OH-MED-P -366685PHARMACY POLICY STATEMENTOhio Medicaid DRUG NAME Rivfloza (nedosiran)BENEFIT TYPE Medical STATUS Prior Authorization Required Rivfloza is an LDHA-directed small interfering RNA indicated to lower urinary oxalate levels in children 9 years of age and older and adults with primary hyperoxaluria type 1 (PH1) and relatively preserved kidney function, e.g., eGFR 30 mL/min/1.73 m2. PH1, which is caused by mutations of the AGXT gene, is a rare autosomal recessive disease that mainly affects the kidneys. It results from buildup of oxalate, which normally is filtered through the kidneys and excreted in the urine. Stone formation (calcium oxalate) in the kidneys and urinary tract occurs, as well as elevated levels of calcium in the kidneys. Eventually, if kidney function declines far enough, oxalate can start to accumulate in other body tissues, leading to a variety of problems (systemic oxalosis).Rivfloza (nedosiran) will be considered for coverage when the following criteria are met:Primary Hyperoxaluria Type 1 (PH1) For initial authorization: 1. Member is at least 9 years of age ; AND 2. Medication must be prescribed by or in consultation with a urologist or nephrologist ; AND 3. Member has a diagnosis of primary hyperoxaluria type 1 confirmed by genetic testing that shows a mutation in the AGXT gene; AND 4. Member has documentation of elevated urinary oxalate levels (24-hour Uox excretion 0.7 mmol (per 1.73 m2 body surface area [BSA] in age
OH-MED-P-366685PHARMACY POLICY STATEMENTOhio Medicaid DRUG NAME Oxlumo (lumasiran)BENEFIT TYPE Medical STATUS Prior Authorization Required Oxlumo is an HAO1-directed small interfering ribonucleic acid (siRNA) indicated for the treatment of primary hyperoxaluria type 1 (PH1) to lower urinary and plasma oxalate levels in pediatric and adult patients. PH1, which is caused by mutations of the AGXT gene, is a rare autosomal recessive disease that mainly affects the kidneys. It results from buildup of oxalate, which normally is filtered through the kidneys and excreted in the urine. Stone formation (calcium oxalate) in the kidneys and urinary tract occurs, as well as elevated levels of calcium in the kidneys. Eventually, if kidney function declines far enough, oxalate can start to accumulate in other body tissues, leading to a variety of problems (systemic oxalosis).Oxlumo (lumasiran) will be considered for coverage when the following criteria are met:Primary Hyperoxaluria Type 1 (PH1)For initial authorization: 1. Medication must be prescribed by or in consultation with a urologist or nephrologist; AND 2. Member has a diagnosis of primary hyperoxaluria type 1 confirmed by genetic testing that shows a mutation in the AGXT gene; AND 3. Member has documentation of elevated urinary or plasma oxalate levels (UOx or POx) ; AND 4. Member has had an inadequate response to vitamin B6 (pyridoxine) after at least 3 months on optimal dose; AND 5. Member does not receive peritoneal dialysis (hemodialysis allowed) ; AND 6. Member has not received a liver transplant ; AND 7. Oxlumo will not be used in combination with Rivfloza. 8. Dosage allowed/Quantity limit: SubQ as below: Body Weight* Loading Dose Maintenance Dose (begin 1 month after the last loading dose) Less than 10 kg 6 mg/kg once monthly for 3 doses 3 mg/kg once monthly 10 kg to less than 20 kg 6 mg/kg once monthly for 3 doses 6 mg/kg once every 3 months (quarterly) 20 kg and above 3 mg/kg once monthly for 3 doses 3 mg/kg once every 3 months (quarterly) If all the above requirements are met , the medication will be approved for 6 months . OH-MED-P-366685For reauthorization : 1. Chart notes must show reduced level of urinary or plasma oxalate compared to baseline; AND 2. Member has maintained s table kidney function (i.e., no clinically significant decline of eGFR ); AND 3. Member has not received a liver transplant and is not on peritoneal dialysis. If all the above requirements are met, the medication will be approved for an additional 12 months . CareSource considers Oxlumo (lumasiran) not medically necessary for the treatment of conditions that are not listed in this document. For any other indication, please refer to the Off-Label policy.DATE ACTION/DESCRIPTION12/08/2020 New policy for Oxlumo created. 05/27/2022 Transferred to new template. Updated billing code. Updated references. Added increased fluid intake. In renewal, changed or stable kidney function to and stable kidney function and revised description. 10/18/2022 Changed initial approval duration from 12 months to 6 months. Updated and added references; updated criteria per expanded product labeling which addresses plasma oxalate and use in severe renal disease and hemo dialysis populations; peritoneal dialysis remains excluded . 10/18/2023 Added reference. Removed biopsy option for diagnosis confirmation. Removed urinary alkalinization trial requirement. Defined non-response to vitamin B6. Added no concurrent use with Rivfloza. 02/13/2024 Removed hyperhydration requirement. 06/25/2024 Removed specific level of oxalate reduction from B6 trial. 8/15/2024 Approved by ODM References: 1. Oxlumo (lumasiran) [prescribing information]. Cambridge, MA: Alnylam Pharmaceuticals Inc; 2023. 2. Cochat P, Hulton S, Acquaviva C, et al: Primary hyperoxaluria Type 1: indications for screening and guidance for diagnosis and treatment. Nephrol Dial Transplant 2012;27:1729-1736 doi: 10.1093/ndt/gfs078. 3. Milliner DS, Harris PC, Sas DJ, et al. Primary Hyperoxaluria Type 1. 2002 Jun 19 [Updated 2022 Feb 10]. In: Adam MP, Ardinger HH, Pagon RA, et al., editors. GeneReviews [Internet]. Seattle (WA): University of Washington, Seattle; 1993-2022. Available from: https://www.ncbi.nlm.nih.gov/books/NBK1283/4. Gupta A, Somers MJG, Baum MA. Treatment of primary hyperoxaluria type 1. Clin Kidney J. 2022;15(Suppl 1):i9-i13. Published 2022 May 17. doi:10.1093/ckj/sfab232 5. Hulton SA, Groothoff JW, Frishberg Y, et al. Randomized Clinical Trial on the Long-Term Efficacy and Safety of Lumasiran in Patients With Primary Hyperoxaluria Type 1. Kidney Int Rep. 2021;7(3):494-506. Published 2021 Dec 11. doi:10.1016/j.ekir.2021.12.001 6. Hayes W, Sas DJ, Magen D, et al. Efficacy and safety of lumasiran for infants and young children with primary hyperoxaluria type 1: 12-month analysis of the phase 3 ILLUMINATE-B trial [published online ahead of print, 2022 Aug 1]. Pediatr Nephrol . 2022;10.1007/s00467-022-05684-1. doi:10.1007/s00467-022-05684-1 7. Michael M, Groothoff JW, Shasha-Lavsky H, et al. Lumasiran for Advanced Primary Hyperoxaluria Type 1: Phase 3 ILLUMINATE-C Trial [published online ahead of print, 2022 Jul 14]. Am JKidney Dis . 2022;S0272-6386(22)00771-5. doi:10.1053/j.ajkd.2022.05.012 8. Groothoff JW, Metry E, Deesker L, et al. Clinical practice recommendations for primary hyperoxaluria: an expert consensus statement from ERKNet and OxalEurope. Nat Rev Nephrol. 2023;19(3):194-211. doi:10.1038/s41581-022-00661-1 OH-MED-P -3666859. Ohio Administrative Code. (2022, February 23). 5160-1-01 (C) Medicaid medical necessity: definitions and principles. Retrieved February 22 2023 from codes.ohio.gov. 10. Ohio Administrative Code. (2022, July 18). 5160-26-03 Managed care: covered services. Retrieved February 22, 2023 from codes.ohio.gov. 11. Ohio Administrative Code. (2020, January 1). 5160-9-03 Pharmacy services: covered drugs and associated limitations. Retrieved February 22, 2023 from codes.ohio.gov. Effective date: 10/01/2024 Revised date: 06/25/2024
OH-MED-P -366685PHARMACY POLICY STATEMENTOhio Medicaid DRUG NAME Xenpozyme (olipudase alfa-rpcp )BENEFIT TYPE Medical STATUS Prior Authorization Required Xenpozyme, approved by the FDA in 2022, is a hydrolytic lysosomal sphingomyelin-specific enzyme indicated for treatment of non central nervous system manifestations of acid sphingomyelinase deficiency (ASMD) inadult and pediatric patients . Xenpozyme provides the enzyme that is deficient or absent in ASMD. Without the enzyme acid sphingomyelinase (ASM), a complex lipid called sphingomyelin builds up in cells which can lead to multiorgan symptoms such as decreased lung function, enlarged liver and spleen, decreased platelet count, and growth delay in children, among many other manifestations . ASMD , a lysosomal storage disorder, is also known as Niemann-Pick disease and can be differentiated as type A, A/B, and B. Xenpozyme has not been studied in patients with ASMD type A. It is not expected to cross the blood-brain barrier or improve CNS manifestations. ASMD is an extremely rare genetic disease with fewer than 120 ASMD diagnoses in the U.S. Signs and symptoms may present in infancy, childhood, or adulthood with about two-thirds of diagnoses in the U.S. in pediatrics. Xenpozyme was the first approved treatment for ASMD. Xenpozyme ( olipudase alfa-rpcp ) will be considered for coverage when the following criteria are met: Acid Sphingomyelinase D eficiency (ASMD)For initial authorization: 1. Medication must be prescribed by or in consultation with a physician knowledgeable in the management of ASMD , such as an endocrinologist, hepatologist, or pulmonologist ; AND 2. Member has a confirmed diagnosis of ASMD with documentation of at least one of the following: a) ASM enzyme activity
OH-MED-P-366685PHARMACY POLICY STATEMENTOhio Medicaid DRUG NAME Vyepti (eptinezumab-jjmr)BENEFIT TYPE Medical STATUS Prior Authorization Required Vyepti is a calcitonin gene-related peptide receptor antagonist initially approved by the FDA in 2020. It is indicated for the preventive treatment of migraine in adults. Vyepti works as a humanized immunoglobulin G1 (IgG1) monoclonal antibody that binds to the calcitonin gene-related peptide (CGRP) ligand and blocks its binding to the receptor. The efficacy of Vyepti was evaluated as a preventive treatment of episodic and chronic migraine in two randomized, multicenter, placebo-controlled studies, both with 6-month double-blind periods: one study in patients with episodic migraine (Study 1) and one study in patients with chronic migraine (Study 2). Patients treated with Vyepti in both trials had greater decreases from baseline in mean monthly migraine days over Months 1-3 compared to placebo-treated patients.Vyepti (eptinezumab-jjmr) will be considered for coverage when the following criteria are met:Chronic Migraine Headache ProphylaxisFor initial authorization: 1. Member is 18 years old or older; AND 2. Medication is being prescribed for prevention of chronic migraine, defined as both of the following: a) 15 headache days per month for at least 3 months b) 8 migraine days per month for at least 3 months; AND 3. Member has tried and failed at least 1 of the following prophylactic medications for 8 weeks: a) Beta blocker (e.g., metoprolol, timolol, or propranolol) b) Calcium channel blocker (e.g., verapamil) c) Antidepressant (e.g., amitriptyline or venlafaxine) d) Anticonvulsant (e.g ., topiramate or valproic acid) e) Candesartan ; AND 4. Member has tried and failed a preferred CGRP; AND 5. If the dosage requested is 300mg, member must have a 3-month trial of the 100mg Vyepti dose; AND 6. Medication is not being used in combination with botulinum toxin therapy or any other prophylactic CGRP product ( e.g., Aimovig, Ajovy, or Emgality). 7. Dosage allowed: 100mg administered intravenously every 3 months. A dose of 300mg may also be used. Quantity Limit: 300mg (3 vials) per 84 days. If all the above requirements are met, the medication will be approved for 6 months. For reauthorization : 1. Chart notes have been provided showing improvement in migraine frequency and severity (e.g., reduced migraine days, reduced use of medications for acute migraines attacks). If all the above requirements are met, the medication will be approved for an additional 12 months . OH-MED-P-366685Episodic Migraine Headache ProphylaxisFor initial authorization: 1. Member is 18 years old or older; AND 2. Medication is being prescribed for prevention of episodic migraine, defined as both of the following: a. 14 headache days per month for at least 3 months b. 4 or more migraine days per month for at least 3 months that cause significant impairment to quality of life ( i.e., requiring bed rest, missed school/work) ; AND 3. Member has tried and failed at least 1 of the following prophylactic medications for 8 weeks: a. Beta blocker (e.g., metoprolol, timolol, or propranolol) b. Calcium channel blocker (e.g., verapamil) c. Antidepressant (e.g., amitriptyline or venlafaxine) d. Anticonvulsant (e.g., topiramate or valproic acid) e. Candesartan; AND 4. Member has tried and failed a preferred CGRP; AND 5. If the dosage requested is 300mg , member must have a 3-month trial of the 100mg Vyepti dose; AND 6. Medication is not being used in combination with botulinum toxin therapy or any other prophylactic CGRP product ( e.g., Aimovig, Ajovy, or Emgality ). 7. Dosage allowed: 100mg (1 vial) administered intravenously every 3 months. A dose of 300mg may also be used. Quantity Limit: 300mg (3 vials) per 84 days. If all the above requirements are met , the medication will be approved for 6 months . For reauthorization : 1. Chart notes have been provided showing improvement in migraine frequency and severity (e.g., reduced migraine days, reduced use of medications for acute migraines attacks). If all the above requirements are met , the medication will be approved for an additional 12 months . CareSource considers Vyepti (eptinezumab-jjmr) not medically necessary for the treatment of conditions that are not listed in this document. For any other indication, please refer to the Off-Label policy.DATE ACTION/DESCRIPTION05/22 /20 20 New policy for Vyepti created.05/05/2022 Transferred to new template . Updated references. Removed prescriber specialty and abortive trials . Added quantity limit. 06/30/2023 Removed Botox trial and the following: Member does not have ANY of the following: Medication overuse headache; History of hemiplegic headache, ophthalmoplegic migraine, and migraine with brainstem aura (basilar-type migraine); Member was older than 50 years of age at migraine onset. Removed CGRP trial from the 300 mg dosage request for initial and reauthorization criteria. Added preferred CGRP trial. 04/29/2024 Updated references. Changed from 2 prior prophylactic trials to 1 and added candesartan to list of trial options (per AHS 2024 statement). 8/15/2024 Approved by ODM References: 1. Vyepti [package insert]. Deerfield, IL: Lundbeck Seattle BioPharmaceuticals, Inc. ; 2022. OH-MED-P -3666852. Ashina M, Saper J, Cady R, Schaeffler B, Biondi D, Hirman J, Pederson S, Allan B, Smith J. Eptinezumab in episodic migraine: the randomized, double-blind, placebo-controlled PROMISE-1 study. Cephalalgia. 2020 Mar; 40(3):241-254. 3. Lipton RB, Goadsby PJ, Smith J, Schaeffler BA, Biondi DM, Hirman J, Pederson S, Allan B, Cady R. Efficacy and safety of eptinezumab in patients with chronic migraine. PROMISE-2. Neurology . 2020 Mar 31; 94(13):e31364-e1377 4. Buse D, Manack A, Serrano D, et al. Headache impact of chronic and episodic migraine: results from the American Migraine Prevalence and Prevention study. Headache. 2012;52(1):3 17. doi:10.1111/j.1526-4610.2011.02046.x 5. Headache Classification Committee of the International Headache Society (IHS) The International Classification of Headache Disorders. Cephalalgia. 2018 Jan;38(1):1-211. 6. The American Headache Society Position Statement on Integrating New Migraine Treatments into Clinical Practice. Headache: The Journal of Head and Face Pain. 2019;59: 1-18. 7. Ailani J, Burch R, et al. Consensus Statement: The American Headache Society Consensus Statement: Update on integrating new migraine treatments into clinical practice. Headache. 2021 Jul;61(7):1021-1039. 8. Charles AC, Digre KB, Goadsby PJ, Robbins MS, Hershey A; American Headache Society. Calcitonin gene-related peptide-targeting therapies are a first-line option for the prevention of migraine: An American Headache Society position statement update. Headache. 2024;64(4):333-341. doi:10.1111/head.14692 Effective date: 10/01/2024 Revised date: 04/29/2024
OH-MED-P -366685PHARMACY POLICY STATEMENTOhio Medicaid DRUG NAME Triptodur (triptorelin)BENEFIT TYPE Medical STATUS Prior Authorization Required Triptodur , initially approved by the FDA in 2000, is a gonadotropin releasing hormone (GnRH) agonist indicated for the treatment of pediatric patients 2 years and older with central precocious puberty . CPP refers to early activation of the hypothalamic pituitary gonadal axis which results in premature development of secondary sexual characteristics. Most cases are seen in girls without an identifiable cause. In contrast, about half of cases in boys do have an identifiable cause. When left untreated, CPP can result in early epiphyseal fusion and a substant ial compromise in adult height. The goal of treatment is preservation of height therefore GnRH agonists are the standard of care .Triptodur (triptorelin) will be considered for coverage when the following criteria are met:Central Precocious Puberty (CPP )For initial authorization: 1. Member is 2 years old or older; AND 2. Medication must be prescribed by or in consultation with an endocrinologist; AND 3. Member has early onset of pubertal symptoms before the age of 8 years f or females or 9 years for male s; AND 4. Member has confirmed diagnosis of central precocious puberty, as evidenced by BOTH of the following: a. Pubertal response to a gonadotropin releasing hormone (GnRH) stimulation test OR pubertal levels of basal luteinizing hormones (LH); b. Advanced bone age for chronological age; AND 5. Members baseline LH level, sex steroid level (estradiol or testosterone), and height are submitted with chart notes. 6. Dosage allowed/Quantity limit: Inject 22.5 mg intramuscularly once every 24 weeks. Quantity Limit: 1 kit per 24 weeks . If all the above requirements are met , the medication will be approved for 6 months . For reauthorization : 1. Chart notes have been provided showing efficacy of response (e.g., slowed growth rate, slowed bone age advancement, LH and sex steroid hormone levels have been suppressed or reduced to prepubertal levels) ; AND 2. If member is 11 years or older for females or 12 years or older for males, prescriber must provide a clinical reason for continuing medication beyond the recommended age for resuming puberty . If member meets all the reauthorization requirements above, the medication will be approved for an additional 12 months. OH-MED-P -366685 CareSource considers Triptodur (triptorelin) not medically necessary for the treatment of conditions that are not listed in this document. For any other indication, please refer to the Off-Label policy.DATE ACTION/DESCRIPTION10/11/2019 New Policy for Triptodur created. 07/28/2020 Diagnostic requirements (#3) updated to require both conditions: advanced bone age and GnRH stimulation test or pubertal hormone levels; removed ruled out diagnoses; removed list of secondary puberty signs and symptoms (redundancy); removed baseline weight; specified baseline LH hormones; Added requirement for discontinuation of treatment in reauth; added prescriber requirement. 03/18/2024 Updated references; r emoved requirement of estradiol/testosterone level from LH testing; simplified bone age requirement from 1 year or greater to advanced; increased reauthorization length to 12 months; rephrased quantity limit to be in line with package size. 8/15/2024 Approved by ODM References: 1. Triptodur [package insert]. Woburn, MA: Azurity Pharmaceuticals, Inc .; 2022. 2. ClinicalTrials.gov Identifier: NCT00564850. Efficacy and Safety Study of Pamoate of Triptorelin in Children With Precocious Puberty (DECAPUB). Available at: https://clinicaltrials.gov/ct2/show/NCT00564850. 3. Fuqua JS. Treatment and outcomes of precocious puberty: an update. JClin Endocrinol Metab. 2013;98(6):2198-2207. doi:10.1210/jc.2013-1024 4. Chen M, Eugster EA. Central Precocious Puberty: Update on Diagnosis and Treatment. Paediatr Drugs . 2015;17(4):273-281. 5. Carel JC, Eugster EA, Rogol A, et al; ESPE-LWPES GnRH Analogs Consensus Conference Group. Consensus statement on the use of gonadotropin-releasing hormone analogs in children. Pediatrics. 2009;123(4). 6. Kletter GB, Klein KO, Wong YY. A pediatrician's guide to central precocious puberty. Clin Pediatr (Phila). 2015;54(5):414-424. doi:10.1177/0009922814541807 7. Zevin EL, Eugster EA. Central precocious puberty: a review of diagnosis, treatment, and outcomes. Lancet Child Adolesc Health. 2023;7(12):886-896. doi:10.1016/S2352-4642(23)00237-7 Effective date: 10/01/2024 Revised date: 03/19/2024
OH-MED-P -366685PHARMACY POLICY STATEMENTOhio Medicaid DRUG NAME Spevigo ( spesolimab-sbzo )BENEFIT TYPE Medical or pharmacy STATUS Prior Authorization Required Spevigo, approved by the FDA in 2022, is an interleukin-36 receptor antagonist indicated for the treatment of generalized pustular psoriasis (GPP) in adults and pediatric patients 12 years of age and older and weighing at least 40 kg. It was the first treatment specifically approved for GPP flares and the first IL-36 receptor antagonist to be approved. GPP is a rare, potentially life-threatening neutrophilic skin condition, and individuals with GPP typically experience episodes of widespread eruptions of painful, sterile pustules. While the severity of GPP flares can vary, if left untreated they can be l ife-threatening due to complications such as sepsis and multisystem organ failure. A preceding history of plaque psoriasis may or may not be present in individuals presenting with GPP.Spevigo (spesolimab-sbzo) will be considered for coverage when the following criteria are met:Generalized Pustular Psoriasis (GPP)For initial authorization: 1. Member is at least 12 years of age and weighs at least 40 kg ; AND 2. Medication must be prescribed by or in consultation with a dermatologist; AND 3. Member has a diagnosis of GPP; AND 4. Member is not experiencing a flare and has ALL the following: a. History of at least TWO GPP flares of moderate-to-severe intensity ; b. Generalized Pustular Psoriasis Physician Global Assessment (GPPPGA ) total score of 0 or 1 ; c. Member has a history of flaring while on concomitant treatment for GPP or a history of flaring upon dose reduction or discontinuation of these concomitant medications (such as retinoids, cyclosporine, methotrexate, etc.) ; OR 5. Member has an acute flare of GPP of moderate to severe intensity, defined by ALL the following: a. GPPPGA total score of at least 3; b. GPPPGA pustulation sub score of at least 2; c. Presence of fresh pustules (new appearance or worsening of pustules); d. At least 5% of body surface area covered with erythema and the presence of pustules; AND 6. Member has had a negative tuberculosis test within the past 12 months. 7. Dosage allowed/Quantity limit : a. If member is experiencing a flare: Administer a single 900 mg (2 vials) dose by intravenous infusion over 90 minutes. If flare symptoms persist, administer an additional intravenous 900 mg dose one week after the initial dose. Quantity Limit: 4 vials per 21 days. b. If member is not experiencing a flare: Administer a subcutaneous loading dose of 600 mg (four 150 mg injections), followed by 300 mg (two 150 mg injections) subcutaneously 4 weeks later and every 4 weeks thereafter. Quantity Limit: 6 syringes per 30 days for loading dose and 2 syringes per 30 days for maintenance dosing. OH-MED-P -366685If all the above requirements are met the medication will be approved for 3 weeks for members with flares and 12 weeks for members who are not experiencing a flare. For reauthorization : 1. If the request is for members experiencing a flare, the medication will not be approved for continuous use. 2. If the request is for members not experiencing a flare, c hart notes have been provided showing an improvement in signs and symptoms of disease (such as decrease in number of flares etc.) CareSource considers Spevigo ( spesolimab-sbzo) not medically necessary for the treatment of conditions that are not listed in this document. For any other indication, please refer to the Off-Label policy.DATE ACTION/DESCRIPTION09/21/2022 New policy for Spevigo created. 05/13/2024 Lowered age limit from 18 to 12 and added that member must be at least 40 kg; removed exclusion that member cannot have pustulation restricted to psoriatic plaques; changed quantity limit for flares from per 365 days to per 21 days ; added pharmacy benefit option for syringe; added criteria for if member is not experiencing a flare; added subcutaneous dosing information ; added references 8/15/2024 Approved by ODM References: 1. Spevigo [package insert]. Ridgefield, CT: Boehringer Ingelheim Pharmaceuticals, Inc.; 2024. 2. Bachelez H, Choon SE, Marrakchi S, et al. Trial of Spesolimab for Generalized Pustular Psoriasis. NEngl JMed. 2021;385(26):2431-2440. doi:10.1056/NEJMoa2111563 3. Navarini AA, Burden AD, Capon F, et al. European consensus statement on phenotypes of pustular psoriasis. JEur Acad Dermatol Venereol . 2017;31(11):1792-1799. doi:10.1111/jdv.14386 Hoegler KM, John AM, Handler MZ, Schwartz RA. Generalized pustular psoriasis: a review and update on treatment. JEur Acad Dermatol Venereol . 2018;32(10):1645-1651. doi:10.1111/jdv.14949 4. Morita A, Strober B, Burden AD, et al. Efficacy and safety of subcutaneous spesolimab for the prevention of generalised pustular psoriasis flares (Effisayil 2): an international, multicentre, randomised, placebo-controlled trial. Lancet. 2023;402(10412):1541-1551. doi:10.1016/S0140-6736(23)01378-8 5. Kodali N, Blanchard I, Kunamneni S, Lebwohl MG. Current management of generalized pustular psoriasis. Exp Dermatol. 2023;32(8):1204-1218. doi:10.1111/exd.14765 Effective date: 10/01/2024 Revised date: 05/13/2024
OH-MED-P -366685PHARMACY POLICY STATEMENTOhio Medicaid DRUG NAME Ryplazim (plasminogen, human-tvmh )BENEFIT TYPE Medica l STATUS Prior Authorization Required Ryplazim is a plasma-derived human plasminogen indicated for the treatment of patients with plasminogen deficiency type 1 (hypoplasminogenemia). It was approved by the FDA on June 4, 2021 and is the first approved treatment for plasminogen deficiency type 1. Individuals with this disease lack a protein called plasminogen, which is responsible for the ability of the body to break down fibrin clots. Plasminogen deficiency leads to an accumulation of fibrin, causing the development of growths (lesions) that can impair normal tissue and organ function and may lead to blindness when these lesions affect the eyes. Ligneous conjunctivitis (LC) appears to be the most common clinical manifestation and is characterized by inflamed, woody growths on the conjunctival membr anes that, if left untreated, can result in visual impairment or blindness. Treatment with Ryplazim temporarily increases plasminogen levels in blood. The effectiveness and safety of Ryplazim (plasminogen) is primarily based on one single-arm, open-label (unblinded) clinical trial enrolling 15 adult and pediatric patients with plasminogen deficiency type 1. All patients received Ryplazim administered every two to four days for 48 weeks. The effectiveness of Ryplazim was demonstrated by at least 50% improvement of their lesions in all 11 patients who had lesions at baseline, and abs ence of recurrent or new lesions in any of the 15 patients through the 48 weeks of treatment.Ryplazim (plasminogen, human-tvmh) will be considered for coverage when the following criteria are met:HypoplasminogenemiaFor initial authorization: 1. Member must be at least 11 months old; AND 2. Medication must be prescribed by or in consultation with a hematologist; AND 3. Member has a documented history of disease-related lesions and symptoms consistent with a diagnosis of hypoplasminogenemia; AND 4. Documentation of baseline plasminogen activity level 45%. 5. Dosage allowed/Quantity limit: 6.6 mg/kg body weight given intravenously every 2 to 4 days If all the above requirements are met , the medication will be approved for 12 weeks . For reauthorization : Ryplazim will be reauthorized when chart notes show at least ONE of the following: a) Absence of recurrent or new lesions; b) Decrease in the lesion number and/or size ; c) Increase in trough plasminogen activity level by at least 10% from baseline. If all the above requirements are met , the medication will be approved for an additional 12 months. OH-MED-P -366685 CareSource considers Ryplazim (plasminogen, human-tvmh) not medically necessary for the treatment of conditions that are not listed in this document. For any other indication, please refer to the Off-Label policy.DATE ACTION/DESCRIPTION10/06 /20 21 Ryplazim policy creation 05/17/2024 Added reference (Shapiro, et al 2023); removed geneticist as prescriber option; added in consultation with for prescriber. 8/15/2024 Approved by ODM References: 1. Ryplazim [package insert]. Laval, Quebec, CA; Prometric Bioproduction, Inc.; 2021. 2. Shapiro, Amy D. et al. Plasminogen replacement therapy for the treatment of children and adults with congenital plasminogen deficiency . Blood. 2018 Mar 22;131(12):1301-1310 3. Shapiro AD, Nakar C, Parker JM, Thibaudeau K, Crea R, Sandset PM. Plasminogen, human-tvmh for the treatment of children and adults with plasminogen deficiency type 1. Haemophilia . 2023;29(6):1556-1564. doi:10.1111/hae.14849 4. Shapiro AD, et al. An international registry of patients with plasminogen deficiency (HISTORY). Haematologica. 2020;105(3):554-561Effective date: 10/01/2024 Revised date: 05/17/2024
OH-MED-P-366685PHARMACY POLICY STATEMENTOhio Medicaid DRUG NAME Leqvio (inclisiran)BENEFIT TYPE Medical STATUS Prior Authorization Required Leqvio, approved by the FDA in 2021, is a small interfering RNA (siRNA) directed to proprotein convertase subtilisin kexin type 9 (PCSK9) messenger RNA ( mRNA) indicated as an adjunct to diet and statin therapy for the treatment of adults with primary hyperlipidemia, including heterozygous familial hypercholesterolemia (HeFH), to reduce low density lipoprotein cholesterol (LDL-C). Leqvio is a first-in-class siRNA that targets PCSK9 to inhibit its production in the liver, prolonging LDL receptor activity . Following induction, it is administered every 6 months by a healthcare professional, which may be beneficial for adherence in comparison to the PCSK9 monoclonal antibody (mAb) drugs. It was originally approved for the atherosclerotic cardiovascular disease (ASCVD) and HeFH populations, and later to include primary hyperlipidemia patients with increased risk for ASCVD.Leqvio (inclisiran) will be considered for coverage when the following criteria are met:H eterozygous Familial H ypercholesterolemia (HeFH)For initial authorization: 1. Member is at least 18 years of age; AND 2. Medication must be prescribed by or in consultation with cardiologist, endocrinologist, or lipid specialist; AND 3. Member has a diagnosis of HeFH as documented by one of the following: a) Genetic testing (presence of LDL-R, ApoB, or PCSK9 mutation) b) Dutch Lipid Network score greater than 8 points c) Definite per Simon Broome criteria (i.e., LDL > 190 at baseline AND tendon xanthoma OR LDL-R, ApoB, or PCSK9 mutation) ; AND 4. Member has a lipid panel within the past 90 days showing LDL of 100 or greater; AND 5. Members LDL is elevated despite at least a 3-month adherent trial of high intensity or max tolerated statin therapy in combination with ezetimibe (unless there is documentation of clearly established statin intolerance or statin contraindicationsee note*) ; AND 6. Leqvio will be taken as an adjunct to diet and maximum tolerated statin therapy; AND 7. Member will NOT be concomitantly taking a PCSK9 mA b (e.g., Repatha, Praluent) . 8. Dosage allowed/Quantity limit: 284 mg as a single subQ injection initially, again at 3 months, and every 6 months thereafter. (QL: 2 syringes per 84 days for the first fill, then 1 syringe per 180 days thereafter; 1 syringe = 1.5 mL) * Note : If not on statin therapy, member must have documented contraindication to all statin drugs or documentation of intolerance to at least 2 different statins, including low/moderate intensity or alternate dosing such as every other day . If all the above requirements are met , the medication will be approved for 6 months .OH-MED-P-366685For reauthorization :1. Documentation in chart notes must demonstrate clinically meaningful LDL reduction compared to pre-treatment baseline. If all the above requirements are met, the medication will be approved for an additional 12 months. Primary HyperlipidemiaFor initial authorization: 1. Member is at least 18 years of age; AND 2. Member has a diagnosis of ASCVD (e.g., coronary heart disease [CHD], cardiovascular disease [CVD], or peripheral arterial disease [PAD] ), OR, has a risk factor (e.g., hypertension, diabetes, chronic kidney disease); AND 3. Member has a lipid panel within the past 90 days showing one of the following: a) LDL of 70 or greater b) LDL of 55 or greater and very high risk, i.e., history of multiple major ASCVD events (acute coronary syndrome within past 12 months, history of MI, stroke, or symptomatic PAD) or 1 major ASCVD event and multiple high-risk conditions; AND 4. Members LDL is elevated despite at least a 3-month adherent trial of high intensity or max tolerated statin therapy in combination with ezetimibe (unless there is documentation of clearly established statin intolerance or statin contraindicationsee note*) ; AND 5. Leqvio will be taken as an adjunct to diet and maximum tolerated statin therapy; AND 6. Member will NOT be concomitantly taking a PCSK9 mA b (e.g., Repatha, Praluent) . 7. Dosage allowed/Quantity limit: 284 mg as a single subQ injection initially, again at 3 months, and every 6 months thereafter. (QL: 2 syringes per 84 days for the first fill, then 1 syringe per 180 days thereafter; 1 syringe = 1.5 mL) * Note : If not on statin therapy, member must have documented contraindication to all statin drugs or documentation of intolerance to at least 2 different statins, including low/moderate intensity or alternate dosing such as every other day . If all the above requirements are met , the medication will be approved for 6 months .For reauthorization :1. Documentation in chart notes must demonstrate clinically meaningful LDL reduction compared to pre-treatment baseline. If all the above requirements are met, the medication will be approved for an additional 12 months. CareSource considers Leqvio (inclisiran) not medically necessary for the treatment of conditions that are not listed in this document. For any other indication, please refer to the Off-Label policy.DATE ACTION/DESCRIPTION01/24/2022 New policy created for Leqvio . 07/28/2023 Updated policy for label expansion to include primary hyperlipidemia/primary prevention (increased risk for ASCVD) rather than just the original ASCVD/secondary prevention population. Updated concomitant exclusion terminology to say PCSK9 mAB instead of PCSK9 inhibitor. Added detail to statin intolerance note regarding low/moderate intensity and alternate dosing. Changed LDL cutoff to 90 days instead of OH-MED-P-36668530. Differentiated LDL cutoff of 55 for very high risk population. Added reference s.Added Simon Broome as option to meet diagnosis for HeFH. 05/03/2024 Added QL. 8/15/2024 Approved by ODM References: 1. Leqvio [prescribing information]. Novartis Pharmaceuticals Corporation; 202 3. 2. Raal FJ, Kallend D, Ray KK, et al. Inclisiran for the Treatment of Heterozygous Familial Hypercholesterolemia. NEngl JMed. 2020;382(16):1520-1530. doi:10.1056/NEJMoa1913805 3. Ray KK, Wright RS, Kallend D, et al. Two Phase 3 Trials of Inclisiran in Patients with Elevated LDL Cholesterol. NEngl JMed. 2020;382(16):1507-1519. doi:10.1056/NEJMoa1912387 4. McGowan MP, Hosseini Dehkordi SH, Moriarty PM, Duell PB. Diagnosis and Treatment of Heterozygous Familial Hypercholesterolemia. JAm Heart Assoc . 2019;8(24):e013225. doi:10.1161/JAHA.119.013225 5. Grundy SM, Stone NJ, Bailey AL, et al. 2018 AHA/ACC Guideline on the Management of Blood Cholesterol. JACC . 2018;73(24)doi:10.1016/j.jacc.2018.11.002. 6. Writing Committee, Lloyd-Jones DM, Morris PB, et al. 2022 ACC Expert Consensus Decision Pathway on the Role of Nonstatin Therapies for LDL-Cholesterol Lowering in the Management of Atherosclerotic Cardiovascular Disease Risk: A Report of the American College of Cardiology Solution Set Oversight Committee [published correction appears in JAm Coll Cardiol. 2023 Jan 3;81(1):104]. JAm Coll Cardiol . 2022;80(14):1366-1418. doi:10.1016/j.jacc.2022.07.006 7. Albosta MS, Grant JK, Taub P, Blumenthal RS, Martin SS, Michos ED. Inclisiran: A New Strategy for LDL-CLowering and Prevention of Atherosclerotic Cardiovascular Disease. Vasc Health Risk Manag. 2023;19:421-431. Published 2023 Jul 6. doi:10.2147/VHRM.S338424 Effective date: 10/01/2024 Revised date: 05/03/2024
OH-MED-P -366685PHARMACY POLICY STATEMENTOhio Medicaid DRUG NAME Lamzede (velmanase alfa-tycv)BENEFIT TYPE Medical STATUS Prior Authorization Required Lamzede, approved by the FDA in 2023, is recombinant human lysosomal alpha-mannosidase indicated for the treatment of non-central nervous system manifestations of alpha-mannosidosis in adult and pediatric patients. Alpha-mannosidosis (AM) is a rare, progressive lysosomal storage disorder caused by pathogenic variants in the MAN2B1 gene, resulting in accumulation of mannose-rich oligosaccharides. Lamzede is an enzyme replacement therapy (ERT) intended to provide alpha-mannosidase, the enzyme that is deficient in AM. It is the first approved treatment for AM but does not cross the blood brain barrier and therefore it not expected to benefit the neurological aspects of the disease. In a Phase 3 c linical trial, 3-minute stair climbing test (3MSCT), 6-minute walking test (6MWT) and forced vital capacity (FVC) numerically favored the Lamzede group and results were supported by a reduction in serum oligos accharide concentration.Lamzede (velmanase alfa-tycv) will be considered for coverage when the following criteria are met:A lpha-M annosidosisFor initial authorization: 1. Medication must be prescribed by or in consultation with a metabolic or genetics specialist, or other specialist experienced with lysosomal storage disorders; AND 2. Member has a diagnosis of alpha-mannosidosis confirmed by enzyme assay showing alpha-mannosidase activity less than 10% of normal; AND 3. Members disease is mild to moderate, without significant central nervous system (CNS) manifestations; AND 4. Member has NOT had a bone marrow transplant or HSCT. 5. Dosage allowed/Quantity limit: 1 mg/kg (actual body weight) once every week as IV infusion. If all the above requirements are met , the medication will be approved for 12 months. For reauthorization : 1. Chart notes must show improvement or stabilized signs and symptoms of disease demonstrated by at least one of the following: a) Clinically significant reduction of serum oligosaccharide concentration from baseline b) Stable or improved 3MSCT, 6MWT, or FVC If all the above requirements are met , the medication will be approved for an additional 12 months . CareSource considers Lamzede (velmanase alfa-tycv) not medically necessary for the treatment of conditions that are not listed in this document. For any other indication, please refer to the Off-Label policy. OH-MED-P -366685DATE ACTION/DESCRIPTION04/13/2023 New policy for Lamzede created.04/25/2024 Annual review; no updates.8/15/2024 Approved by ODM References: 1. Lamzede [prescribing information]; Chiesi USA, Inc.; 2023. 2. Borgwardt L, Guffon N, Amraoui Y, et al. Efficacy and safety of Velmanase alfa in the treatment of patients with alpha-mannosidosis: results from the core and extension phase analysis of a phase III multicentre, double-blind, randomised, placebo-controlled trial. JInherit Metab Dis . 2018;41(6):1215-1223. doi:10.1007/s10545-018-0185-0 3. Lund AM, Borgwardt L, Cattaneo F, et al. Comprehensive long-term efficacy and safety of recombinant human alpha-mannosidase (velmanase alfa) treatment in patients with alpha-mannosidosis. JInherit Metab Dis . 2018;41(6):1225-1233. doi:10.1007/s10545-018-0175-2 4. Guffon N, Tylki-Szymanska A, Borgwardt L, et al. Recognition of alpha-mannosidosis in paediatric and adult patients: Presentation of a diagnostic algorithm from an international working group. Mol Genet Metab. 2019;126(4):470-474. doi:10.1016/j.ymgme.2019.01.024 5. Malm D, Nilssen . Alpha-Mannosidosis. 2001 Oct 11 [Updated 2019 Jul 18]. In: Adam MP, Mirzaa GM, Pagon RA, et al., editors. GeneReviews [Internet]. Seattle (WA): University of Washington, Seattle; 1993-2023. Available from: https://www.ncbi.nlm.nih.gov/books/NBK1396/Effective date: 10/01/2024 Revised date: 04/25/2024
OH-MED-P -366685PHARMACY POLICY STATEMENTOhio Medicaid DRUG NAME Kanuma (sebelipase alfa)BENEFIT TYPE Medical STATUS Prior Authorization Required Kanuma, approved by the FDA in 2015, is a recombinant human lysosomal acid lipase ( a lysosomal enzyme) indicated for the treatment of Lysosomal Acid Lipase (LAL) deficiency . LAL deficiency is a lysosomal storage disorder caused by a genetic defect of the LIPA gene that results in decreased or absent activity of the LAL enzyme. Deficient LAL enzyme activity results in progressive complications due to the lysosomal accumulation of cholesteryl esters and triglycerides in multiple organs, including the liver, spleen, intestine, and the walls of blood vessels . Accumulation of lipids in the liver can lead to fibrosis and cirrhosis. Lipid accumulation in the intestinal walls can lead to malabsorption and growth failure. Dyslipidemia and accelerated atherosclerosis also occur. Kanuma works as an enzyme replacement therapy (ERT) and catalyzes the lysosomal hydrolysis of cholesteryl esters and triglycerides to break them down. It is the first drug to be approved for LAL-D and the first treatment to target the underlying pathology . Previous management focused on strategies like lipid lowering medications, diet modification, liver transplant , or stem cell transplant. Wolman disease is the early infantile-onset phenotype of LAL-D which is more severe and rapidly progressive, with survival not expected beyond infancy. Kanuma significantly prolongs the life expectancy of these patients. Cholesterol ester storage disease (CESD) has a more variable phenotype with onset during childhood or adulthood. Kanuma has proven benefits for these patients as well.Kanuma (sebelipase alfa) will be considered for coverage when the following criteria are met:Lysosomal Acid Lipase (LAL) DeficiencyFor initial authorization: 1. Member is at least 1 month of age; AND 2. Medication must be prescribed by or in consultation with a hepatologist, cardiologist, metabolic specialist , or geneticist ; AND 3. Member has a diagnosis of LAL deficiency confirmed by at least one of the following methods : a) Deficient LAL enzyme activity (assay) b) Identification of biallelic pathogenic mutations of the LIPA gene (genetic testing) ; AND 4. Member demonstrates at least one clinical feature of LAL-Dsuch as dyslipidemia, elevated transaminases (ALT, AST), impaired growth or failure to thrive, malabsorption, hepatomegaly, adrenal calcification (in Wolmans disease), or advanced liver disease. 5. Dosage allowed/Quantity limit: Infants with rapidly progressive LAL-Dwithin first 6 months of life: Starting dose of 1 mg/kg once weekly via IV infusion. If clinical response is suboptimal, may increase to 3 mg/kg once weekly, and further to 5 mg/kg once weekly if needed. Pediatric and adult LAL-D: 1 mg/kg IV infusion every other week. If clinical response is suboptimal, may increase to 3 mg/kg every other week. If all the above requirements are met , the medication will be approved for 6 months. OH-MED-P -366685For reauthorization :1. Chart notes must document a positive clinical response to treatment such as improved lipid profile (LDL-c, triglycerides ), liver biomarkers (ALT, AST), liver volume, or growth /weight z-scores (if pediatric) . If all the above requirements are met , the medication will be approved for an additional 12 months. CareSource considers Kanuma (sebelipase alfa) not medically necessary for the treatment of conditions that are not listed in this document. For any other indication, please refer to the Off-Label policy.DATE ACTION/DESCRIPTION04/11/2018 New policy for Kanuma created. 01/10/2022 Transferred to new template. Updated references. Corrected max dose from 3 mg/kg to 5 mg/kg; added ped/adult dosing section which was missing. Changed prescribed by to by or in consultation with; also removed general specialist, added geneticist . Amended age minimum from 8 months to 1 month. Added tests for diagnostic confirmation. Removed all criteria that listed manifestations from a specific clinical trial and was divided by age; replaced with general list of manifestations. Specified renewal criteria. 04/18/2024 Annual review; no updates. 8/15/2024 Approved by ODM References: 1. Kanuma [package inset]. New Haven, CT: Alexion Pharmaceuticals Inc.; 2021. 2. Hoffman EP, et al. Lysosomal acid lipase deficiency. In: ed. Adam MP, et al. GeneReviews [Internet]. Seattle (WA): University of Washington, Seattle; 1993-2018. 2015 Jul 30 [Updated 2016 Sep 1]. 3. Wilson DP, Patni N. Lysosomal Acid Lipase Deficiency. [Updated 2020 Jan 18]. In: Feingold KR, Anawalt B, Boyce A, et al., editors. Endotext [Internet]. South Dartmouth (MA): MDText.com, Inc.; 2000- . Available from: https://www.ncbi.nlm.nih.gov/books/NBK395569/4. Vijay S, Brassier A, Ghosh A, et al. Long-term survival with sebelipase alfa enzyme replacement therapy in infants with rapidly progressive lysosomal acid lipase deficiency: final results from 2 open-label studies [published correction appears in Orphanet JRare Dis. 2021 Mar 1;16(1):113]. Orphanet JRare Dis . 2021;16(1):13. Published 2021 Jan 6. doi:10.1186/s13023-020-01577-4 5. Malinov V, Balwani M, Sharma R, et al. Sebelipase alfa for lysosomal acid lipase deficiency: 5-year treatment experience from a phase 2 open-label extension study. Liver Int. 2020;40(9):2203-2214. doi:10.1111/liv.14603 6. Burton BK, Feillet F, Furuya KN, Marulkar S, Balwani M. SEBELIPASE ALFA IN CHILDREN AND ADULTS WITH LYSOSOMAL ACID LIPASE DEFICIENCY: FINAL RESULTS OF THE ARISE STUDY [published online ahead of print, 2021 Nov 10]. JHepatol . 2021;S0168-8278(21)02171-1. doi:10.1016/j.jhep.2021.10.026 7. Demaret T, Lacaille F, Wicker C, et al. Sebelipase alfa enzyme replacement therapy in Wolman disease: a nationwide cohort with up to ten years of follow-up. Orphanet JRare Dis . 2021;16(1):507. Published 2021 Dec 14. doi:10.1186/s13023-021-02134-3 Effective date: 10/01/2024 Revised date: 04/18/2024
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