IN-MED-P -366647a; Issued Date: 6/1/2023 OMPP Approved: 5/16/2023PHARMACY POLICY STATEMENTIndiana Medicaid DRUG NAME Veopoz (Pozelimab)BENEFIT TYPE Medical STATUS Prior Authorization Required Veopoz is a complement inhibitor that was FDA approved in August 2023 for the treatment of a CD55-deficient protein losing enteropathy, in adult and pediatric patients. Veopoz is the first FDA-approved agent for the treatment of CHAPLE disease. The study that led to its approval was an open-label, single arm study with 10 participants whose primary endpoint was met in November 2021. CHAPLE disease or complement hyperactivation angiopathic thrombosis and protein-losing enteropathy, is a rare, inherited disorder that causes an overactivity of the complement system. The complement system is a portion of the immune system that increases the ability of phagocytic cells and antibodies to combat various microbes and damaged cellular components. With this disorder, a mutation of the CD55 gene can cause the complement system to attack and disrupt its own cells. This condition can be characterized by impaired growth, edema, and severe thrombotic vascular occlusion that can be life-threatening and cause subsequent death.Veopoz (Pozelimab) will be considered for coverage when the following criteria are met:Complement Hyperactive, Angiopathic Thrombosis, and Protein-LosingEnteropathy (CHAPLE) Disease For initial authorization: 1. Member is at least 1 year of age or older; AND 2. Medication must be prescribed by or in consultation with a geneticist, hematologist, gastroenterologist or an immunologist; AND 3. Member has a diagnosis of CHAPLE disease confirmed by BOTH of the following: a) Genotypic analysis confirming biallelic loss of function mutations to the CD55 gene; b) History of protein-losing enteropathy (PLE); AND 4. Member must have a baseline test confirming hypoalbuminemia; AND 5. Member must have meningococcal vaccination at least 2 weeks prior to therapy start date; AND 6. Veopoz will NOT be used in combination with eculizumab. 7. Dosage allowed/Quantity limit: Quantity limit: 16 mL per 28 days. Loading Dose Maintenance Dose Maximum Maintenance Dose Single 30 mg/kg dose 10 mg/kg dose once weekly May be increased to 12 mg/kg once weekly if inadequate response after at least 3 weekly doses 800 mg once weekly If all the above requirements are met, the medication will be approved for 6 months.IN-MED-P -366647a; Issued Date: 6/1/2023 OMPP Approved: 5/16/2023For reauthorization :1. Chart notes must show an improvement of at least ONE of the following symptoms: daily bowel movement frequency, edema, or abdominal pain; AND 2. Normalization of serum albumin. If all the above requirements are met, the medication will be approved for an additional 12 months. CareSource considers Veopoz (Pozelimab) 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/DESCRIPTION11/06/2023 New policy for Veopoz created. References: 1. Veopoz [package insert]. Regeneron Pharmaceuticals, Inc.; 2023. 2. Ozen A, Comrie WA, Ardy RC, et al. CD55 deficiency, early-onset protein-losing enteropathy, and thrombosis. NEngl JMed. 2017;377:52-61. 3. Regeneron Pharmaceuticals. Open-Label Efficacy and Safety Study of Pozelimab in Patients With CD55-Deficient Protein-Losing Enteropathy (CHAPLE Disease). https://clinicaltrials.gov/study/NCT04209634. Published October 2023 4. FDA approves first treatment for CD55-deficient protein-losing enteropathy (CHAPLE disease). U.S. Food and Drug Administration. https://www.fda.gov/drugs/news-events-human-drugs/fda-approves-first-treatment-cd55-deficient-protein-losing-enteropathy-chaple-disease. Accessed 28 Oct 2023. 5. Biopharma Dealmakers. Methods of Diagnosing and Treating CHAPLE, A Newly Identified Orphan Disease: Collaborative Research and Licensing Opportunity. https://www.nature.com/articles/d43747-020-00626-y. Accessed October 25, 2023 Effective date: 04/01/2024 Revised date: 11/06/2023
IN-MED-P -366647a; Issued Date: 6/1/2023 OMPP Approved: 5/16/2023PHARMACY POLICY STATEMENTIndiana Medicaid DRUG NAME Jynarque (tolvaptan)BENEFIT TYPE Pharmacy STATUS Prior Authorization Required Jynarque, initially approved by the FDA in 2018, is a selective vasopressin V2-receptor antagonist indicated to slow kidney function decline in adults at risk of rapidly progressing autosomal dominant polycystic kidney disease (ADPKD) . Decreased binding of vasopressin to the V2-receptor in the kidney lowers adenylate cyclase activity resulting in a decrease in intracellular cAMP concentrations. Decreased cAMP concentrations prevent aquaporin 2 containing vesicles from fusing with the plasma membrane, which in turn causes an increase in urine water excretion, an increase in free water clearance (aquaresis) and a decrease in urine osmolality. ADPKD is a genetic disorder that leads to the formation of cysts in the kidneys. It is sometimes referred to as adult PKD as it is usually diagnosed between 30 and 50 years of age. Symptoms include high blood pressure, flank pain, blood in the urine and poor function of the kidneys that gets worse over time. Organs other than the kidneys can become involved, but ultimately ADPKD leads to end stage renal disease. Jynarque is only available through a Risk Evaluation and Mitigation Strategy (REMS) program due to the potential for fatal liver injury.Jynarque (tolvaptan) will be considered for coverage when the following criteria are met:Autosomal D ominant Polycystic K idney D isease (ADPKD )For initial authorization: 1. Member is at least 18 years of age ; AND 2. Medication must be prescribed by or in consultation with a nephrologist; AND 3. Member has autosomal dominant polycystic kidney disease (ADPKD) confirmed by imaging (e.g. ultrasound, CT scan, or MRI scan) or genetic testing documented in chart notes; AND 4. Member is at risk of rapidly progressing disease, defined as having ONE of the following: a) Mayo classification 1D or 1E; b) Mayo classification 1C with ONE of the following: i) PROPKD score > 6 in patients with genetic data available; ii) Early hypertension/urological manifestations; iii) Truncating PKD1 mutation; iv) Family history (onset dialysis 16.5 cm in a patie nt
IN-MED-P -366647a; Issued Date: 6/1/2023 OMPP Approved: 5/16/2023PHARMACY POLICY STATEMENTIndiana Medicaid DRUG NAME Xalkori (crizotinib )BENEFIT TYPE Pharmacy STATUS Prior Authorization Required Xalkori, originally approved by the FDA in 2011, is a small molecule tyrosine kinase inhibitor (TKI). As of 2022, it is indicated for adult and pediatric patients 1 year of age and older with unresectable, recurrent, or refractory inflammatory myofibroblastic tumor (IMT) that is ALK-positive. It is also indicated to treat specific types of non-small cell lung ca ncer (NSCLC) and anaplastic large cell lymphoma (ALCL). IMTs are a rare but usually benign type of mesenchymal neoplasm that can be found in any age, although they most often develop in children or young adults. While these tumors typically affect the abdominal cavity, any area of the body can be affected. Surgery is the initial standard of care treatment, but surgery may not be possible in some cases, and tumors may recur after surgery. Malignant IMTs are uncommon, especially when ALK-positive. ALK rearrangements have been implicated in giving rise to oncogenic fusion proteins in at least 50% of IMTs. Expression of ALK fusion proteins contributes to cell proliferation and tumor survival. ALK is a type of tyrosine kinase and has proven to be a sui table target for systemic therapy with high response rates.Xalkori (crizotinib) will be considered for coverage when the following criteria are met:Inflammatory M yofibroblastic Tumor (IMT) For initial authorization: 1. Member is at least 1 year of age ; AND 2. Medication must be prescribed by or in consultation with a hematologist/oncologist; AND 3. Member has a documented diagnosis of unresectable, recurrent, or refractory IMT ; AND 4. Test results show the members tumor is ALK-positive . 5. Dosage allowed/Quantity limit: Adult: 250 mg orally twice daily Pediatric: 280 mg/m 2 orally twice daily (refer to body surface area table in prescribing information) Capsule QL: 120 per 30 days Pellet QL: 240 per 30 days If all the above requirements are met , the medication will be approved for 3 months . For reauthorization :1. Chart notes must show a positive clinical response to treatment such as tumor shrinkage and/or symptomatic improvement. If all the above requirements are met , the medication will be approved for an additional 12 months .Lung Cancer or LymphomaIN-MED-P -366647a; Issued Date: 6/1/2023 OMPP Approved: 5/16/2023Any request for cancer must be submitted through NantHealth/Eviti portal.CareSource considers Xalkori (crizotinib ) 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/DESCRIPTION 09/28/2023 New policy for Xalkori created. References: 1. Xalkori [prescribing information]. Pfizer Inc.; 2023. 2. Schffski P, Kubickova M, Wozniak A, et al. Long-term efficacy update of crizotinib in patients with advanced, inoperable inflammatory myofibroblastic tumour from EORTC trial 90101 CREATE. Eur JCancer . 2021;156:12-23. doi:10.1016/j.ejca.2021.07.016 3. Butrynski JE, D'Adamo DR, Hornick JL, et al. Crizotinib in ALK-rearranged inflammatory myofibroblastic tumor. NEngl JMed. 2010;363(18):1727-1733. doi:10.1056/NEJMoa1007056 4. Moss YP, Voss SD, Lim MS, et al. Targeting ALK With Crizotinib in Pediatric Anaplastic Large Cell Lymphoma and Inflammatory Myofibroblastic Tumor: A Children's Oncology Group Study. JClin Oncol. 2017;35(28):3215-3221. doi:10.1200/JCO.2017.73.4830 5. Siemion K, Reszec-Gielazyn J, Kisluk J, Roszkowiak L, Zak J, Korzynska A. What do we know about inflammatory myofibroblastic tumors? – A systematic review. Adv Med Sci. 2022;67(1):129-138. doi:10.1016/j.advms.2022.02.002 6. Trahair T, Gifford AJ, Fordham A, et al. Crizotinib and Surgery for Long-Term Disease Control in Children and Adolescents With ALK-Positive Inflammatory Myofibroblastic Tumors. JCO Precis Oncol . 2019;3:PO.18.00297. Published 2019 May 16. doi:10.1200/PO.18.00297 7. National Comprehensive Cancer Network (NCCN) Clinical Practice Guidelines in Oncology. Soft Tissue Sarcoma. Version 2.2023 April 25, 2023. Accessed September 28, 2023. Available at https://www.nccn.org/professionals/physician_gls/pdf/sarcoma.pdf . Effective date: 04/01/2024 Revised date: 09/28/2023
IN-MED-P -366647a; Issued Date: 6/1/2023 OMPP Approved: 5/16/2023PHARMACY POLICY STATEMENTIndiana Medicaid DRUG NAME Visudyne (verteporfin)BENEFIT TYPE Medical STATUS Prior Authorization Required Visudyne is a light activated drug used in photodynamic therapy (PDT) to treat certain cases of choroidal neovascularization (CNV). A course of therapy is a 2-step process. First, Visudyne is administered. Second, Visudyne is activated with light from a nonthermal diode laser. Photoactivation of Visudyne is controlled by the light dose delivered. Patients must avoid exposure of skin or eyes to direct sunlight or bright indoor light for 5 days following the procedure. CNV is the creation of new blood vessels in the choroid layer of the eye and can lead to vision loss. Age-related macular degeneration (AMD) is the most common cause of CNV.Visudyne (verteporfin) will be considered for coverage when the following criteria are met:C horoidal N eovascularization (CNV) For initial authorization: 1. Member is at least 18 years of age; AND 2. Medication must be prescribed by or in consultation with an ophthalmologist; AND 3. Member has a documented diagnosis of predominantly classic subfoveal choroidal neovascularization (CNV) due to one of the following: a) Wet a ge-related macular degeneration (AMD) b) Pathologic myopia c) Presumed ocular histoplasmosis; AND 4. Member has tried and failed bevacizumab; AND 5. Member does NOT have predominantly occult subfoveal CNV . 6. Dosage allowed/Quantity limit: 6 mg/m 2 body surface area IV If all the above requirements are met , the medication will be approved for 3 months (1 dose per eye) . For reauthorization :1. Chart notes must document positive clinical response (e.g., slowed progression of vision loss) following photodynamic treatment; AND 2. Choroidal neovascular leakage has recurred as detected on fluorescein angiography (FA) or optical coherence tomography (OCT). If all the above requirements are met , the medication will be approved for an additional 3 months (1 dose per eye) .IN-MED-P -366647a; Issued Date: 6/1/2023 OMPP Approved: 5/16/2023 CareSource considers Visudyne (verteporfin) 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/DESCRIPTION10/19/2021 New policy created for Visudyne . 10/04/2023 Revised /added references. References: 1. Visudyne [prescribing information]. Bausch & Lomb; 2023 . 2. Soubrane G, Bressler NM. Treatment of subfoveal choroidal neovascularisation in age related macular degeneration: focus on clinical application of verteporfin photodynamic therapy. Br JOphthalmol . 2001;85(4):483-495. doi:10.1136/bjo.85.4.483 3. Wormald R, Evans J, Smeeth L, Henshaw K. Photodynamic therapy for neovascular age-related macular degeneration. Cochrane Database Syst Rev . 2007;(3):CD002030. Published 2007 Jul 18. doi:10.1002/14651858.CD002030.pub3 4. Flaxel CJ, Adelman RA, Bailey ST, et al. Age-Related Macular Degeneration Preferred Practice Pattern [published correction appears in Ophthalmology. 2020 Sep;127(9):1279]. Ophthalmology . 2020;127(1):P1-P65. doi:10.1016/j.ophtha.2019.09.024 5. Zhu Y, Zhang T, Xu G, Peng L. Anti-vascular endothelial growth factor for choroidal neovascularisation in people with pathological myopia. Cochrane Database Syst Rev . 2016;12(12):CD011160. Published 2016 Dec 15. doi:10.1002/14651858.CD011160.pub2 6. Chen Y, Han X, Gordon I, et al. A systematic review of clinical practice guidelines for myopic macular degeneration. JGlob Health . 2022;12:04026. Published 2022 Mar 26. doi:10.7189/jogh.12.04026 7. Thuruthumaly C, Yee DC, Rao PK. Presumed ocular histoplasmosis. Curr Opin Ophthalmol . 2014;25(6):508-512. doi:10.1097/ICU.0000000000000100 Effective date: 04/01/2024 Revised date: 10/04/2023
IN-MED-P -366647a; Issued Date: 6/1/2023 OMPP Approved: 5/16/2023PHARMACY POLICY STATEMENTIndiana Medicaid DRUG NAME Ocaliva (obeticholic acid )BENEFIT TYPE Pharmacy STATUS Prior Authorization Required Ocaliva, approved by the FDA in 2016, is a farnesoid Xreceptor (FXR) agonist indicated for the treatment of adult s with primary biliary cholangitis (PBC) without cirrhosis , OR with compensated cirrhosis who do not have evidence of portal hypertension, either in combination with ursodeoxycholic acid (UDCA) with an inadequate response to UDCA or as monotherapy in patients unable to tolerate UDCA . PBC is a progressive , autoimmune liver disease that leads to scarring and inflammation of the small bile ducts. It primarily affects women and is characterized by fatigue, pruritis, and jaundice. A ntimitochondrial antibody (AMA ) is found in 95% of PBC patients. FXR signaling protects hepatocytes against bile acid toxicity by impairing bile acid synthesis and stimulating choleresis . Ursodiol (ursodeoxycholic acid [UDCA]) is the first-line treatment for PBC. It improves biochemical indices, delays histologic progression, and improves survival. A ccelerated approval of Ocaliva for PBC was based on a reduction in alkaline phosphatase (ALP). An improvement in survival or disease-related symptoms has not been established.Ocaliva (obeticholic acid) will be considered for coverage when the following criteria are met:Primary Biliary Cholangitis (PBC)For initial authorization: 1. Member is at least 18 years of age ; AND 2. Medication must be prescribed by or in consultation with a hepatologist or gastroenterologist; AND 3. Member has a diagnosis of PBC confirmed by at least 2 of the following a) Biochemical evidence of cholestasis based on ALP elevation b) Presence of AMA or other PBC-specific antibodies, including sp100 or gp210 c) Histologic evidence of nonsuppurative cholangitis and destruction of small or medium-sized bile ducts on biopsy ; AND 4. Member had an inadequate response to UDCA after 1 year of treatment OR the member has documentation of intolerance to UDCA; AND 5. UDCA will be continued in combination with Ocaliva unless the patient has documented intolerance; AND 6. Member does NOT have any of the following: a) Decompensated cirrhosis (e.g., Child-Pugh Bor C) b) Prior decompensation event c) Compensated cirrhosis with evidence of portal hypertension (e.g., ascites, gastroesophageal varices, persistent thrombocytopenia) d) Complete biliary obstruction. 7. Dosage allowed/Quantity limit: 5 mg once daily for 3 months, then may increase to 10 mg once daily if an adequate reduction in ALP and/or total bilirubin has not been achieved. (QL: 30 tablets per 30 days) IN-MED-P -366647a; Issued Date: 6/1/2023 OMPP Approved: 5/16/2023If all the above requirements are met , the medication will be approved for 6 months .For reauthorization :1. Chart notes must show improved (decreased) ALP and/or total bilirubin compared to baseline. If all the above requirements are met , the medication will be approved for an additional 12 months.CareSource considers Ocaliva (obeticholic acid) 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/DESCRIPTION06/14/2023 New policy for Ocaliva created. References: 1. Ocaliva [prescribing information]. Intercept Pharmaceuticals, Inc.; 2022. 2. Horwich BH, Han H. Diagnosis and Treatment of Primary Biliary Cholangitis: A Patient-Friendly Summary of the 2018 AASLD Practice Guidance. Clin Liver Dis (Hoboken). 2021;18(5):255-259. Published 2021 Sep 13. doi:10.1002/cld.1158 3. Lindor KD, Bowlus CL, Boyer J, Levy C, Mayo M. Primary Biliary Cholangitis: 2018 Practice Guidance from the American Association for the Study of Liver Diseases. Hepatology. 2019;69(1):394-419. doi:10.1002/hep.30145 4. Lindor KD, Bowlus CL, Boyer J, Levy C, Mayo M. Primary biliary cholangitis: 2021 practice guidance update from the American Association for the Study of Liver Diseases. Hepatology. 2022;75(4):1012-1013. doi:10.1002/hep.32117 5. Nevens F, Andreone P, Mazzella G, et al. A Placebo-Controlled Trial of Obeticholic Acid in Primary Biliary Cholangitis. NEngl JMed. 2016;375(7):631-643. doi:10.1056/NEJMoa1509840 6. Hirschfield GM, Mason A, Luketic V, et al. Efficacy of obeticholic acid in patients with primary biliary cirrhosis and inadequate response to ursodeoxycholic acid. Gastroenterology . 2015;148(4):751-61.e8. doi:10.1053/j.gastro.2014.12.005 7. Krupa K, Hapshy V, Nguyen H, et al. Obeticholic Acid. [Updated 2023 Jan 17]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2023 Jan- . Available from: https://www.ncbi.nlm.nih.gov/books/NBK567735/8. Hirschfield GM, Dyson JK, Alexander GJM, et al. The British Society of Gastroenterology/UK-PBC primary biliary cholangitis treatment and management guidelines. Gut . 2018;67(9):1568-1594. doi:10.1136/gutjnl-2017-315259 Effective date: 01/01/2024 Revised date: 06/14/2023
IN-MED-P -366647a; Issued Date: 6/1/2023 OMPP Approved: 5/16/2023PHARMACY POLICY STATEMENTIndiana Medicaid DRUG NAME Gattex (teduglutide )BENEFIT TYPE Pharmacy STATUS Prior Authorization Required Gattex, approved by the FDA in 2012, is a glucagon-like peptide-2 (GLP-2) analog indicated for the treatment of adults and pediatric patients 1 year of age and older with Short Bowel Syndrome (SBS) who are dependent on parenteral support. SBS can result from a variety of different conditions that require surgical removal of portions of the small intestine including Crohns disease, cancer, and vascular events. Many patients with SBS cannot absorb nutrients from food sources and need to be o n parenteral support. Long-term parenteral support can negatively impact patients quality of life. Approval for Gattex was based on a clinical response defined as achieving at least 20% reduction in weekly parenteral nutrition/intravenous volume from baseline. At week 24, the mean reduction in weekly parenteral nutrition/intravenous volume was significant at 4.4 liters for Gattex-treated patients versus 2.3 liters for placebo-treated patients.Gattex (teduglutuide ) will be considered for coverage when the following criteria are met:Short Bowel Syndrome (SBS)For initial authorization: 1. Member is at least 1 year of age (must weigh at least 10 kg) ; AND 2. Medication must be prescribed by or in consultation with a gastroenterologist; AND 3. Member has a diagnosis of SBS confirmed by
IN-MED-P-366647a; Issued Date: 6/1/2023 OMPP Approved: 5/16/2023PHARMACY POLICY STATEMENTIndiana Medicaid DRUG NAME Ultomiris (ravulizumab-cwvz)BENEFIT TYPE Medical , Pharmacy STATUS Prior Authorization Required Ultomiris was originally approved by the FDA in 2018 for the treatment of p aroxysmal nocturnal h emoglobinuria (PNH ). It is a terminal complement inhibitor that specifically binds to the complement protein C5 with high affinity, thereby inhibiting its cleavage to C5a (the proinflammatory anaphylatoxin) and C5b (the initiating subunit of the membrane attack complex [MAC or C5b-9]) thus preventing MAC formation. Ultomiris was engineered from an earlier product, Soliris, to have a longer half-life allowing for extended dosing intervals (every 2 weeks vs. every 8 weeks) . Ultomiris and Soliris are virtually identical aside from Ultomiris having the longer half-life. Ultomiris is also approved for the treatment of atypical hemolytic uremic syndrome (aHUS) to inhibit complement-mediated thrombotic microangiopathy (TMA) . Ultomiris is also approved for the treatment of generalized myasthenia gravis (gMG) who are anti-acetylcholine receptor (AChR) antibody-positive .Ultomiris (ravulizumab-cwvz) will be considered for coverage when the following criteria are met:Atypical Hemolytic Uremic Syndrome (aHUS) For initial authorization: 1. Medication is prescribed by or in consultation with a hematologist or nephrologist; AND 2. Member has a diagnosis of aHUS supported by ALL of the following: a) Thrombocytopenia (platelet count 10% (to rule out TTP); AND 5. Member has received meningococcal vaccine. 6. Dosage allowed/Quantity limit: IV infusion: loading dose followed by maintenance doses starting 2 weeks later, based on body weight, per prescribing information. See appendix 1. Subcutaneous (adults only) : 490 mg once weekly (8 cartons per 28 days) If all the above requirements are met , the medication will be approved for 6 months . IN-MED-P-366647a; Issued Date: 6/1/2023 OMPP Approved: 5/16/2023For reauthorization :1. Chart notes must demonstrate hematologic normalization as evidenced by increased platelet count or LDH maintained below upper limit of normal; AND 2. Improved or preserved kidney function. If all the above requirements are met, the medication will be approved for an additional 12 months. Paroxysmal Nocturnal Hemoglobinuria (PNH)For initial authorization: 1. Medication is prescribed by or in consultation with a hematologist; AND 2. Member has a documented diagnosis of PNH as confirmed by flow cytometry; AND 3. Member has a lactate dehydrogenase (LDH) level >1.5x upper limit of normal (ULN); AND 4. Member has at least one PNH-related sign/symptom e.g., fatigue, hemoglobin
IN-MED-P -366647a; Issued Date: 6/1/2023 OMPP Approved: 5/16/2023PHARMACY POLICY STATEMENTIndiana Medicaid DRUG NAME Cablivi (caplacizumab-yhdp )BENEFIT TYPE Medical or Pharmacy STATUS Prior Authorization Required Cablivi, approved by the FDA in 2019, is a von Willebrand factor (vWF) -directed antibody fragment indicated for the treatment of adult patients with acquired thrombotic thrombocytopenic purpura (aTTP), in combination with plasma exchange and immunosuppress ive therapy. It targets the A1-domain of vWF, and inhibits the interaction between vWF and platelets, thereby reducing both vWF-mediated platelet adhesion and platelet consumption. Cabliv i (caplacizumab-yhdp) will be considered for coverage when the following criteria are met:A cquired Thrombotic Thrombocytopenic Purpura (aTTP)For initial authorization: 1. Member is at least 18 years of age ; AND 2. Medication must be prescribed by or in consultation with a hematologist; AND 3. Member has known or highly likely diagnosis of severe aTTP with ALL of the following: a) Lab results showing platelet count less than 100,000; b) Microangiopathic hemolytic anemia (MAHA) confirmed by presence of schistocytes on blood smear; c) Testing shows an ADAMTS13 activity level less than 20%;OR d) Testing has been ordered and results are pending; AND e) Documentation of a PLASMIC score of 6-7 (high risk) OR a French score of 2-3; AND 4. Cablivi was initiated inpatient with plasma exchange and will be continued in combination with immunosuppressive therapy (i.e. glucocorticoids, rituximab) as indicated. 5. Dosage allowed/Quantity limit: 11 mg once daily. Quantity Limit: 30 vials per 30 days. If all the above requirements are met , the medication will be approved for 30 days. For reauthorization : 1. Platelet count normalized (at least 150,000) for at least 2 days during treatment; AND 2. Member was able to stop daily plasma exchange within 5 days of platelet normalization; AND 3. ADAMTS13 activity remains less than 20%; AND 4. Member has not experienced more than 2 recurrences (need to restart plasma exchange) of aTTP during treatment (within the same episode or acute event). If all the above requirements are met , the medication will be approved for an additional 28 days . CareSource considers Cablivi (caplacizumab-yhdp) 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. IN-MED-P -366647a; Issued Date: 6/1/2023 OMPP Approved: 5/16/2023DATE ACTION/DESCRIPTION07/15/2020 New policy for Cablivi created.06/27/2023 Policy transferred to new template; updated references; Changed PLASMIC score documentation to high risk only (6-7) and required only if ADAMTS13 activity level is pending; added French score option to PLASMIC score requirement; added requirement for reauthorization that plasma exchange was stopped within 5 days of platelet normalization; changed ADAMTS13 activity level requirement from less than 10% to less than 20%.References: 1. Cablivi [package insert]. Cambridge, MA: Genzyme Corporation; 2023. 2. George JN, Cuker A.Acquired TTP: Initial treatment. UpToDate. http://www.uptodate.com. Updated September 30, 2019. Accessed July 15, 2020. 3.Zheng XL, Vesely SK, Cataland SR, et al. ISTH guidelines for treatment of thrombotic thrombocytopenic purpura. JThromb Haemost. 2020;18(10):2496-2502. doi:10.1111/jth.15010Z 4. Zheng XL, Vesely SK, Cataland SR, et al. ISTH guidelines for the diagnosis of thrombotic thrombocytopenic purpura [published correction appears in JThromb Haemost. 2021 May;19(5):1381]. JThromb Haemost. 2020;18(10):2486-2495. doi:10.1111/jth.15006 5. Scully M, Cataland SR, Peyvandi F, et al. Caplacizumab Treatment for Acquired Thrombotic Thrombocytopenic Purpura. NEngl JMed. 2019;380(4):335-346. doi:10.1056/NEJMoa1806311 6. Peyvandi F, Scully M, Kremer Hovinga JA, et al. Caplacizumab for Acquired Thrombotic Thrombocytopenic Purpura. NEngl JMed. 2016;374(6):511-522. doi:10.1056/NEJMoa1505533 7. Coppo P, Cuker A, George JN. Thrombotic thrombocytopenic purpura: Toward targeted therapy and precision medicine. Res Pract Thromb Haemost. 2018;3(1):26-37. Published 2018 Nov 16. doi:10.1002/rth2.12160 8. Assessment report (Cablivi dossier). European Medicines Agency. https://www.ema.europa.eu/en/documents/assessment-report/cablivi-epar-public-assessment-report_en.pdf. Published 2018. Accessed August 20, 2020. 9. 2021 Georgia Code Title 33 Insurance Chapter 20A-Managed Health Care Plans Article 2-Patient's Right to Independent Review 33-20A-31 Definitions. Justia US Law. Accessed April 25, 2023. https://law.justia.com/codes/georgia/2021/title-33/chapter-20a/article-2/section-33-20a-31/.Effective date: 01/01/2024 Revised date: 06/27/2023
IN-MED-P -366647a; Issued Date: 6/1/2023 OMPP Approved: 5/16/2023PHARMACY POLICY STATEMENTIndiana Medicaid DRUG NAME Alpha 1-Proteinase Inhibitor (Aralast NP,Glassia, Prolastin C, Zemaira [human]) BENEFIT TYPE Medical (Pharmacy allowed for Glassia) STATUS Prior Authorization Required Alpha1-proteinase inhibitor (alpha1 antitrypsin) from pooled human plasma donors acts as augmentation therapy for maintenance treatment in adults with clinical evidence of emphysema due to severe alpha1-antitrypsin deficiency (AATD) . The available products are Aralast NP, Glassia, Prolastin C, and Zemaira, with none of them being clinically preferred over the others. Prolastin was the first, approved by the FDA in 1987 (and later replaced by Prolastin C). The goal of this therapy is to restore and maintain alpha1-antitrypsin to protective levels and slow the progression of lung damage and emphysema by inhibiting proteases such as neutrophil elastase . Alpha-1 antitrypsin deficiency (A ATD) is a hereditary disorder caused by pathogenic mutations in the SERPINA1 gene responsible for producing the protein alpha-1 antitrypsin (AAT) and leads to low levels of AAT . This deficiency results in an imbalance that allows relatively unopposed protease activity to cause destruction in the lungs . The liver, and less likely the skin (panniculitis) , can also be affected .Alpha1-Proteinase Inhibitor (Aralast NP, Glassia, Prolastin C, Zemaira [human]) will be considered for coverage when the following criteria are met:Alpha 1-Antitrypsin Deficiency (AATD)For initial authorization: 1. Member is at least 18 years of age ; AND 2. Medication must be prescribed by or in consultation with a pulmonologist; AND 3. Member has a diagnosis of clinically evident emphysema due to severe AATD; AND 4. Member is a never-smoker or has been a non-smoker for at least 3 months; AND 5. Member is in compliance with any prescribed supportive therapy (at least one) (e.g., bronchodilators, pulmonary rehabilitation, oxygen); AND 6. Chart notes must include lab reports showing ALL of the following: a) Pre-treatment alpha1-antitrypsin (AAT) serum level less than 11 micromol/L (or equivalent ) b) High risk genotype ( e.g., Pi*ZZ, Pi*ZNull, Pi*NullNull) c) Pre-treatment FEV1 is 65% predicted or less; AND 7. Member has NOT had a liver transplant. Dosage allowed/Quantity limit: 60 mg/kg once weekly IV infusion. If all the above requirements are met , the medication will be approved for 6 months. IN-MED-P -366647a; Issued Date: 6/1/2023 OMPP Approved: 5/16/2023For reauthorization :1. Member continues to abstain from smoking; AND 2. At least ONE of the following: a) AAT level at or above protective threshold (11 micromol/L) b) Slowed rate of FEV1 decline per spirometry results c) CT densitometry demonstrates slowed progression of anatomic lung disease If all the above requirements are met , the medication will be approved for an additional 12 months . CareSource considers Alpha 1-Proteinase Inhibitor (Aralast NP, Glassia, Prolastin C, Zemaira [human]) 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/DESCRIPTION07/14/2020 Transferred to new template; revised and updated content . 06/29/2023 Transferred to new template. Updated and added references. Removed lower FEV limit and rate of decline. Added liver transplant exclusion. References: 1. 2021 Georgia Code Title 33 Insurance Chapter 20A-Managed Health Care Plans Article 2-Patient’s Right to Independent Review 33-20A-31 Definitions . Justia US Law. Accessed April 25, 2023. https://law.justia.com/codes/georgia/2021/title-33/chapter-20a/article-2/section-33-20a-31/.2. Stoller JK. Treatment of alpha-1-antitrypsin deficiency. UpToDate. http://www.uptodate.com. Updated July 13, 2020. Accessed July 13,2020. 3. Marciniuk DD, Hernandez P, Balter M, et al. Alpha-1 antitrypsin deficiency targeted testing and augmentation therapy: a Canadian Thoracic Society clinical practice guideline [published correction appears in Can Respir J. 2012 Jul-Aug;19(4):272]. Can Respir J. 2012;19(2):109-116. doi:10.1155/2012/920918 4. Sandhaus RA, Turino G, Brantly ML, et al. The Diagnosis and Management of Alpha-1 Antitrypsin Deficiency in the Adult. Chronic Obstructive Pulmonary Diseases: Journal of the COPD Foundation. 2016;3(3):668-682. doi:10.15326/jcopdf.3.3.2015.0182 5. Miravitlles M, Dirksen A, Ferrarotti I, et al. European Respiratory Society statement: diagnosis and treatment of pulmonary disease in 1-antitrypsin deficiency. Eur Respir J 2017; 50: 1700610 [https://doi.org/10.1183/13993003.00610-2017]. 6. Gtzsche PC, Johansen HK. Intravenous alpha 1 antitrypsin augmentation therapy for treating patients with alpha 1 antitrypsin deficiency and lung disease. Cochrane Database of Systematic Reviews 2016, Issue 9. Art. No.: CD007851. DOI: 10.1002/14651858.CD007851.pub3. 7. Global Initiative for Chronic Obstructive Lung Disease (GOLD). Global Strategy for Prevention, Diagnosis, and Management of COPD : 2023 Report. www.goldcopd.org (Accessed June 28, 2023). 8. Aralast NP [prescribing information]. Takeda Pharmaceuticals U.S.A., Inc.; 2023. 9. Glassia [prescribing information]. Takeda Pharmaceuticals U.S.A., Inc.; 2022. 10. Prolastin C[prescribing information]. Grifols Therapeutics LLC; 2020. 11. Zemaira [prescribing information]. CSL Behring LLC; 2022. 12. Chapman KR, Chorostowska-Wynimko J, Koczulla AR, Ferrarotti I, McElvaney NG. Alpha 1 antitrypsin to treat lung disease in alpha 1 antitrypsin deficiency: recent developments and clinical implications. Int JChron Obstruct Pulmon Dis . 2018;13:419-432. Published 2018 Jan 31. doi:10.2147/COPD.S149429 13. Dummer J, Dobler CC, Holmes M, et al. Diagnosis and treatment of lung disease associated with alpha one-antitrypsin deficiency: A position statement from the Thoracic Society of Australia and New Zealand. Respirology . 2020;25(3):321-335. doi:10.1111/resp.13774 Effective date: 01/01/2024 Revised date: 06/29/2023
IN-MED-P-366647a; Issued Date: 6/1/2023 OMPP Approved: 5/16/2023PHARMACY POLICY STATEMENTIndiana Medicaid DRUG NAME Procysbi and Cystagon (cysteamine bitartrate); Cystaran and Cystadrops (cysteamine hydrochloride solution)BENEFIT TYPE Pharmacy STATUS Prior Authorization Required Cysteamine bitartrate , approved by the FDA in 1994 as immediate release Cystagon and in 2013 as extended release Procysbi, is indicated for the treatment of nephropathic cystinosis. Cystinosis is a rare inherited metabolic disease in which cystine builds up as damaging crystals in organs including the kidneys and eyes. It is caused by CTNS gene mutations that result in defective cystinosin transport protein that keeps cystine from effectively exiting lysosomes. Manifestations can include Fanconi syndrome, renal failure, photophobia due to cystine deposits in the cornea, and endocrine disruption such as hypothyroidism or hypogonadism . Nephropathic (infantile) cystinosis is the most common and most severe type of cystinosis. The adult non-nephropathic ocular form affects the cornea only. Cysteamine depletes white blood cell (WBC) cystine levels to slow the progression of disease manifestations such as end stage renal disease. Systemic cysteamine does not affect the cornea, thus cysteamine eye drops are indicated when corneal cystine crystals are present. Cysteamine hydrochloride eye drops are supplied as Cystaran or Cystadrops. Cystadrops can be administered less frequently but has a higher incidence of side effects.Cysteamine products will be considered for coverage when the following criteria are met:Nephropathic Cystinosis (Procysbi or Cystagon)For initial authorization: 1. Member must be 1 year of age or older if the request is for Procysbi (no limit for Cystagon); AND 2. Medication must be prescribed by or in consultation with a nephrologist or endocrinologist ; AND 3. Member has a documented diagnosis of nephropathic cystinosis confirmed by an elevated WBC cystine concentration greater than 2 nmol cystine/mg protein (lab report must include reference values) AND at least one of the following: a) CTNS gene mutation b) Presence of corneal crystals, as shown by slit lamp exam performed by an ophthalmologist; AND 4. If the request is for Procysbi, ALL the following that are applicable must be documented in chart notes: a) Inability to reach target cystine level (
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