]. Plainsboro, NJ : Novo Nordisk Inc. ; June 2018. 29. Novoseven RT [package insert]. Bagsvaerd, Denmark: Novo Nordisk A/S.; May 2014. 30. NuwiQ [package insert] . Hoboke n, NJ: Octapharma USA Inc.; Sept 2015. 31. Obizur [package insert] . Westlake Village, CA: Baxter Healthcare Corporation.; Oct 2014 32. Profilnine [package insert]. Los Angeles, CA: Grifols Biologicals Inc. ; Aug 2010. 33. Rebinyn [package insert]. Plainsboro, NJ : Novo Nordisk Inc. ; May 2017. 34. Recombinate [package insert] Westlake Village, CA: Baxter Healthcare Corporation.; Dec 2010. 35. RiaSTAP [package insert] Kankakee, IL: CSL Behring LLC.; Dec 2011. 36. Rixubis [package insert] . Westlake Village, CA : Baxalta US Inc. ; Sept 2014. 37. Tretten [package insert]. Bagsvaerd, Denmark: Novo Nordisk A/S.; Apr 2014. 38. VonVendi [package insert]. Westlake Village, CA: Baxalta US Inc.; Dec 2015. 39. Wilate [package insert] . Hoboken, NJ: Octapharma USA Inc.; Aug 2010. 40. Xyntha [package insert] . Philadelphia, PA: Wyeth Pharmaceuticals Inc.; Oct 2014. 41. Jivi [package insert]. Whippany, NJ: Bayer HealthCare LLC ; August 2018. 42. Esperoct [package insert]. Plainsboro, NJ: Novo Nordisk Inc. ; February, 2019. Effective date: 09/26/2019 Revised date: 08/06/2019

Kymriah (tisagenlecleucel)

PHARMACY POLICY STATEMENT Kentucky Medicaid DRUG NAME Kymriah (tisagenlecleucel) BILLING CODE Q2042 (1 unit = 250 million Tcells) BENEFIT TYPE Medical SITE OF SERVICE ALLOWED Outpatient Hospital COVERAGE REQUIREMENTS Prior Authorization Required (Non-Preferred Product) QUANTITY LIMIT see Dosage allowed below LIST OF DIAGNOSES CONSIDERED NOT MEDICALLY NECESSARY Click Here Kymriah ( tisagenlecleucel) is a non-preferred product and will only be con sidered for coverage under the medical benefit when the following criteria are met: Members must be clinically diagnosed with one of the following disease states and meet their individual criteria as stated. ACUTE LYMPHOBLASTIC LEUKEMIA (ALL) for autologous use only For initial authorization: 1. Member is 3-25 years of age and has documentation of CD19 tumor expression; AND 2. Member has B-cell acute lymphoblastic leukemia that is refractory or in second or later relapse as defined by one of the following: a) 2nd or greater Bone Marrow (BM) relapse; b) Any BM relapse after allogeneic stem cell transplantation ( SCT) and must be > 6 months from SCT at the time of CAR-T cell immunotherapy infusion; c) Refractory as defined by not achieving a complete remission (CR) after 2 cycles of a standard chemotherapy regimen chemotherapy regimen or chemorefractory as defined by not achieving a CR after 1 cycle of standard chem otherapy for relapse leukemia; d) Member with Philadelphia chromosome positive (Ph+) acute lymphoblastic leukemia that is intolerant to or have failed 2 lines of t yrosine kinase inhibitor (TKI) therapy ( e.g. imatinib mesylate (Gleevec), dasatinib (Sprycel), nilotinib (Tasigna) or ponatinib (Iclus ig)), or if TKI therapy is contraindicated ; e) Member is not eligible for allogeneic SCT; AND 3. Member has been screened for hepatitis Bvirus (HBV), hepatitis Cvirus (HCV), and human immunodeficiency virus (HIV) prior to collection of cells (leukapheresis); A ND 4. Healthcare facility /provider has enrolled in the Kymriah REMS and has training on the management of cytokine release syndrome (CR S) and neurological toxicities; AND 5. Member must be premedicated with acetaminophen and an H1-antihistamine, and tocilizumab (Actemra) must be available in healthcare facility prior to infusion; AND 6. Member has a life expectancy > 12 weeks; AND 7. Member does not have history of ALL of the following: a) Prior CAR-Ttherapy; b) Concomitant genetic syndrome (e.g., Fanconi anemia, Kostmann syndrome, Shwachman syndrome or any other known bone marrow failure syndrome ); c) Burkitt's lymphoma/leukemia; d) Malignancy, except carcinoma in situ of the skin or cervix treated with curative intent and with no evidence of active disease; e) Prior treatment wit h gene therapy product ; f) Presence of Grade 2 to 4 acute or extensive chronic graft-versus-host disease (GVHD) ; g) Active or latent hepatitis Bor active hepatitis Cor HIV. 8. Dosage allowed: Weight 50 kg or less: administer 0.2 to 5.0 x 106CAR-positive viable Tcells per kg body weight intravenously. Weight above 50 kg: administer 0.1 to 2.5 x 108 total CAR-positive viable Tcells (non-weight based) intravenously . Note: Treatment will not be authorized unless the requesting facility has provided documentation th at they are contracted with Novartis for the Novartis Outcomes agreement . If member meets all the requirements listed above, the medication will be approved for 3 months. For reauthorization: 1. Kymriah will not be reauthorized for continued therapy . LARGE B-CELL LYMPHOMA for autologous use only For initial authorization: 1. Member is being use for adult member (18 years old or older ) with has relapsed or refractory large B-cell lymphoma (diffuse large B-cell lymphoma (DLBCL) not otherwise specified, high grade B-cell lymphoma, or DLBCL arising from follicular lymphoma); AND 2. Member has received 2 lines of chemotherapy, including rituximab and anthracycline, or relapsed following autologous hematopoietic stem cell transplantation (HSCT); AND 3. Member does not have ALL of the following: a) Active central nervous system malignancy; b) Prior allogenic HSCT; c) ECOG performance status 2; d) Creatinine clearance 5 times normal; f) Cardiac ejection fraction 12 w eeks; AND 7. Member has not received prior CAR-T therapy. 8. Dosage allowed: Administer 0.6 to 6.0 x 108 CAR-positive viable Tcells . Note: Treatment will not be authorized unless the requesting facility has provided documentation that they are contracted with Novartis for the Novartis Outcomes agreement . If member meets all the requirements listed above, the medication will be approved for 3 months. For reauthorization: 1. Kymriah will not be reauthorized for continued therapy. CareSource considers Kymriah (tisagenlecleucel) not medically necessary for the treatment of the following disease states based on a lack of robust clinical controlled trials showing superior efficacy compared to currently available treatments: Primary central nervous system l ymphoma DATE ACTION/DESCRIPTION 10/24/2017 New policy for Kymriah created. 08/27/2018 New indication of Large B-cell lymphoma was added. Criteria expanded for ALL diagnosis for members disease history requirement. References: 1. Kymriah [package insert]. East Hanover, NJ; Novartis Pharmaceuticals Corp., May 2018. 2. The Leukemia & Lymphoma Society (LLS). Ph-Positive ALL Therapy. Available at https://www.lls.org/leukemia/acute-lymphoblastic-leukemia/treatment/ph-positive-all-therapy. 3. ClinicalTrials.gov. Identifier NCT02228096. Study of Efficacy and Safety of CTL019 in Pediatric ALL Patients. Available at https://clinicaltrials.gov/ct2/show/NCT02228096?term=tisagenlecleucel&rank=1. Accessed in October, 2017. 4. Maude SL, et al. Tisagenlecl eucel in Children and Young Adults with B-Cell Lymphoblastic Leukemia. NEngl JMed. 2018;378(5):439-448. [PubMed 29385370] 5. Schuster SJ, et al. Primary analysis of Juliet: a global, pivotal, phase 2 trial of CTL019 in adult patients with relapsed or refrac tory diffuse large B-cell lymphoma. Blood. 2017;130(s1):577 [Abstract 577 from 2017 ASH annual meeting]. 6. NCCN Guidelines. Acute Lymphoblastic Leukemia. V.1.2018 7. NCCN Guidelines. Non-Hodgkins Lymphoma. V.4.2018. Effective date: 09/07/2018 Revised date: 08/2 7/2018

Zolgensma (onasemnogene abeparvovec-xioi)

PHARMACY POLICY STATEMENT Kentucky Medicaid DRUG NAME Zolgensma (onasemnogene abeparvovec-xioi) BILLING CODE TBD BENEFIT TYPE Medical SITE OF SERVICE ALLOWED Outpatient Hospital COVERAGE REQUIREMENTS Prior Authorization Required (Non-Preferred Product) QUANTITY LIMIT 1.1 1014 vector genomes per kilogram (vg/kg) of body weight LIST OF DIAGNOSES CONSIDERED NOT MEDICALLY NECESSARY Click Here Zolgensma (onasemnogene abeparvovec-xioi) is a non-preferred product and will only be con sidered for coverage under the medical benefit when the following criteria are met: Members must be clinically diagnosed with one of the following disease states and meet their individual criteria as stated. SPINAL MUSCULAR ATROPHY (SMA) For initial authorization: 1. Member is less than 12 months old at the time of infusion and has documented onset of symptoms before 6 months of age; AND 2. Member has docu mented diagnosis of SMA type I confirmed by ALL of the following diagnostic test results (both a and b ): a) The mutation or deletion of genes in chromosome 5q resulting in one of the following: i) homozygous gene deletion OR mutation (e.g., homozygous deletion of exon 7 at locus 5q13); ii) compound heterozygous mutation (e.g., deletion of SMN1 exon 7 (allele 1) and mutation of SMN1 (allele 2)); b) Genetic testing confirming 2 copies of SMN2; AND 3. Medication must be prescribed by or in consultation with a neurologist; AND 4. Member does not have advanced SMA (e.g., complete paralysis of limbs, permanent ventilator dependence) ; AND 5. Medication must not be concomitantly used with Spinraza (discontinuation of Spinraza prior to Zolgensma therapy is required) ; AND 6. On day one prior t o Zolgensma infusion member will receive systemic corticosteroids equivalent to oral prednisolone at 1 mg/kg of body weight per day (for a total of 30 days); AND 7. Member has documented ALL of the following: a) Liver function tests (clinical exam, AST, ALT, total bilirubin, prothrombin time); b) Platelet counts and troponin-I; c) Baseline testing for the presence of anti-AAV9 antibodies ( titer must be 1:50); AND 8. Member has documentation of baseline of at least one of the following exams (based on patient age and motor ability): a) Hammersmith Infant Neurological Exam (HINE) (infant to early childhood); b) Hammersmith Functional Motor Scale Expanded (HFMSE); c) Upper Limb Module (ULM) Test (Non ambulatory); d) Childrens Hospital of Philadelphia Infant Test of Neur omuscular Disorders (CHOP INTEND); AND 9. Members gestational age is 35 weeks ; AND 10. Member must be up-to-date on childhood vaccinations and prophylaxis against respiratory syncytial virus ; AND 11. Member has no t have any of the following: a) Signs of aspiration; b) Active viral infection ; c) Concomitant use of drugs for treatment of myopathy or neuropathy, agents used to treat diabetes mellitus, or ongoing immunosuppressive therapy or immunosuppressive therapy within 3 months (e.g. , corticosteroids, cyclosporine, tacrol imus, methotrexate, cyclophosphamide, intravenous immunoglobulin, rituximab) ; d) Tracheostomy (i.e., invasive ventilatory support ) or required of non-invasive ventilatory support while awake over the 7 days ; e) Upper or lower respiratory infection requiring medi cal attention, medical intervention, or increase in supportive care of any manner within 4 weeks prior to request. 12. Dosage allowed: 1.1 1014 vector genomes (vg) per kg of body weight . Note: Use of Zolgensma in premature neonates before reaching full term gestational age is not recommended because concomitant treatment with corticosteroids may adversely affect neurological development . If member meets all the requirements listed above, the medication will be approved for 1 month. For reauthorization: 1. Zolgensma will not be reauthorized for continuous use. CareSource considers Zolgensma (onasemnogene abeparvovec-xioi) not medically necessary for the treatment of the diseases that are not listed in this document.DATE ACTION/DESCRIPTION 05/31/2019 New policy for Zolgensma (onasemnogene abeparvovec-xioi) created. References: 1. Zolgensma [prescribing information]. Bannockburn, IL: AveXis, Inc; 2019. 2. AveXis, Inc. Gene Transfer Clinical Trial for Spinal Muscular Atrophy Type 1. In: ClinicalTrials.gov [Internet]. Bethesda (MD): National Library of Medicine (US). Available from: https://clinicaltrials.gov/ct2/show/NCT02122952?term=ZOLGENSMA&rank=8. Identifier: NCT02122952. 3. Mendell JR, AlZaidy S, Shell R, et al. Single-Dose Gene-Replacement Therapy for Spinal Muscular Atrophy . NEngl JMed 2017 ;377:1713-22. 4. Kolb SJ, Coffey CS, Yankey JW, et al. Natural history of infantile-onset spinal muscular atrophy. Ann Neurol. 2017;82(6):883 891. 5. Govoni A, Gagliardi D, Comi GP, Corti S. Time is motor neuron: therapeutic window and its correlation with path ogenic mechanisms in spinal muscular atrophy. Mol Neur obiol. 2018;55(8):6307 6318. 6. Stifani N. Motor neurons and the generation of spinal motor neuron diversity. Front Cell Neurosci. 2014;8:293. 7. Prior TW. Perspectives and diagnostic considerations in spin al muscular atrophy. Genet Med. 2010;12(3):145 152.8. Farrar MA, et al. Emerging therapies and challenges in spinal muscular atrophy . Ann Neurol 2017;81(3):355 368. 9. De Sanctis R, et al.Developmental milestones in type I spinal muscular atrophy . Neuromusc Disord 2016;26(11):754 759. 10. Lowes LP, et al. Impact of age and motor function in a phase 1/2A study of infants with SMA Type 1 receiving single-dose gene replacement therapy. Pediatric Neurology (2019). 11. Waldrop MA, et al. Current Treatment Options in Neurology SMA Therapeutics. Curr Treatment Options Beurology. 2019;21(6):25. Effective date: 07/01/2019 Revised date: 05/31/2019

Emgality (galcanezumab-gnlm)

PHARMACY POLICY STATEMENT Kentucky Medicaid DRUG NAME Emgality (galcanezumab-gnlm) BILLING CODE Must use valid NDC code BENEFIT TYPE Pharmacy SITE OF SERVICE ALLOWED Home COVERAGE REQUIREMENTS Prior Authorization Required (Non-Preferred Product) Alternative preferred product includes Botox QUANTITY LIMIT see Dosage Allowed below LIST OF DIAGNOSES CONSIDERED NOT MEDICALLY NECESSARY Click Here Emgality (galcanezumab-gnlm) is a non-preferred product and will only be considered for coverage under the pharmacy benefit when the following criteria are met: Members must be clinically diagnosed with one of the following disease states and meet their individual criteria as stated. MIGRAINE HEADACHE PROPHYLAXIS For initial authorization: 1. Member is 18 years of age or older with a history of migraine attacks with or without aura; AND 2. Member has documented history of 15 headache days per month for more than 3 months, of which 8 days were migraine days characterized as 5 attacks lasting 4-72 hours with both of the following: a) Tw o or more of the following: i) Aggravation by or causing avoidance of routine physical activity; ii) Moderate or severe pain intensity; iii) Pulsating quality; iv) Unilateral location; b) One or more of the following: i) Nausea or vomiting; ii) Photophobia and phonophobia; AND 3. Medication must be prescribed by neurologist or a headache specialist; AND 4. Other prophylactic therapeutic options have been ineffective or not tolerated for trial of at least 30 days , as indicated by two or more of the following: a) Beta-blockers; b) Antidepressants such as amitriptyline, nortriptyline, doxepin, or protriptyline; c) Anticonvulsant medications such as topiramate or valproic acid; d) Calcium channel blockers; AND 5. Abortive therapeutic options ( i.e., ergotamine, triptans, combination analgesics , or simple analgesics) have been ineffective or not tolerated for at least 30 days (for a minimum of 8 or more days per month ); AND 6. Medication is not being used in combination with botulinum toxin therapy ; AND 7. Member does not have ANY of the following: a) Medication overuse headache; b) Pregnant or nursing female; c) History of cluster or hemiplegic headache,=ophthalmoplegic migraine, and migraine with brainstem aura (basilar-type migraine) ; d) Member was older than 50 years of age at migraine onset . 8. Dosage allowed: Subcutaneously, 240 mg loading dose (administered as two consecutive injections of 120 mg each), followed by monthly doses of 120 mg . Note: Emgality is considered experimental and investigational as combination therapy with Botox, Ajovy or Aimovig because the safety and effectiveness of these combinations has not been established. If member meets all the requirements listed above, the medication will be approved for 6 months. For reauthorization: 1. Member has improvement in prevention of migraines documented in chart notes (e.g., reduced migraine frequency, reduced use of medication for acute migraines attacks) . If member meets all the reauthorization requirements above, the medication will be approved for an additional 12 months . CareSource considers Emgality (galcanezumab-gnlm) not medically necessary for the treatment of the following disease states based on a lack of robust clinical controlled trials showing superior efficacy compared to currently available treatments: Cluster or hemiplegic migraine headache DATE ACTION/DESCRIPTION 03/05/2019 New policy for Emgality created. References: 1. Emgality [package insert]. Indianapolis, IN: Eli Lilly and Company; September, 2018. 2. ICHD-3 The International Classification of Headache Disorders. www.ichd-3.org. 3. Katsarava Z, Buse DC, Manack AN, Lipton RB. Defining the Differences Between Episodic Migraine and Chronic Migraine. Current Pain and Headache Reports. 2012;16(1):86-92. doi:10.1007/s11916-011-0233-z. 4. ClinicalTrials.gov. Identifier: NCT02614183.Evaluation of Galcanezumab in the Prevention of Episodic Migraine-the EVOLVE-1 Study (EVOLVE-1) . Available at: https://clinicaltrials.gov/ct2/show/NCT02614183?term=NCT02614183&rank=1. 5. ClinicalTrials.gov. Identifier: NCT02614196. Evaluation of Efficay & Safety of Galcanezumab in the Prevention of Episodic Migraine-the EVOLVE-2 Study (EVOLVE-2). Available at: https://clinicaltrials.gov/ct2/show/NCT02614196?term=NCT02614196&rank=1. 6. Detke HC, et al. Galcanezumab in chronic migraine: The randomized, double-blind, placebo-control led REGAIN study. Neurology. 2018;91(24):e2211-e2221. Effective date: 07/01/2019 Revised date: 03/05/2019

Ajovy (fremanezumab-vfrm)

PHARMACY POLICY STATEMENT Kentucky Medicaid DRUG NAME Ajovy (fremanezumab-vfrm) BILLING CODE Must use valid NDC code BENEFIT TYPE Pharmacy SITE OF SERVICE ALLOWED Home COVERAGE REQUIREMENTS Prior Authorization Required (Non-Preferred Product) Alternative preferred product includes Botox QUANTITY LIMIT see Dosage allowed below LIST OF DIAGNOSES CONSIDERED NOT MEDICALLY NECESSARY Click Here Ajovy (fremanezumab-vfrm) is a non-preferred product and will only be con sidered for coverage under the pharmacy benefit when the following criteria are met: Members must be clinically diagnosed with one of the following disease states and meet their individual criteria as stated. MIGRAINE HEADACHE PROPHYLAXIS For initial authorization: 1. Member is 18 years of age or older with a history of migraine attacks with or without aura; AND 2. Member has documented history of 15 headache days per month for more than 3 months, of which 8 days were migraine days characterized as 5 attacks lasting 4-72 hours with both of the following: a) Tw o or more of the following: i) Aggravation by or causing avoidance of routine physical activity; ii) Moderate or severe pain intensity; iii) Pulsating quality; iv) Unilateral location; b) One or more of the following: i) Nausea or vomiting; ii) Photophobia and phonophobia; AND 3. Medication must be prescribed by neurologist or a headache specialist; AND 4. Other prophylactic therapeutic options have been ineffective or not tolerated for trial of at least 30 days , as indicated by two or more of the following: a) Beta-blockers; b) Calcium channel blockers; c) Antidepressants such as amitriptyline, nortriptyline, doxepin, or protriptyline; d) Anticonvulsant medications such as topiramate or valproic acid; AND 5. Abortive therapeutic options ( i.e., ergotamine, triptans, combination analgesics , or simple analgesics) have been ineffective or not tolerated for at least 30 days (for a mi nimum of 8 or more days per month ); AND 6. Medication is not being used in combination with botulinum toxin therapy ; AND 7. Member does not have ANY of the following: a) Medication overuse headache; b) Pregnant or nursing female; c) Evidence or medical history of clinically significant psychiatric issues, including any suicide attempt in the past, or suicidal ideation with a specific plan in the past 2 years; d) History of clinically significant cardiovascul ar disease or vascular ischemia, deep vein thrombosis, or pul monary embolism ; e) Known infection or history of human immunodeficiency virus, tuberculosis, or chronic hepatitis Bor Cinfection ; f) Past or current history of cancer in the last 5 years, except for appropriately treated non-melanoma skin carcinoma; g) Member was older than 50 years of age at migraine onset . 8. Dosage allowed: Subcutaneously 225 mg monthly, or 675 mg every 3 months (quarterly) . Note: Ajovy is considered experimental and investigational as combination therapy with Botox, Aimovig or Emgality because the safety and effectiveness of these combinations has not been established. If member meets all the requirements listed above, the medication will be approved for 6 months. For reauthorization: 1. Member has improvement in prevention of migraines documented in chart notes (e.g., reduced migraine frequency, reduced use of medication f or acute migraines attacks). If member meets all the reauthorization requirements above, the medication will be approved for an additional 12 months . CareSource considers Ajovy (fremanezumab-vfrm) not medically necessary for the treatment of the diseases that are not listed in this document. DATE ACTION/DESCRIPTION 03/05/2019 New policy for Ajovy created. References: 1. Ajovy [package insert]. North Wales, PA: Teva Pharmaceuticals USA, Inc; January 2019. 2. ICHD-3 The International Classification of Headache Disorders. www.ichd-3.org. 3. Katsarava Z, Buse DC, Manack AN, Lipton RB. Defining the Differences Between Episodic Migraine and Chronic Migraine. Current Pain and Headache Reports. 2012;16(1):86-92. doi:10.1007/s11916-011-0233-z. 4. ClinicalTrials.gov. Identifier: NCT02621931. Comparing Efficacy and Safety of 2 Dose Regimens of Subcutaneous Administration of TEV-48125 Versus Plac ebo for the Preventive Treatment of Chronic Migraine. Available at:https://clinicaltrials.gov/ct2/show/NCT02621931?term=02621931&rank=1. 5. ClinicalTrials.gov. Identifier: NCT02629861. Efficacy and Safety of 2 Dose Regimens of TEV-48125 Versus Placebo for the Preventive Treatment of Episodic Migraine. Available at: https://clinicaltrials.gov/ct2/show/NCT02629861?term=02629861&rank=1. Effective date: 07/01/2019 Revised date: 03/05/2019

Aimovig (erenumab-aooe)

PHARMACY POLICY STATEMENT Kentucky Medicaid DRUG NAME Aimovig (erenumab-aooe) BILLING CODE Must use valid NDC code BENEFIT TYPE Pharmacy SITE OF SERVICE ALLOWED Home COVERAGE REQUIREMENTS Prior Authorization Required (Non-Preferred Product) Alternative preferred product includes Botox QUANTITY LIMIT up to 140 mg per month LIST OF DIAGNOSES CONSIDERED NOT MEDICALLY NECESSARY Click Here Aimovig (erenumab-aooe) is a non-preferred product and will only be con sidered for coverage under the pharmacy benefit when the following criteria are met: Members must be clinically diagnosed with one of the following disease states and meet their individual criteria as stated. MIGRAINE HEADACHE PROPHYLAXIS For initial authorization: 1. Memb er is 18 years of age or older with a history of migraine attacks with or without aura; AND 2. Member has documented history of 15 headache days per month for more than 3 months, of which 8 days were migraine days characterized as 5 attacks lasting 4-72 hours with both of the following: a) Tw o or more of the following: i) Aggravation by or causing avoidance of routine physical activ ity; ii) Moderate or severe pain intensity; iii) Pulsating quality; iv) Unilateral location; b) One or more of the following: i) Nausea or vomiting; ii) Photophobia and phonophobia; AND 3. Medication must be prescribed by neurologist or a headache specialist; AND 4. Other prophylactic therapeutic options have been ineffective or not tolerated for trial of at least 30 days , as indicated by two or more of the following: a) Beta-blockers; b) Calcium channel blockers; c) Antidepressants such as amitriptyline, nortriptyline, doxepin, or protriptyline; d) Anticonvulsant medications such as topiramate or valproic acid ; AND 5. Abortive therapeutic options (i.e., ergotamine, triptans, combination analgesics , or simple analgesics) have been ineffecti ve or not tolerated for at least 30 days ( for a minimum of 8 or more days per month ); AND 6. Member has not received botulinum toxin injection for headache prophylaxis in the past 4 months ; AND 7. Member does not have ANY of the following: a) Medication overuse headache; b) Pregnant or nursing female; c) History of cluster or hemiplegic headache; d) Cardiac or hepatic disease; e) Member was older than 50 years of age at migraine onset. 8. Dosage allowed: 70 mg subcutaneous injection once a month. Some patients may benefit from a dosage of 140 mg once monthly. The 140 mg dose is administered once monthly as two consecutive injections of 70 mg each. Note: Aimovig is considered experimental and investigational as combination therapy with Botox, Ajovy or Emgality because the safety and effectiveness of these combinations has not been established. If member meets all the requirements listed above, the medication will be approved for 6 months. For reauthorization: 1. Member has improvement in prevention of migraines documented in chart notes (e.g., reduced migraine frequency, reduced use of medication for acute migraines attacks) ; AND 2. Member has not received botulinum toxin injection for headache prophylaxis in the past 4 months . If member meets all the reauthorization requirements above, the medication will be approved for an additional 12 months. CareSource considers Aimovig (erenumab-aooe) not medically necessary for the treatment of the following disease states based on a lack of robust clinical controlled trials showing superior efficacy compared to currently available treatments: Cluster or hemiplegic migraine headache DATE ACTION/DESCRIPTION 08/03/2018 New policy for Aimovig created. 03/05/2019 Criterion on pregnant or nursing females added. Initial authorization length increased to 6 months and reauthorization length increased to 12 months. References: 1. Aimovig [package insert]. Thousand Oaks, CA: Amgen Inc.; May 2018. 2. ClinicalTrials.gov. Identifier: NCT 03096834. A Study Evaluating the Effectiveness of AMG 334 Injection in Preventing Migraines in Adults Having Failed Other Therapies (LIBERTY). Available: https://clinicaltrials.gov/ct2/show/NCT03096834?term=NCT03096834&rank=1. 3. ClinicalTrials.gov. Identifier: NCT 02456740. Study to Evaluate the Efficacy and Safety of AMG 334 in Migraine Prevention (STRIVE). Available at: https://clinicaltrials.gov/ct2/show/NCT02456740?term=NCT+02456740&rank=1. 4. ICHD-3 The International Classification of Headache Disorders. www.ichd-3.org. 5. Katsarava Z, Buse DC, Manack AN, Lipton RB. Defining the Differences Between Episodic Migraine and Chronic Migraine. Current Pain and Headache Reports. 2012;16(1):86-92. doi:10.1007/s11916-011-0233-z. 6. ClinicalTrials.gov. Identifier: NCT 02066415. A Study to Evaluate the Efficacy and Safety of AMG 334 in Chronic Migraine Prevention. Available at: https://clinicaltrials.gov/ct2/show/NCT02066415?term=NCT+02066415&rank=1. 7. Tepper S, et al. Safety and efficacy of erenumab for preventive treatment of chronic migraine: a randomized, double-blind, placebo-controlled phase 2 trial. The Lancet Neurology. 2017;16(6): 425-434. Effective date: 07/01/2019 Revised date: 03/05/2019

Spravato (esketamine)

PHARMACY POLICY STATEMENT Kentucky Medicaid DRUG NAME Spravato (esketamine) BILLING CODE Must use valid NDC code BENEFIT TYPE Pharmacy or Medical SITE OF SERVICE ALLOWED Office COVERAGE REQUIREMENTS Prior Authorization Required (Non-Preferred Product) LIST OF DIAGNOSES CONSIDERED NOT MEDICALLY NECESSARY Click Here Spravato (esketamine) is a non-preferred product and will only be con sidered for coverage under the medical or pharmacy benefit when the following criteria are met: Members must be clinically diagnosed with the following disease state and meet the individual criteria as stated. TREATMENT RESISTANT DEPRESSION For initial authorization: 1. Member has diagnosis of treatment resistant depression; AND 2. Member is 18 years old or older; AND 3. Medication must be used in conjunction with an oral antidepressant ( e.g., citalopram, escitalopram, fluoxetine, paroxetine, sertraline, duloxetine, venlafaxine, amitriptyline, nortriptyline, bupropion, trazodone) ; AND 4. Member has had a 30-day trial and failure of at least two of the following: a) Selective Serotonin Reuptake Inhibi tor; b) Selective Norepinephrine Reuptake Inhibitor ; c) Tricyclic Antidepressant ; d) Monoamine Oxidase Inhibitor ; e) Bupropion; f) Mirtazapine ; g) Trazodone; AND 5. Documentation of the members baseline depression status using an appropriate rating scale ( e.g., PHQ-9, Clinically Useful Depression Outcome Scale, Quick Inventory of Depressive Symptomatology-Sel f Report 16 Item, MADRS, HAM-D) . 6. Dosage allowed: Weeks 1-4: Maximum of 8 kits per month for 56 mg device and 7 kits per month for 84 mg device; Weeks 5-8: Maximum o f 4 kits per month. If member meets all the requirements listed above, the medication will be approved for 2 months.For reauthorization: 1. Documented maintenance of clinical improvement in depression symptoms as measured by improvement from baseline score on an appropriate rating scale. 2. Dosage allowed: Dose: 4 kits per 28 days . Note: Healthcare site, dispensing pharmacy, and patient must all be enrolled in the Spravato REMS program. If member meets all the reauthorization requirements above, the medication will be approved for an additional 1 year. CareSource considers Spravato (esketamine) not medically necessary for the treatment of the diseases that are not listed in this document. DATE ACTION/DESCRIPTION 05/23/2019 New policy for Spravato created. References: 1. Spravato [package insert]. Titusville, NJ: Janssen Pharmaceuticals, Inc.; May, 2019. 2. A Randomized, Double-blind, Multicenter, Active-controlled Study to Evaluate the Efficacy, Safety, and Tolerability of Flexible Doses of Intranasal Esketamine Plus an Oral Antidepressant in Adult Subjects With Treatment-resistant Depression (TRANSFORM-2). Janssen Research & Development. NCT0241858. April 2019. Available at: https://clinicaltrials.gov/ct2/show/NCT02418585?term=02418585&rank=1. 3. An Open-label Long-term Extension Safety Study of Intranasal Esketamine in Treatment-resistant Depression (SUSTAIN-3). Janssen Research & Development. NCT02493868. May 2019. Available at: https://clinicaltrials.gov/ct2/show/NCT02493868?term=02493868&rank=1. 4. Gelenberg A., Freeman M., Markowitz J., et. al. Practice guideline for the treatment of patients with major depressive disorder . Am JPsychiatry. May 2010. Available at: https://psychiatryonline.org/pb/assets/raw/sitewide/practice_guidelines/guidelines/mdd.pdf. Effective da te: 07/01/2019 Revised date: 05/31/2019