May 2018 policy update bulletin

Medical & Administrative Policy Updates

UnitedHealthcare respects the expertise of the physicians, health care professionals, and their staff who participate in our network. Our goal is to support you and your patients in making the most informed decisions regarding the choice of quality and cost-effective care, and to support practice staff with a simple and predictable administrative experience. The Policy Update Bulletin was developed to share important information regarding Oxford® Medical and Administrative Policy.*

*Where information in this bulletin conflicts with applicable state and/or federal law, UnitedHealthcare follows such applicable federal and/or state law

Oxford ® Oxford Medical and Administrative Policy Updates Overview

This bulletin provides complete details on Oxford® Clinical, Policy Update Classifications Administrative and Reimbursement Policy updates. The inclusion of New a health service (e.g., test, drug, device or procedure) in this New clinical coverage criteria and/or documentation review bulletin indicates only that UnitedHealthcare has recently adopted a requirements have been adopted for a health service (e.g., test, drug, new policy and/or updated, revised, replaced or retired an existing device or procedure) policy; it does not imply that Oxford® provides coverage for the Updated health service. In the event of an inconsistency or conflict between An existing policy has been reviewed and changes have not been made the information provided in this bulletin and the posted policy, the to the clinical coverage criteria or documentation review requirements; provisions of the posted policy will prevail. Note that most benefit however, items such as the clinical evidence, FDA information, and/or plan documents exclude from benefit coverage health services list(s) of applicable codes may have been updated identified as investigational or unproven/not medically necessary. Physicians and other health care professionals may not seek or Revised collect payment from a member for services not covered by the An existing policy has been reviewed and revisions have been made to applicable benefit plan unless first obtaining the member’s written the clinical coverage criteria and/or documentation review requirements consent, acknowledging that the service is not covered by the Replaced benefit plan and that they will be billed directly for the service. An existing policy has been replaced with a new or different policy

A complete library of Oxford® Medical and Retired Administrative Policies is available at The health service(s) addressed in the policy are no longer being OxfordHealth.com > Providers > Tools & Resources > managed or are considered to be proven/medically necessary and are Medical Information > Medical and Administrative Policies. therefore not excluded as unproven/not medically necessary services, unless coverage guidelines or criteria are otherwise documented in

another policy Tips for using the Policy Update Bulletin:  From the table of contents, click the policy title to be Note: The absence of a policy does not automatically indicate or imply directed to the corresponding policy update summary. coverage. As always, coverage for a health service must be determined in accordance with the member’s benefit plan and any applicable  From the policy updates table, click the policy title to view a federal or state regulatory requirements. Additionally, UnitedHealthcare complete copy of a new, updated, or revised policy. reserves the right to review the clinical evidence supporting the safety and effectiveness of a medical technology prior to rendering a coverage determination.

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Clinical Policy Updates Page NEW  Benlysta® (Belimumab) - Effective May 1, 2018 ...... 7  Crysvita® (Burosumab-Twza) - Effective May 1, 2018 ...... 7  Enzyme Replacement Therapy - Effective May 1, 2018 ...... 8 UPDATED  Chelation Therapy for Non-Overload Conditions - Effective May 1, 2018 ...... 14  Cochlear Implants - Effective Jun. 1, 2018 ...... 14  Computerized Dynamic Posturography - Effective May 1, 2018 ...... 15  Deep Brain and Cortical Stimulation - Effective May 1, 2018 ...... 15  Embolization of the Ovarian and Iliac Veins for Pelvic Congestion Syndrome - Effective May 1, 2018 ...... 15  Infertility Diagnosis and Treatment - Effective Jun. 1, 2018 ...... 15  Injectable Chemotherapy Drugs: Application of NCCN Clinical Practice Guidelines - Effective Jun. 1, 2018 ...... 15  Otoacoustic Emissions Testing - Effective Jun. 1, 2018 ...... 15  Site of Service Guidelines for Certain Outpatient Surgical Procedures - Effective May 1, 2018 ...... 16  Thermography - Effective May 1, 2018 ...... 16  Vaccines - Effective May 1, 2018 ...... 16 REVISED  Ablative Treatment for Spinal Pain - Effective Jun. 1, 2018 ...... 16  Actemra® (Tocilizumab) Injection for Intravenous Infusion - Effective Jun. 1, 2018 ...... 18  Benlysta® (Belimumab) - Effective Aug. 1, 2018 ...... 20  Chromosome Microarray Testing (Non-Oncology Conditions) - Effective Jun. 1, 2018 ...... 21  Drug Coverage Criteria - New and Therapeutic Equivalent Medications - Effective Jun. 1, 2018 ...... 24  Drug Coverage Guidelines - Effective May 1, 2018 ...... 24 o Adagen (Pegademase Bovine) ...... 24 o Aldurazyme® (Laronidase) ...... 24 o Benlysta (Belimumab) ...... 24 o Crysvita (Burosumab-Twza) ...... 25 o Elaprase (Idursulfase) ...... 25 o Fabrazyme® (Agalsidase Beta) ...... 25 o Kanuma (Sebelipase Alfa) ...... 26 o Lumizyme (Alglucosidase Alfa) ...... 26 o Mepsevii (Vestronidase Alfa-Vjbk) ...... 26 o Naglazyme (Galsulfase) ...... 26

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o Vimizim (Elosulfase Alfa) ...... 26  Drug Coverage Guidelines - Effective Jun. 1, 2018 ...... 26 o Actemra (Tocilizumab) ...... 26 o Actiq (Brand Only) (Fentanyl Citrate) ...... 26 o Afstyla (Antihemophilic Factor [Recombinant] Single Chain) ...... 26 o Atorvastatin (Generic Lipitor) 10mg, 20mg...... 26 o Belsomra (Suvorexant) ...... 26 o Benznidazole ...... 26 o Bupropion (SR) (Generic Zyban) ...... 27 o Carospir Suspension (Spironolactone)...... 27 o Chantix (Varenicline Tartrate) ...... 27 o Cimduo (Lamivudine/Tenofovir DF) ...... 27 o Cimzia (Certolizumab Pegol) ...... 27 o Cosentyx (Secukinumab) ...... 27 o Daraprim (Pyrimethamine) ...... 27 o Enbrel (Etanercept) ...... 27 o Ergomar (Ergotamine Tartrate) ...... 27 o Farydak (Panobinostat) ...... 27 o Fentanyl Citrate (Generic Actiq) ...... 27 o Grastek (Timothy Grass Pollen Allergen Extract) ...... 28 o Jakafi (Ruxolitinib) ...... 28 o Kineret (Anakinra) ...... 28 o Korlym (Mifepristone) ...... 28 o Lidocaine Patch ...... 28 o Lemtrada (Alemtuzumab) ...... 28 o Linzess (Linaclotide) ...... 28 o Lynparza (Olaparib) ...... 28 o Lyrica CR (Pregabalin) ...... 28 o Mekinist (Trametinib) ...... 28 o Migranal (Dihydro-Ergotamine) (Brand) ...... 28 o Migranal (Dihydro-Ergotamine) (Generic) ...... 28 o Movantik (Naloxegol)...... 28 o Nicotine OTC Products ...... 28 o Ninlaro (Ixazomib) ...... 28 o Odactra (House Dust Mite Allergen Extract) ...... 29 o Oralair (Sweet Vernal, Orchard, Perennial Rye, Timothy and Kentucky Blue Grass, Mixed Pollens Allergen Extract) ...... 29 o Prolia, Xgeva (Denosumab) ...... 29 o Radicava (Edaravone)...... 29 o Ragwitek (Short Ragweed Pollen Allergen Extract) ...... 29 o Rexulti (Brexpiprazole) ...... 29

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o Rhopressa (Netarsudil) ...... 29 o Rozerem (Ramelteon) ...... 29 o Simponi (Golimumab) ...... 29 o Simponi Aria (Golimumab) ...... 29 o Simvastatin (Generic Zocor) 5mg, 10mg, 20mg, 40mg ...... 29 o Sutent (Sunitinib) ...... 30 o Symfi (Efavirenz/Lamivudine/Tenofovir Disoproxil Fumarate) ...... 30 o Smyfi Lo (Efavirenz/Lamivudine/Tenofovir Disoproxil Fumarate) ...... 30 o Symproic (Naldemedine) ...... 30 o Tafinlar (Dabrafenib) ...... 30 o Tamiflu Capsules (Brand Only) (Oseltamivir Phosphate) ...... 30 o Test Strips and Meters (Diabetic) ...... 30 o Trulance (Plecanatide) ...... 30 o Viberzi (Eluxadoline) ...... 30 o Vyzulta (Latanoprostene Bunod) ...... 30 o Zelboraf (Vemurafenib) ...... 30 o Zolpimist (Zolpidem Tartrate) ...... 30 o Zypitamag (Pitavastatin) ...... 30  Electrical Stimulation for the Treatment of Pain and Muscle Rehabilitation - Effective Jun. 1, 2018 ...... 31  Entyvio® (Vedolizumab) - Effective Jun. 1, 2018 ...... 34  Enzyme Replacement Therapy - Effective Aug. 1, 2018 ...... 35  Exondys 51™ (Eteplirsen) - Effective Jun. 1, 2018 ...... 43  Ilaris® (Canakinumab) - Effective Jun. 1, 2018 ...... 44  Infliximab (Remicade®, Inflectra™, Renflexis™) - Effective Jun. 1, 2018 ...... 47  Ocrevus™ (Ocrelizumab) - Effective Jun. 1, 2018 ...... 50  Orencia® (Abatacept) Injection for Intravenous Infusion - Effective Jun. 1, 2018 ...... 53  Preventive Care Services - Effective Jun. 1, 2018 ...... 55  Radicava™ (Edaravone) - Effective Jun. 1, 2018 ...... 59  Radiology Procedures Requiring Precertification for eviCore healthcare Arrangement - Effective Jun. 1, 2018 ...... 59  Simponi Aria® (Golimumab) Injection for Intravenous Infusion - Effective Jun. 1, 2018 ...... 60 Administrative Policy Updates NEW  New York & Connecticut Participating Surgeons Using Non- Participating Providers for Intraoperative Neuro-Monitoring (IONM) - Effective Jun. 1, 2018 62 UPDATED  Accreditation Requirements for Radiology Services - Effective May 1, 2018 ...... 64

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REVISED  Ambulance Services - Effective Jun. 1, 2018 ...... 65 Reimbursement Policy Updates UPDATED  Maximum Frequency Per Day - Effective May 7, 2018 ...... 69  Services and Modifiers Not Reimbursable to Healthcare Professionals - Effective May 7, 2018 ...... 69

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Policy Title Effective Date Coverage Rationale NEW Benlysta® May 1, 2018 This policy refers only to Benlysta (belimumab) injection for intravenous infusion for the treatment of systemic lupus (Belimumab) erythematosus (SLE). Benlysta (belimumab) for self-administered subcutaneous injection is obtained under the pharmacy benefit and is indicated systemic lupus erythematosus.

Benlysta (belimumab) is proven and medically necessary for the treatment of systemic lupus erythematosus when all of the following criteria are met:  Diagnosis of active systemic lupus erythematosis; and  One of the following: o Anti-nuclear antibody (ANA) titer ≥ 1:80 o Anti-double-stranded DNA (anti-dsDNA) level ≥ 30 IU/mL] and  Currently receiving at least one standard of care treatment for active systemic lupus erythematosus (e.g., antimalarials, corticosteroids, or immunosuppressants); and  Benlysta is initiated and titrated according to US Food and Drug Administration labeled dosing for SLE up to a maximum of 10mg/kg every 4 weeks.

Benlysta is unproven and not medically necessary for:  Severe active lupus nephritis  Severe active central nervous system (CNS) lupus  Use in combination with other biologics or intravenous cyclophosphamide  Waldenström macroglobulinemia  Sjögren's syndrome  Rheumatoid arthritis

Crysvita® May 1, 2018 Crysvita (burosumab) is proven for the treatment of X-linked hypophosphatemia (XLH). (Burosumab-Twza) Crysvita (burosumab) is medically necessary for the treatment of XLH when the following criteria are met:  For initial therapy, all of the following: o Diagnosis of XLH, confirmed by one of the following: . Genetic testing . Elevated Serum fibroblast growth factor 23 (FGF23) level > 30 pg/mL and o Prescribed by, or in consultation with, a specialist experienced in the treatment of metabolic bone disorders; and o Serum phosphorus is below the normal range for age; and o Presence of clinical signs and symptoms of the disease (e.g rickets, growth retardation, musculoskeletal pain, bone fractures ); and o Dosing is in accordance with the United States Food and Drug Administration approved labeling; and o Initial authorization will be for no more than 12 months.  For continuation therapy, all of the following:

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Policy Title Effective Date Coverage Rationale NEW Crysvita® May 1, 2018 o Patient has previously received treatment with burosumab; and (Burosumab-Twza) o Prescribed by, or in consultation with, a specialist experienced in the treatment of metabolic bone disorders; (continued) and o Patient has experienced normalization of serum phosphate while on therapy; and o Patient has experienced a positive clinical response to burosumab (e.g., enhanced height velocity, improvement in skeletal deformities, reduction of fractures, reduction of generalized bone pain); and o Dosing is in accordance with the United States Food and Drug Administration approved labeling; and o Reauthorization will be for no more than 12 months.

Enzyme May 1, 2018 This policy refers to the following enzyme replacement therapy products: Replacement  Adagen (pegademase bovine) Therapy  Aldurazyme (laronidase)  Elaprase (idursulfase)  Fabrazyme (agalsidase beta)  Kanuma (sebelipase alfa)  Lumizyme (alglucosidase alfa)  Mepsevii (vestronidase alfa-vjbk)  Naglazyme (galsulfase)  Vimizim (elosulfase alfa)

Adagen (pegademase bovine) is medically necessary for the treatment of severe combined immunodeficiency disease (SCID) associated with a deficiency of adenosine deaminase (ADA) when the following criteria are met:  For initial therapy, all of the following: o Diagnosis of SCID; and o Deficiency of adenosine deaminase is confirmed by any of the following: . Deficiency or absence of ADA in plasma, lysed erythrocytes, fibroblasts (cultured from amniotic fluid), or chorionic villus . Increase in deoxyadenosine triphosphate (dATP) levels in erythrocyte lysates compared to laboratory standard . Decrease in ATP concentration in erythrocytes . Molecular genetic confirmation of mutations in both alleles of the ADA1 gene . Positive screening by T cell receptor excision circles (TRECs); and o One of the following: . Patient is not a suitable candidate for hematopoietic cell transplantation (HCT) . Patient has failed HCT; and o Dosing is in accordance with the United States Food and Drug Administration approved labeling: dosing is started at 10 U/kg for the first dose, and titrated up to a maximum dose of 30 U/kg per week; and

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Policy Title Effective Date Coverage Rationale NEW Enzyme May 1, 2018 o Initial authorization will be for no more than 12 months. Replacement  For continuation therapy, all of the following: Therapy o Patient has previously received treatment with pegademase therapy; and (continued) o Patient has experienced a positive clinical response to pegademase therapy (e.g., normalization of plasma ADA activity, erythrocyte dATP levels, improvement of disease symptoms, etc.); and o Dosing is in accordance with the United States Food and Drug Administration approved labeling: appropriate maintenance dosing, up to a maximum dose of 30 U/kg per week; and o Reauthorization will be for no more than 12 months.

Aldurazyme (laronidase) is medically necessary for the treatment of Mucopolysaccharidosis I (MPS I) when the following criteria are met:  For initial therapy, all of the following: o Diagnosis of any of the MPS I syndromes confirmed by one the following: . Hurler variant (severe mucopolysaccharidosis I; also MPS IH) . Hurler-Scheie variant (attenuated mucopolysaccharidosis I; also MPS IHS) . Scheie variant (attenuated mucopolysaccharidosis I; also MPS IS); and o Diagnosis of MPS I is confirmed by either of the following: . Deficiency or absence of fibroblast or leukocyte enzyme activity of alpha-L-iduronidase enzyme activity . Molecular genetic confirmation of mutations in the alpha-L-iduronidase gene; and o Presence of clinical signs and symptoms of the disease (e.g., asymptomatic with affected older sibling, cardiac abnormalities, corneal clouding, dysostosis multiplex, hepatomegaly, restrictive lung disease, etc.); and o Dosing is in accordance with the United States Food and Drug Administration approved labeling: Administered dose does not exceed 0.58 mg/kg intravenously once every week; and o Initial authorization will be for no more than 12 months.  For continuation therapy, all of the following: o Patient has previously received treatment with laronidase therapy; and o Patient has experienced a positive clinical response to laronidase therapy (e.g., improved endurance, improved functional capacity, reduced urine dermatan sulfate/heparan sulfate excretion, etc.); and o Dosing is in accordance with the United States Food and Drug Administration approved labeling: administered dose does not exceed 0.58 mg/kg intravenously once every week; and o Reauthorization will be for no more than 12 months.

Elaprase (idursulfase) is medically necessary for the treatment of Mucopolysaccharidosis II (MPS II, Hunter Syndrome) when the following criteria are met:  For initial therapy, all of the following: o Diagnosis of MPS II confirmed by one the following:

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Policy Title Effective Date Coverage Rationale NEW Enzyme May 1, 2018 . Deficiency in iduronate 2-sulfatase enzyme activity as measured in fibroblasts or leukocytes combined Replacement with normal enzyme activity level of another sulfatase Therapy . Molecular genetic testing for deletion or mutations in the iduronate 2-sulfatase gene; (continued) and o Presence of clinical signs and symptoms of the disease (e.g., hepatosplenomegaly, skeletal deformities, dysostosis, neurocognitive decline, cardiovascular disorders, etc.); and o Dosing is in accordance with the United States Food and Drug Administration approved labeling: administered dose does not exceed 0.5 mg/kg intravenously once every week; and o Initial authorization will be for no more than 12 months.  For continuation therapy, all of the following: o Patient has previously received treatment with idursulfase therapy; and o Patient has experienced a positive clinical response to idursulfase therapy (e.g., improved endurance, improved functional capacity, reduced spleen volume, reduced urine GAG excretion, etc.); and o Dosing is in accordance with the United States Food and Drug Administration approved labeling: administered dose does not exceed 0.5 mg/kg intravenously once every week; and o Reauthorization will be for no more than 12 months.

Fabrazyme (agalsidase beta) is medically necessary for the treatment of Fabry disease when the following criteria are met:  For initial therapy, all of the following: o Diagnosis of Fabry disease as confirmed by one the following: . Absence or deficiency (< 5% of mean) of normal alpha-galactosidase A (α-Gal A) enzyme activity) in leukocytes, dried blood spots, or serum analysis . Molecular genetic testing for deletion or mutations in the galactosidase alpha gene; and o Presence of clinical signs and symptoms of the disease (e.g., Acroparesthesias, angiokeratomas, whorls, anhidrosis/hypohidrosis, renal disease, exercise/heat/cold intolerance, etc.); and o Dosing is in accordance with the United States Food and Drug Administration approved labeling: administered dose does not exceed 1 mg/kg intravenously every two weeks; and o Initial authorization will be for no more than 12 months.  For continuation therapy, all of the following: o Patient has previously received treatment with agalsidase therapy; and o Patient has experienced a positive clinical response to agalsidase therapy (e.g., improved renal function, reduction in mean plasma GL-3 levels, decreased GL-3 inclusions, etc.); and o Dosing is in accordance with the United States Food and Drug Administration approved labeling: administered dose does not exceed 1 mg/kg intravenously every two weeks; and o Reauthorization will be for no more than 12 months.

Kanuma (sebelipase alfa) is medically necessary for the treatment of Lysosomal acid lipase deficiency

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Policy Title Effective Date Coverage Rationale NEW Enzyme May 1, 2018 [LAL-D, Wolman disease (WD), cholesteryl ester disease (CESD)] when the following criteria are met: Replacement  For initial therapy, all of the following: Therapy o Diagnosis of lysosomal acid lipase deficiency [LAL-D, Wolman disease (WD), cholesteryl ester disease (continued) (CESD)] as confirmed by one the following: . Absence or deficiency lysosomal acid lipase activity by dried blood spot test . Molecular genetic testing for deletion or mutations in the lipase A, lysosomal acid type (LIPA) gene; and o Presence of clinical signs and symptoms of the disease (e.g., abdominal distention, hepatosplenomegaly, liver fibrosis, ascities, etc.); and o Dosing is in accordance with the United States Food and Drug Administration approved labeling by one of the following: . For rapidly progressive disease presenting within the first 6 months of life: administered initial starting dose is 1 mg/kg intravenously once weekly, up to a maximum of 3 mg/kg once weekly . Pediatric and adult patients with disease: administered dose does not exceed 1 mg/kg intravenously every other week; and o Initial authorization will be for no more than 12 months.  For continuation therapy, all of the following: o Patient has previously received treatment with sebelipase therapy; and o Patient has experienced a positive clinical response to sebelipase therapy [e.g., improved disease symptoms, improvement of laboratory values (LFTs, cholesterol, triglycerides), etc.]; and o Dosing is in accordance with the United States Food and Drug Administration approved labeling by one of the following: . For rapidly progressive disease presenting within the first 6 months of life: administered dose is 1 mg/kg intravenously once weekly, up to a maximum of 3 mg/kg once weekly . Pediatric and adult patients with disease: administered dose does not exceed 1 mg/kg intravenously every other week; and o Reauthorization will be for no more than 12 months.

Lumizyme (alglucosidase alfa) is medically necessary for the treatment of Pompe disease when the following criteria are met:  For initial therapy, one of the following: o All of the following for infantile-onset Pompe disease: . Diagnosis of infantile-onset Pompe disease as confirmed by one the following: - Absence or deficiency (<1% of the lab specific normal mean) acid alpha-glucosidase deficiency (GAA) activity in skin fibroblasts - Molecular genetic testing for deletion or mutations in the GAA gene; and

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Policy Title Effective Date Coverage Rationale NEW Enzyme May 1, 2018 . Presence of clinical signs and symptoms of the disease (e.g., cardiac hypertrophy, respiratory distress, Replacement skeletal muscle weakness, etc.); and Therapy . Dosing is in accordance with the United States Food and Drug Administration approved labeling: (continued) administered dose does not exceed 20 mg/kg intravenously every two weeks; and . Initial authorization will be for no more than 12 months; or o All of the following for late-onset (non-infantile) Pompe disease: . Diagnosis of late-onset Pompe disease as confirmed by one the following: - Absence or deficiency (<40% of the lab specific normal mean) acid alpha-glucosidase deficiency (GAA) activity in lymphocytes, fibroblasts or muscle - Molecular genetic testing for deletion or mutations in the GAA gene; and . Presence of clinical signs and symptoms of the disease (e.g., cardiac hypertrophy, respiratory distress, skeletal muscle weakness, etc.); and . Dosing is in accordance with the United States Food and Drug Administration approved labeling: administered dose does not exceed 20 mg/kg intravenously every two weeks; and . Initial authorization will be for no more than 12 months.  For continuation therapy, all of the following: o Patient has previously received treatment with alglucosidase therapy; and o Patient has experienced a positive clinical response to alglucosidase therapy (e.g., improved respiratory/cardiac function, improved endurance, etc.); and o Dosing is in accordance with the United States Food and Drug Administration approved labeling: administered dose does not exceed 20 mg/kg intravenously every two weeks; and o Reauthorization will be for no more than 12 months.

Mepsevii (vestronidase alfa-vjbk) is proven and medically necessary for the treatment of Mucopolysaccharidosis VII (Sly syndrome) when the following criteria are met:  For initial therapy, all of the following: o Diagnosis of Mucopolysaccharidosis VII confirmed by either of the following: . Absence or deficiency of fibroblast or leukocyte enzyme activity of beta glucuronidase . Molecular genetic confirmation of mutations in the GUSB gene. and o Presence of clinical signs and symptoms of the disease (e.g., enlarged liver and spleen, joint limitations, airway obstruction or pulmonary problems, limitation of mobility while still ambulatory, etc.); and o Dosing is in accordance with the United States Food and Drug Administration approved labeling: Administered dose does not exceed 4 mg/kg intravenously once every two weeks; and o Initial authorization will be for no more than 12 months.  For continuation therapy, all of the following: o Patient has previously received treatment with vestronidase therapy; and

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Policy Title Effective Date Coverage Rationale NEW Enzyme May 1, 2018 o Patient has experienced a positive clinical response to vestronidase therapy (e.g., improved endurance, Replacement improved functional capacity, improved pulmonary function, etc.); and Therapy o Dosing is in accordance with the United States Food and Drug Administration approved labeling: (continued) Administered dose does not exceed 4 mg/kg intravenously once every two weeks; and o Reauthorization will be for no more than 12 months.

Naglazyme (galsulfase) is medically necessary for the treatment of Mucopolysaccharidosis VI (Maroteaux-Lamy syndrome) when the following criteria are met:  For initial therapy, all of the following: o Diagnosis of Mucopolysaccharidosis VI confirmed by either of the following: . Absence or deficiency of fibroblast or leukocyte enzyme activity of N-acetylgalactosamine 4-sulfatase (arylsulfatase) . Molecular genetic confirmation of mutations in the ASB gene (5q13-q14); and o Presence of clinical signs and symptoms of the disease (e.g., kyphoscoliosis, genu valgum, pectus carinatum, gait disturbance, growth deficiency, etc.); and o Dosing is in accordance with the United States Food and Drug Administration approved labeling: administered dose does not exceed 1 mg/kg intravenously once every week; and o Initial authorization will be for no more than 12 months.  For continuation therapy, all of the following: o Patient has previously received treatment with galsulfase therapy; and o Patient has experienced a positive clinical response to galsulfase therapy (e.g., improved endurance, improved functional capacity, reduced urine dermatan sulfate excretion, etc.); and o Dosing is in accordance with the United States Food and Drug Administration approved labeling: administered dose does not exceed 1 mg/kg intravenously once every week; and o Reauthorization will be for no more than 12 months.

Vimizim (elosulfase alfa) is medically necessary for the treatment of Mucopolysaccharidosis type IVA (MPS IVA; Morquio A syndrome) when the following criteria are met:  For initial therapy, all of the following: o Diagnosis of Morquio A syndrome confirmed by either of the following: . Absence or deficiency of fibroblast or leukocyte GALNS enzyme activity . Molecular genetic testing for mutations in the GALNS gene (16q24.3); and o Presence of clinical signs and symptoms of the disease (e.g., kyphoscoliosis, genu valgum, pectus carinatum, gait disturbance, growth deficiency, etc.); and o Dosing is in accordance with the United States Food and Drug Administration approved labeling: administered dose does not exceed 2 mg/kg IV once every week; and o Initial authorization will be for no more than 12 months.

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Policy Title Effective Date Coverage Rationale NEW Enzyme May 1, 2018  For continuation therapy, all of the following: Replacement o Patient has previously received treatment with elosulfase alfa therapy; and Therapy o Patient has experienced a positive clinical response to elosulfase alfa therapy (e.g., improved endurance, (continued) improved functional capacity, reduced urine keratan sulfate excretion); and o Dosing is in accordance with the United States Food and Drug Administration approved labeling: administered dose does not exceed 2 mg/kg IV once every week; and o Reauthorization will be for no more than 12 months.

Policy Title Effective Date Summary of Changes UPDATED Chelation Therapy May 1, 2018  Updated coverage rationale; replaced language indicating: for Non-Overload o “[The listed service] is proven and medically necessary” with “[the listed service] is proven and/or medically Conditions necessary” o “[The listed services] are unproven and not medically necessary” with “[the listed services] are unproven and/or not medically necessary”  Updated supporting information to reflect the most current description of services, clinical evidence, and references

Cochlear Implants Jun. 1, 2018  Replaced references to “patient” with “individual”  Updated benefit considerations: o Replaced reference to “Covered Health Service” with “Covered Health Care Service” o Replaced language indicating “cochlear implant monitoring (remapping and reprogramming of implant) and rehabilitation following the cochlear implant surgery is usually billed as aural rehabilitation and is not covered as a speech therapy benefit” with “cochlear implant monitoring (remapping and reprogramming of implant) and rehabilitation following the cochlear implant surgery is usually billed as aural rehabilitation and is covered as an outpatient rehabilitation therapy benefit”  Updated coverage rationale; replaced language indicating: o “[The listed service] is proven and medically necessary” with “[the listed service] is proven and/or medically necessary” o “[The listed service] is unproven and not medically necessary” with “[the listed service] is unproven and/or not medically necessary” o “There is insufficient high quality evidence in the published clinical literature demonstrating the safety and efficacy of cochlear hybrid implants in the management of individuals with severe hearing loss” with “there is insufficient high quality evidence in the published clinical literature demonstrating the efficacy of cochlear hybrid implants in the management of individuals with severe hearing loss”  Updated list of applicable HCPCS codes: o Added V5273 o Removed L8621, L8622, L8623, L8624, and L8629  Updated supporting information to reflect the most current clinical evidence and references

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Policy Title Effective Date Summary of Changes UPDATED Computerized May 1, 2018  Updated non-coverage rationale; replaced language indicating “[the listed service] is unproven and not medically Dynamic necessary” with “[the listed service] is unproven and/or not medically necessary” Posturography  Updated supporting information to reflect the most current clinical evidence and references

Deep Brain and May 1, 2018  Updated coverage rationale: Cortical Stimulation o Replaced references to: . “Patients” with “individuals” . “Patient population” with “population” or “study population” . “Patient selection criteria” with “selection criteria” o Replaced language indicating: . “[The listed services] are proven and medically necessary” with “[the listed services] are proven and/or medically necessary” . “[The listed services] are unproven and not medically necessary” with “[the listed services] are unproven and/or not medically necessary” o Removed reference to specific directional deep brain stimulation device/product name (Infinity™ DBS System) o Added reference link to the U.S. Food and Drug Administration (FDA) section of the policy for information regarding directional deep brain stimulation devices  Updated supporting information to reflect the most current clinical evidence and FDA information

Embolization of the May 1, 2018  Updated non-coverage rationale; replaced language indicating “[the listed service] is unproven and not medically Ovarian and Iliac necessary” with “[the listed service] is unproven and/or not medically necessary” Veins for Pelvic  Updated supporting information to reflect the most current description of services, clinical evidence, and Congestion references Syndrome Infertility Diagnosis Jun. 1, 2018  Added definition of: and Treatment o Preimplantation Genetic Diagnosis (PGD) o Preimplantation Genetic Screening (PGS)  Updated supporting information to reflect the most current references

Injectable Jun. 1, 2018  Updated list of applicable HCPCS codes: Chemotherapy o Added J0202 Drugs: Application o Removed J9010 of NCCN Clinical Practice Guidelines Otoacoustic Jun. 1, 2018  Updated list of related policies; added reference link to the policy titled Preventive Care Services Emissions Testing  Updated coverage rationale o Replaced language indicating: . “[The listed service] is proven and medically necessary” with “[the listed service] is proven and/or

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Clinical Policy Updates

Policy Title Effective Date Summary of Changes UPDATED Otoacoustic Jun. 1, 2018 medically necessary” Emissions Testing . “[The listed services] are not proven and medically necessary” with “[the listed services] are unproven (continued) and/or not medically necessary” o Replaced reference(s)to: . “Patients” with “individuals” . “Patient populations” with “populations”  Updated list of applicable ICD-10 diagnosis codes; added F44.6, F68.10, F68.12, F68.13, H91.01, H91.02, H91.03, and H91.09  Updated supporting information to reflect the most current description of services, clinical evidence, and references

Site of Service May 1, 2018  Updated supporting information to reflect the most current references; no change to coverage rationale or list of Guidelines for applicable codes Certain Outpatient Surgical Procedures Thermography May 1, 2018  Updated non-coverage rationale; replaced language indicating “[the listed service] is unproven or not medically necessary” with “[the listed service] is unproven and/or not medically necessary”  Updated list of applicable CPT codes: o Modified table heading; removed descriptor classifying codes as “non-reimbursable”  Updated supporting information to reflect the most current clinical evidence, FDA information, and references

Vaccines May 1, 2018  Updated coverage rationale; replaced reference to “ACIP definitive (‘shall’) recommendation” with “ACIP definitive (e.g., should, shall, is) recommendation”  Updated supporting information to reflect the most current references

Policy Title Effective Date Summary of Changes Coverage Rationale REVISED Ablative Treatment Jun. 1, 2018  Updated list of related policies; Thermal radiofrequency ablation of facet joint nerves is proven and for Spinal Pain added reference link to the medically necessary for chronic cervical, (C3-4 and below), thoracic policy titled Office Based and lumbar pain when confirmed by: Program  Positive response to medial branch block at the side and level of the  Revised conditions of proposed ablation coverage/precertification  Confirmation of needle placement by fluoroscopic guided imaging requirements; replaced language  Operative notes document: indicating “pulsed radiofrequency o Temperature 60 degrees celsius or more ablation (unlisted CPT code o Duration of ablation at least 40 seconds 64999) requires Medical Director review in all sites of service” A repeat thermal radiofrequency ablation of the same facet joint with “CPT codes 64633, 64635, nerves is proven and medically necessary when:

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Clinical Policy Updates

Policy Title Effective Date Summary of Changes Coverage Rationale REVISED Ablative Treatment Jun. 1, 2018 and 64999 (when used for  Performed at a frequency of six months or longer (maximum of 2 times for Spinal Pain pulsed radiofrequency ablation) over a 12 month period), and (continued) require precertification with  There has been a 50% or greater documented reduction in pain for 10 to Medical Director review in all 12 weeks following the previous ablation. sites of service” Thermal radiofrequency ablation of facet joint nerves is unproven and not medically necessary:  When there has been no positive response to medial branch block injection; or  When performed more frequently than every six months

For additional information regarding frequency guidelines, refer to the Clinical Evidence section of the policy.

Documentation requirements for the aforementioned procedures must

include:

 Temperature of procedure

 Duration of ablation

 Specific identification of side and level of medial branch blocks

 Specific identification of side and level of ablation

 Percentage of pain relief with prior ablation if applicable

 Duration of improvement from previous ablation if applicable.

Thermal radiofrequency ablation is unproven and not medically

necessary for treating ALL other pain indications including but not

limited to:

 Diabetic neuropathy

 Sacroiliac pain

 Complex regional pain syndrome or regional pain disorders and

syndromes in the absence of spinal pain

 Definitive clinical and/or imaging findings identifying a condition requiring

surgical treatment

 Identified specific causes of spinal pain (e.g., disc herniation) requiring

definitive treatment

Studies of radiofrequency ablation for other conditions were limited, uncontrolled, and insufficient to support conclusions regarding efficacy or duration of effect. Additional well-designed, longer-term randomized controlled trials are required to evaluate the safety and efficacy of radiofrequency ablation and to compare this technique with other medical or

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Clinical Policy Updates

Policy Title Effective Date Summary of Changes Coverage Rationale REVISED Ablative Treatment Jun. 1, 2018 surgical therapies for pain. for Spinal Pain (continued) The following ablation procedures are unproven and not medically necessary for treating spinal pain:  Pulsed radiofrequency therapy of the facet nerves of the cervical, thoracic, or lumbar region, sacral nerve root or dorsal root ganglion  Endoscopic radiofrequency ablation (rhizotomy)  Cryoablation (cryodenervation, cryoneurolysis, cryosurgery, or cryoanesthesia)  Chemical ablation (including but not limited to alcohol, phenol or sodium morrhuate  Laser ablation (including pulsed, continuous, or low level)

There is insufficient evidence to establish the efficacy of the ablation therapies bulleted immediately above to reduce or relieve spinal pain. Studies are limited by small sample size retrospective and case series studies. The clinical value needs to be examined in well-designed, randomized controlled trials with large sample size and long term follow-up.

Actemra® Jun. 1, 2018  Revised conditions of Please refer to Injectable Chemotherapy Drugs: Application of NCCN Clinical (Tocilizumab) coverage/precertification Practice Guidelines for updated information based upon the National Injection for requirements; added language Comprehensive Cancer Network (NCCN) Drugs & Biologics Compendium® Intravenous to indicate: (NCCN Compendium®) for oncology indications. Infusion o Requests for hospital outpatient facility infusion of This policy refers only to Actemra (tocilizumab) injection for intravenous Actemra require additional infusion for the treatment of rheumatoid arthritis, polyarticular juvenile precertification with review idiopathic arthritis, systemic juvenile idiopathic arthritis, and cytokine release by a Medical Director or their syndrome. Actemra for self-administered subcutaneous injection is obtained designee; refer to the policy under the pharmacy benefit and is indicated in the treatment of rheumatoid titled Specialty Medication arthritis and giant cell arteritis. Administration - Site of Care Review Guidelines Actemra is proven and medically necessary for the treatment of:

 Polyarticular juvenile idiopathic arthritis when ALL of the following criteria are met: o Diagnosis of polyarticular juvenile idiopathic arthritis (PJIA); and o Actemra is initiated and titrated according to US Food and Drug Administration labeled dosing for polyarticular juvenile idiopathic arthritis up to a maximum of (or equivalent dose and interval schedule):

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Clinical Policy Updates

Policy Title Effective Date Summary of Changes Coverage Rationale REVISED Actemra® Jun. 1, 2018 . 10mg/kg every 4 weeks for patients weighing < 30kg (Tocilizumab) . 8mg/kg every 4 weeks for patients weighing ≥ 30kg; Injection for and Intravenous o Patient is not receiving Actemra in combination with either of the Infusion following: (continued) . Biologic disease-modifying antirheumatic drug (DMARD) [e.g., Enbrel (etanercept), Humira (adalimumab), Cimzia (certolizumab), Simponi (golimumab)] . Janus kinase inhibitor [e.g., Xeljanz (tofacitinib)]

 Rheumatoid arthritis when ALL of the following criteria are met: o Diagnosis of moderate to severely active rheumatoid arthritis (RA); and o History of failure, contraindication, or intolerance to at least one non-biologic DMARD [e.g., methotrexate, leflunomide, sulfasalazine, hydroxychloroquine, minocycline, etc.]; and o Actemra is initiated and titrated according to US Food and Drug Administration labeled dosing for rheumatoid arthritis up to a maximum of 800mg every 4 weeks (or equivalent dose and interval schedule); and o Patient is not receiving Actemra in combination with either of the following: . Biologic DMARD [e.g., Enbrel (etanercept), Humira (adalimumab), Cimzia (certolizumab), Simponi (golimumab)] . Janus kinase inhibitor [e.g., Xeljanz (tofacitinib)]

 Systemic juvenile idiopathic arthritis when ALL of the following criteria are met: o Diagnosis of systemic juvenile idiopathic arthritis (SJIA); and o Actemra is initiated and titrated according to US Food and Drug Administration labeled dosing for systemic juvenile idiopathic arthritis up to a maximum of (or equivalent dose and interval schedule): . 12mg/kg every 2 weeks for patients weighing < 30kg . 8mg/kg every 2 weeks for patients weighing ≥ 30kg; and o Patient is not receiving Actemra in combination with either of the following: . Biologic DMARD [e.g., Enbrel (etanercept), Humira (adalimumab), Cimzia (certolizumab), Simponi (golimumab)]

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Clinical Policy Updates

Policy Title Effective Date Summary of Changes Coverage Rationale REVISED Actemra® Jun. 1, 2018 . Janus kinase inhibitor [e.g., Xeljanz (tofacitinib)] (Tocilizumab) Injection for  Cytokine release syndrome when ALL of the following criteria are Intravenous met: Infusion o Diagnosis of chimeric antigen receptor (CAR) T cell-induced cytokine (continued) release syndrome (CRS); and o Actemra is prescribed according to US Food and Drug Administration labeled dosing for CRS: . 12mg/kg for patients weighing < 30kg . 8mg/kg for patients weighing ≥ 30kg; up to a maximum of 800mg per infusion and o Actemra is prescribed for a maximum of 4 doses

Benlysta® Aug. 1, 2018  Updated list of related policies; This policy refers only to Benlysta (belimumab) injection for intravenous (Belimumab) added reference link to the infusion for the treatment of systemic lupus erythematosus (SLE). Benlysta policy titled Specialty Medication (belimumab) for self-administered subcutaneous injection is obtained under Administration – Site of Care the pharmacy benefit and is indicated systemic lupus erythematosus. Review Guidelines  Revised conditions of Benlysta (belimumab) is proven and medically necessary for the coverage/precertification treatment of systemic lupus erythematosus when all of the following requirements to indicate: criteria are met: o Precertification with review  Diagnosis of active systemic lupus erythematosis; and by a Medical Director or their  One of the following: designee through Oxford’s o Anti-nuclear antibody (ANA) titer ≥ 1:80 Medical Management is o Anti-double-stranded DNA (anti-dsDNA) level ≥ 30 IU/mL] required and o New Jersey Small Group  Currently receiving at least one standard of care treatment for active members should refer to systemic lupus erythematosus (e.g., antimalarials, corticosteroids, or their certificate of coverage immunosuppressants); and for precertification guidelines  Benlysta is initiated and titrated according to US Food and Drug and quantity limit guidelines Administration labeled dosing for SLE up to a maximum of 10mg/kg o Requests for hospital every 4 weeks. outpatient facility infusion of Benlysta require additional Benlysta is unproven and not medically necessary for: precertification with review  Severe active lupus nephritis by a Medical Director or their  Severe active central nervous system (CNS) lupus designee; refer to the policy  Use in combination with other biologics or intravenous cyclophosphamide titled Specialty Medication  Waldenström macroglobulinemia Administration - Site of Care  Sjögren's syndrome

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Clinical Policy Updates

Policy Title Effective Date Summary of Changes Coverage Rationale REVISED Benlysta® Aug. 1, 2018 Review Guidelines  Rheumatoid arthritis (Belimumab) (continued) Chromosome Jun. 1, 2018  Changed policy title; previously Genome-wide comparative genomic hybridization microarray testing Microarray Testing titled Chromosome Microarray or single nucleotide polymorphism (SNP) chromosomal microarray (Non-Oncology Testing analysis is proven and/or medically necessary for evaluating an Conditions)  Updated list of related policies: embryo/fetus in the following cases: o Added reference link to the  Women undergoing invasive prenatal testing (i.e., amniocentesis, policy titled Molecular chorionic villus sampling or fetal tissue sampling) Oncology Testing for Cancer  Intrauterine Fetal Demise or Stillbirth Diagnosis, Prognosis, and Treatment Decisions Genome-wide comparative genomic hybridization microarray testing o Removed reference link to or SNP chromosomal microarray analysis is proven and/or medically the policy titled Gene necessary for evaluating individuals with one or more of the Expression Tests for Cardiac following: Indications  Multiple anomalies not specific to a well-delineated genetic syndrome and  Revised coverage rationale: cannot be identified by a clinical evaluation alone o Replaced reference to  Non-syndromic Developmental Delay/Intellectual Disability “patients” with “individuals”  Autism spectrum disorders o Replaced language indicating: Genome-wide comparative genomic hybridization microarray testing . “[The listed services] are or SNP chromosomal microarray analysis are unproven and/or not proven and medically medically necessary for all other populations and conditions necessary” with “[the including but not limited to the following: listed services] are  For evaluating an embryo/fetus in the following cases: proven and/or medically o Preimplantation Genetic Diagnosis (PGD) in embryos necessary” o Preimplantation Genetic Screening (PGS) in embryos . “Genome-wide  Epilepsy comparative genomic hybridization microarray There is insufficient evidence in the clinical literature demonstrating that testing and single genome-wide comparative genomic hybridization microarray testing or SNP nucleotide polymorphism chromosomal microarray analysis has a role in clinical decision-making or (SNP) chromosomal has a beneficial effect on health outcomes for other indications such as PGD microarray analysis are in embryos, PGS in embryos, or epilepsy. Further studies are needed to unproven and not determine the analytic validity, clinical validity and clinical utility of this test medically necessary for for indications other than those listed above as proven/medically necessary. all other patient populations and Note: Genome-wide comparative genomic hybridization microarray testing conditions [not listed as or SNP chromosomal microarray analysis for the evaluation of cancer is

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Clinical Policy Updates

Policy Title Effective Date Summary of Changes Coverage Rationale REVISED Chromosome Jun. 1, 2018 proven and/or medically addressed in the policy titled Molecular Oncology Testing for Cancer Microarray Testing necessary]” with Diagnosis Prognosis, and Treatment Decisions. (Non-Oncology “genome-wide Conditions) comparative genomic Genetic Counseling (continued) hybridization microarray Genetic counseling is strongly recommended prior to this test in order to testing or SNP inform persons being tested about the advantages and limitations of the test chromosomal microarray as applied to a unique person. analysis are unproven and/or not medically necessary for all other populations and conditions [not listed as proven and/or medically necessary]” o Updated list of populations and conditions for which genome-wide comparative genomic hybridization microarray testing or SNP chromosomal microarray analysis is unproven and/or not medically necessary: . Added epilepsy . Removed diagnosis, management, and prognosis of cancer . Replaced “preimplantation genetic diagnosis or screening in embryos” with “Preimplantation Genetic Diagnosis (PGD) and Preimplantation Genetic Screening (PGS) in embryos” o Modified language pertaining to clinical evidence/study findings to indicate there is insufficient evidence in the

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Clinical Policy Updates

Policy Title Effective Date Summary of Changes Coverage Rationale REVISED Chromosome Jun. 1, 2018 clinical literature Microarray Testing demonstrating that genome- (Non-Oncology wide comparative genomic Conditions) hybridization microarray (continued) testing or SNP chromosomal microarray analysis has a role in clinical decision- making or has a beneficial effect on health outcomes for other indications such as PGD in embryos, PGS in embryos, or epilepsy o Added language to indicate genome-wide comparative genomic hybridization microarray testing or SNP chromosomal microarray analysis for the evaluation of cancer is addressed in the policy titled Molecular Oncology Testing for Cancer Diagnosis Prognosis, and Treatment Decisions  Added definition of: o Preimplantation Genetic Diagnosis (PGD) o Preimplantation Genetic Screening (PGS) o Prenatal Diagnosis  Updated list of applicable CPT codes: o Added 81479 o Removed 0004M  Reformatted list of applicable ICD-10 diagnosis codes; transferred content to embedded Excel file format  Updated supporting information to reflect the most current

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Clinical Policy Updates

Policy Title Effective Date Summary of Changes Coverage Rationale REVISED Chromosome Jun. 1, 2018 description of services, clinical Microarray Testing evidence, and references (Non-Oncology Conditions) (continued) Drug Coverage Jun. 1, 2018  Revised list of medications Refer to the policy for complete details on Drug Coverage Criteria - New and Criteria - New and requiring precertification through Therapeutic Equivalent Medications. Therapeutic the pharmacy benefit manager Equivalent (PBM): Medications o Added Cimduo, Rhopressa, Symfi, Symfi Lo, Tamiflu Capsules (brand only), and Zypitamag o Removed Actiq, Carospir Suspension, and Lyrica CR

Policy Title Effective Date Drug/Medication Status Summary of Changes REVISED Drug Coverage May 1, 2018 Adagen (Pegademase New  Added language to indicate precertification is required through the Guidelines Bovine) Pharmacy Benefit Manager (PBM)  Added precertification guidelines; refer to Precertification Guidelines: Enzyme Replacement Therapy for complete details Aldurazyme® Revised  Added language to indicate precertification is not required however it is (Laronidase) strongly recommended o While no penalty will be imposed for failure to request a pre-service review, if you do not request one, a medical necessity review will be conducted post-service to determine coverage o It is the referring physician’s responsibility to provide medical documentation to demonstrate clinical necessity for the medication o Beginning Aug. 1, 2018, precertification will be required  Added precertification guidelines; refer to Precertification Guidelines: Enzyme Replacement Therapy for complete details Benlysta (Belimumab) Revised  Added language to indicate precertification is not required however it is strongly recommended o While no penalty will be imposed for failure to request a pre-service review, if you do not request one, a medical necessity review will be conducted post-service to determine coverage o It is the referring physician’s responsibility to provide medical

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Policy Title Effective Date Drug/Medication Status Summary of Changes REVISED Drug Coverage May 1, 2018 Benlysta (Belimumab) Revised documentation to demonstrate clinical necessity for the medication Guidelines (continued) o Beginning Aug. 1, 2018, precertification will be required (continued)  Added precertification guidelines; refer to Precertification Guidelines: Enzyme Replacement Therapy for complete details Crysvita (Burosumab- New  Added language to indicate precertification is not required however it is Twza) strongly recommended o While no penalty will be imposed for failure to request a pre-service review, if you do not request one, a medical necessity review will be conducted post-service to determine coverage o It is the referring physician’s responsibility to provide medical documentation to demonstrate clinical necessity for the medication o Beginning Aug. 1, 2018, precertification will be required o Added precertification guidelines; refer to the following policies for complete details: o Precertification Guidelines: Crysvita (Burosumab-Twza) o Precertification Guidelines: Review at Launch for New to Market Medications Elaprase (Idursulfase) Revised  Added language to indicate precertification is not required however it is strongly recommended o While no penalty will be imposed for failure to request a pre-service review, if you do not request one, a medical necessity review will be conducted post-service to determine coverage o It is the referring physician’s responsibility to provide medical documentation to demonstrate clinical necessity for the medication o Beginning Aug. 1, 2018, precertification will be required  Added precertification guidelines; refer to Precertification Guidelines: Enzyme Replacement Therapy for complete details Fabrazyme® (Agalsidase Revised  Added language to indicate precertification is not required however it is Beta) strongly recommended o While no penalty will be imposed for failure to request a pre-service review, if you do not request one, a medical necessity review will be conducted post-service to determine coverage o It is the referring physician’s responsibility to provide medical documentation to demonstrate clinical necessity for the medication o Beginning Aug. 1, 2018, precertification will be required  Added precertification guidelines; refer to Precertification Guidelines: Enzyme Replacement Therapy for complete details

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Clinical Policy Updates

Policy Title Effective Date Drug/Medication Status Summary of Changes REVISED Drug Coverage May 1, 2018 Kanuma (Sebelipase New  Added language to indicate precertification is required through the Guidelines Alfa) Pharmacy Benefit Manager (PBM) (continued)  Added precertification guidelines; refer to Precertification Guidelines: Enzyme Replacement Therapy for complete details Lumizyme (Alglucosidase New  Added language to indicate precertification is required through the Alfa) Pharmacy Benefit Manager (PBM)  Added precertification guidelines; refer to Precertification Guidelines: Enzyme Replacement Therapy for complete details Mepsevii (Vestronidase New  Added language to indicate precertification is required through the Alfa-Vjbk) Pharmacy Benefit Manager (PBM)  Added precertification guidelines; refer to Precertification Guidelines: Enzyme Replacement Therapy for complete details Naglazyme (Galsulfase) New  Added language to indicate precertification is required through the Pharmacy Benefit Manager (PBM)  Added precertification guidelines; refer to Precertification Guidelines: Enzyme Replacement Therapy for complete details Vimizim (Elosulfase Alfa) New  Added language to indicate precertification is required through the Pharmacy Benefit Manager (PBM)  Added precertification guidelines; refer to Precertification Guidelines: Enzyme Replacement Therapy for complete details Drug Coverage Jun. 1, 2018  Revised conditions of coverage; added instruction to refer to the policy titled Supply Limits: New Jersey Benefit Guidelines Maximum Limits for details on applicable benefit guidelines for Jersey (NJ) plan members Actemra (Tocilizumab) Updated  Updated step therapy guidelines; refer to Step Therapy Guidelines: Actemra (Tocilizumab) for complete details Actiq (Brand Only) Revised  Removed therapeutic equivalent guidelines and corresponding reference (Fentanyl Citrate) link to policy titled Drug Coverage Criteria - New and Therapeutic Equivalent Medications  Updated medication/drug name to include “Brand Only” Afstyla (Antihemophilic Updated  Updated prior authorization/medical necessity guidelines; refer to Prior Factor [Recombinant] Authorization/Medical Necessity Guidelines: Afstyla for complete details Single Chain) Atorvastatin (Generic Updated  Updated prior authorization/notification guidelines; refer to Prior Lipitor) 10mg, 20mg Authorization/Notification Guidelines: Cardiovascular Disease Prevention Zero Cost Share for complete details Belsomra (Suvorexant) Updated  Updated step therapy guidelines; refer to Step Therapy Guidelines: Belsomra for complete details Benznidazole New  Added language to indicate precertification is required through the Pharmacy Benefit Manager (PBM)  Added prior authorization/notification guidelines; refer to Prior

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Clinical Policy Updates

Policy Title Effective Date Drug/Medication Status Summary of Changes REVISED Drug Coverage Jun. 1, 2018 Benznidazole New Authorization/Notification Guidelines: Benznidazole for complete details Guidelines (continued) (continued) Bupropion (SR) (Generic Revised  Updated medication/drug name to include “SR” Zyban)  Added supply limit guidelines; refer to Supply Limit Guidelines: HCR Tobacco Cessation - Supply Limits Override - NJ Fully Insured for complete details Carospir Suspension Revised  Added prior authorization/medical necessity guidelines; refer to Prior (Spironolactone) Authorization/Medical Necessity Guidelines: Carospir for complete details  Removed therapeutic equivalent guidelines and corresponding reference link to policy titled Drug Coverage Criteria - New and Therapeutic Equivalent Medications Chantix (Varenicline Revised  Added supply limit guidelines; refer to Supply Limit Guidelines: HCR Tartrate) Tobacco Cessation - Supply Limits Override - NJ Fully Insured for complete details Cimduo (Lamivudine/ New  Added language to indicate precertification is required through the Tenofovir DF) Pharmacy Benefit Manager (PBM)  Added therapeutic equivalent guidelines; refer to Therapeutic Equivalent Guidelines: Drug Coverage Criteria - New and Therapeutic Equivalent Medications for complete details Cimzia (Certolizumab Updated  Updated prior authorization/notification guidelines; refer to Prior Pegol) Authorization/Notification Guidelines: Cimzia (Certolizumab Pegol) for complete details Cosentyx (Secukinumab) Updated  Updated prior authorization/notification guidelines; refer to Prior Authorization/Notification Guidelines: Cosentyx for complete details Daraprim Revised  Revised prior authorization/medical necessity guidelines; refer to Prior (Pyrimethamine) Authorization/Medical Necessity Guidelines: Daraprim for complete details Enbrel (Etanercept) Updated  Updated prior authorization/notification guidelines; refer to Prior Authorization/Notification Guidelines: Enbrel (Etanercept) for complete details Ergomar (Ergotamine Updated  Updated prior authorization/medical necessity guidelines; refer to Prior Tartrate) Authorization/Medical Necessity Guidelines: Migranal for complete details Farydak (Panobinostat) Revised  Revised prior authorization/notification guidelines; refer to Prior Authorization/Notification Guidelines: Farydak for complete details Fentanyl Citrate (Generic New  Added language to indicate precertification is required through the Actiq) Pharmacy Benefit Manager (PBM)  Added prior authorization/notification guidelines; refer to Prior Authorization/Notification Guidelines: Actiq (Fentanyl Citrate) for complete details

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Policy Title Effective Date Drug/Medication Status Summary of Changes REVISED Drug Coverage Jun. 1, 2018 Grastek (Timothy Grass Updated  Updated prior authorization/medical necessity guidelines; refer to Prior Guidelines Pollen Allergen Extract) Authorization/Medical Necessity Guidelines: Grastek (Timothy Grass (continued) Pollen Allergen Extract) for complete details Jakafi (Ruxolitinib) Revised  Revised prior authorization/notification guidelines; refer to Prior Authorization/Notification Guidelines: Jakafi for complete details Kineret (Anakinra) Updated  Updated prior authorization/notification guidelines; refer to Prior Authorization/Notification Guidelines: Kineret (Anakinra) for complete details Korlym (Mifepristone) Updated  Updated prior authorization/notification guidelines; refer to Prior Authorization/Notification Guidelines: Korlym for complete details Lidocaine Patch Updated  Updated prior authorization/notification guidelines; refer to Prior Authorization/Notification Guidelines: Lidocaine Patch for complete details Lemtrada Updated  Updated list of applicable HCPCS codes; replaced J9010 with J0202 (Alemtuzumab) Linzess (Linaclotide) Updated  Updated prior authorization/notification guidelines; refer to Prior Authorization/Notification Guidelines: Linzess (Linaclotide) for complete details Lynparza (Olaparib) Revised  Revised prior authorization/notification guidelines; refer to Prior Authorization/Notification Guidelines: Lynparza for complete details Lyrica CR (Pregabalin) Revised  Added step therapy guidelines; refer to Step Therapy Guidelines: Lyrica CR for complete details  Removed therapeutic equivalent guidelines and corresponding reference link to policy titled Drug Coverage Criteria - New and Therapeutic Equivalent Medications Mekinist (Trametinib) Revised  Revised prior authorization/notification guidelines; refer to Prior Authorization/Notification Guidelines: Mekinist (Trametinib) for complete details Migranal (Dihydro- Updated  Updated prior authorization/medical necessity guidelines; refer to Prior Ergotamine) (Brand) Authorization/Medical Necessity Guidelines: Migranal for complete details Migranal (Dihydro- Updated  Updated prior authorization/medical necessity guidelines; refer to Prior Ergotamine) (Generic) Authorization/Medical Necessity Guidelines: Migranal for complete details Movantik (Naloxegol) Updated  Updated prior authorization/notification guidelines; refer to Prior Authorization/Notification Guidelines: Movantik for complete details Nicotine OTC Products Revised  Added supply limit guidelines; refer to Supply Limit Guidelines: HCR Tobacco Cessation - Supply Limits Override - NJ Fully Insured for complete details Ninlaro (Ixazomib) Updated  Updated prior authorization/notification guidelines; refer to Prior Authorization/Notification Guidelines: Ninlaro for complete details

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Policy Title Effective Date Drug/Medication Status Summary of Changes REVISED Drug Coverage Jun. 1, 2018 Odactra (House Dust Updated  Updated prior authorization/medical necessity guidelines; refer to Prior Guidelines Mite Allergen Extract) Authorization/Medical Necessity Guidelines: Odactra for complete details (continued) Oralair (Sweet Vernal, Updated  Updated prior authorization/medical necessity guidelines; refer to Prior Orchard, Perennial Rye, Authorization/Medical Necessity Guidelines: Oralair (Sweet Vernal, Timothy and Kentucky Orchard, Perennial Rye, Timothy and Kentucky Blue Grass, Mixed Pollens Blue Grass, Mixed Allergen Extract) for complete details Pollens Allergen Extract) Prolia, Xgeva Revised  Revised coverage guidelines to indicate precertification is required (Denosumab) through Oxford’s Medical Management Radicava (Edaravone) Revised  Added notation to indicate administration of Radicava in a hospital outpatient facility (including any ambulatory infusion suite associated with the hospital) requires precertification with review by a Medical Director or their designee; refer to Specialty Medication Administration – Site of Care Review Guidelines Ragwitek (Short Updated  Updated prior authorization/medical necessity guidelines; refer to Prior Ragweed Pollen Allergen Authorization/Medical Necessity Guidelines: Ragwitek (Short Ragweed Extract) Pollen Allergen Extract) for complete details Rexulti (Brexpiprazole) Revised  Revised prior authorization/medical necessity guidelines; refer to Prior Authorization/Medical Necessity Guidelines: Rexulti for complete details Rhopressa (Netarsudil) New  Added language to indicate precertification is required through the Pharmacy Benefit Manager (PBM)  Added therapeutic equivalent guidelines; refer to Therapeutic Equivalent Guidelines: Drug Coverage Criteria - New and Therapeutic Equivalent Medications for complete details Rozerem (Ramelteon) Updated  Updated step therapy guidelines; refer to Step Therapy Guidelines: Rozerem (Ramelteon) for complete details Simponi (Golimumab) Updated  Updated prior authorization/notification guidelines; refer to Prior Authorization/Notification Guidelines: Simponi (Golimumab) for complete details Simponi Aria Updated  Updated list of related policies: (Golimumab) o Modified reference link Precertification Guidelines: Simponi Aria® (Golimumab) Injection for Intravenous Infusion to reflect title change o Added reference link to Precertification Guidelines: Specialty Medication Administration – Site of Care Review Guidelines (previously listed under Notes section only) Simvastatin (Generic Updated  Updated prior authorization/notification guidelines; refer to Prior Zocor) 5mg, 10mg, Authorization/Notification Guidelines: Cardiovascular Disease Prevention 20mg, 40mg Zero Cost Share for complete details

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Policy Title Effective Date Drug/Medication Status Summary of Changes REVISED Drug Coverage Jun. 1, 2018 Sutent (Sunitinib) Revised  Revised prior authorization/notification guidelines; refer to Prior Guidelines Authorization/Notification Guidelines: Sutent for complete details (continued) Symfi (Efavirenz/ New  Added language to indicate precertification is required through the Lamivudine/Tenofovir Pharmacy Benefit Manager (PBM) Disoproxil Fumarate)  Added therapeutic equivalent guidelines; refer to Therapeutic Equivalent Guidelines: Drug Coverage Criteria - New and Therapeutic Equivalent Medications for complete details Smyfi Lo New  Added language to indicate precertification is required through the (Efavirenz/Lamivudine/ Pharmacy Benefit Manager (PBM) Tenofovir Disoproxil  Added therapeutic equivalent guidelines; refer to Therapeutic Equivalent Fumarate) Guidelines: Drug Coverage Criteria - New and Therapeutic Equivalent Medications for complete details Symproic (Naldemedine) New  Added prior authorization/notification guidelines; refer to Prior Authorization/Notification Guidelines: Symproic for complete details Tafinlar (Dabrafenib) Revised  Revised prior authorization/notification guidelines; refer to Prior Authorization/Notification Guidelines: Tafinlar for complete details Tamiflu Capsules (Brand Revised  Added language to indicate precertification is required through the Only) (Oseltamivir Pharmacy Benefit Manager (PBM) Phosphate)  Added therapeutic equivalent guidelines; refer to Therapeutic Equivalent Guidelines: Drug Coverage Criteria - New and Therapeutic Equivalent Medications for complete details Test Strips and Meters Revised  Updated prior authorization/notification guidelines; refer to Prior (Diabetic) Authorization/Notification Guidelines: Test Strips for complete details  Added notation to indicate Contour Next test strips do not require precertification Trulance (Plecanatide) Revised  Revised prior authorization/medical necessity guidelines; refer to Prior Authorization/Medical Necessity Guidelines: Trulance for complete details Viberzi (Eluxadoline) Updated  Updated prior authorization/medical necessity guidelines; refer to Prior Authorization/Medical Necessity Guidelines: Viberzi for complete details Vyzulta (Latanoprostene Revised  Added step therapy guidelines; refer to Step Therapy Guidelines: Vyzulta Bunod) for complete details Zelboraf (Vemurafenib) Revised  Revised prior authorization/notification guidelines; refer to Prior Authorization/Notification Guidelines: Zelboraf for complete details Zolpimist (Zolpidem Updated  Updated step therapy guidelines; refer to Step Therapy Guidelines: Tartrate) Zolpimist (Zolpidem Tartrate) for complete details Zypitamag (Pitavastatin) New  Added language to indicate precertification is required through the Pharmacy Benefit Manager (PBM)  Added therapeutic equivalent guidelines; refer to Therapeutic Equivalent Guidelines: Drug Coverage Criteria - New and Therapeutic Equivalent

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Clinical Policy Updates

Policy Title Effective Date Drug/Medication Status Summary of Changes REVISED Drug Coverage Jun. 1, 2018 Medications for complete details Guidelines (continued)

Policy Title Effective Date Summary of Changes Coverage Rationale REVISED Electrical Jun. 1, 2018  Replaced references to When used for walking, functional electrical stimulation (FES), a Stimulation for the “patients” with “individuals” form of neuromuscular electrical stimulation (NMES) is proven Treatment of Pain  Revised coverage rationale; and/or medically necessary when used as one component of a and Muscle replaced language indicating: comprehensive rehabilitation program in persons with paralyzed Rehabilitation o “[The listed services] are lower limbs due to spinal cord injury (SCI) with ALL of the following proven and medically characteristics: necessary” with “[the listed  Intact lower motor units (L1 and below) (both muscle and peripheral services] are proven and/or nerves); medically necessary”  Muscle and joint stability for weight bearing at upper and lower o “[The listed services] are extremities that can demonstrate balance and control to maintain an unproven and not medically upright support posture independently; necessary” with “[the listed  Demonstrate brisk muscle contraction to NMES and have sensory services] are unproven perception of electrical stimulation (ES) sufficient for muscle contraction; and/or not medically  Possess high motivation, commitment and cognitive ability to use such necessary” devices for walking; o “Peripheral subcutaneous  Able to transfer independently and can demonstrate independent field stimulation (PSFS) or standing tolerance for at least 3 minutes; peripheral nerve field  Demonstrate hand and finger function to manipulate controls; stimulation (PNFS) is  Post recovery from SCI and restorative surgery of at least 6 months; unproven and not medically  No hip and knee degenerative disease and no history of long bone necessary for treating pain” fracture secondary to osteoporosis. with “peripheral subcutaneous field FES is unproven and/or not medically necessary for treating ANY stimulation (PSFS) or other indication not listed above as proven and medically necessary, peripheral nerve field including but not limited to: stimulation (PNFS) is  Disuse muscle atrophy in persons with SCI. unproven and/or not  Disuse muscle atrophy in persons with multiple sclerosis (MS). medically necessary for all  Gait disorders (e.g., foot drop) of central neurologic origin, including but indications, including but not not limited to stroke or MS. limited to pain and opioid management” Further studies are needed to confirm that FES promotes bone o “Evidence for the remineralization and prevents or reverses muscle atrophy. Only a few studies effectiveness of PSFS or have looked at FES as a modality of treatment of MS, and the results are

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Policy Title Effective Date Summary of Changes Coverage Rationale REVISED Electrical Jun. 1, 2018 PNFS based on controlled limited and conflicting regarding whether FES improves treatment outcomes Stimulation for the studies is lacking; in MS when offered in addition to other rehabilitative treatment modalities. Treatment of Pain randomized controlled trials There is insufficient evidence in the peer reviewed literature that use of FES and Muscle (RCTs) are needed to will improve health outcomes in individuals with gait disorders. Published Rehabilitation evaluate the efficacy of this studies have included small heterogeneous patient populations, short-term (continued) treatment” with “evidence follow-ups, and various treatment protocols, outcome measures, and FES for the effectiveness of PSFS devices. or PNFS based on controlled studies is limited; additional NMES is proven and/or medically necessary for treating Disuse studies are needed to muscle atrophy if: evaluate the efficacy of this  The nerve supply to the muscle is intact; and treatment”  The disuse muscle atrophy is not of neurological origin but originates  Updated supporting information from conditions such as casting, splinting or contractures; or to reflect the most current  To improve wrist and finger function and prevent or correct shoulder clinical evidence, FDA subluxation in persons with partial paralysis following stroke. information, and references NMES is unproven and/or not medically necessary for treating ANY other indication not listed above as proven and medically necessary. There is insufficient evidence in the peer reviewed literature that use of ES will improve health outcomes for the treatment of multiple conditions other than those identified above as proven. Overall, studies in the form of RCTs and case series included small, heterogeneous patient populations and short- term follow-ups. Some systematic reviews have reported that no improvement was seen with NMES, outcomes were conflicting and/or in some cases, when improvement was noted, the effects did not last. Heterogeneity of treatment regimens and outcome measures make it difficult to establish that NMES resulted in meaningful clinical outcomes (e.g., decrease pain, functional improvement, improvement in quality of life (QOL) and ability to carry out activities of daily living (ADLs) for these other conditions and indications.

Interferential therapy (IFT) is unproven and/or not medically necessary for treating the following indications:  For the treatment of musculoskeletal disorders or injuries.  For stimulating healing of nonsurgical soft tissue injuries.  To facilitate the healing of bone fractures.

There is limited evidence from the available studies to conclude that IFT reduces the pain or promotes healing of bone fractures, musculoskeletal or

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Policy Title Effective Date Summary of Changes Coverage Rationale REVISED Electrical Jun. 1, 2018 nonsurgical soft tissue injuries. Although a few studies reported some Stimulation for the improvement in pain or disability following IFT for these conditions, none of Treatment of Pain the double-blind, randomized, placebo-controlled studies reported a positive and Muscle treatment effect of IFT for nonsurgical soft tissue injuries or bone fractures. Rehabilitation (continued) Pulsed electrical stimulation (PES) is unproven and/or not medically necessary for treating osteoarthritis (OA). There is insufficient evidence to conclude that PES provides health benefits to individuals with OA. RTCs are necessary to assess the durability of this procedure in comparison to other types of treatment.

Peripheral subcutaneous field stimulation (PSFS) or peripheral nerve field stimulation (PNFS) is unproven and/or not medically necessary for all indications, including but not limited to pain and opioid management. Evidence for the effectiveness of PSFS or PNFS based on controlled studies is limited. Additional studies are needed to evaluate the efficacy of this treatment.

Microcurrent electrical nerve stimulation (MENS) therapy is unproven and/or not medically necessary. There is insufficient evidence to conclude that MENS is safe and effective. Robust clinical trials are needed to evaluate this therapy in comparison to other types of treatment.

Percutaneous electrical nerve stimulation (PENS) or percutaneous neuromodulation therapy (PNT) is unproven and/or not medically necessary for treating pain. There is limited evidence in the peer reviewed literature to support that PENS or PNT will improve health outcomes in individuals with pain. RCTs assessing larger patient groups and long-term follow up are needed to further clarify its role.

Dorsal root ganglion (DRG) stimulation is unproven and/or not medically necessary. There is limited evidence in the peer reviewed literature to support that DRG stimulation will improve health outcomes in individuals with pain. RCTs assessing larger patient groups and long-term follow up are needed to further clarify its role.

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Policy Title Effective Date Summary of Changes Coverage Rationale REVISED Entyvio® Jun. 1, 2018  Updated list of related policies; Entyvio (vedolizumab) is proven and medically necessary for the (Vedolizumab) added reference link to the treatment of: policy titled Acquired Rare Disease Drug Therapy Exception Crohn's disease when all of the following criteria are met: Process  For initial therapy, all of the following:  Revised conditions of o Diagnosis of moderately to severely active Crohn’s disease (CD); coverage/precertification and requirements; added language o One of the following: to indicate: . History of failure, contraindication, or intolerance to at least one o Requests for hospital of the following conventional therapies: outpatient facility infusion of - Tumor necrosis factor (TNF) blocker [e.g., Humira Entyvio require additional (adalimumab), Cimzia (certolizumab)] precertification with review - Immunomodulator (e.g., azathioprine, 6-mercaptopurine) by a Medical Director or their - Corticosteroid designee; refer to the policy . Corticosteroid dependent (e.g., unable to successfully taper titled Specialty Medication corticosteroids without a return of the symptoms of CD) Administration - Site of Care and Review Guidelines o Entyvio is initiated and titrated according to US Food and Drug  Updated supporting information Administration (FDA) labeled dosing for Crohn’s disease up to a to reflect the most current maximum of 300mg every 8 weeks (or equivalent dose and interval references schedule); and o Patient is not receiving Entyvio in combination with either of the following: . Tumor necrosis factor (TNF) blocker [e.g., Humira (adalimumab), Cimzia (certolizumab)] . Tysabri (natalizumab); and o Initial authorization will be for no more than 14 weeks  For continuation therapy, all of the following: o Documentation of positive clinical response to Entyvio; and o Entyvio dosing for Crohn’s disease is in accordance with the FDA labeled dosing up to a maximum of 300mg every 8 weeks (or equivalent dose and interval schedule); and o Reauthorization will be for no more than 12 months.

Ulcerative colitis when all of the following criteria are met:  For initial therapy, all of the following: o Diagnosis of moderately to severely active ulcerative colitis (UC); and o One of the following:

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Policy Title Effective Date Summary of Changes Coverage Rationale REVISED Entyvio® Jun. 1, 2018 . History of failure, contraindication, or intolerance to at least one (Vedolizumab) of the following conventional therapies: (continued) - Tumor necrosis factor (TNF) blocker [e.g., Humira (adalimumab), Simponi (golimumab)] - Immunomodulator (e.g., azathioprine, 6-mercaptopurine) - Corticosteroid . Corticosteroid dependent (e.g., unable to successfully taper corticosteroids without a return of the symptoms of UC) and o Entyvio is initiated and titrated according to US Food and Drug Administration labeled dosing for ulcerative colitis up to a maximum of 300mg every 8 weeks (or equivalent dose and interval schedule); and o Patient is not receiving Entyvio in combination with either of the following: . Tumor necrosis factor (TNF) blocker [e.g., Humira (adalimumab), Simponi (golimumab)] . Tysabri (natalizumab); and o Initial authorization will be for no more than 14 weeks.  For continuation therapy, all of the following: o Documentation of positive clinical response to Entyvio; and o Entyvio dosing for ulcerative colitis is in accordance with the FDA labeled dosing up to a maximum of 300mg every 8 weeks (or equivalent dose and interval schedule); and o Reauthorization will be for no more than 12 months.

Enzyme Aug. 1, 2018  Updated list of related policies; This policy refers to the following enzyme replacement therapy products: Replacement added reference link to the  Adagen (pegademase bovine) Therapy policy titled Specialty Medication  Aldurazyme (laronidase) Administration – Site of Care  Elaprase (idursulfase) Review Guidelines  Fabrazyme (agalsidase beta)  Revised conditions of  Kanuma (sebelipase alfa) coverage/precertification  Lumizyme (alglucosidase alfa) requirements to indicate:  Mepsevii (vestronidase alfa-vjbk) o Precertification with review  Naglazyme (galsulfase) by a Medical Director or their  Vimizim (elosulfase alfa) designee through Oxford’s Medical Management is Adagen (pegademase bovine) is medically necessary for the required treatment of severe combined immunodeficiency disease (SCID) o New Jersey Small Group associated with a deficiency of adenosine deaminase (ADA) when the

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Clinical Policy Updates

Policy Title Effective Date Summary of Changes Coverage Rationale REVISED Enzyme Aug. 1, 2018 members should refer to following criteria are met: Replacement their certificate of coverage  For initial therapy, all of the following: Therapy for precertification guidelines o Diagnosis of SCID; and (continued) and quantity limit guidelines o Deficiency of adenosine deaminase is confirmed by any of the o Requests for hospital following: outpatient facility infusion of . Deficiency or absence of ADA in plasma, lysed erythrocytes, enzyme replacement therapy fibroblasts (cultured from amniotic fluid), or chorionic villus products addressed in this . Increase in deoxyadenosine triphosphate (dATP) levels in policy require additional erythrocyte lysates compared to laboratory standard precertification with review . Decrease in ATP concentration in erythrocytes by a Medical Director or their . Molecular genetic confirmation of mutations in both alleles of the designee; refer to the policy ADA1 gene titled Specialty Medication . Positive screening by T cell receptor excision circles (TRECs); Administration - Site of Care and Review Guidelines o One of the following: . Patient is not a suitable candidate for hematopoietic cell transplantation (HCT) . Patient has failed HCT; and o Dosing is in accordance with the United States Food and Drug Administration approved labeling: dosing is started at 10 U/kg for the first dose, and titrated up to a maximum dose of 30 U/kg per week; and o Initial authorization will be for no more than 12 months.  For continuation therapy, all of the following: o Patient has previously received treatment with pegademase therapy; and o Patient has experienced a positive clinical response to pegademase therapy (e.g., normalization of plasma ADA activity, erythrocyte dATP levels, improvement of disease symptoms, etc.); and o Dosing is in accordance with the United States Food and Drug Administration approved labeling: appropriate maintenance dosing, up to a maximum dose of 30 U/kg per week; and o Reauthorization will be for no more than 12 months.

Aldurazyme (laronidase) is medically necessary for the treatment of Mucopolysaccharidosis I (MPS I) when the following criteria are met:  For initial therapy, all of the following: o Diagnosis of any of the MPS I syndromes confirmed by one the

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Policy Title Effective Date Summary of Changes Coverage Rationale REVISED Enzyme Aug. 1, 2018 following: Replacement . Hurler variant (severe mucopolysaccharidosis I; also MPS IH) Therapy . Hurler-Scheie variant (attenuated mucopolysaccharidosis I; also (continued) MPS IHS) . Scheie variant (attenuated mucopolysaccharidosis I; also MPS IS); and o Diagnosis of MPS I is confirmed by either of the following: . Deficiency or absence of fibroblast or leukocyte enzyme activity of alpha-L-iduronidase enzyme activity . Molecular genetic confirmation of mutations in the alpha-L- iduronidase gene; and o Presence of clinical signs and symptoms of the disease (e.g., asymptomatic with affected older sibling, cardiac abnormalities, corneal clouding, dysostosis multiplex, hepatomegaly, restrictive lung disease, etc.); and o Dosing is in accordance with the United States Food and Drug Administration approved labeling: Administered dose does not exceed 0.58 mg/kg intravenously once every week; and o Initial authorization will be for no more than 12 months.  For continuation therapy, all of the following: o Patient has previously received treatment with laronidase therapy; and o Patient has experienced a positive clinical response to laronidase therapy (e.g., improved endurance, improved functional capacity, reduced urine dermatan sulfate/heparan sulfate excretion, etc.); and o Dosing is in accordance with the United States Food and Drug Administration approved labeling: administered dose does not exceed 0.58 mg/kg intravenously once every week; and o Reauthorization will be for no more than 12 months.

Elaprase (idursulfase) is medically necessary for the treatment of Mucopolysaccharidosis II (MPS II, Hunter Syndrome) when the following criteria are met:  For initial therapy, all of the following: o Diagnosis of MPS II confirmed by one the following: . Deficiency in iduronate 2-sulfatase enzyme activity as measured in fibroblasts or leukocytes combined with normal enzyme

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Policy Title Effective Date Summary of Changes Coverage Rationale REVISED Enzyme Aug. 1, 2018 activity level of another sulfatase Replacement . Molecular genetic testing for deletion or mutations in the Therapy iduronate 2-sulfatase gene; (continued) and o Presence of clinical signs and symptoms of the disease (e.g., hepatosplenomegaly, skeletal deformities, dysostosis, neurocognitive decline, cardiovascular disorders, etc.); and o Dosing is in accordance with the United States Food and Drug Administration approved labeling: administered dose does not exceed 0.5 mg/kg intravenously once every week; and o Initial authorization will be for no more than 12 months.  For continuation therapy, all of the following: o Patient has previously received treatment with idursulfase therapy; and o Patient has experienced a positive clinical response to idursulfase therapy (e.g., improved endurance, improved functional capacity, reduced spleen volume, reduced urine GAG excretion, etc.); and o Dosing is in accordance with the United States Food and Drug Administration approved labeling: administered dose does not exceed 0.5 mg/kg intravenously once every week; and o Reauthorization will be for no more than 12 months.

Fabrazyme (agalsidase beta) is medically necessary for the treatment of Fabry disease when the following criteria are met:  For initial therapy, all of the following: o Diagnosis of Fabry disease as confirmed by one the following: . Absence or deficiency (< 5% of mean) of normal alpha- galactosidase A (α-Gal A) enzyme activity) in leukocytes, dried blood spots, or serum analysis . Molecular genetic testing for deletion or mutations in the galactosidase alpha gene; and o Presence of clinical signs and symptoms of the disease (e.g., Acroparesthesias, angiokeratomas, whorls, anhidrosis/hypohidrosis, renal disease, exercise/heat/cold intolerance, etc.); and o Dosing is in accordance with the United States Food and Drug Administration approved labeling: administered dose does not exceed 1 mg/kg intravenously every two weeks; and o Initial authorization will be for no more than 12 months.

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Policy Title Effective Date Summary of Changes Coverage Rationale REVISED Enzyme Aug. 1, 2018  For continuation therapy, all of the following: Replacement o Patient has previously received treatment with agalsidase therapy; Therapy and (continued) o Patient has experienced a positive clinical response to agalsidase therapy (e.g., improved renal function, reduction in mean plasma GL-3 levels, decreased GL-3 inclusions, etc.); and o Dosing is in accordance with the United States Food and Drug Administration approved labeling: administered dose does not exceed 1 mg/kg intravenously every two weeks; and o Reauthorization will be for no more than 12 months.

Kanuma (sebelipase alfa) is medically necessary for the treatment of Lysosomal acid lipase deficiency [LAL-D, Wolman disease (WD), cholesteryl ester disease (CESD)] when the following criteria are met:  For initial therapy, all of the following: o Diagnosis of lysosomal acid lipase deficiency [LAL-D, Wolman disease (WD), cholesteryl ester disease (CESD)] as confirmed by one the following: . Absence or deficiency lysosomal acid lipase activity by dried blood spot test . Molecular genetic testing for deletion or mutations in the lipase A, lysosomal acid type (LIPA) gene; and o Presence of clinical signs and symptoms of the disease (e.g., abdominal distention, hepatosplenomegaly, liver fibrosis, ascities, etc.); and o Dosing is in accordance with the United States Food and Drug Administration approved labeling by one of the following: . For rapidly progressive disease presenting within the first 6 months of life: administered initial starting dose is 1 mg/kg intravenously once weekly, up to a maximum of 3 mg/kg once weekly . Pediatric and adult patients with disease: administered dose does not exceed 1 mg/kg intravenously every other week; and o Initial authorization will be for no more than 12 months.  For continuation therapy, all of the following: o Patient has previously received treatment with sebelipase therapy;

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Policy Title Effective Date Summary of Changes Coverage Rationale REVISED Enzyme Aug. 1, 2018 and Replacement o Patient has experienced a positive clinical response to sebelipase Therapy therapy [e.g., improved disease symptoms, improvement of (continued) laboratory values (LFTs, cholesterol, triglycerides), etc.]; and o Dosing is in accordance with the United States Food and Drug Administration approved labeling by one of the following: . For rapidly progressive disease presenting within the first 6 months of life: administered dose is 1 mg/kg intravenously once weekly, up to a maximum of 3 mg/kg once weekly . Pediatric and adult patients with disease: administered dose does not exceed 1 mg/kg intravenously every other week; and o Reauthorization will be for no more than 12 months.

Lumizyme (alglucosidase alfa) is medically necessary for the treatment of Pompe disease when the following criteria are met:  For initial therapy, one of the following: o All of the following for infantile-onset Pompe disease: . Diagnosis of infantile-onset Pompe disease as confirmed by one the following: - Absence or deficiency (<1% of the lab specific normal mean) acid alpha-glucosidase deficiency (GAA) activity in skin fibroblasts - Molecular genetic testing for deletion or mutations in the GAA gene; and . Presence of clinical signs and symptoms of the disease (e.g., cardiac hypertrophy, respiratory distress, skeletal muscle weakness, etc.); and . Dosing is in accordance with the United States Food and Drug Administration approved labeling: administered dose does not exceed 20 mg/kg intravenously every two weeks; and . Initial authorization will be for no more than 12 months; or o All of the following for late-onset (non-infantile) Pompe disease: . Diagnosis of late-onset Pompe disease as confirmed by one the following: - Absence or deficiency (<40% of the lab specific normal mean) acid alpha-glucosidase deficiency (GAA) activity in

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Policy Title Effective Date Summary of Changes Coverage Rationale REVISED Enzyme Aug. 1, 2018 lymphocytes, fibroblasts or muscle Replacement - Molecular genetic testing for deletion or mutations in the GAA Therapy gene; (continued) and . Presence of clinical signs and symptoms of the disease (e.g., cardiac hypertrophy, respiratory distress, skeletal muscle weakness, etc.); and . Dosing is in accordance with the United States Food and Drug Administration approved labeling: administered dose does not exceed 20 mg/kg intravenously every two weeks; and . Initial authorization will be for no more than 12 months.  For continuation therapy, all of the following: o Patient has previously received treatment with alglucosidase therapy; and o Patient has experienced a positive clinical response to alglucosidase therapy (e.g., improved respiratory/cardiac function, improved endurance, etc.); and o Dosing is in accordance with the United States Food and Drug Administration approved labeling: administered dose does not exceed 20 mg/kg intravenously every two weeks; and o Reauthorization will be for no more than 12 months.

Mepsevii (vestronidase alfa-vjbk) is proven and medically necessary for the treatment of Mucopolysaccharidosis VII (Sly syndrome) when the following criteria are met:  For initial therapy, all of the following: o Diagnosis of Mucopolysaccharidosis VII confirmed by either of the following: . Absence or deficiency of fibroblast or leukocyte enzyme activity of beta glucuronidase . Molecular genetic confirmation of mutations in the GUSB gene. and o Presence of clinical signs and symptoms of the disease (e.g., enlarged liver and spleen, joint limitations, airway obstruction or pulmonary problems, limitation of mobility while still ambulatory, etc.); and o Dosing is in accordance with the United States Food and Drug Administration approved labeling: Administered dose does not exceed 4 mg/kg intravenously once every two weeks; and

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Policy Title Effective Date Summary of Changes Coverage Rationale REVISED Enzyme Aug. 1, 2018 o Initial authorization will be for no more than 12 months. Replacement  For continuation therapy, all of the following: Therapy o Patient has previously received treatment with vestronidase therapy; (continued) and o Patient has experienced a positive clinical response to vestronidase therapy (e.g., improved endurance, improved functional capacity, improved pulmonary function, etc.); and o Dosing is in accordance with the United States Food and Drug Administration approved labeling: Administered dose does not exceed 4 mg/kg intravenously once every two weeks; and o Reauthorization will be for no more than 12 months.

Naglazyme (galsulfase) is medically necessary for the treatment of Mucopolysaccharidosis VI (Maroteaux-Lamy syndrome) when the following criteria are met:  For initial therapy, all of the following: o Diagnosis of Mucopolysaccharidosis VI confirmed by either of the following: . Absence or deficiency of fibroblast or leukocyte enzyme activity of N-acetylgalactosamine 4-sulfatase (arylsulfatase) . Molecular genetic confirmation of mutations in the ASB gene (5q13-q14); and o Presence of clinical signs and symptoms of the disease (e.g., kyphoscoliosis, genu valgum, pectus carinatum, gait disturbance, growth deficiency, etc.); and o Dosing is in accordance with the United States Food and Drug Administration approved labeling: administered dose does not exceed 1 mg/kg intravenously once every week; and o Initial authorization will be for no more than 12 months.  For continuation therapy, all of the following: o Patient has previously received treatment with galsulfase therapy; and o Patient has experienced a positive clinical response to galsulfase therapy (e.g., improved endurance, improved functional capacity, reduced urine dermatan sulfate excretion, etc.); and o Dosing is in accordance with the United States Food and Drug Administration approved labeling: administered dose does not exceed 1 mg/kg intravenously once every week; and

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Policy Title Effective Date Summary of Changes Coverage Rationale REVISED Enzyme Aug. 1, 2018 o Reauthorization will be for no more than 12 months. Replacement Therapy Vimizim (elosulfase alfa) is medically necessary for the treatment of (continued) Mucopolysaccharidosis type IVA (MPS IVA; Morquio A syndrome) when the following criteria are met:  For initial therapy, all of the following: o Diagnosis of Morquio A syndrome confirmed by either of the following: . Absence or deficiency of fibroblast or leukocyte GALNS enzyme activity . Molecular genetic testing for mutations in the GALNS gene (16q24.3); and o Presence of clinical signs and symptoms of the disease (e.g., kyphoscoliosis, genu valgum, pectus carinatum, gait disturbance, growth deficiency, etc.); and o Dosing is in accordance with the United States Food and Drug Administration approved labeling: administered dose does not exceed 2 mg/kg IV once every week; and o Initial authorization will be for no more than 12 months.  For continuation therapy, all of the following: o Patient has previously received treatment with elosulfase alfa therapy; and o Patient has experienced a positive clinical response to elosulfase alfa therapy (e.g., improved endurance, improved functional capacity, reduced urine keratan sulfate excretion); and o Dosing is in accordance with the United States Food and Drug Administration approved labeling: administered dose does not exceed 2 mg/kg IV once every week; and o Reauthorization will be for no more than 12 months.

Exondys 51™ Jun. 1, 2018  Revised conditions of Exondys 51™ (eteplirsen) may be covered for the treatment of (Eteplirsen) coverage/precertification Duchenne muscular dystrophy (DMD) in patients who meet ALL of requirements; added language the following criteria: to indicate:  For initial therapy, all of the following: o Requests for hospital o Diagnosis of Duchenne muscular dystrophy by, or in consultation outpatient facility infusion of with, a neurologist with expertise in the diagnosis of DMD; and Exondys 51 require o Submission of medical records (e.g., chart notes, laboratory values) additional precertification confirming the mutation of the DMD gene is amenable to exon 51 with review by a Medical skipping; and

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Policy Title Effective Date Summary of Changes Coverage Rationale REVISED Exondys 51™ Jun. 1, 2018 Director or their designee; o Submission of medical records (e.g., chart notes, laboratory values) (Eteplirsen) refer to the policy titled confirming patient has a 6-Minute Walk Time (6MWT) ≥ 300 meters (continued) Specialty Medication while walking independently (e.g., without side-by-side assist, cane, Administration - Site of Care walker, wheelchair, etc.) prior to beginning Exondys 51 therapy;and Review Guidelines o Exondys 51 is prescribed by, or in consultation with, a neurologist with expertise in the treatment of DMD; and o Exondys 51 dosing for DMD is in accordance with the United States Food and Drug Administration approved labeling: maximum dosing of 30 mg/kg infused once weekly; and o Initial authorization will be for no more than 8 weeks.  For continuation therapy, all of the following: o Exondys 51 is prescribed by, or in consultation with, a neurologist with expertise in the treatment of DMD; and o Submission of medical records (e.g., chart notes, laboratory values) demonstrating that the patient continues to have a 6-Minute Walk Time (6MWT) ≥ 300 meters while walking independently (e.g., without side-by-side assist, cane, walker, wheelchair, etc.). This must be measured no earlier than 4 weeks prior to a continuation request; and o Exondys 51 dosing for DMD is in accordance with the United States Food and Drug Administration approved labeling: maximum dosing of 30 mg/kg infused once weekly; and o Reauthorization will be for no more than 6 months.

Exondys 51 will not be covered for other forms of muscular dystrophy.

Ilaris® Jun. 1, 2018  Revised conditions of Ilaris® (canakinumab) is proven and medically necessary for: (Canakinumab) coverage/precertification requirements; added language The treatment of Cryopyrin-Associated Periodic Syndromes (CAPS) in to indicate: patients who meet ALL of the following criteria: o Requests for hospital  For initial therapy, all of the following: outpatient facility infusion of o One of the following, as diagnosed by, or in consultation with, a Ilaris require additional rheumatologist or immunologist with expertise in the diagnosis of the precertification with review following: by a Medical Director or their . Familial Cold Autoinflammatory Syndrome (FCAS) designee; refer to the policy . Muckle-Wells syndrome (MWS) titled Specialty Medication and Administration - Site of Care o Ilaris dosing for FCAS/MWS is in accordance with the United States Review Guidelines

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Clinical Policy Updates

Policy Title Effective Date Summary of Changes Coverage Rationale REVISED Ilaris® Jun. 1, 2018 Food and Drug Administration approved labeling: maximum dosing of (Canakinumab) 3mg/kg up to 150 mg every 8 weeks; and (continued) o Initial authorization will be for no more than 12 months.  For continuation therapy, all of the following: o Patient is currently on Ilaris therapy for one of the following: . FCAS . MWS and o Ilaris dosing for FCAS/MWS is in accordance with the United States Food and Drug Administration approved labeling: maximum dosing of 3mg/kg up to 150 mg every 8 weeks; and o Documentation of positive clinical response to Ilaris therapy; and o Reauthorization will be for no more than 12 months.

The treatment of Tumor Necrosis Factor (TNF) Receptor-Associated Periodic Syndrome (TRAPS) in patients who meet ALL of the following criteria:  For initial therapy, all of the following: o Diagnosis of TRAPS by, or in consultation with, a rheumatologist or immunologist with expertise in the diagnosis of TRAPS. and o Ilaris dosing for TRAPS is in accordance with the United States Food and Drug Administration approved labeling: maximum dosing of 4 mg/kg up to 300mg every 4 weeks; and o Initial authorization will be for no more than 12 months.  For continuation of therapy, all of the following: o Patient is currently receiving Ilaris therapy for TRAPS; and o Documentation of a positive clinical response to therapy, defined as a decrease in frequency or severity of attacks; and o Ilaris dosing for TRAPS is in accordance with the United States Food and Drug Administration approved labeling: maximum dosing of 4 mg/kg up to 300mg every 4 weeks; and o Reauthorization will be for no more than 12 months.

The treatment of Hyperimmunoglobulin D (Hyper-IgD) Syndrome (HIDS)/Mevalonate Kinase Deficiency (MKD) in patients who meet ALL of the following criteria:  For initial therapy, all of the following: o One of the following, as diagnosed by, or in consultation with, a

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Policy Title Effective Date Summary of Changes Coverage Rationale REVISED Ilaris® Jun. 1, 2018 rheumatologist or immunologist with expertise in the diagnosis of the (Canakinumab) following: (continued) . HIDS . MKD and o Ilaris dosing for HIDS/MKD is in accordance with the United States Food and Drug Administration approved labeling: maximum dosing of 4 mg/kg up to 300mg every 4 weeks; and o Initial authorization will be for no more than 12 months.  For continuation of therapy, all of the following: o Patient is currently receiving Ilaris for one of the following: . HIDS . MKD and o Documentation of a positive clinical response to therapy, defined by a decrease in frequency or severity of attacks; and o Ilaris dosing for HIDS/MKD is in accordance with the United States Food and Drug Administration approved labeling: maximum dosing of 4 mg/kg up to 300mg every 4 weeks; and o Reauthorization will be for no more than 12 months.

The treatment of Familial Mediterranean Fever (FMF) in patients who meet ALL of the following criteria:  For initial therapy, all of the following: o Diagnosis of FMF by, or in consultation with, a rheumatologist or immunologist with expertise in the diagnosis of FMF; and o History of failure, contraindication, or intolerance to colchicine; and o Ilaris dosing for FMF is in accordance with the United States Food and Drug Administration approved labeling: maximum dosing of 4 mg/kg up to 300mg every 4 weeks; and o Initial authorization will be for no more than 12 months.  For continuation of therapy, all of the following: o Patient is currently receiving Ilaris for FMF; and o Documentation of a positive clinical response to therapy, defined by a decrease in index disease flare or normalization of CRP; and o Ilaris dosing for FMF is in accordance with the United States Food and Drug Administration approved labeling: maximum dosing of 4 mg/kg up to 300mg every 4 weeks; and o Reauthorization will be for no more than 12 months.

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Clinical Policy Updates

Policy Title Effective Date Summary of Changes Coverage Rationale REVISED Ilaris® Jun. 1, 2018 The treatment of Systemic Juvenile Idiopathic Arthritis (SJIA) in (Canakinumab) patients who meet ALL of the following criteria: (continued)  For initial therapy, all of the following: o Diagnosis of SJIA by, or in consultation with, a rheumatologist or immunologist with expertise in the diagnosis of SJIA; and o Ilaris dosing for SJIA is in accordance with the United States Food and Drug Administration approved labeling: maximum dosing of 4 mg/kg up to 300mg every 4 weeks; and o Patient is not receiving Ilaris in combination with another biologic [e.g., Actemra]; and o Initial authorization will be for no more than 12 months.  For continuation of therapy, all of the following: o Patient is currently receiving Ilaris for SJIA; and o Documentation of a positive clinical response to therapy; and o Ilaris dosing for SJIA is in accordance with the United States Food and Drug Administration approved labeling: maximum dosing of 4 mg/kg up to 300mg every 4 weeks; and o Patient is not receiving Iliaris in combination with another biologic [e.g., Actemra]; and o Reauthorization will be for no more than 12 months.

Ilaris is not proven or medically necessary for the management or treatment of cardiovascular disease.

Infliximab Jun. 1, 2018  Revised conditions of This policy refers to the following infliximab products: (Remicade®, coverage/precertification  Inflectra™ (infliximab-dyyb) Inflectra™, requirements; added language  Remicade® (infliximab) Renflexis™) to indicate:  Renflexis™ (infliximab-abda) o Requests for hospital outpatient facility infusion of A. Preferred Product Remicade, Inflectra, and Remicade® (infliximab) is the preferred infliximab product. Coverage will Renflexis require additional be provided for Remicade® contingent on the coverage criteria in the precertification with review Diagnosis-Specific Criteria section. by a Medical Director or their designee; refer to the policy Coverage for Inflectra™ (infliximab-dyyb) or Renflexis™ (infliximab- titled Specialty Medication abda) will be provided contingent on the criteria in this section and the Administration - Site of Care coverage criteria in the Diagnosis-Specific Criteria section. In order to Review Guidelines continue coverage, members already on Inflectra™ or Renflexis™ will be required to change therapy to Remicade® unless they meet the criteria in

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Clinical Policy Updates

Policy Title Effective Date Summary of Changes Coverage Rationale REVISED Infliximab Jun. 1, 2018 this section. (Remicade®, Inflectra™, Preferred Product Criteria Renflexis™) Treatment with Inflectra™ (infliximab-dyyb), Renflexis™ (continued) (infliximab-abda) or other infliximab biosimilar is medically necessary for the indications specified in this policy when the following criteria are met: o Both of the following: . One of the following: - Both of the following: o History of a trial of at least 14 weeks of Remicade resulting in minimal clinical response to therapy and residual disease activity. o Physician attests that in their clinical opinion the clinical response would be expected to be superior with Inflectra or other infliximab biosimilar product, than experienced with Remicade. or - Both of the following: o History of intolerance or adverse event to Remicade. o Physician attests that in their clinical opinion the same intolerance or adverse event would not be expected to occur with Inflectra or other infliximab biosimilar product. and . Both of the following: - Patient has NOT had a loss of a favorable response after established maintenance therapy with Remicade or other infliximab product. - Patient has NOT developed neutralizing antibodies to any infliximab product that has led to an attenuation of efficacy of therapy.

B. Diagnosis-Specific Criteria “Infliximab” will be used to refer to all infliximab products.

Infliximab is proven and medically necessary for the treatment of: o Ankylosing spondylitis when the following criterion is met: . Diagnosis of ankylosing spondylitis (AS).

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Clinical Policy Updates

Policy Title Effective Date Summary of Changes Coverage Rationale REVISED Infliximab Jun. 1, 2018 o Crohn’s disease when the following criterion is met: (Remicade®, . One of the following: Inflectra™, - Diagnosis of fistulizing Crohn’s disease (Crohn’s Disease Renflexis™) Activity Index (CDAI) ≥ 220 and ≤ 400); or (continued) - Both of the following: o Diagnosis of moderately to severely active Crohn’s disease; and o History of failure, contraindication, or intolerance to at least one conventional therapy (e.g., corticosteroids, 6- mercaptopurine, azathioprine, methotrexate, etc.). o Noninfectious uveitis when BOTH of the following criteria are met: . Diagnosis of refractory noninfectious uveitis that is causing or threatening vision loss (e.g., noninfectious uveitis associated with Behçet’s or Reiter’s syndromes); and . History of failure, contraindication, or intolerance to ALL of the following: - Topical corticosteroids; - Systemic corticosteroids; - Immunosuppressive drugs (e.g., azathioprine, cyclosporine, or methotrexate). o Plaque psoriasis when BOTH of the following criteria are met: . Diagnosis of chronic severe plaque psoriasis i.e., extensive and/or disabling); and . Patient is a candidate for systemic therapy. o Psoriatic arthritis when the following criterion is met: . Diagnosis of psoriatic arthritis (PsA). o Rheumatoid arthritis when BOTH of the following criteria are met: . Diagnosis of moderately to severely active rheumatoid arthritis (RA); and . One of the following: - Member is receiving concurrent therapy with methotrexate; - History of contraindication or intolerance to methotrexate. o Sarcoidosis when ALL of the following criteria are met: . Diagnosis of sarcoidosis; and . History of failure, contraindication, or intolerance to corticosteroids (e.g., prednisone, methylprednisolone); and . History of failure, contraindication, or intolerance to one

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Clinical Policy Updates

Policy Title Effective Date Summary of Changes Coverage Rationale REVISED Infliximab Jun. 1, 2018 immunosuppressant (e.g., methotrexate, cyclophosphamide, (Remicade®, azathioprine). Inflectra™, o Ulcerative colitis when BOTH of the following criteria are met: Renflexis™) . Diagnosis of moderately to severely active ulcerative colitis (UC); (continued) and . History of failure, contraindication, or intolerance to at least one conventional therapy e.g., 6-mercaptopurine, aminosalicylate, azathioprine, corticosteroids.

There may be other conditions that qualify as serious, rare diseases for which the use of infliximab may be appropriate. Please refer to the Benefit Considerations section of the policy for additional information.

Infliximab is unproven and not medically necessary in the treatment of: o Still’s disease o Sjogren’s syndrome o Graft-vs-host disease o Myelodysplastic syndromes o Undifferentiated spondyloarthropathy o Reiter’s syndrome o Hidradenitis suppurativa o Wegener’s granulomatosis o Juvenile idiopathic arthritis (juvenile rheumatoid arthritis)

Infliximab is unproven and not medically necessary for the treatment of the above conditions because statistically robust randomized controlled trials are needed to address the issue of whether Infliximab has sufficient superiority in clinical efficacy compared to other available treatments to justify the inherent clinical risk in the use of a monoclonal antibody anti- tumor necrosis factor agent.

Ocrevus™ Jun. 1, 2018  Updated list of related policies; Please refer to the policy titled Injectable Chemotherapy Drugs: Application (Ocrelizumab) added reference link to the of NCCN Clinical Practice Guidelines for updated information based upon the policy titled Specialty Medication National Comprehensive Cancer Network (NCCN) Drugs & Biologics Administration - Site of Care Compendium® (NCCN Compendium®) for oncology indications. Review Guidelines  Revised conditions of Ocrevus (ocrelizumab) is proven and medically necessary for: coverage/precertification  Primary Progressive Multiple Sclerosis requirements; added language Ocrevus is medically necessary for the treatment of primary

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Clinical Policy Updates

Policy Title Effective Date Summary of Changes Coverage Rationale REVISED Ocrevus™ Jun. 1, 2018 to indicate: progressive multiple sclerosis (PPMS) when ALL of the following (Ocrelizumab) o Requests for hospital criteria are met: (continued) outpatient facility infusion of o Diagnosis of primary progressive multiple sclerosis (PPMS); and Ocrevus require additional o One of the following: precertification with review . Initial therapy for ocrelizumab when meeting both of the by a Medical Director or their following: designee; refer to the policy - Patient is not receiving ocrelizumab in combination with any titled Specialty Medication of the following: Administration - Site of Care  Disease modifying therapy (e.g., interferon beta Review Guidelines preparations, daclizumab, dimethyl fumarate, glatiramer acetate, natalizumab, fingolimod, or teriflunomide)  B cell targeted therapy (e.g., rituximab, belimumab, ofatumumab)  Lymphocyte trafficking blockers (e.g., alemtuzumab, mitoxantrone) and - Initial dosing: One time 300 mg intravenous course of doses on days 1 and 15. or . Continuation therapy for ocrelizumab when meeting all of the following: - Patient has previously received treatment with ocrelizumab; and - Documentation of positive clinical response to ocrelizumab therapy; and - Patient is not receiving ocrelizumab in combination with any of the following:  Disease modifying therapy (e.g., interferon beta preparations, daclizumab, dimethyl fumarate, glatiramer acetate, natalizumab, fingolimod, or teriflunomide)  B cell targeted therapy (e.g., rituximab, belimumab, ofatumumab)  Lymphocyte trafficking blockers (e.g., alemtuzumab, mitoxantrone) and . Continued dosing: One 600 mg intravenous dose every 6 months.  Relapsing Forms of Multiple Sclerosis Ocrevus is medically necessary for the treatment of relapsing

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Clinical Policy Updates

Policy Title Effective Date Summary of Changes Coverage Rationale REVISED Ocrevus™ Jun. 1, 2018 forms of multiple sclerosis (MS) when BOTH of the following (Ocrelizumab) criteria are met: (continued) o Diagnosis of relapsing forms of multiple sclerosis (MS) (e.g., relapsing-remitting MS, secondary-progressive MS with relapses, progressive-relapsing MS with relapses); and o One of the following: . Initial therapy for ocrelizumab meeting all of the following: - Patient has history of failure following a trial for at least 4 weeks or history of intolerance or contraindication to one of the following:  interferon β-1a (Avonex®, Rebif®, Plegridy™)  interferon β-1b (Betaseron® or Extavia®)  glatiramer acetate (Copaxone®)  dimethyl fumarate (Tecfidera®)  teriflunomide (Aubagio®)  fingolimod (Gilenya®)  alemtuzumab (Lemtrada®)  natalizumab (Tysabri®)  daclizumab (Zinbryta™) and - Patient is not receiving ocrelizumab in combination with any of the following:  Disease modifying therapy (e.g., interferon beta preparations, daclizumab, glatiramer acetate, natalizumab, fingolimod, or teriflunomide)  B cell targeted therapy (e.g., rituximab, belimumab, ofatumumab)  Lymphocyte trafficking blockers (e.g., alemtuzumab, mitoxantrone) and - Initial dosing: One time 300 mg intravenous course of doses on days 1 and 15. or . Continuation therapy for ocrelizumab when meeting all of the following: - Patient has previously received treatment with ocrelizumab; and - Documentation of positive clinical response to ocrelizumab therapy; and

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Clinical Policy Updates

Policy Title Effective Date Summary of Changes Coverage Rationale REVISED Ocrevus™ Jun. 1, 2018 - Patient is not receiving ocrelizumab in combination with any (Ocrelizumab) of the following: (continued)  Disease modifying therapy (e.g., interferon beta preparations, daclizumab, dimethyl fumarate, glatiramer acetate, natalizumab, fingolimod, or teriflunomide)  B cell targeted therapy (e.g., rituximab, belimumab, ofatumumab)  Lymphocyte trafficking blockers (e.g., alemtuzumab, mitoxantrone) and - Continued dosing: One 600 mg intravenous dose every 6 months.

Ocrevus is unproven and not medically necessary for the treatment of:  Lupus nephritis  Rheumatoid arthritis  Systemic lupus erythematosus

Orencia® Jun. 1, 2018  Revised conditions of This policy refers to Orencia (abatacept) injection for intravenous infusion. (Abatacept) coverage/precertification Injection for requirements; added language Orencia is proven and medically necessary for the treatment of: Intravenous to indicate:  Polyarticular juvenile idiopathic arthritis when all of the following Infusion o Requests for hospital criteria are met: outpatient facility infusion of o Diagnosis of moderately to severely active polyarticular juvenile Orencia require additional idiopathic arthritis (PJIA); and precertification with review o Orencia is initiated and titrated according to US Food and Drug by a Medical Director or their Administration labeled dosing for polyarticular juvenile idiopathic designee; refer to the policy arthritis up to a maximum of (or equivalent dose and interval titled Specialty Medication schedule): Administration - Site of Care . 10mg/kg every 4 weeks for patients weighing <75kg Review Guidelines . 1,000mg every 4 weeks for patients weighing ≥75kg and o Member is not receiving Orencia in combination with either of the following: . Biologic disease-modifying antirheumatic drug (DMARD) [e.g., Enbrel (etanercept), Humira (adalimumab), Cimzia (certolizumab), Simponi (golimumab)] . Janus kinase inhibitor [e.g., Xeljanz (tofacitinib)]  Rheumatoid arthritis when all of the following criteria are met:

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Clinical Policy Updates

Policy Title Effective Date Summary of Changes Coverage Rationale REVISED Orencia® Jun. 1, 2018 o Diagnosis of moderately to severely active rheumatoid arthritis; and (Abatacept) o Orencia is initiated and titrated according to US Food and Drug Injection for Administration labeled dosing for rheumatoid arthritis up to a Intravenous maximum of (or equivalent dose and interval schedule): Infusion . 500mg every 4 weeks for patients weighing <60kg (continued) . 750mg every 4 weeks for patients weighing 60kg to 100kg . 1,000mg every 4 weeks for patients weighing >100kg and o Member is not receiving Orencia in combination with either of the following: . Biologic DMARD [e.g., Enbrel (etanercept), Humira (adalimumab), Cimzia (certolizumab), Simponi (golimumab)] . Janus kinase inhibitor [e.g., Xeljanz (tofacitinib)]  Psoriatic arthritis when all of the following criteria are met: o Diagnosis of active psoriatic arthritis (PsA); and o Orencia is initiated and titrated according to US Food and Drug Administration labeled dosing for psoriatic arthritis up to a maximum of (or equivalent dose and interval schedule): . 500mg every 4 weeks for patients weighing <60kg . 750mg every 4 weeks for patients weighing 60kg to 100kg . 1,000mg every 4 weeks for patients weighing >100kg and o Patient is not receiving Orencia in combination with any of the following: . Biologic DMARD [e.g., Enbrel (etanercept), Humira (adalimumab), Cimzia (certolizumab), Simponi (golimumab)] . Janus kinase inhibitor [e.g., Xeljanz (tofacitinib)] . Phosphodiesterase 4 (PDE4) inhibitor [e.g., Otezla (apremilast)]

Orencia is unproven and not medically necessary for the treatment of:  Multiple sclerosis  Systemic lupus erythematosus  Graft versus host disease (GVHD)  Uveitis associated with Behçet's disease

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Clinical Policy Updates

Policy Title Effective Date Summary of Changes Coverage Rationale REVISED Preventive Care Jun. 1, 2018  Revised coverage rationale for Refer to the policy for complete details on the coverage guidelines for Services Women’s Health; added Preventive Care Services. language to indicate the following services are covered under the Preventive Care Services benefit effective Jun. 1, 2018: o Screening for diabetes mellitus for those with a history of gestational diabetes o Screening for urinary continence, annually  Revised list of applicable procedure and diagnosis codes for: Preventive Care Services Diabetes Screening o Updated service description; added instruction to refer to the Screening for Diabetes Mellitus After Pregnancy section of policy for information on additional diabetes screening benefits Gestational Diabetes Mellitus

Screening

o Updated service description;

added instruction to refer to

the Screening for Gestational Diabetes Mellitus and Screening for Diabetes Mellitus After Pregnancy

sections of policy for

information on additional

diabetes screening benefits

Wellness Examinations

o Updated service

description/language

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Clinical Policy Updates

Policy Title Effective Date Summary of Changes Coverage Rationale REVISED Preventive Care Jun. 1, 2018 pertaining to Health Services Resources and Services (continued) Administration (HRSA) coverage requirements; added “screening for urinary incontinence” to list of services included in codes for wellness examinations Expanded Women’s Preventive Health Screening for Gestational Diabetes Mellitus o Updated service description; added instruction to refer to the Screening for Diabetes Mellitus After Pregnancy section of policy for additional information Screening for Diabetes Mellitus After Pregnancy (new to policy)

o Added service description to

indicate:

. The Women’s Preventive Services Initiative [HRSA Requirement (Dec. 2017)] recommends:

• Women with a

history of gestational

diabetes mellitus

(GDM) who are not currently pregnant and who have not previously been

diagnosed with type

2 diabetes mellitus

should be screened for diabetes mellitus • Initial testing should ideally occur within

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Clinical Policy Updates

Policy Title Effective Date Summary of Changes Coverage Rationale REVISED Preventive Care Jun. 1, 2018 the first year Services postpartum and can (continued) be conducted as early as 4-6 weeks postpartum • Women with a negative initial postpartum screening test result should be rescreened at least every 3 years for a minimum of 10 years after pregnancy . See the Gestational Diabetes Mellitus Screening, Diabetes Screening, and Screening for Gestational Diabetes Mellitus sections of policy for additional information o Added list of applicable CPT codes: 36415, 36416, 82947, 82948, 82950, 82951, 82952, and 83036 o Added list of applicable ICD- 10 diagnosis codes: . Z00.00, Z00.01, or Z13.1; and . Z86.32 o Added preventive benefit instructions to indicate: . The service is payable when the listed diagnosis code requirements are met . No benefit age limit applies

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Clinical Policy Updates

Policy Title Effective Date Summary of Changes Coverage Rationale REVISED Preventive Care Jun. 1, 2018 . CPT codes 36415 and Services 36416 are payable when (continued) billed with all of the following: • One of the [listed/required] diabetes screening procedure codes; and • The [listed/required] diagnosis codes . If a diabetes diagnosis code is present in any position, the preventive benefit will not be applied; see the list of applicable diabetes diagnosis codes Screening for Urinary Incontinence (new to policy) o Added service description to indicate the Women’s Preventive Services Initiative recommends screening women for urinary incontinence annually o Added instruction to see the Wellness Examinations section of the policy for applicable codes and preventive benefit instructions  Updated supporting information to reflect the most current references

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Clinical Policy Updates

Policy Title Effective Date Summary of Changes Coverage Rationale REVISED Radicava™ Jun. 1, 2018  Updated list of related policies; Radicava (edaravone) is proven and medically necessary for the (Edaravone) added reference link to the treatment of amyotrophic lateral sclerosis (ALS) in patients who policy titled Specialty Medication meet all of the following criteria: Administration - Site of Care  For initial therapy, all of the following: Review Guidelines o Submission of medical records (e.g., chart notes, previous medical  Revised conditions of history, diagnostic testing including: imaging, nerve conduction coverage/precertification studies, laboratory values) to support the following: requirements; added language Diagnosis of “definite” or “probable” ALS per the EL Escorial / revised to indicate: Airlie House diagnostic criteria, and prescribed by, or in consultation o Requests for hospital with, a neurologist with expertise in the diagnosis of ALS; and outpatient facility infusion of o Submission of the most recent ALS Functional Rating Scale-Revised Radicava require additional (ALSFRS-R) score confirming that the patient has scores ≥ 2 in all precertification with review items of the ALSFRS-R criteria at the start of treatment; and by a Medical Director or their o Submission of medical records (e.g., chart notes, laboratory values) designee; refer to the policy confirming that the patient has a % forced vital capacity (%FVC) ≥ titled Specialty Medication 80% at the start of treatment; and Administration - Site of Care o Radicava dosing for ALS is in accordance with the United States Food Review Guidelines and Drug Administration approved labeling; and  Updated coverage rationale; o Initial authorization will be for no more than 6 cycles (64 doses over reformatted/clarified coverage 168 days). criterion addressing applicable  For continuation therapy, all of the following: diagnosis and treating physician o Diagnosis of “definite” or “probable” ALS per the revised EL Escorial and Airlie House diagnostic criteria, and prescribed by, or in consultation with, a neurologist with expertise in the diagnosis of ALS; and o Patient is currently receiving Radicava therapy; and o Patient is not dependent on invasive ventilation or tracheostomy; and o Radicava dosing for ALS is in accordance with the United States Food and Drug Administration approved labeling; and o Authorization will be for no more than 6 cycles (60 doses over 168 days).

Radiology Jun. 1, 2018  Reformatted and revised the Refer to the policy for complete details on Radiology Procedures Requiring Procedures Oxford Radiology Prior Precertification for eviCore healthcare Arrangement. Requiring Notification/Authorization Precertification for Crosswalk Table: eviCore healthcare o Transferred content to Arrangement embedded Excel file format o Added language to indicate

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Clinical Policy Updates

Policy Title Effective Date Summary of Changes Coverage Rationale REVISED Radiology Jun. 1, 2018 precertification given with Procedures CPT code 74177 will be Requiring allowed for claims submitted Precertification for with CPT codes 72194 and eviCore healthcare 74170 Arrangement (continued)

Simponi Aria® Jun. 1, 2018  Revised conditions of This policy refers only to Simponi Aria (golimumab) injection for intravenous (Golimumab) coverage/precertification infusion for the treatment of ankylosing spondylitis, psoriatic arthritis, and Injection for requirements; added language rheumatoid arthritis. Simponi, for self-administered subcutaneous injection, Intravenous to indicate: is obtained under the pharmacy benefit and is indicated in the treatment of Infusion o Requests for hospital rheumatoid arthritis, psoriatic arthritis, ankylosing spondylitis and ulcerative outpatient facility infusion of colitis. Simponi-Aria require additional precertification Simponi Aria is proven and/or medically necessary for the treatment with review by a Medical of: Director or their designee;  Ankylosing spondylitis when all of the following criteria are met: refer to the policy titled o Diagnosis of active ankylosing spondylitis (AS); and Specialty Medication o Simponi Aria is initiated and titrated according to US Food and Drug Administration - Site of Care Administration labeled dosing for ankylosing spondylitis, up to a Review Guidelines maximum of 2mg/kg every 8 weeks (or equivalent dose and interval schedule); and o Patient is not receiving Simponi Aria in combination with either of the following: . Biologic disease-modifying antirheumatic drug (DMARD) [e.g., Enbrel (etanercept), Humira (adalimumab), Cimzia (certolizumab), Orencia (abatacept)] . Janus kinase inhibitor [e.g., Xeljanz (tofacitinib)]  Psoriatic arthritis when all of the following criteria are met: o Diagnosis of active psoriatic arthritis (PsA); and o Simponi Aria is initiated and titrated according to US Food and Drug Administration labeled dosing for psoriatic arthritis up to a maximum of 2mg/kg every 8 weeks (or equivalent dose and interval schedule); and o Patient is not receiving Simponi Aria in combination with either of the following: . Biologic disease-modifying antirheumatic drug (DMARD) [e.g., Enbrel (etanercept), Humira (adalimumab), Cimzia (certolizumab), Orencia (abatacept)]

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Clinical Policy Updates

Policy Title Effective Date Summary of Changes Coverage Rationale REVISED Simponi Aria® Jun. 1, 2018 . Janus kinase inhibitor [e.g., Xeljanz (tofacitinib)] (Golimumab) . Phosphodiesterase 4 (PDE4) inhibitor [e.g., Otezla (apremilast)] Injection for  Rheumatoid arthritis when all of the following criteria are met: Intravenous o Diagnosis of moderately to severely active rheumatoid arthritis (RA); Infusion and (continued) o One of the following: . Patient is receiving concurrent therapy with methotrexate . History of contraindication or intolerance to methotrexate and o Simponi Aria is initiated and titrated according to US Food and Drug Administration labeled dosing for rheumatoid arthritis up to a maximum of 2mg/kg every 8 weeks (or equivalent dose and interval schedule); and o Patient is not receiving Simponi Aria in combination with either of the following: . Biologic disease-modifying antirheumatic drug (DMARD) [e.g., Enbrel (etanercept), Humira (adalimumab), Cimzia (certolizumab), Orencia (abatacept)] . Janus kinase inhibitor [e.g., Xeljanz (tofacitinib)]

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Administrative Policy Updates

Policy Title Effective Date Administrative Guidelines NEW New York & Jun. 1, 2018 Notice of Revision: The following Administrative Policy has been modified; revisions to the original policy update Connecticut announcement are outlined in red below. Please take note of the amended guidelines to be applied beginning Jun. Participating 1, 2018. Surgeons Using Non- Participating NY and CT Participating Providers Using Non-Participating IONM Providers Providers for The following procedures and responsibilities apply to Participating Providers located in NY and CT when providing Intraoperative services to members enrolled on NY and/or CT products in an outpatient or inpatient facility setting that involve Neuro-Monitoring intraoperative neuro-monitoring (IONM). (IONM) Services performed by a Participating Provider located in NY or CT and meeting the following criteria must ensure that the IONM provider utlized is participating with the Oxford network.  Member is enrolled on a NY or CT product; and  Service is being provided in an outpatient or inpatient facility setting; and  Involve intraoperative neuro-monitoring (IONM).

Outpatient and Inpatient Facility Place Codes

Place Code Description 15 Mobile diagnostic unit 19 Off campus - outpatient hospital

21 Inpatient Hospital

22 On campus - outpatient hospital 24 Ambulatory surgical center 99 Other unlisted factilty

If the Participating Provider intends to utilize an IONM provider that does not participate in the Oxford network, the

provider is required to:

 Verbally discuss options and financial impact with the Member

o The Participating Provider must review this policy and the Non-Participating Provider Consent Form with the

Member.

. The discussion must explain Participating and Non-Participating IONM Provider alternatives and provide

the Member with an understanding of all the providers involved in the Member’s care.

. The discussion must include a conversation explaining the financial impact of using a Non-Participating

IONM Provider.

. A copy of the Non-Participating Provider Consent Form must be provided to the Member.

o The discussion must occur no more than 90 days, and no less than 14 days before, the scheduled date of the

procedure.

o If the Member does not sign the form at the end of the discussion, explain that it needs to be completed and

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Administrative Policy Updates

Policy Title Effective Date Administrative Guidelines NEW New York & Jun. 1, 2018 returned no less than 14 days before the scheduled date of the procedure. Connecticut o The discussion must then be noted in the Member’s medical record. Participating  Obtain a completed Non-Participating Provider Consent Form Surgeons Using o The member will need to agree or disagree to receive IONM services from a Non-Participating Provider by Non- Participating marking the appropriate box on the Non-Participating Provider Consent Form. The member must then sign Providers for and date the form and return the form to the Participating Provider no less than 14 days before the Intraoperative scheduled date of the procedure. If the Member: Neuro-Monitoring . Does not agree to the use of a Non-Participating IONM Provider: Following the discussion, if the (IONM) Participating Provider: (continued) - Is unable to locate a Participating IONM Provider, they must contact the health plan for assistance in locating a Participating IONM Provider. - Still wants to recommend the Non-Participating IONM Provider, they must contact Oxford to request and initiate an In-Network Exception request. . Does agree to the use of a Non-Participating IONM Provider: The Participating Provider must ensure that the Member understands the financial obligations of using a Non-Participating IONM Provider. - For Members with out-of-network benefits: Non-Participating IONM Providers will be paid at the out-of-network benefit level. Out-of-network cost shares and deductibles will apply. In addition, Members may be responsible to the Non-Participating IONM Provider for any amount above the amount paid by the health plan, as determined by the Member’s out-of-network benefit; or - For Members with only in-network benefits: Non-Participating IONM Provider claims will be denied because the Member has no coverage for services provided by Non-Participating Providers. Members will therefore be responsible for the entire cost of the service(s). o The Participating Provider must then sign and date the form to acknowledge the Member’s decision. o The Non-Participating Provider Consent Form must be kept on file by the Participating Provider. o A separate Non-Participating Provider Consent Form is required for every service when the Participating Provider wants to refer to or involve a Non-Participating IONM Provider in a member’s care. o The Non-Participating Provider Consent Form will only be valid for 90 days from the date of member signature. o Oxford may request a copy of the completed Non-Participating Provider Consent Form from the Participating Provider (who is required to keep the form on file) in order to conduct standard business. . When requested, the Participating Provider must provide a copy of the Non-Participating Provider Consent Form within 15 days of the request. . If a copy of the completed Non-Participating Provider Consent Form is not received within 15 days of the request, the Participating Provider’s claim will be denied administratively for failure to comply with this protocol. . In these instances, the Participating Provider is prohibited from balance billing the Member. . Any payment previously made for the surgical service will be subject to recovery. The Participating provider cannot balance bill the member for claims denied for administrative reasons.

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Administrative Policy Updates

Policy Title Effective Date Administrative Guidelines NEW New York & Jun. 1, 2018 Participating IONM Providers Connecticut When a Participating Provider performs services in an outpatient or inpatient facility setting using a Participating Participating IONM Provider, there will be no additional requirements to fulfill. A Non-Participating Provider Consent Form is not Surgeons Using required. Non- Participating Providers for Non-Compliance With This Policy Intraoperative Oxford may request a copy of the completed Non-Participating Provider Consent Form from the Participating Provider Neuro-Monitoring (who is required to keep the form on file) in order to conduct standard business. When requested: (IONM)  The Participating Provider must provide a copy of the Non-Participating Provider Consent Form within 15 days of (continued) the request.  If a copy of the completed Non-Participating Provider Consent Form is not received within 15 days of the request, as proof that they discussed the member’s options for selecting a Participating or Non-Participating IONM Provider, in advance of the service, the Participating Provider’s claim will be denied administratively for failure to comply with the protocol.  In these instances, the Participating Provider is prohibited from balance billing the member. Any payment previously made for the service will be subject to recovery. The Participating Provider cannot balance bill the member for claims denied for administrative reasons.

In-Network Exception Requests If requesting an In-Network Exception to have a Non-Participating IONM Provider covered as if they were participating with the Oxford network, the Participating Provider must make the exception request. The exception request will not be accepted from the Non-Participating IONM Provider.  The In-Network Exception request must be made no less than 14 days in advance of the scheduled procedure in order to avoid delays in care and alleviate potential complications with the patient’s required preparations for the procedure.  If the Participating Provider requests an In-Network Exception less than 14 days in advance of the scheduled procedure, the In-Network Exception request will be processed per Oxford’s standard guidelines, however the Participating Provider will receive an administrative denial for their claim for failure to follow protocol.

Policy Title Effective Date Summary of Changes UPDATED Accreditation May 1, 2018  Updated policy guidelines: Requirements for o Modified list of modalities/procedures for AIUM accreditation; removed echocardiography (previously listed in Radiology Services error; no change to accreditation guidelines) o Updated reference link to eviCore healthcare Accreditation Fax Cover Sheet; redirected from “Prior Authorization and Notification App” to “eviCore healthcare website”

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Administrative Policy Updates

Policy Title Effective Date Summary of Changes Administrative Guidelines REVISED Ambulance Jun. 1, 2018  Changed policy title; previously Note: Refer to Oxford’s Ambulance policy for additional information Services titled Transportation Services regarding the reimbursement of ambulance transportation services.  Revised conditions of coverage/precertification Indications for Coverage guidelines; added language to Emergency Ambulance (Ground, Water, or Air) indicate: Coverage includes Emergency ambulance transportation (including wait time o Precertification with review and treatment at the scene) by a licensed ambulance service from the by a Medical Director or their location of the sudden illness or injury, to the nearest hospital where services designee is required for all can be performed. requests for out-of-the- country transportation Emergency transportation to an acute care hospital and/or hospital o In the event precertification Emergency facility does not require notification, precertification or for air or water certification. transportation is not feasible due to time constraints The following Emergency ambulance services are covered: related to medical  Ground ambulance transportation requiring basic life support or emergencies, Oxford will advanced life support require review of clinical  Treatment at the scene (paramedic services) without ambulance notes post-service and prior transportation to payment  Wait time associated with covered ambulance transportation  Revised coverage rationale:  To a hospital that provides a required higher level of care that was not o Replaced references to: available at the original hospital . “Patient” with “member” . “Transportation” with Air Ambulance

“ambulance” As a general guideline, when it would take a ground ambulance 30-60

Emergency Ambulance minutes or more to transport a member whose medical condition at the time

(Ground, Water, or Air) of pick-up required immediate and rapid transport due to the nature and/or

o Added language to indicate severity of the member’s illness/injury, air transportation may be

the following Emergency appropriate.

ambulance services are

covered: Air Ambulance transportation should meet the following criteria:

. Ground ambulance  The member’s destination is an acute care hospital, and

transportation requiring  The member’s condition is such that the ground ambulance (basic or

basic life support or advanced life support) would endanger the member’s life or health, or

advanced life support  Inaccessibility to ground ambulance transport or extended length of time

. Treatment at the scene required to transport the member via ground ambulance transportation

(paramedic services) could endanger the member, or

without ambulance  Weather or traffic conditions make ground ambulance transportation

transportation impractical, impossible, or overly time consuming.

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Administrative Policy Updates

Policy Title Effective Date Summary of Changes Administrative Guidelines REVISED

Ambulance Jun. 1, 2018 . Wait time associated Additional Information Services with covered ambulance Emergency ambulance coverage includes supplies that are needed for (continued) transportation advanced life support or basic life support to stabilize a member’s medical . To a hospital that condition. provides a required

higher level of care that Non-Emergency Ambulance (Ground or Air) was not available at the original hospital Coverage includes Non-Emergency ambulance transportation by a licensed o Removed duplicative ambulance service (either ground or Air Ambulance), between health care language pertaining to facilities only when the transport meets one of the following: precertification requirements  From an out-of-network hospital or facility to the closest Network (see the Conditions of hospital when Covered Health Care Services are required. Coverage section of the  To the closest Network hospital or facility that provides the required policy) Covered Health Care Services that were not available at the original Non-Emergency Ambulance hospital (Ground or Air)  From a Short-Term Acute Care Facility to the closest Network Long-Term o Modified language to indicate Acute Care Facility (LTAC), Network Inpatient Rehabilitation Facility, or coverage includes non- other Network Sub-Acute facility where the required Covered Health Care Emergency ambulance Services can be delivered. transportation by a licensed ambulance service (either Additional Information ground or air ambulance, as  Non-emergent transportation and is covered only when the member’s we determine appropriate) specific benefit document includes coverage for non-emergent between facilities only when ambulance/transportation and/or coverage is required due to federal or the transport meets one of state mandates. the following:  Ambulance transportation that is done for convenience of the patient is . From an out-of-Network not covered. Please see the Coverage Limitations and Exclusions section Hospital to the closest of the policy for more information on non-covered ambulance Network Hospital when transportation. Covered Health Care Services are required Coverage Limitations and Exclusions . To the closest Network The following services are not eligible for coverage: Hospital or facility that  Ambulance services from providers that are not properly licensed to be provides the required performing the ambulance services rendered. Covered Health Care  Air Ambulance transportation that does not meet the covered indications Services that were not in the Air Ambulance criteria listed above. available at the original  Non-ambulance transportation. Non-ambulance transportation is not Hospital or facility covered even if rendered in an Emergency situation. Examples include . From a Short-Term but are not limited to:

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Administrative Policy Updates

Policy Title Effective Date Summary of Changes Administrative Guidelines REVISED Ambulance Jun. 1, 2018 Acute Care Facility to the o Commercial or private airline or helicopter Services closest Network Long- o A police car ride to a hospital (continued) Term Acute Care Facility o Medi-van or wheel chair van transportation (LTAC), Network o Taxi ride, bus ride, etc. Inpatient Rehabilitation  Ambulance transportation when other mode of transportation is Facility, or other Network appropriate. Except as indicated under the Indications for Coverage Sub-Acute Facility where section of the policy, ambulance services when transportation by other the required Covered means would not endanger the member’s health are not covered. Health Care Services can  Ambulance transportation to a home, residential, domiciliary or custodial be delivered facility is not covered. o Added reference link to the  Ambulance transportation for member convenience or other Coverage Limitations and miscellaneous reasons for member and/or family. Examples include but Exclusions section of the are not limited to: policy for additional o Member wants to be at a certain hospital or facility for information on non-covered personal/preference reasons ambulance transportation o Member is in foreign country, or out of state, and wants to come Coverage Limitations and home for a surgical procedure or treatment (this includes those Exclusions recently discharged from inpatient care) o Replaced reference to “air o Member is going for routine service and is medically able to use ambulance” with “air another mode of transportation ambulance transportation” o Member is deceased and family wants transportation to the coroner’s o Removed language office or mortuary indicating ambulance  Ambulance transportation deemed not appropriate. Examples include but transportation that violates are not limited to: the notification criteria listed o Hospital to home in the Indications for o Home to physician’s office Coverage section of the o Home (e.g., residence, nursing home, domiciliary or custodial policy is not covered facility) to a hospital for a scheduled service o Modified list of examples of excluded ambulance Out-of-Country Transportation transportation for member When a member has traveled outside of the United States, Mexico, Canada convenience or other and the U.S. Territories, Emergency transportation to the nearest hospital miscellaneous reasons to and/or hospital Emergency facility does not require notification, reflect/include: precertification or certification. However, Oxford should be notified of an . Member wants to be at a admission within 48 hours or as soon as possible, consistent with the certain hospital or facility member's certificate. for personal/ preference reasons Refer to the policy titled Emergency Room Visits (Including Coverage for . Member is in foreign Members Outside of the United States for additional information on coverage

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Policy Title Effective Date Summary of Changes Administrative Guidelines REVISED Ambulance Jun. 1, 2018 country, or out of state, for services received outside of the United States, Mexico, Canada, and the Services and wants to come home U.S. Territories. (continued) for a surgical procedure or treatment (this includes those recently discharged from inpatient care) . Member is going for a routine service and is medically able to use another mode of transportation . Member is deceased and family wants transportation to the coroner’s office or mortuary Out-of-Country Transportation o Removed duplicative language pertaining to precertification requirements (see the Conditions of Coverage section of the policy)  Updated definitions: o Added definition of “Emergency” o Removed definition of: . Air Ambulance . Fixed Wing Aircraft . Rotary Wing Aircraft  Updated list of applicable HCPCS codes; revised description for A0427

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Reimbursement Policy Updates

Policy Title Effective Date Summary of Changes UPDATED Maximum May 7, 2018  Updated Maximum Frequency Per Day Code List (attachment file designating the maximum frequency per day Frequency Per Day value assignments for CDT, CPT, and HCPCS codes) to reflect quarterly code edits: o Added 0012M, 0013M, 0035U, 0036U, 0037U, 0038U, 0039U, 0040U, 0041U, 0042U, 0043U, 0044U, 0492T, 34711, 64913, 86008, 99494, A4225, A9515, A9587, E0953, E0954, J1428, J1555, J1726, J2182, J2326, J2350, J2786, K0903, Q2041, Q5103, and Q5104 o Revised value assignments for 0394T, 0395T, 0398T, 0450T, 22854, 22859, 23101, 64636, 74713, 93592, 95886, A0384, A0392, A0396, A0422, A4224, A4337, A5200, A9575, B4081, B4082, B4083, B4164, B4172, B4176, B4178, B4180, H0003, J0638, J1575, and J3060  Updated list of Codes Restricting Modifiers LT and RT (attachment file listing codes that allow up to the MFD limit with "bilateral" or "unilateral or bilateral" in the description or where the concept of laterality does not apply) to reflect quarterly code edits; added 0012M, 0013M, 0035U, 0036U, 0037U, 0038U, 0039U, 0040U, 0041U, 0042U, 0043U, 0044U, Q2041, Q5103, and Q5104

Services and May 7, 2018  Updated list of Status E and X Codes to reflect quarterly code edits; added G9873, G9874, G9875, G9876, Modifiers Not G9877, G9878, G9879, G9880, G9881, G9882, G9883, G9884, G9885, G9890, and G9891 Reimbursable to Healthcare Professionals

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