POLICY

APPLICABLE PRODUCTS

NEW POLICY OVERVIEW OR UPDATED POLICY REVISIONS

Rituximab (Rituxan, Riabni, Ruxience, Truxima, Rituxan Hycela) (CP.PHAR.260)

Ambetter

Policy updates include:

o Added off-label criteria for Wiskott-Aldrich syndrome infection prophylaxis associated with Waskyra gene therapy

Lisocabtagene maraleucel (Breyanzi) (CP.PHAR.483)

Ambetter

Policy updates include:

o Added new indication for marginal zone lymphoma

Etuvetidigene Autotemcel (Waskyra) (CP.PHAR.735)

Ambetter

Policy includes:

· Requests for indications not approved by the FDA are reviewed with the off-label use policy HIM.PHAR.154 for Ambetter

· Initial Approval Criteria: Wiskott-Aldrich Syndrome (WAS) (must meet all):

o Diagnosis of WAS confirmed by the presence of a WAS genetic mutation and one of the following:

§ Severe WAS gene mutation;

§ Absent or markedly reduced WAS protein expression;

§ Severe WAS clinical phenotype defined as a Zhu, Ochs, or Zhu-Ochs clinical score of 3 or higher;

§ Clinically significant disease as evidenced by documented classic clinical manifestations of WAS (e.g., microthrombocytopenia with bleeding, recurrent or severe infections, eczema, immune dysfunction or autoimmunity);

o Prescribed by or in consultation with a medical geneticist, transplant specialist, or specialist with expertise in treating WAS (e.g., hematologist, immunologist);

o Age at least 6 months;

o Member has no available human leukocyte antige (HLA)-matched related stem cell donor;

o Transplant specialist attestation that member is clinically stable and eligible to undergo myeloablative conditioning and hematopoietic stem cell transplantation (HSCT);

o If member has previously received allogeneic HSCT, both of the following:

§ It has been greater than 6 months since the transplant;

§ There is no evidence of residual cells of donor origin;

o Member has not received prior hematopoietic stem cell gene therapy;

o Dose is a single infusion containing a minimum of 7 x 106 CD34+ cells per kg.

o Approval duration: 3 months (one time infusion per lifetime

· Continued Therapy: Wiskott-Aldrich Syndrome

o Re-authorization is not permitted as Waskyra is indicated to be dosed one time only.

o Approval duration: Not applicable

Aficamten (Myqorzo) (CP.PHAR.766)

Ambetter

Policy includes:

· Requests for indications not approved by the FDA are reviewed with the off-label use policy for the relevant line of business: HIM.PHAR.154 for Ambetter

· Initial Approval Criteria: Obstructive Hypertrophic Cardiomyopathy (oHCM) (must meet all):

o Diagnosis of oHCM;

o Member exhibits New York Heart Association (NYHA) Class II to III symptoms, including but not limited to: effort-related dyspnea or chest pain, or syncope or near syncope attributed to left ventricular outflow tract obstruction;

o Prescribed by or in consultation with a cardiologist;

o Age at least 18 years;

o Member has left ventricular hypertrophy with maximal left ventricular wall thickness of one of the following:

§ at least 15 mm;

§ at least 13 mm if member has familial HCM or in conjunction with a positive genetic test;

o Member has a left ventricular ejection fraction (LVEF) at least 55%;

o Member has peak left ventricular outflow tract (LVOT) gradients of both of the following:

§ at least 30 mmHg at rest;

§ at least 50 mmHg with provocation;

o Failure of TWO of the following at up to maximally indicated doses, unless clinically significant adverse effects are experienced or all are contraindicated:

§ Non-vasodilating beta-blocker (e.g., atenolol, metoprolol, bisoprolol, propranolol);

§ Non-dihydropyridine calcium channel blocker (e.g., verapamil, diltiazem);

§ Add-on disopyramide therapy after failure of beta-blocker or calcium channel blocker monotherapy;

o Myqorzo is not prescribed concurrently with Camzyos®; 10. Dose does not exceed 20 mg per day.

o Approval duration: 12 months

· Continued Therapy: Obstructive Hypertrophic Cardiomyopathy (must meet all):

o Member meets one of the following:

§ Currently receiving medication via Centene benefit or member has previously met initial approval criteria;

§ Member is currently receiving medication and is enrolled in a state and product with continuity of care regulations;

o Member is responding positively to therapy as evidenced by improvement in obstructive HCM symptoms;

o Myqorzo is not prescribed concurrently with Camzyos;

o If request is for a dose increase, new dose does not exceed 20 mg per day.

o Approval duration: 12 months

Lerodalcibep-liga (Lerochol) (CP.PHAR.768)

Ambetter

Policy includes:

· Requests for indications not approved by the FDA are reviewed with the off-label use policy: HIM.PHAR.154 for Ambetter.

· Initial Approval Criteria: Primary Hypercholesterolemia (including heterozygous familial hypercholesterolemia (HeFH)) and Cardiovascular (CV) Event Risk Reduction (must meet all):

o Diagnosis of one of the following:

§ HeFH, and both of the following:

· Baseline low density lipoprotein cholesterol (LDL-C) (prior to any lipid-lowering pharmacologic therapy) was one of the following:

o If age less than 20 years: at least 160 mg/dL;

o If age at least 20 years: at least 190 mg/dL;

· HeFH diagnosis is confirmed by one of the following:

o World Health Organization (WHO)/Dutch Lipid Network familial hypercholesterolemia diagnostic criteria score of greater than 8 as determined by requesting provider;

o Definite diagnosis per Simon Broome criteria;

o Primary hypercholesterolemia that is not HeFH, and both of the following:

§ Documentation of one of the following:

· Presence of a genetically mediated form of primary hypercholesterolemia as evidenced by confirmatory genetic testing results;

· A diagnosis of secondary hypercholesterolemia has been ruled out with absence of all of the following potential causes of elevated cholesterol:

o Poor diet;

o Hypothyroidism;

o Obstructive liver disease;

o Renal liver disease;

o Nephrosis;

o Medications that have had a clinically relevant contributory effect on the current degree of the member’s elevated lipid levels including, but not limited to: glucocorticoids, sex hormones, antipsychotics, antiretrovirals, immunosuppressive agents, retinoic acid derivatives;

§ Baseline LDL-C (prior to any lipid-lowering pharmacologic therapy) was at least 190 mg/dL;

o Increased risk for CV events as evidenced by a history of atherosclerotic cardiovascular disease (ASCVD) including any one of the following conditions (i-vii):

§ Acute coronary syndromes;

§ Clinically significant coronary heart disease (CHD) diagnosed by invasive or noninvasive testing (such as coronary angiography, stress test using treadmill, stress echocardiography, or nuclear imaging);

§ Coronary or other arterial revascularization;

§ Myocardial infarction;

§ Peripheral arterial disease presumed to be of atherosclerotic origin;

§ Stable or unstable angina;

§ Stroke or transient ischemic attack (TIA);

o Prescribed by or in consultation with a cardiologist, endocrinologist, or lipid specialist;

o Age at least 18 years;

o Failure of an 8-week trial of a preferred PCSK9 inhibitor*, if applicable, at up to maximally indicated doses, unless contraindicated or clinically significant adverse effects are experienced;^

Prior authorization may be required for PCSK9 inhibitors

o For members on statin therapy, both of the following:*

§ Lerochol is prescribed in conjunction with a statin at the maximally tolerated dose;

§ Member has been adherent for at least the last 8 weeks to maximally tolerated doses of one of the following statin regimens:

· A high intensity statin;

· A moderate or low intensity statin, and member has one of the following:

o Previous use of one high-intensity statin (i.e., atorvastatin at least 40 mg daily; rosuvastatin at least 20 mg daily [as a single-entity or as a combination product]) for a minimum of 8 weeks continuously and LDL-C remained at least 70 mg/dL;

o Member has tried both rosuvastatin and atorvastatin and has experienced skeletal-muscle related symptoms on both agents which also resolved upon discontinuation;

o For members not on statin therapy, member meets one of the following:*

§ Statin therapy is contraindicated:

§ For members who are statin intolerant, both of the following:

· Member has tried at least two statins, one of which must be hydrophilic (pravastatin, fluvastatin, or rosuvastatin);

· Member meets one of the following:

· Member has documented statin risk factors;

· Member is statin intolerant due to statin-associated muscle symptoms (SAMS) and meets both of the following:

o Documentation of intolerable SAMS persisting at least two weeks, which disappeared with discontinuing the statin therapy and recurred with a statin re-challenge;

o Documentation of re-challenge with titration from lowest possible dose and/or intermittent dosing frequency (e.g., 1 to 3 times weekly);

o Member has been adherent to ezetimibe therapy used concomitantly with a statin at the maximally tolerated dose for at least the last 4 months, unless contraindicated per Appendix F or member has a history of ezetimibe intolerance (e.g., associated diarrhea or upper respiratory tract infection);*

o Documentation of recent (within the last 60 days) LDL-C of one of the following:

§ If member has ASCVD:

· at least 70 mg/dL;

· at least 55 mg/dL, and member is at very high risk;

§ If member has severe primary hypercholesterolemia (including HeFH): at least 100 mg/dL;

o Treatment plan does not include coadministration with Leqvio^®, Juxtapid^®, Repatha^®, or Praluent^®;

o Dose does not exceed 300 mg per month.

o Approval duration: 12 months

· Continued Therapy: Primary Hypercholesterolemia (including HeFH) and Cardiovascular Event Risk Reduction (must meet all):

o Member meets one of the following:

§ Currently receiving medication via Centene benefit or member has previously met initial approval criteria;

§ Member is currently receiving medication and is enrolled in a state and product with continuity of care regulations;

o If statin tolerant, documentation of adherence to a statin at the maximally tolerated dose;*

o Member is responding positively to therapy as evidenced by lab results within the last 3 months showing an LDL-C reduction since initiation of Lerochol therapy;

o Treatment plan does not include coadministration with Leqvio, Juxtapid, Repatha, or Praluent;

o If request is for a dose increase, new dose does not exceed 300 mg per month.

o Approval duration: 12 months

Anitocabtagene autoleucel (KITE-772)_PEPP (CP.PHAR.769_PEPP)

Ambetter

Policy includes:

· Criteria will mirror the clinical information from the prescribing information once FDA-approved

· Requests for indications not approved by the FDA are reviewed with the off-label use policy HIM.PHAR.154 for Ambetter

· Initial Approval Criteria*: Multiple Myeloma (MM)* (must meet all):

*Criteria will mirror the clinical information from the prescribing information once FDA-approved

*Only for initial treatment dose; subsequent doses will not be covered.

o Diagnosis of relapsed or refractory MM;*

o Prescribed by or in consultation with an oncologist or hematologist;

o Age at least 18 years;

o One of the following:*

§ Member has measurable disease as evidenced by one of the following assessed within the last 30 days:

· Serum M-protein at least 1.0 g/dL;

· Urine M-protein at least 200 mg/24 h;

· Serum free light chain (FLC) assay: involved FLC level at least 10 mg/dL (100 mg/L) provided serum FLC ratio is abnormal;

§ Member has progressive disease, as defined by the International Myeloma Working Group (IMWG) response criteria, assessed within 60 days following the last dose of the last anti-myeloma drug regimen received;

o Member has received at least 3 prior lines of therapy, that included all of the following:*

§ One immunomodulatory dru (IMiD) (e.g., Revlimid^®, Pomalyst^®, Thalomid^®);

§ One proteasome inhibitor (PI) (e.g., bortezomib, Kyprolis^®, Ninlaro^®);

§ One anti-CD38 antibody (e.g., Darzalex^®/Darzalex Faspro^™, Sarclisa^®);

*Prior authorization may be required. Induction with or without hematopoietic stem cell transplant, consolidation and maintenance therapy is considered a single line of therapy.

o Member does not have active central nervous system (CNS) involvement by malignancy, or history or presence of clinically relevant CNS pathology (e.g., epilepsy, seizure, paresis, aphasia, stroke, severe brain injuries, dementia, Parkinson's disease, cerebellar disease, organic brain syndrome, or psychosis);*

o Member has not previously received treatment with anti-BCMA targeted therapy (e.g., Blenrep^™, Tecvayli^™);*

o Member has not previously received treatment with chimeric antigen receptor (CAR) T-cell immunotherapy (e.g., Abecma^®, Breyanzi^®, Carvykti^™, Kymriah^™, Tecartus^™, Yescarta^™);*

o KITE-772 is not prescribed concurrently with other CAR T-cell immunotherapy (e.g., Abecma, Breyanzi, Carvykti, Kymriah, Tecartus, Yescarta); *

o Dose does not exceed 115 x 10⁶ CAR-positive viable T cells.*

o Approval duration: 3 months (1 dose only, with 4 doses of tocilizumab (Actemra) if requested at up to 800 mg per dose)

· Continued Therapy: Multiple Myeloma:

*Criteria will mirror the clinical information from the prescribing information once FDA-approved

o Continued therapy will not be authorized as KITE-772 is indicated to be dosed one time only.

o Approval duration: Not applicable

HSPCs, Tregs, and Tcons (Orca-T)_PEPP (CP.PHAR.770_PEPP)

Ambetter

Policy includes:

· Criteria will mirror the clinical information from the prescribing information once FDA-approved

· Requests for indications not approved by the FDA are reviewed with the off-label use policy HIM.PHAR.154 for Ambetter.

· Initial Approval Criteria*: Acute Myeloid Leukemia (AML), Acute Lymphoblastic Leukemia (ALL), and Myelodysplastic Syndromes (MDS) (must meet all):

*Criteria will mirror the clinical information from the prescribing information once FDA-approved

o Diagnosis of one of the following:*

§ AML or ALL, and one of the following:

· Disease is in complete remission (CR);

· Disease is in complete remission with incomplete hematologic recovery (CRi);

§ MDS, and both of the following:

· Member has at most 10% blast burden in the bone marrow;

· One of the following:

o Member is indicated for allogeneic hematopoietic stem cell transplantation (HSCT) per 2017 International Expert Panel recommendations;

o Member has therapy-related/secondary MDS per the World Health Organization classification of myeloid malignancies;

o Prescribed by or in consultation with a hematologist, oncologist, or transplant specialist;

o Age at least 18 years;*

o Member has a matched donor (related or unrelated) who is an 8/8 match for human leukocyte antigen (HLA)-A, -B, -C, and -DRB1;*

o Transplant specialist attestation that member is clinically stable and eligible to undergo myeloablative conditioning and allogeneic HSCT;*

o Member has not received prior allogeneic HSCT or Orca-T transplant;*

o Member will receive standard of care therapy for prophylaxis of graft-versus-host disease (e.g., tacrolimus, methotrexate, post-transplant cyclophosphamide, anti-thymocyte globulin, sirolimus, alemtuzumab, mycophenolate mofetil, ursodiol) following Orca-T transplant;*

o Dose does not exceed the FDA-approved maximum.*

o Approval duration: 3 months (one-time transplant per lifetime)

• Continued Therapy*: Acute Myeloid Leukemia, Acute Lymphoblastic Leukemia, and Myelodysplastic Syndromes

*Criteria will mirror the clinical information from the prescribing information once FDA-approved

o Continued therapy will not be authorized as there is no evidence to support repeat Orca-T transplants.*

o Approval duration: Not applicable

Semaglutide (Wegovy) (CP.PMN.295)

Ambetter

Policy updates include:

· Added new formulation Wegovy tablets to policy

o For cardiovascular event prevention, revised language for members with concurrent type 2 diabetes mellitus language from "failure" to "member has received optimal diabetic standard of care therapy as evidenced by a trial" to align with verbiage in metabolic dysfunction-associated steatohepatitis criterion

Etripamil (Cardamyst) (CP.PMN.306)

Ambetter

Policy includes:

· Requests for indications not approved by the FDA are reviewed with the off-label use policy for the relevant line of business: HIM.PHAR.154 for Ambetter

· Initial Approval Criteria: Paroxysmal Supraventricular Tachycardia (PSVT) (must meet all):

o Diagnosis of PSVT confirmed by electrocardiogram (ECG);

o Prescribed by or in consultation with a cardiologist or electrophysiologist;

o Age at least 18 years;

o Member has a history of sustained, symptomatic episodes of PSVT (i.e., typically lasting approximately 20 minutes or longer);

o One of the following:

§ Member has experienced at least one episode of PSVT that required hospitalization or visit to an emergency department/urgent care within the last 12 months;

§ Member is currently receiving oral pharmacologic therapy for prevention of PSVT episodes (e.g., diltiazem, verapamil, metoprolol, atenolol, propranolol, nadolol, flecainide, propafenone);

o If member is currently receiving oral pharmacologic therapy for prevention of PSVT episodes, Cardamyst is prescribed concurrently with ongoing oral pharmacologic therapy for prevention;

o Provider attestation that catheter ablation has been considered but is not appropriate for the member at this time;

o At the time of request, member has none of the following contraindications:

§ Wolff-Parkinson-White syndrome;

§ Lown-Ganong-Levine syndrome;

§ Manifest pre-excitation (delta wave) on a 12-lead ECG;

§ Second degree atrioventricular (AV) Mobitz 2 block or higher degree of AV block;

§ New York Heart Association (NYHA) Class II to IV heart failure;

§ Sick sinus syndrome without a permanent pacemaker;

o Request does not exceed 4 nasal spray devices per month;

o Dose does not exceed both of the following in a 24-hour period:

§ 140 mg;

§ 2 nasal spray devices.

o Approval duration: 12 months (up to 4 nasal spray devices per month)

· Continued Therapy: Paroxysmal Supraventricular Tachycardia (must meet all):

o Member meets one of the following:

§ Currently receiving medication via Centene benefit or member has previously met initial approval criteria;

§ Member is currently receiving medication and is enrolled in a state and product with continuity of care regulations;

o Member is responding positively to therapy;

o If member was previously receiving oral pharmacologic therapy for prevention of PSVT episodes, Cardamyst is prescribed concurrently with ongoing oral pharmacologic therapy for prevention;

o Request does not exceed 4 nasal spray devices per month;

o If request is for a dose increase, new dose does not exceed both of the following in a 24-hour period:

§ 140 mg;

§ 2 nasal spray devices.

o Approval duration: 12 months (up to 4 nasal spray devices per month)

Immune Globulins (HIM.PA.178)

Ambetter

Policy updates include:

o Added off-label measles post-exposure prophylaxis criteria for intravenous immunoglobulin

Depemokimab-ulaa (Exdensur) (HIM.PA.179)

Ambetter

Policy includes:

· Requests for indications not approved by the FDA are reviewed with the off-label use policy for the relevant line of business: HIM.PHAR.154 for Ambetter

· Initial Approval Criteria: Severe Asthma (must meet all):

o Diagnosis of asthma;

o Member has an absolute blood eosinophil count at least 150 cells/mcL within the past 3 months;

o Prescribed by or in consultation with an allergist, immunologist, or pulmonologist;

o Age at least 12 years;

o Member has experienced at least 2 exacerbations within the last 12 months, requiring one of the following despite adherent use of controller therapy (i.e., medium- to high-dose inhaled corticosteroid [ICS] plus either a long acting beta-2 agonist [LABA] or leukotriene modifier [LTRA] if LABA contraindication/intolerance):

§ Oral/systemic corticosteroid treatment (or increase in dose if already on oral corticosteroid);

§ Urgent care/emergency room (ER) visit or hospital admission;

o Failure of Dupixent^® and Fasenra^®, each used for at least 4 consecutive months at up to maximally indicated doses, unless clinically significant adverse effects are experienced or both are contraindicated;^

*Prior authorization may be required for Dupixent and Fasenra

o Exdensur is prescribed concurrently with an ICS plus either a LABA or LTRA;

o Exdensur is not prescribed concurrently with Cinqair^®, Fasenra, Nucala^®, Dupixent, Xolair^®, or Tezspire^®;

o Dose does not exceed 100 mg every 6 months.

o Approval duration: 12 months

· Continued Therapy: Severe Asthma (must meet all):

o Member meets one of the following:

§ Currently receiving medication via Centene benefit or member has previously met initial approval criteria;

§ Member is currently receiving medication and is enrolled in a state and product with continuity of care regulations;

o Demonstrated adherence to asthma controller therapy (an ICS plus either an LABA or LTRA) as evidenced by proportion of days covered (PDC) of 0.8 in the last 6 months (i.e., member has received asthma controller therapy for at least 5 of the last 6 months);

o Member is responding positively to therapy (examples may include but are not limited to: reduction in exacerbations or corticosteroid dose, improvement in forced expiratory volume over one second since baseline, reduction in the use of rescue therapy);

o Exdensur is not prescribed concurrently with Cinqair, Fasenra, Nucala, Dupixent, Xolair, or Tezspire;

o If request is for a dose increase, new dose does not exceed 100 mg every 6 months.

o Approval duration: 12 months

Olezarsen (Tryngolza) (CP.PHAR.689)

Ambetter

Policy includes:

· Criteria will mirror the clinical information from the prescribing information once FDA-approved

· Requests for indications not approved by the FDA are reviewed with the off-label use policy for the relevant line of business: HIM.PHAR.154 for Ambetter

· Initial Approval Criteria*: Hypertriglyceridemia (must meet all):

*Criteria will mirror the clinical information from the prescribing information once FDA-approved

o Diagnosis of hypertriglyceridemia as evidenced by fasting triglycerides at least 500 mg/dL (lab must be dated within 90 days);*

o Prescribed by or in consultation with a cardiologist, endocrinologist, or lipid specialist;

o Age at least 18 years;*

o Failure of at least 3 consecutive month trial of both of the following at up to maximally indicated doses, unless clinically significant adverse effects are experienced or all are contraindicated;

§ Fibrate therapy;

§ Omega-3 fatty acids therapy^;

^Prior authorization may be required for omega-3 fatty acids

o Member is concurrently receiving standard of care lipid-lowering treatment (e.g., statins, ezetimibe, fibrates, omega-3 fatty acids, niacin), if clinically appropriate;

o Dose does not exceed 80 mg per month.*

o Approval duration: 12 months

· Continued Therapy*: Hypertriglyceridemia (must meet all):

*Criteria will mirror the clinical information from the prescribing information once FDA-approved

o Member meets one of the following:

§ Currently receiving medication via Centene benefit or member has previously met initial approval criteria;

§ Member is currently receiving medication and is enrolled in a state and product with continuity of care regulations;

o Member is responding positively to therapy as evidenced by reduction in fasting triglycerides from baseline;

o Member is concurrently receiving standard of care lipid-lowering treatment (e.g., statins, ezetimibe, fibrates, omega-3 fatty acids, niacin), if clinically appropriate;

o If request is for a dose increase, new dose does not exceed 80 mg per month.*

o Approval duration: 12 months

Glepaglutide (ZP1848) (CP.PHAR.694)

Ambetter

Policy includes:

· Requests for indications not approved by the FDA are reviewed with the off-label use policy for the relevant line of business: HIM.PHAR.154 for Ambetter

· Criteria will mirror the clinical information from the prescribing information once FDA-approved

· Initial Approval Criteria*: Short Bowel Syndrome (SBS) (must meet all):

*Criteria will mirror the clinical information from the prescribing information once FDA-approved

o Diagnosis of SBS;*

o Prescribed by or in consultation with a gastroenterologist;

o Age at least 18 years;*

o Member is currently receiving parenteral support (i.e., parenteral nutrition and/or intravenous fluids) at least 3 days per week;*

o Glepaglutide is not prescribed concurrently with Gattex®;

o Dose does not exceed 20 mg per week.*

o Approval duration: 12 months

· Continued Therapy*: Short Bowel Syndrome (must meet all):

*Criteria will mirror the clinical information from the prescribing information once FDA-approved

o Member meets one of the following:

§ Currently receiving medication via Centene benefit or member has previously met initial approval criteria;

§ Member is currently receiving medication and is enrolled in a state and product with continuity of care regulations;

o Requirement for parenteral nutrition or other intravenous support has decreased since initiation of glepaglutide;*

o Glepaglutide is not prescribed concurrently with Gattex;

o If request is for a dose increase, new dose does not exceed 20 mg per week.*

o Approval duration: 12 months

Ataluren (Translarna) (CP.PHAR.710)

Ambetter

Policy includes:

· Requests for indications not approved by the FDA are reviewed with the off-label use policy for the relevant line of business: HIM.PHAR.154 for Ambetter

· Criteria will mirror the clinical information from the prescribing information once FDA-approved

· Initial Approval Criteria*: Duchenne Muscular Dystrophy (DMD) (must meet all):

*Criteria will mirror the clinical information from the prescribing information once FDA-approved

o Diagnosis of nonsense mutation DMD (nmDMD) confirmed by genetic testing;*

o Prescribed by or in consultation with a neurologist;

o Age at least 5 years;*

o Member has all of the following assessed within the last 30 days:

§ Ambulatory function (e.g., ability to walk with or without assistive devices, not wheelchair dependent) with a 6-minute walk test (6MWT) distance at least 150 m;

§ Stable cardiac function with left ventricular ejection fraction (LVEF) greater than 40%;

§ Stable pulmonary function with predicted forced vital capacity (FVC) at least 50%;

o Inadequate response (as evidenced by a significant decline in 6MWT, LVEF, or FVC) despite adherent use of an oral corticosteroid (e.g., prednisone, Emflaza®, Agamree®) for at least 12 months, unless contraindicated or clinically significant adverse effects are experienced;

*Prior authorization is required for Emflaza and Agamree

o Translarna is prescribed concurrently with an oral corticosteroid, unless contraindicated or clinically significant adverse effects are experienced;

o Translarna is not prescribed concurrently with exon-skipping therapies (e.g., Amondys 45®, Exondys 51®, Vyondys 53®, Viltepso®);

o Member has not previously received gene replacement therapy for DMD (e.g., Elevidys®);

o Documentation of member’s current body weight in kg;

o Dose does not exceed 40 mg/kg per day.*

o Approval duration: 6 months

· Continued Therapy*: Duchenne Muscular Dystrophy (must meet all):

*Criteria will mirror the clinical information from the prescribing information once FDA-approved

o Currently receiving medication for nmDMD or member has previously met initial approval criteria;

o Member is responding positively to therapy as evidenced by one of the following:

§ All of the following assessed within the last 6 months (i, ii, and iii):

· Ambulatory function (e.g., ability to walk with or without assistive devices, not wheelchair dependent) with a 6MWT distance at least 150 m;

· Stable cardiac function with LVEF greater than 40%;

· Stable pulmonary function with predicted FVC at least 50%;

§ Member has received this medication via a healthcare insurer without meeting the requirements above (see criterion 2a), and medical record shows improved or stable LVEF and FVC, assessed within the last 6 months;

o Member has been assessed by a neurologist within the last 6 months;

o Translarna is prescribed concurrently with an oral corticosteroid, unless contraindicated or clinically significant adverse effects are experienced;

o Translarna is not prescribed concurrently with exon-skipping therapies (e.g., Amondys 45, Exondys 51, Vyondys 53, Viltepso);

o Member has not previously received gene replacement therapy for DMD (e.g., Elevidys);

o Documentation of member’s current body weight in kg;

o If request is for a dose increase, new dose does not exceed 40 mg/kg per day.*

o Approval duration: 6 months

Copper Histidinate (CUTX-101) (CP.PHAR.714)

Ambetter

Policy includes:

· Requests for indications not approved by the FDA are reviewed with the off-label use policy for the relevant line of business: HIM.PHAR.154 for Ambetter

· Criteria will mirror the clinical information from the prescribing information once FDA-approved

· Initial Approval Criteria*: Menkes Disease (must meet all):

*Criteria will mirror the clinical information from the prescribing information once FDA-approved

o Diagnosis of Menkes disease;*

o Diagnosis is confirmed by one of the following methods:*

§ Genetic testing demonstrating mutation in the ATP7A gene;

§ Both of the following:

· Biochemically with one of the following:

o Low serum copper levels (less than 75 mcg/dL);

o Low ceruloplasmin;

o Abnormal plasma catecholamine levels;

· Clinically based on signs of abnormal hair color/texture, seizures, hypotonia, or developmental delay;

o Prescribed by or in consultation with a neonatologist, neurologist, or specialist with expertise in the management of metabolic disorders (e.g., pediatric geneticist);

o Dose does not exceed one of the following:*

§ Age at most 12 months: 2,900 mcg per day;

§ Age greater than 12 months: 1,450 mcg per day.

o Approval duration: 6 months*

o Continued Therapy*: Menkes Disease (must meet all):

*Criteria will mirror the clinical information from the prescribing information once FDA-approved

§ Member meets one of the following:

· Currently receiving medication via Centene benefit or member has previously met initial approval criteria;

· Member is currently receiving medication and is enrolled in a state and product with continuity of care regulations;

§ Member is responding positively to therapy;*

§ If request is for a dose increase, new dose does not exceed one of the following:*

· Age at most 12 months: 2,900 mcg per day;

· Age greater than 12 months: 1,450 mcg per day.

§ Approval duration: 12 months

Deramiocel (CAP-1002) (CP.PHAR.716)

Ambetter

Policy includes:

· Criteria will mirror the clinical information from the prescribing information once FDA-approved

· Requests for indications not approved by the FDA are reviewed with the off-label use policy for the relevant line of business: HIM.PHAR.154 for Ambetter

· Initial Approval Criteria*: Duchenne Muscular Dystrophy (DMD) (must meet all):

*Criteria will mirror the clinical information from the prescribing information once FDA-approved

o Diagnosis of DMD confirmed by genetic testing;*

o One of the following:

§ Prescribed by or in consultation with a neurologist;

§ Member is being treated at a certified Duchenne care center or a Muscular Dystrophy Association care center;*

o Age at least 10 years;*

o Member has a performance upper limb (PUL 2.0) entry item score of 2 – 5;*

o One of the following:*

§ Member is non-ambulatory;

§ If member is late ambulatory (i.e., ability to walk with or without assistive devices, not wheelchair dependent) defined as a 10-meter walk time of greater than 10 seconds, age is less than 18 years;

o Member meets both of the following assessed within the last 30 days:*

§ Stable cardiac function with left ventricular ejection fraction (LVEF) at least 35%;

§ Stable pulmonary function with predicted forced vital capacity (FVC) at least 35%;

o Member has been on a stable dose of an oral corticosteroid (e.g., prednisone, Emflaza®*, Agamree®*) for at least 6 months, unless contraindicated or clinically significant adverse effects are experienced;*

*Prior authorization is required for Emflaza and Agamree

o CAP-1002 is prescribed concurrently with an oral corticosteroid, unless contraindicated or clinically significant adverse effects are experienced;*

o Member has not received prior stem cell therapy;*

o Member has not previously received gene replacement therapy for DMD (e.g., Elevidys®);*

o If member is currently on an exon-skipping therapy (e.g., Amondys 45™, Exondys 51®, Viltepso™, Vyondys 53™), member must discontinue therapy prior to CAP-1002;

o Dose does not exceed 150 million cells every 3 months.*

o Approval duration: 6 months

· Continued Therapy*: Duchenne Muscular Dystrophy (must meet all):

*Criteria will mirror the clinical information from the prescribing information once FDA-approved

o Member meets one of the following:

§ Currently receiving medication via Centene benefit or member has previously met initial approval criteria;

§ Member is currently receiving medication and is enrolled in a state and product with continuity of care regulations;

o Member is responding positively to therapy as evidenced by, including but not limited to, improvement or stabilization in member’s PUL score, LVEF, and FVC ;*

o Member has been assessed by a neurologist within the last 6 months;

o CAP-1002 is prescribed concurrently with an oral corticosteroid, unless contraindicated or clinically significant adverse effects are experienced;

o Member has not received prior stem cell therapy;*

o Member has not previously received gene replacement therapy for DMD (e.g., Elevidys);*

o CAP-1002 is not prescribed concurrently with exon-skipping therapies (e.g., Amondys 45, Exondys 51, Viltepso, Vyondys 53);*

o If request is for a dose increase, new dose does not exceed 150 million cells every 3 months.*

o Approval duration: 6 months

Mozafancogene Autotemcel (RP-L102) (CP.PHAR.719)

Ambetter

Policy includes:

· Criteria will mirror the clinical information from the prescribing information once FDA-approved

· Requests for indications not approved by the FDA are reviewed with the off-label use policy for the relevant line of business: HIM.PHAR.154 for Ambetter

· Initial Approval Criteria*: Fanconi Anemia (FA) (must meet all):

Criteria will mirror the clinical information from the prescribing information once FDA-approved

· *Only for initial treatment dose; subsequent doses will not be covered.

o Diagnosis of FA complementation group A as evidenced by both of the following;*

§ FANCA gene mutation confirmed by genetic testing;

§ One of the following:

· Positive chromosome breakage test in peripheral blood;

· Documentation of potential somatic mosaicism (e.g., negative, or equivocal chromosome breakage test) and medically significant decrease in at least one blood cell lineage over time;

o Prescribed by or in consultation with a transplant specialist, hematologist, or geneticist;

o Age at least 1 year and at most 12 years;*

o Weight at least 8 kg;*

o One of the following:

§ Member has no available human leukocyte antigen (HLA)-matched (i.e., full HLA-matching of all evaluated alleles) donor;

§ Member has an available HLA-matched donor, and both of the following:

· Provider submits medical rationale that allogeneic hematopoietic stem cell transplantation (HSCT) is not feasible (e.g., donor unable to undergo donation procedure because of medical impairments);

· ii. Member understands the risks and benefits of alternative therapeutic options such as allogeneic HSCT;

o Transplant specialist attestation that member is clinically stable and eligible to undergo myeloablative conditioning and HSCT;

o Member has not received prior allogeneic HSCT;

o Member has not received prior gene therapy;

o Both of the following:

§ Dose does not exceed a single administration;

§ Dose contains a minimum of 3 x 105CD34+ cells/kg.*

o Approval duration: 6 months (one time infusion per lifetime)

· Continued Therapy*: Fanconi Anemia

*Criteria will mirror the clinical information from the prescribing information once FDA-approved

o Continued therapy will not be authorized as RP-L102 is indicated to be dosed one time only.

o Approval duration: Not applicable

Plozasiran (ARO-APOC3) (CP.PHAR.721)

Ambetter

Policy includes:

· Criteria will mirror the clinical information from the prescribing information once FDA-approved

· Requests for indications not approved by the FDA are reviewed with the off-label use policy for the relevant line of business: HIM.PHAR.154 for Ambetter

· Initial Approval Criteria*: Familial Chylomicronemia Syndrome (FCS) (must meet all):

*Criteria will mirror the clinical information from the prescribing information once FDA-approved

o Diagnosis of FCS as evidenced by all of the following:*

§ Fasting triglycerides at least 880 mg/dL or at least 10 mmol/L (lab must be dated within 90 days);

§ One of the following:

· Genetic testing confirms the presence a loss-of-function mutation in a FCS-causing gene (e.g., LPL, APOC2, APOA5, GPIHBP1, LMF1);

· History of pancreatitis;

· Family history of hypertriglyceridemia;

· History of recurrent abdominal pain without other explainable cause;

§ Documentation of nonresponse to both of the following, at up to maximally indicated doses, unless clinically significant adverse effects are experienced or all are contraindicated:

· Fibrates (e.g., fenofibric acid, fenofibrate, fenofibrate micronized, gemfibrozil);

· Omega-3 fatty acids (e.g., omega-3-acid-ethyl esters, icosapent ethyl);

o Prescribed by or in consultation with an endocrinologist, lipid specialist, or cardiologist;

o Age at least 18 years;*

o ARO-APOC3 is not prescribed concurrently with Tryngolza™;

o Dose does not exceed 50 mg every 3 months.*

o Approval duration: 6 months

· Continued Therapy*: Familial Chylomicronemia Syndrome (must meet all):

*Criteria will mirror the clinical information from the prescribing information once FDA-approved

· Familial Chylomicronemia Syndrome (must meet all):

o Member meets one of the following:

§ Currently receiving medication via Centene benefit or member has previously met initial approval criteria;

§ Member is currently receiving medication and is enrolled in a state and product with continuity of care regulations;

o Member is responding positively to therapy as evidenced by reduction in fasting triglycerides from baseline;

o If request is for a dose increase, new dose does not exceed 50 mg every 3 months.*

o Approval duration: 12 months

Rebisufligene Etisparvovec (UX111) (CP.PHAR.722)

Ambetter

Policy includes:

· Criteria will mirror the clinical information from the prescribing information once FDA-approved

· Requests for indications not approved by the FDA are reviewed with the off-label use policy for the relevant line of business: HIM.PHAR.154 for Ambetter

· Initial Approval Criteria*: Mucopolysaccharidosis IIIA: Sanfilippo Syndrome Type A (must meet all):

*Criteria will mirror the clinical information from the prescribing information once FDA-approved

o Diagnosis of MPS IIIA confirmed by both of the following:*

§ Genetic confirmation of homozygous or compound heterozygous mutations in the SGSH gene;

§ Leukocyte assay demonstrating undetectable or significantly reduced SGSH enzyme activity;

o Prescribed by or in consultation with a specialist with expertise in lysosomal storage diseases (e.g., medical geneticist, pediatric endocrinologist) or neurologist;

o Age at most 4 years;*

o Member does not have anti-AAV9 antibody titers at least 1:100;*

o Member does not have either of the following via genetic testing:*

§ Two nonsense or null variants of the SGSH gene;

§ One or more S298P mutation(s) in the SGSH gene;

o Member has not previously received hematopoietic stem cell transplantation;*

o Member has not previously received cell or gene therapy;*

o Documentation of member’s current weight in kg;

o Dose does not exceed 3 × 1013vector genomes/kg.*

o Approval duration: 3 months (one infusion per lifetime)

· Continued Therapy*: Mucopolysaccharidosis IIIA: Sanfilippo Syndrome Type A

*Criteria will mirror the clinical information from the prescribing information once FDA-approved

o Re-authorization is not permitted as UX111 is indicated to be dosed one time only.

o Approval duration: Not applicable

Sodium Dichloroacetate (SL-1009) (CP.PHAR.724)

Ambetter

Policy includes:

· Requests for indications not approved by the FDA are reviewed with the off-label use policy for the relevant line of business: HIM.PHAR.154 for Ambetter

· Criteria will mirror the clinical information from the prescribing information once FDA-approved

· Initial Approval Criteria*: Pyruvate Dehydrogenase Complex Deficiency (must meet all):

*Criteria will mirror the clinical information from the prescribing information once FDA-approved

o Diagnosis of PDCD confirmed by the presence of clinical or metabolic features of PDCD and one of the following (a or b; see Appendix D):*

§ Known pathogenic mutation of a gene that is specifically associated with PDCD;

§ Both of the following:

· A variant of uncertain significance for a mutation in a gene that is specifically associated with PDCD;

· Enzyme assay demonstrating a deficiency of pyruvate dehydrogenase complex activity;

o Prescribed by or in consultation with an endocrinologist, geneticist, neurologist, pediatrician, or metabolic disease specialist;*

o Age at most 17 years;*

o Member is currently on a ketogenic diet (i.e., a high fat, low carbohydrate diet) and will continue this diet during treatment with SL-1009;*

o Dose does not exceed the FDA-approved maximum dose.*

o Approval duration: 6 months

· Continued Therapy*: Pyruvate Dehydrogenase Complex Deficiency (must meet all):

*Criteria will mirror the clinical information from the prescribing information once FDA-approved

o Member meets one of the following:

§ Currently receiving medication via Centene benefit or member has previously met initial approval criteria;

§ Member is currently receiving medication and is enrolled in a state and product with continuity of care regulations;

o Member is responding positively to therapy;*

o Member is currently on a ketogenic diet and will continue this diet during treatment with SL-1009;*

o If request is for a dose increase, new dose does not exceed the FDA-approved maximum dose.*

o Approval duration: 12 months

Rexlemestrocel-L (Revascor) (CP.PHAR.728)

Ambetter

Policy includes:

· Criteria will mirror the clinical information from the prescribing information once FDA-approved

· Requests for indications not approved by the FDA are reviewed with the off-label use policy for the relevant line of business: HIM.PHAR.154 for Ambetter

· Initial Approval Criteria*

*Criteria will mirror the clinical information from the prescribing information once FDA-approved

o Advanced Heart Failure (must meet all):^^If member has an LVAD, please refer to criteria set I.B below.

§ Diagnosis of advanced HFrEF with all of the following characteristics (a-e):*

· High-risk defined as one of the following (i, ii, or iii):

o At least 1 heart failure hospitalization in the last 9 months;

o At least 1 outpatient urgent care heart failure visit requiring intravenous diuretic, vasodilator, and/or positive inotropic therapy in the last 9 months;

o Plasma levels of N-terminal pro–B-type natriuretic peptide (NT-proBNP) greater than 1,000 pg/mL (greater than 1,200 pg/mL for members with atrial fibrillation);

· Left ventricular ejection fraction (LVEF) at most 40% by 2-dimensional echocardiogram or at most 35% by multigated acquisition scan;

· New York Heart Association (NYHA) functional class II or III;

· Presence of ischemic cardiomyopathy;

· Presence of inflammation as evidenced by baseline plasma high-sensitivity C-reactive protein (CRP) at least 2 mg/L;

§ Prescribed by or in consultation with a cardiologist;

§ Age at least 18 years;*

§ Member is receiving stable (i.e., no changes in dose for at least the last month), optimally tolerated dosages of guideline-directed medical therapies for HFrEF that includes all of the following, unless clinically significant adverse effects are experienced or all are contraindicated (a, b, c, and d; Appendix B):*

· Beta-blocker;

· Angiotensin receptor/neprilysin inhibitor (ARNI), angiotensin-converting enzyme (ACE) inhibitor, or angiotensin-receptor blocker (ARB);

· Mineralocorticoid antagonist;

· Sodium-glucose cotransporter 2 (SGLT2) inhibitor;

§ Member has not previously received any stem cell therapy;*

§ Dose does not exceed a single transendocardial injection.*

§ Approval duration: 3 months (one transendocardial injection per lifetime)

o End-Stage Heart Failure (must meet all):

§ Diagnosis of end-stage chronic HFrEF with all of the following characteristics:*

· LVEF at most 40% by 2-dimensional echocardiogram or at most 35% by multigated acquisition scan;

· NYHA functional class III or IV;

· Presence of ischemic cardiomyopathy;

§ Prescribed by or in consultation with a cardiologist;

§ Age at least 18 years;*

§ Member has an implanted LVAD, or is scheduled to receive an LVAD and will receive Revascor at the time of LVAD implantation;*

§ Member is receiving stable (i.e., no changes in dose for at least the last month), optimally tolerated dosages of guideline-directed medical therapies for HFrEF that includes all of the following, unless clinically significant adverse effects are experienced or all are contraindicated (a, b, c, and d; see Appendix B):*

· Beta-blocker;

· ARNI, ACE inhibitor, or ARB;

· Mineralocorticoid antagonist;

· SGLT2 inhibitor;

§ Member has not previously received any stem cell therapy;*

§ Dose does not exceed a single transendocardial injection.*

§ Approval duration: 3 months (one transendocardial injection per lifetime)

· Continued Therapy*

*Criteria will mirror the clinical information from the prescribing information once FDA-approved

o Heart Failure

§ Continued therapy will not be authorized as Revascor is indicated to be dosed one time only.*

§ Approval duration: Not applicable

Vatiquinone (PTC743) (CP.PHAR.729)

Ambetter

Policy includes:

· Requests for indications not approved by the FDA are reviewed with the off-label use policy for the relevant line of business: HIM.PHAR.154 for Ambetter

· Criteria will mirror the clinical information from the prescribing information once FDA-approved

· Initial Approval Criteria*: Friedreich’s Ataxia (must meet all):

*Criteria will mirror the clinical information from the prescribing information once FDA-approved

o Diagnosis of FA;*

o Documentation of genetic testing that shows a GAA triplet-repeat expansion in the frataxin (FXN) gene; *

o Prescribed by or in consultation with a neurologist;

o Age at least 7 years;*

o Recent (within the last 30 days) baseline modified Functional Assessment Rating Scale (mFARS) score; *

o Recent (within the last 30 days) baseline ventricular ejection fraction at least 50%;*

o PTC743 is not prescribed concurrently with Skyclarys®;*

o Dose does not exceed 1,200 mg per day.*

o Approval duration: 6 months

· Continued Therapy*: Friedreich’s Ataxia (must meet all):

*Criteria will mirror the clinical information from the prescribing information once FDA-approved

o Member meets one of the following:

§ Currently receiving medication via Centene benefit or member has previously met initial approval criteria;

§ Member is currently receiving medication and is enrolled in a state and product with continuity of care regulations;

o Member is responding positively to therapy as evidenced by, including but not limited to, improvement in any of the following parameters: FA symptoms or mFARS score; *

o If request is for a dose increase, new dose does not exceed 1,200 mg per day.*

o Approval duration: 12 months

Clemidsogene Lanparvovec (RGX-121) (CP.PHAR.734)

Ambetter

Policy includes:

· Criteria will mirror the clinical information from the prescribing information once FDA-approved

· Requests for indications not approved by the FDA are reviewed with the off-label use policy for the relevant line of business: HIM.PHAR.154 for Ambetter

· Initial Approval Criteria*: Mucopolysaccharidosis II: Hunter Syndrome (must meet all):

*Criteria will mirror the clinical information from the prescribing information once FDA-approved

o Diagnosis of MPS II (Hunter syndrome) confirmed by both of the following:*

§ Genetic confirmation of pathogenic or likely pathogenic mutation(s) in the iduronate-2-sulfatase (IDS) gene;

§ Enzyme assay demonstrating a deficiency of IDS activity;

o Prescribed by or in consultation with a specialist with expertise in lysosomal storage diseases (e.g., medical geneticist, pediatric endocrinologist) or a neurologist;*

o Age at least 4 months to at most 5 years;*

o Member has or is expected to have a neuronopathic form of MPS II as evidenced by one of the following:*

§ Neurocognitive testing demonstrating neuronopathic involvement;

§ Documented mutation(s) in IDS gene known to result in a neuronopathic phenotype;

§ Both of the following:

· Member has a relative clinically diagnosed with neuronopathic MPS II who has the same IDS gene mutation as the member;

· Geneticist determines member has inherited a neuronopathic form of MPS II;

o Member has not previously received hematopoietic stem cell transplantation;*

o Member has not previously received an AAV-based gene therapy product;*

o Dose does not exceed 2.9 x 1011 genome copies/g brain mass.*

o Approval duration: 3 months (one intracisternal injection per lifetime)

· Continued Therapy*: Mucopolysaccharidosis II: Hunter Syndrome

*Criteria will mirror the clinical information from the prescribing information once FDA-approved

o Re-authorization is not permitted as RGX-121 is indicated to be dosed one time only.

o Approval duration: Not applicable

Relacorilant (CP.PHAR.736)

Ambetter

Policy includes:

· Requests for indications not approved by the FDA are reviewed with the off-label use policy for the relevant line of business: HIM.PHAR.154 for Ambetter

· Criteria will mirror the clinical information from the prescribing information once FDA-approved

· Initial Approval Criteria: Cushing’s Syndrome (must meet all):

*Criteria will mirror the clinical information from the prescribing information once FDA-approved

o Diagnosis of Cushing’s syndrome;*

o Member has one of the following:*

§ Type 2 diabetes mellitus;

§ Impaired glucose tolerance as evidenced by plasma glucose at least 140 and less than 200 mg/dL on 2-hour oral glucose tolerance test in the last 30 days;

§ Uncontrolled hypertension as evidenced by one of the following in the last 30 days:

· Mean systolic blood pressure at least 135 to at most 170 mmHg;

· Mean diastolic blood pressure at least 85 to at most 110 mmHg;

o Prescribed by or in consultation with an endocrinologist;

o Age at least 18 years;*

o Member meets one of the:*

§ Surgery was not curative;

§ Member is not eligible for surgery;

o Dose does not exceed 400 mg per day.*

o Approval duration: 6 months

· Continued Therapy*: Cushing’s Syndrome (must meet all):

*Criteria will mirror the clinical information from the prescribing information once FDA-approved

o Member meets one of the following:

§ Currently receiving medication via Centene benefit or member has previously met initial approval criteria;

§ Member is currently receiving medication and is enrolled in a state and product with continuity of care regulations;

o Member is responding positively to therapy;

o If request is for a dose increase, new dose does not exceed 400 mg per day.*

o Approval duration: 12 months

Apitegromab (SRK-015) (CP.PHAR.737)

Ambetter

Policy includes:

· Criteria will mirror the clinical information from the prescribing information once FDA-approved

· Requests for indications not approved by the FDA are reviewed with the off-label use policy for the relevant line of business: HIM.PHAR.154 for Ambetter

· Initial Approval Criteria*: Spinal Muscular Atrophy

*Criteria will mirror the clinical information from the prescribing information once FDA-approved

o Diagnosis of SMA confirmed by the presence of one of the following:*

§ Homozygous deletions of SMN1 gene (e.g., absence of the SMN1 gene);

§ Homozygous mutation in the SMN1 gene (e.g., biallelic mutations of exon 7);

§ Compound heterozygous mutation in the SMN1 gene (e.g., deletion of SMN1 exon 7 (allele 1) and mutation of SMN1 (allele 2));

o Documentation of genetic testing confirming no more than 4 copies of SMN2 gene;*

o Prescribed by or in consultation with a neurologist; 

o Age at least 2 years;*

o Member meets one of the following:*

§ Has been receiving Spinraza® for at least 10 months;

§ Has been receiving Evrysdi® for at least 6 months; 

o Member is concurrently receiving treatment with Spinraza or Evrysdi;* 

o Member has not been previously treated with Zolgensma®;*

o Documentation of baseline Hammersmith functional motor scale expanded (HFMSE) score;

o Member does not require tracheostomy or invasive or noninvasive ventilation for greater than 16 hours/day continuously for at least 14 days;*

o Documentation of member’s current weight in kg;*

o Dose does not exceed 20 mg/kg every 4 weeks.*

o Approval duration: 6 months

· Continued Therapy*: Spinal Muscular Atrophy

* Criteria will mirror the clinical information from the prescribing information once FDA-approved

o Currently receiving medication for SMA with 1 to 4 copies of the SMN2 gene, or member has previously met initial approval criteria;*

o Member does not require tracheostomy or invasive or noninvasive ventilation for greater than 16 hours/day continuously for at least 14 days;*

o Member is responding positively to therapy as evidenced by one of the following:*

§ Must demonstrate HFMSE score improvement or maintenance of previous score improvement from baseline;

§ Member has not had a decline in motor function test score(s) from baseline AND medical justification demonstrates and supports that member is responding positively to therapy;

o Member is concurrently receiving treatment with Spinraza or Evrysdi;*

o Member has not been previously treated with Zolgensma;*

o Documentation of member’s current weight in kg;*

o If request is for a dose increase, new dose does not exceed 20 mg/kg every 4 weeks.*

o Approval duration: 12 months

Doxecitine and Doxribtimine (MT1621) (CP.PHAR.738)

Ambetter

Policy includes:

· Requests for indications not approved by the FDA are reviewed with the off-label use policy for the relevant line of business: HIM.PHAR.154 for Ambetter

· Criteria will mirror the clinical information from the prescribing information once FDA-approved

· Initial Approval Criteria*: Thymidine Kinase 2 Deficiency

*Criteria will mirror the clinical information from the prescribing information once FDA-approved

o Diagnosis of TK2d as evidenced by a mutation in the thymidine kinase 2 (TK2) gene;*

o Prescribed by or in consultation with a neurologist or metabolic disease specialist;* 

o Age of symptom onset at most 12 years (e.g., proximal muscle weakness, respiratory weakness, facial diplegia);*

o Documentation of member’s current weight in kg;*

o Dose does not exceed doxecitine 400 mg/kg and doxribtimine 400 mg/kg per day.*

o Approval duration: 6 months

· Continued Therapy*: Thymidine Kinase 2 Deficiency

*Criteria will mirror the clinical information from the prescribing information once FDA-approved

o Member meets one of the following:

§ Currently receiving medication via Centene benefit or member has previously met initial approval criteria;

§ Member is currently receiving medication and is enrolled in a state and product with continuity of care regulations;

o Member is responding positively to therapy as evidenced by, including but not limited to, improvement in any of the following parameters: improvement in motor function, walking ability, sitting ability, respiratory symptoms, nutritional status (e.g., body mass index), quality of life;*

o Documentation of member’s current weight in kg;*

o If request is for a dose increase, new dose does not exceed doxecitine 400 mg/kg and doxiribtimine 400 mg/kg per day.*

o Approval duration: 12 months

Navepegritide (TransCon CNP) (CP.PHAR.746)

Ambetter

Policy includes:

· Criteria will mirror the clinical information from the prescribing information once FDA-approved

· Requests for indications not approved by the FDA are reviewed with the off-label use policy for the relevant line of business: HIM.PHAR.154 for Ambetter

· Initial Approval Criteria*: Achondroplasia (must meet all):'

*Criteria will mirror the clinical information fANVe prescribing information once FDA-approved

o Diagnosis of achondroplasia with genetic testing confirming a mutation in the fibroblast growth receptor 3 (FGFR3) gene;

o Prescribed by or in consultation with a pediatric endocrinologist;

o Age between 2 and 11 years;

o At the time of request, radiographic evidence indicates open epiphyses (growth plates);

o Documentation of baseline annualized growth velocity, calculated based on standing height measured over the course of 6 months prior to request;

o Documentation of member's current weight (in kg);

o TransCon CNP is not prescribed concurrently with any human growth hormone products (e.g., Genotropin®, Humatrope®, Norditropin®, Nutropin AQ®, Omnitrope®, Saizen®, Zomacton®);

o TransCon CNP is not prescribed concurrently with Voxzogo;

o Dose does not exceed 100 mcg/kg per week.

o Approval duration: 6 months

· Continued Therapy*: Achondroplasia (must meet all):

*Criteria will mirror the clinical information from the prescribing information once FDA-approved

o Member meets one of the following:

§ Currently receiving medication via Centene benefit or member has previously met initial approval criteria;

§ Member is currently receiving medication and is enrolled in a state and product with continuity of care regulations;

o Member is responding positively to therapy as evidenced by improvement in annualized growth velocity from baseline;

o Radiographic evidence within the last four months indicates that the member continues to have open epiphyses (growth plates);

o Documentation of member's current weight (in kg);

o If request is for a dose increase, new dose does not exceed 100 mcg/kg per week.

o Approval duration: 6 months

Tabelecleucel (Tab-cel) (CP.PHAR.747)

Ambetter

Policy includes:

· Criteria will mirror the clinical information from the prescribing information once FDA-approved

· Requests for indications not approved by the FDA are reviewed with the off-label use policy for the relevant line of business: HIM.PHAR.154 for Ambetter

· Initial Approval Criteria*: Post-Transplant Lymphoproliferative Disease

*Criteria will mirror the clinical information from the prescribing information once FDA-approved

o Diagnosis of PTLD;*

o Prescribed by or in consultation with an oncologist, hematologist, or transplant specialist; 

o Age at least 2 years;*

o Member has previously received a hematopoietic cell transplantation or a solid organ transplant;

o Disease is EBV seropositive;

o One of the following:

o Disease is refractory;

o Member has relapsed after at least one line of therapy that includes rituximab or a rituximab biosimilar (see Appendix B for examples);* *Prior authorization may be required for rituximab or rituximab biosimilar

o Tab-cel is not prescribed concurrently with chimeric antigen receptor (CAR) T-cell immunotherapy (e.g., Abecma®, Carvykti®, Breyanzi®, Kymriah™, Tecartus®, Yescarta®);

o Documentation of member’s current body weight in kg;

o Request meets one of the following:*

§ Dose does not exceed 2 x 106 viable T cells/kg (per dose) on days 1, 8, and 15 of a 35 day cycle;*

§ Dose is supported by practice guidelines or peer-reviewed literature for the relevant off-label use (prescriber must submit supporting evidence). *Prescribed regimen must be FDA-approved or recommended by NCCN

o Approval duration: 6 months

· Continued Therapy*: Post-Transplant Lymphoproliferative Disease 

*Criteria will mirror the clinical information from the prescribing information once FDA-approved

o Currently receiving medication via Centene benefit, or documentation supports that member is currently receiving Tab-cel for a covered indication and has received this medication for at least 30 days;

o Member has not switched at least 4 times to a Tab-cel lot with a different human leukocyte antigen (HLA) restriction;

o Member has not achieved 2 consecutive complete responses to the same Tab-cel lot;

o Member has not achieved 3 consecutive partial responses to the same Tab-cel lot;

o Member has not received at least 15 cycles (45 doses) of Tab-cel;

o Tab-cel is not prescribed concurrently with CAR T-cell immunotherapy (e.g., Abecma, Carvykti, Breyanzi, Kymriah, Tecartus, Yescarta);

o If request is for a dose increase, documentation of member’s current body weight in kg;

o If request is for a dose increase, request meets one of the following:*

§ New dose does not exceed 2 x 106 viable T cells/kg (per dose) on days 1, 8, and 15 of a 35 day cycle;*

§ New dose is supported by practice guidelines or peer-reviewed literature for the relevant off-label use (prescriber must submit supporting evidence). *Prescribed regimen must be FDA-approved or recommended by NCCN

o Approval duration: 6 months 

Tividenofusp Alfa (DNL310) (CP.PHAR.748)

Ambetter

Policy includes:

· Criteria will mirror the clinical information from the prescribing information once FDA-approved

· Requests for indications not approved by the FDA are reviewed with the off-label use policy for the relevant line of business: HIM.PHAR.154 for Ambetter

· Initial Approval Criteria*: Mucopolysaccharidosis II (MPS II): Hunter Syndrome

*Criteria will mirror the clinical information from the prescribing information once FDA-approved

o Diagnosis of MPS II (Hunter syndrome) confirmed by one of the following:

§ Enzyme assay demonstrating a deficiency of iduronate 2-sulfatase (IDS) activity;

§ Genetic confirmation of pathogenic or likely pathogenic mutation(s) in the IDS gene;

o Age at least 3 months;* 

o DNL310 is not prescribed concurrently with Elaprase®;*

o Documentation of member’s current weight (in kg);

o Dose does not exceed FDA-labeled maximum dose.*

o Approval duration: 12 months 

· Continued Therapy*: Mucopolysaccharidosis II: Hunter Syndrome

*Criteria will mirror the clinical information from the prescribing information once FDA-approved

o Member meets one of the following:

§ Currently receiving medication via Centene benefit or member has previously met initial approval criteria;

§ Member is currently receiving medication and is enrolled in a state and product with continuity of care regulations;

o Member is responding positively to therapy as evidenced by improvement or stabilization in the individual member’s MPS II manifestation profile;*

o DNL310 is not prescribed concurrently with Elaprase;*

o Documentation of member’s current weight (in kg);

o If request is for a dose increase, new dose does not exceed FDA-labeled maximum dose.*

o Approval duration: 12 months 

Sonpiretigene Isteparvovec (MCO-010) (CP.PHAR.749)

Ambetter

Policy includes:

· Criteria will mirror the clinical information from the prescribing information once FDA-approved

· Requests for indications not approved by the FDA are reviewed with the off-label use policy for the relevant line of business: HIM.PHAR.154 for Ambetter

· Initial Approval Criteria*: Retinitis Pigmentosa (RP)

*Criteria will mirror the clinical information from the prescribing information once FDA-approved

o Diagnosis of RP as confirmed by genetic testing;*

o Prescribed by an ophthalmologist or retina specialist;* 

o Age at least 18 years;*

o Member has significant vision loss as evidenced by both of the following:

§ Best-corrected visual acuity (BCVA) worse than 1.9 logMAR in the eye receiving MCO-010;

§ BCVA less than 1.6 logMAR in the eye not receiving treatment;

o Provider attestation that treatment with MCO-010 will only be given in the eye with the lowest visual acuity;*

o Member has not previously been treated with MCO-010;*

o Dose does not exceed FDA maximum dose.*

o Approval duration: 4 weeks (1 lifetime dose)

· Continued Therapy*: Retinitis Pigmentosa

*Criteria will mirror the clinical information from the prescribing information once FDA-approved

o Continued therapy will not be authorized as MCO-010 is indicated to be a one-time application per lifetime.*

o Approval duration: Not applicable

Idebenone (Brand Name) (CP.PHAR.752)

Ambetter

Policy includes:

· Requests for indications not approved by the FDA are reviewed with the off-label use policy for the relevant line of business: HIM.PHAR.154 for Ambetter

· Criteria will mirror the clinical information from the prescribing information once FDA-approved

· Initial Approval Criteria*: Leber’s Hereditary Optic Neuropathy (LHON)

*Criteria will mirror the clinical information from the prescribing information once FDA-approved

o Diagnosis of LHON;*

o Genetic testing confirms the presence of one of the following):*

§ m.11778G>A mutation in the MT-ND4 gene; 

§ m.3460G>A mutation in the MT-ND1 gene; 

§ m.14484T>C mutation in the MT-ND6 gene; 

o Prescribed by or in consultation with an ophthalmologist; 

o Age at least 12 years;*

o Documentation of member’s baseline visual acuity (VA) using the Early Treatment of Diabetic Retinopathy Study (ETDRS) chart;*

o Member has impaired VA in at least one eye due to LHON;*

o Onset of vision loss due to LHON occurred within the last 5 years;*

o Dose does not exceed 900 mg (6 tablets) per day.*

o Approval duration: 12 months

· Continued Therapy*: Leber’s Hereditary Optic Neuropathy

*Criteria will mirror the clinical information from the prescribing information once FDA-approved

o Member meets one of the following:

§ Currently receiving medication via Centene benefit or member has previously met initial approval criteria;

§ Member is currently receiving medication and is enrolled in a state and product with continuity of care regulations;

o Member is responding positively to therapy as evidenced by one of the following:*

§ For the first re-authorization request, one of the following:

o If member had an off-chart VA (i.e., unable to read any letter on the ETDRS chart) at baseline, improvement of VA to reading at least 5 letters on the ETDRS chart (i.e., VA is at most 1.6 logMAR);

o If member had an on-chart VA (i.e., able to read letters on the ETDRS chart) at baseline, improvement of VA by at least 10 additional letters (i.e., change of -0.2 logMAR) on the ETDRS chart;

o iMaintenance of VA from baseline without deterioration to legal blindness (i.e., VA remains less than 1.0 logMAR) at the most recent visit on the ETDRS chart;

§ For second or subsequent re-authorization requests: Improvement or maintenance of VA from the last request at the most recent visit on the ETDRS chart;

o If request is for a dose increase, new dose does not exceed 900 mg (6 tablets) per day.*

o Approval duration: 12 months

DB-OTO (CP.PHAR.757)

Ambetter

Policy includes:

· Criteria will mirror the clinical information from the prescribing information once FDA-approved

· Requests for indications not approved by the FDA are reviewed with the off-label use policy for the relevant line of business: HIM.PHAR.154 for Ambetter

· Initial Approval Criteria*: Congenital Hearing Loss

*Criteria will mirror the clinical information from the prescribing information once FDA-approved

o Diagnosis of profound congenital hearing loss as evidenced by both of the following:*

§ Genetic testing confirms biallelic pathogenic or likely pathogenic OTOF mutation;

§ Member meets all of the following in the requested treatment ear(s):

o Profound hearing loss defined by an average audiometric threshold of greater than 90 dB hearing level;

o Absent auditory brainstem response (ABR);

o Intact outer hair cell function as evidenced by one of the following:

§ Presence of otoacoustic emissions (OAE); 

§ Presence of a cochlear microphonic;

o Prescribed by or in consultation with an otolaryngologist;

o Age less than 18 years;*

o Member does not have a history of a cochlear implant in the requested treatment ear(s);

o Member has not previously been treated with DB-OTO in the requested treatment ear(s);

o Member has not received prior gene therapy;

o Dose does not exceed 7.2 x 1012 vector genomes (vg) per ear.* 

o Approval duration: 3 months (one lifetime injection per ear

· Continued Therapy*: Congenital Hearing Loss

*Criteria will mirror the clinical information from the prescribing information once FDA-approved

o Re-authorization is not permitted. Members must meet the initial approval criteria.

o Approval duration: Not applicable

Bitopertin (Brand Name) (CP.PHAR.758)

Ambetter

Policy includes:

· Requests for indications not approved by the FDA are reviewed with the off-label use policy for the relevant line of business: HIM.PHAR.154 for Ambetter

· Criteria will mirror the clinical information from the prescribing information once FDA-approved

· Initial Approval Criteria*: Erythropoietic Protoporphyria (EPP) and X-Linked Protoporphyria (XLP)

*Criteria will mirror the clinical information from the prescribing information once FDA-approved

o Diagnosis of EPP or XLP confirmed by both of the following tests:*

o Elevated total erythrocyte protoporphyrin (e.g., 300 to 5,000 mcg/dL vs. normal at 80 mcg/dL);

o Erythrocyte fractionation shows at least 50% metal-free vs. zinc protoporphyrin (certified laboratories include University of Texas Medical Branch at Galveston - Porphyria Center, and Mayo Medical Laboratories);

o Prescribed by or in consultation with a dermatologist;

o Age at least 12 years;*

o Evidence of EPP/XLP-associated acute non-blistering cutaneous reactions (e.g., pain, stinging, redness, swelling, blanching) following exposure to sun;*

o Sun avoidance and use of sunscreen, protective clothing, and pain medication have proven inadequate in controlling EPP/XLP-associated painful skin reactions;*

o Member does not have any of the following:

§ Aspartate aminotransferase and alanine transaminase at least 2 times upper limit of normal (ULN);

§ Total bilirubin at least ULN;

§ History of liver transplantation;

§ An inherited or acquired red cell disease associated with anemia (e.g., sickle cell anemia, iron-deficiency anemia, aplastic anemia, autoimmune hemolytic anemia, thalassemias);

o Bitopertin is not prescribed concurrently with Scenesse®;*

o Dose does not exceed 60 mg per day.*

o Approval duration: 12 months

· Continued Therapy*: Erythropoietic Protoporphyria and X-Linked Protoporphyria

*Criteria will mirror the clinical information from the prescribing information once FDA-approved

o Member meets one of the following:

o Currently receiving medication via Centene benefit or member has previously met initial approval criteria;

o Member is currently receiving medication and is enrolled in a state and product with continuity of care regulations;

o Member is responding positively to therapy as evidenced by any of the following:

o Reduction in acute non-blistering cutaneous reactions (e.g., pain, stinging, redness, swelling, blanching) following exposure to sun;
Increase in pain-free period during direct sunlight exposure;

o Bitopertin is not prescribed concurrently with Scenesse;

o If request is for a dose increase, new dose does not exceed 60 mg per day.*

o Approval duration: 12 months

Nanoencapsulated Sirolimus plus Pegadricase (NASP) (CP.PHAR.760)

Ambetter

Policy includes:

· Criteria will mirror the clinical information from the prescribing information once FDA-approved

· Requests for indications not approved by the FDA are reviewed with the off-label use policy for the relevant line of business: HIM.PHAR.154 for Ambetter

· Initial Approval Criteria*: Chronic Gout

Criteria will mirror the clinical information from the prescribing information once FDA-approved

o Diagnosis of chronic gout;*

o Age at least 21 years;*

o Positive for symptomatic gout with one or more of the following:

§ At least 3 gout flares in the previous 18 months;

§ At least 1 gout tophus;

§ Chronic gouty arthritis;

o Failure to normalize uric acid to less than 6 mg/dL with allopurinol and febuxostat at maximally indicated doses, each used for at least 3 months unless clinically significant adverse effects are experienced or both are contraindicated;

o Failure of probenecid, at maximally indicated doses, in combination with allopurinol or febuxostat unless clinically significant adverse effects are experienced or all are contraindicated;

o NASP is not prescribed concurrently with oral urate-lowering agents (e.g., allopurinol, febuxostat, probenecid) or injectable urate-lowering agents (e.g., pegloticase);*

o Dose does not exceed 0.2 mg/kg of pegadricase and 0.15 mg/kg of sirolimus every 28 days.*

o Approval duration: 12 months

· Continued Therapy*: Chronic Gout

Criteria will mirror the clinical information from the prescribing information once FDA-approved

o Member meets one of the following:

§ Currently receiving medication via Centene benefit or member has previously met initial approval criteria;

§ Member is currently receiving medication and is enrolled in a state and product with continuity of care regulations;

o Member is responding positively to therapy as evidenced by a decrease in plasma uric acid levels;

o NASP is not prescribed concurrently with oral urate-lowering agents (e.g., allopurinol, febuxostat, probenecid) or injectable urate lowering agents (e.g., pegloticase); *

o If request is for a dose increase, new dose does not exceed 0.2 mg/kg of pegadricase and 0.15 mg/kg of sirolimus every 28 days.*

o Approval duration: 12 months

Semaglutide (Wegovy, NN9932) (CP.PMN.295)

Ambetter

Policy includes:

· Requests for indications not approved by the FDA are reviewed with the off-label use policy for the relevant line of business: HIM.PHAR.154 for Ambetter

· Criteria will mirror the clinical information from the prescribing information once FDA-approved

· Initial Approval Criteria*: Heart Failure (HF)

*Criteria will mirror the clinical information from the prescribing information once FDA-approved

o Request is for Wegovy;

o Diagnosis of chronic HF of New York Heart Association (NYHA) Class II, III, or IV;

o Prescribed by or in consultation with a cardiologist;

o Age at least 18 years;

o Body mass index (BMI) at least 30 kg/m2;*

o Member has a left ventricular ejection fraction (LVEF) at least 50%;*

o Member is receiving stable (i.e., no changes in dose for at least the last month) optimally tolerated dosages of guideline-directed medical therapies for heart failure with preserved ejection fraction (HFpEF) that includes all of the following classes, unless clinically significant adverse effects are experienced or all are contraindicated:

§ Sodium-glucose cotransporter 2 (SGLT2) inhibitor;

§ Secondary therapies, if applicable: loop diuretic, mineralocorticoid antagonist (MRA), and/or angiotensin receptor-neprilysin inhibitor (ARNI) or angiotensin receptor blocker (ARB) (see Appendix B for examples);

o For members with concurrent type 2 diabetes mellitus (T2DM), both of the following:**

§ Failure of at least 3 consecutive months of Ozempic ®, Trulicity ®, and Victoza ®, unless clinically significant adverse effects are experienced or all are contraindicated;* *Prior authorization may be required

§ If member is currently receiving a glucagon-like peptide-1 (GLP-1) receptor agonist and is requesting to switch to Wegovy therapy, medical justification* supports necessity for Wegovy;

*Intolerance due to common adverse effects of the GLP-1 receptor agonist class such as gastrointestinal symptoms is not considered acceptable medical justification

o Wegovy is not prescribed concurrently with other semaglutide-containing products or any other GLP-1 receptor agonist(s);

o Documentation support’s member’s participation in a physician-directed weight loss program that involves a reduced calorie diet, increased physical activity, and behavioral modification that meets both of the following:*

§ Been actively enrolled in a physician-directed weight loss program for at least 6 months;

§ Will continue to be enrolled in a physician-directed weight loss program while concomitantly prescribed Wegovy;

o Documentation of member’s baseline body weight in kg;

o Dose does not exceed the following:*

§ Week 1 through 4: 0.25 mg once weekly;

§ Week 5 through 8: 0.5 mg once weekly;

§ Week 9 through 12: 1 mg once weekly;

§ Week 13 through 16: 1.7 mg once weekly;

§ Week 17 and onward: 2.4 mg once weekly.

o Approval duration: 6 months

· Initial Approval Criteria*: Weight Management

o Use of Wegovy or NN9932 for the treatment of weight management is a benefit exclusion and will not be authorized.

o Approval duration: Not applicable

· Continued Therapy* Heart Failure

*Criteria will mirror the clinical information from the prescribing information once FDA-approved 

o Member meets one of the following:

§ Currently receiving medication via Centene benefit or member has previously met initial approval criteria;

§ Member is currently receiving medication and is enrolled in a state and product with continuity of care regulations;

o Member is responding positively to therapy as evidenced by one of the following:

§ If this is the first renewal request, both of the following:

o Member has lost at least 5% of baseline body weight;

o Improvement in any of the following parameters: heart failure symptom frequency (e.g., fatigue, dyspnea, edema), physical limitations, and exercise function;

§ If this is a second or subsequent renewal request, both of the following:

o Member has lost weight and/or maintained weight loss on therapy;

o Stabilization or improvement in any of the following parameters: heart failure symptom frequency (e.g., fatigue, dyspnea, edema), physical limitations, and exercise function;

o Documentation of member’s current body weight in kg;

o Provider attestation that member is currently receiving guideline-directed medical therapies for HFpEF;

o Wegovy is not prescribed concurrently with other semaglutide-containing products or any other GLP-1 receptor agonist(s);

o Documentation that member is actively enrolled in a weight loss program that involves a reduced calorie diet, increased physical activity, and behavioral modification adjunct to therapy;

o Request meets both of the following:

o Dose does not exceed 2.4 mg once weekly;

o After the initial dose escalation period, maintenance dose is at least 1.7 mg once weekly.

o Approval duration: 12 months

· Continued Therapy*: Weight Management

o Use of Wegovy or NN9932 for the treatment of weight management is a benefit exclusion and will not be authorized.

o Approval duration: Not applicable

Glucagon-Like Peptide-1 (GLP-1) Receptor Agonists (HIM.PA.53)

Ambetter

Policy includes:

· Requests for indications not approved by the FDA are reviewed with the off-label use policy HIM.PHAR.154 for Ambetter

· Criteria will mirror the clinical information from the prescribing information once FDA-approved

· Initial Approval Criteria*: Preferred glucagon-like peptide-1 (GLP-1) receptor agonist (RA) Therapy*

*Criteria will mirror the clinical information from the prescribing information once FDA-approved

*If request is for a GLP-1 RA other than liraglutide (Victoza), Trulicity, Ozempic, Rybelsus, Soliqua, or Xultophy, please refer to criteria set I.B below.

o Diagnosis of type 2 diabetes mellitus;

o Request is for liraglutide (Victoza), Trulicity, Ozempic, Rybelsus, Soliqua, or Xultophy;

o Age is one of the following:

§ Trulicity, liraglutide (Victoza): at least 10 years;

§ Ozempic, Rybelsus, Soliqua, Xultophy: at least 18 years;

o If request is for Victoza, member must use generic liraglutide, unless contraindicated or clinically significant adverse effects are experienced;

o Requested product is not prescribed concurrently with another GLP-1 receptor agonist;

o Dose does not exceed the FDA-approved maximum recommended dose.

o Approval duration: 12 months

· Initial Approval Criteria*: Type 2 Diabetes Mellitus*

* If request is for liraglutide (Victoza), Trulicity, Ozempic, Rybelsus, Soliqua, or Xultophy, please refer to criteria set I.A above for preferred GLP-1 RA Therapy.

o Diagnosis of type 2 diabetes mellitus;

o Request is for Adlyxin, Bydureon, Bydureon BCise, Byetta, or Mounjaro;

o Age is one of the following:

§ Bydureon BCise: at least 10 years;

§ Adlyxin, Byetta, Mounjaro: at least 18 years;

o Member meets one of the following:

§ Failure of at least 3 consecutive months of metformin as evidenced by HbA1c at least 7%, unless contraindicated or clinically significant adverse effects are experienced;

§ For antidiabetic medication-naïve members, requested agent is approvable if intended for concurrent use with metformin due to HbA1c at least 8.5% (drawn within the past 3 months);

§ Request is for an agent with proven cardiovascular or renal benefit (Ozempic, Rybelsus*, Trulicity, liraglutide [Victoza]), and member has established atherosclerotic cardiovascular disease (ASCVD), indicators of high ASCVD risk, heart failure with preserved ejection fraction, chronic kidney disease, or symptomatic PAD*;

§ Member has metabolic dysfunction-associated steatotic liver disease (MASLD), and:

o Member is overweight (body mass index [BMI] 25-29.9 kg/m2) or obese (BMI at least 30 kg/m2);

§ Member has metabolic dysfunction-associated steatohepatitis (MASH), and:

o Failure of at least 3 consecutive month trial of pioglitazone, unless contraindicated or clinically significant adverse effects are experienced;

o Failure of all of the following, unless clinically significant adverse effects are experienced or all are contraindicated:

§ At least 3 consecutive months of each of the following:

o Liraglutide (Victoza);

o Trulicity;

o Ozempic or Rybelsus;

§ Sodium-glucose co-transporter 2 (SGLT2) inhibitor, unless the member has MASLD or MASH;

o Requested product is not prescribed concurrently with another GLP-1 receptor agonist;

o Dose does not exceed the FDA-approved maximum recommended dose.

o Approval duration: 12 months

· Continued Therapy

o Member meets one of the following:

§ Currently receiving medication via Centene benefit or member has previously met initial approval criteria;

§ Member is currently receiving medication and is enrolled in a state and product with continuity of care regulations;

o Member is responding positively to therapy;

o If request is for Victoza, member must use generic liraglutide, unless contraindicated or clinically significant adverse effects are experienced;

o Requested product is not prescribed concurrently with another GLP-1 receptor agonist;

o If request is for a dose increase, new dose does not exceed the FDA-approved maximum recommended dose.

o Approval duration: 12 months

Epinephrine (Epipen, Epipen Jr, Neffy, Auvi-Q) (CP.PCH.55)

Ambetter

Policy updates include:

· Revised initial approval duration to 12 months

· Added quantity limit template statement to initial and continued therapy criteria

· Added Appendix D information for epinephrine dosing in pediatric patients weighing 7.5 kg to 15 kg

Caplacizumab-yhdp (Cablivi) (CP.PHAR.416)

Ambetter

Policy updates include:

· Updated with pediatric age extension of at least 12 years

Selinexor (Xpovio) (CP.PHAR.431)

Ambetter

Policy updates include:

· Added new 80 mg strength tablet

· Extended initial approval durations from 6 months to 12 months for this maintenance medication for a chronic condition

Narsoplimab (Yartemlea) (CP.PHAR.527)

Ambetter

Policy includes:

· Requests for indications not approved by the FDA are reviewed with the off-label use policy HIM.PHAR.154 for Ambetter

· Initial Approval Criteria: Hematopoietic Stem Cell Transplant-Associated Thrombotic Microangiopathy (TA-TMA) (must meet all):

o Diagnosis of hematopoietic stem cell TA-TMA;

o Prescribed by or in consultation with a hematologist or transplant specialist;

o Age at least 2 years;

o Member has signs of persistent TMA as evidenced by presence of all of the following for at least 2 weeks after modification or discontinuation of calcineurin inhibitor therapy (e.g., cyclosporine, tacrolimus):

§ Platelet count less than 150 x 109/L;

§ Evidence of hemolysis (e.g., serum lactate dehydrogenase [LDH] above the upper limit of normal, presence of schistocytes);

§ Serum creatinine at least 2 times pre-transplantation baseline or member requires dialysis;

o Documentation that member does not have any of the following:

§ A disintegrin and metalloproteinase with thrombospondin type 1 motif, member 13 (ADAMTS13) deficiency;

§ Shiga toxin E. coli related hemolytic uremic syndrome (STEC-HUS);

§ Atypical hemolytic uremic syndrome (aHUS);

o Yartemlea is not prescribed concurrently with Soliris®/Bkemv™/Epysqli® or Ultomiris®;

o Dose does not exceed one of the following:

§ Weight at least 50 kg: 370 mg once weekly;

§ Weight less than 50 kg: 4 mg/kg once weekly.

o Approval duration: 8 weeks

· Continued Therapy: Hematopoietic Stem Cell Transplant-Associated Thrombotic Microangiopathy (must meet all):

o Member meets one of the following:

o Currently receiving medication via Centene benefit or member has previously met initial approval criteria;

o Member is currently receiving medication and is enrolled in a state and product with continuity of care regulations;

o Member is responding positively to therapy as evidenced by, including but not limited to, improvement in any of the following parameters:

§ Improved measures of hemolysis (e.g., normalization of LDH, decrease or absence of schistocytes);

§ Increased or stabilized platelet counts;

§ Improved or stabilized serum creatinine or estimated glomerular filtration rate (eGFR);

§ Reduced need for dialysis;

o Member has not received Yartemlea for greater than 16 weeks;

o Yartemlea is not prescribed concurrently with Soliris/Bkemv/Epysqli or Ultomiris;

o If request is for a dose increase, new dose does not exceed one of the following:

§ Weight at least 50 kg: 370 mg twice weekly;

§ Weight less than 50 kg: 4 mg/kg twice weekly.

o Approval duration: Up to 16 weeks total

Amivantamab-vmjw, Amivantamab-Hyaluronidase-lpuj (Rybrevant, Rybrevant Faspro) (CP.PHAR.544)

Ambetter

Policy updates include:

· Added new formulation of Rybrevant Faspro

· Removed the EGFR mutations G719X, S768I, and L861Q from criteria per National Comprehensive Cancer Network (NCCN)

· Added additional options for combination with Lazcluze as subsequent therapy and for brain metastases per National Comprehensive Cancer Network (NCCN)

· Replaced Rybrevant-specific maximum dose criteria with a reference to the indicated regimen in section V

· Revised initial approval duration for to 12 months

Niraparib and Abiraterone (Akeega) (CP.PHAR.645)

Ambetter

Policy updates include:

· Added new indication in BRCA2-mutated metastatic castration-sensitive prostate cancer

Ferric maltol (Accrufer) (CP.PMN.213)

Ambetter

Policy updates include:

· Updated with pediatric age extension to at least 10 years