UnitedHealthcare Community Plan of Kentucky

Medical Policy Update Bulletin: June 2021

In This Issue

Medical Policy Updates Page Updated • Pharmacogenetic Testing – Effective Jun. 1, 2021 ...... 3 Revised • Articular Cartilage Defect Repairs – Effective Jul. 1, 2021 ...... 3 • Cell-Free Fetal DNA Testing – Effective Jul. 1, 2021 ...... 6 • Implanted Electrical Stimulator for Spinal Cord – Effective Jul. 1, 2021 ...... 8 • Lower Extremity Invasive Diagnostic and Endovascular Procedures – Effective Jul. 1, 2021 ...... 9 Replaced/Retired • Femoroacetabular Impingement Syndrome – Effective Jun. 1, 2021 ...... 11 • Otoacoustic Emissions Testing – Effective Jun. 1, 2021 ...... 11 Medical Benefit Drug Policy Updates New • Long-Acting Injectable Antiretroviral Agents for HIV – Effective Jul. 1, 2021 ...... 12 • Oxlumo™ (Lumasiran) – Effective Jul. 1, 2021 ...... 13 Revised • Antiemetics for Oncology – Effective Jul. 1, 2021 ...... 14 • Benlysta® (Belimumab) – Effective Jul. 1, 2021 ...... 21 • Complement Inhibitors (Soliris® & Ultomiris®) – Effective Jul. 1, 2021 ...... 24 • Gonadotropin Releasing Hormone Analogs – Effective Jul. 1, 2021 ...... 30 • Infliximab (Remicade®) – Effective Jul. 1, 2021 ...... 32 • Intravenous Bisphosphonates/ Bone Resorption Inhibitors (Zoledronic Acid & Pamidronate Disodium) – Effective Jul. 1, 2021 ...... 33 • Rituximab (Riabni™, Rituxan®, Ruxience®, & Truxima®) – Effective Jul. 1, 2021 ...... 35

Page 1 of 65 UnitedHealthcare Community Plan of Kentucky Medical Policy Update Bulletin: June 2021

In This Issue

• Spinraza® (Nusinersen) – Effective Jul. 1, 2021 ...... 41 • White Blood Cell Colony Stimulating Factors – Effective Jul. 1, 2021 ...... 45 • Xiaflex® (Collagenase, Clostridium, Histolyticum) – Effective Jul. 1, 2021 ...... 48 • Xolair® (Omalizumab) – Effective Jul. 1, 2021 ...... 50 • Zolgensma® (Onasemnogene Abeparvovec-Xioi) – Effective Jul. 1, 2021 ...... 56 Coverage Determination Guideline Updates Updated • Breast Reconstruction Post Mastectomy and Poland Syndrome – Effective Jun. 1, 2021 ...... 60 • Rhinoplasty and Other Nasal Surgeries – Effective Jun. 1, 2021 ...... 60 Revised • Ambulance Services – Effective Jul. 1, 2021 ...... 60 • Chiropractic Services – Effective Jul. 1, 2021 ...... 64

Page 2 of 65 UnitedHealthcare Community Plan of Kentucky Medical Policy Update Bulletin: June 2021

Medical Policy Updates

Updated Policy Title Effective Date Summary of Changes Pharmacogenetic Jun. 1, 2021 Coverage Rationale

Testing Updated list of examples of unproven and not medically necessary pharmacogenetic Multi-Gene Panels for genetic polymorphisms; removed “NeurolDgenetix”

Revised Policy Title Effective Date Summary of Changes Coverage Rationale Articular Cartilage Jul. 1, 2021 Coverage Rationale Autologous chondrocyte transplantation (ACT) is proven and medically Defect Repairs Revised language to indicate: necessary for treating individuals with a symptomatic full-thickness articular cartilage defect. o Autologous chondrocyte

transplantation (ACT) is proven and medically necessary for ACT is unproven and not medically necessary for treating individuals with the

treating individuals with a following indications due to insufficient evidence of efficacy:

symptomatic full-thickness Treatment of joints other than the knee articular cartilage defect Growth plates have not closed History of partial-thickness defects o ACT is unproven and not medically necessary for Osteochondritis dissecans (OCD) treating individuals with the Malignancy in the bone, cartilage, fat or muscle of the treated limb following indications due to Active infection in the affected knee Instability of the knee insufficient evidence of efficacy: History of total meniscectomy

. Treatment of joints other Repeat ACT

than the knee Active inflammatory degenerative, rheumatoid or osteoarthritis

. Growth plates have not As initial or first line of surgical therapy closed . History of partial-thickness Microfracture repair to treat full and partial thickness chondral defects of the defects knee is proven and medically necessary. . Osteochondritis dissecans (OCD) For medical necessity clinical coverage criteria for ACT and microfracture repair, . Malignancy in the bone, refer to the InterQual® Client Defined 2020, CP: Procedures, Articular Cartilage

cartilage, fat or muscle of Defect Repairs (Custom) - UHG.

the treated limb ® . Active infection in the Click here to view the InterQual criteria. affected knee . Instability of the knee Osteochondral Autograft and Allograft transplantation is proven and

Page 3 of 65 UnitedHealthcare Community Plan of Kentucky Medical Policy Update Bulletin: June 2021

Medical Policy Updates

Revised Policy Title Effective Date Summary of Changes Coverage Rationale Articular Cartilage Jul. 1, 2021 . History of total medically necessary for treating individuals with cartilage defects of the Defect Repairs meniscectomy knee. (continued) . Repeat ACT . Active inflammatory For medical necessity clinical coverage criteria for Osteochondral Autograft and degenerative, rheumatoid Allograft transplantation, refer to the InterQual® 2020, Apr. 2020 Release, CP:

or osteoarthritis Procedures:

. As initial or first line of • Arthroscopy or Arthroscopically Assisted Surgery, Knee

surgical therapy • Arthroscopy or Arthroscopically Assisted Surgery, Knee (Pediatric)

o Microfracture repair is proven • Arthrotomy, Knee and medically necessary to treat full and partial thickness Click here to view the InterQual® criteria. chondral defects of the knee o For medical necessity clinical Focal articular cartilage repair is unproven and not medically necessary for coverage criteria for ACT and treating individuals with any of the following due to insufficient evidence of

microfracture repair, refer to efficacy:

the InterQual® Client Defined Osteochondral Autograft and Allograft transplantation for all other

2020, CP: Procedures, indications than those listed above

Articular Cartilage Defect Use of minced articular cartilage repair (whether synthetic, allograft or Repairs (Custom) - UHG autograft) for treating osteochondral defects of the knee

o Osteochondral Autograft and Use of cryopreserved viable Osteochondral Allograft products (e.g., Allograft transplantation is Cartiform) proven and medically Microfracture repair of the knee with any of the following indications: necessary for treating o Misalignment of the knee individuals with cartilage Osteoarthritis o defects of the knee; for Systemic immune-mediated disease, disease-induced arthritis, or o medical necessity clinical cartilage disease

coverage criteria, refer to the o Unwilling or unable to participate in post-operative physical InterQual® 2020, Apr. 2020 rehabilitation program

Release, CP: Procedures:

. Arthroscopy or

Arthroscopically Assisted

Surgery, Knee . Arthroscopy or

Page 4 of 65 UnitedHealthcare Community Plan of Kentucky Medical Policy Update Bulletin: June 2021

Medical Policy Updates

Revised Policy Title Effective Date Summary of Changes Coverage Rationale Articular Cartilage Jul. 1, 2021 Arthroscopically Assisted Defect Repairs Surgery, Knee (Pediatric) (continued) . Arthrotomy, Knee o Focal articular cartilage repair is unproven and not medically

necessary for treating individuals with any of the

following due to insufficient

evidence of efficacy: . Osteochondral Autograft and Allograft transplantation for all other indications than those listed above

. Use of minced articular

cartilage repair (whether

synthetic, allograft or

autograft) for treating osteochondral defects of the knee . Use of cryopreserved viable Osteochondral

Allograft products (e.g., Cartiform)

. Microfracture repair of the

knee with any of the

following indications:

Misalignment of the knee

Osteoarthritis

Systemic immune- mediated disease, disease-induced

Page 5 of 65 UnitedHealthcare Community Plan of Kentucky Medical Policy Update Bulletin: June 2021

Medical Policy Updates

Revised Policy Title Effective Date Summary of Changes Coverage Rationale Articular Cartilage Jul. 1, 2021 arthritis, or cartilage Defect Repairs disease (continued) Unwilling or unable to participate in post- operative physical rehabilitation program Cell-Free Fetal DNA Jul. 1, 2021 Coverage Rationale DNA-based noninvasive prenatal tests of fetal aneuploidy are proven and Testing Revised list of unproven and not medically necessary as screening tools for trisomy 21 (Down syndrome), medically necessary indications; trisomy 18 (Edwards syndrome) or trisomy 13 (Patau syndrome) for individuals with a singleton pregnancy in any one of the following added “non-amplified or sequenced cell-free DNA-based circumstances:

Maternal age or oocyte age of 35 years or older at delivery; or noninvasive prenatal tests (e.g., Vanadis)” Fetal ultrasound findings indicating an increased risk of aneuploidy; or

Applicable Codes History of a prior pregnancy with a trisomy; or

Added ICD-10 diagnosis codes Positive first- or second-trimester screening test results for aneuploidy; or

O09.00, O09.01, O09.02, O09.03, Parental balanced Robertsonian translocation with an increased risk of fetal O09.10, O09.11, O09.12, O09.13, trisomy 13 or trisomy 21; or O09.211, O09.212, O09.213, Screening after pre-test counseling from a board-certified genetic counselor or from the prenatal care physician or healthcare professional using Shared O09.219, O09.30, O09.31, O09.32, Decision-Making (SDM) O09.33, O09.40, O09.41, O09.42,

O09.43, O09.611, O09.612, O09.613, O09.619, O09.621, Due to insufficient evidence of efficacy, DNA-based noninvasive prenatal

O09.622, O09.623, O09.629, tests are unproven and not medically necessary for any of the following:

O09.70, O09.71, O09.72, O09.73, Conditions including, but not limited to, the following:

O09.811, O09.812, O09.813, o Multiple gestation pregnancies

O09.819, O09.821, O09.822, o Twin zygosity O09.823, O09.829, O09.891, o Repeat testing due to low fetal fraction O09.892, O09.893, O09.899, o Screening for the following: . Aneuploidy other than trisomies 21, 18, or 13 O09.90, O09.91, O09.92, O09.93, . Microdeletions O09.A0, O09.A1, O09.A2, O09.A3, O26.20, O26.21, O26.22, O26.23, . Single gene disorders

O26.841, O26.842, O26.843, . Fetal RhD status

O26.849, O26.851, O26.852, Non-amplified or sequenced cell-free DNA-based noninvasive prenatal tests

Page 6 of 65 UnitedHealthcare Community Plan of Kentucky Medical Policy Update Bulletin: June 2021

Medical Policy Updates

Revised Policy Title Effective Date Summary of Changes Coverage Rationale Cell-Free Fetal DNA Jul. 1, 2021 O26.853, O26.859, O26.891, (e.g., Vanadis) Testing O26.892, O26.893, O26.899, (continued) O26.90, O26.91, O26.92, O26.93, Genetic Counseling O28.0, O28.1, O28.2, O28.4, O28.8, Genetic counseling is strongly recommended prior to fetal screening or prenatal O99.210, O99.211, O99.212, diagnosis in order to inform persons being tested about the advantages and O99.213, O99.280, O99.281, limitations of the test as applied to a unique person. O99.282, O99.283, O99.284,

O99.285, O99.310, O99.311, O99.312, O99.313, O99.320, O99.321, O99.322, O99.323, O99.330, O99.331, O99.332, O99.333, O99.340, O99.341, O99.342, O99.343, O99.810, O99.814, Z34.00, Z34.01, Z34.02,

Z34.03, Z34.80, Z34.81, Z34.82,

Z34.83, Z34.90, Z34.91, Z34.92,

Z34.93, Z36.0, Z36.1, Z36.2, Z36.3, Z36.4, Z36.5, Z36.81, Z36.82, Z36.83, Z36.84, Z36.85, Z36.86, Z36.87, Z36.88, Z36.89, Z36.8A, Z3A.09, Z3A.10, Z3A.11, Z3A.12, Z3A.13, Z3A.14, Z3A.15, Z3A.16, Z3A.17, Z3A.18, Z3A.19, Z3A.20, Z3A.21, Z3A.22, Z3A.23, Z3A.24,

Z3A.25, Z3A.26, Z3A.27, Z3A.28,

Z3A.29, Z3A.30, Z3A.31, Z3A.32, Z3A.33, Z3A.34, Z3A.35, Z3A.36, Z3A.37, Z3A.38, Z3A.39, Z3A.40, Z3A.41, Z3A.42, and Z3A.49 Supporting Information Updated Clinical Evidence and References sections to reflect the most current information

Page 7 of 65 UnitedHealthcare Community Plan of Kentucky Medical Policy Update Bulletin: June 2021

Medical Policy Updates

Revised Policy Title Effective Date Summary of Changes Coverage Rationale Implanted Electrical Jul. 1, 2021 Template Update Implanted electrical stimulators for spinal cord, including high-frequency Stimulator for Spinal Removed CMS section dorsal column stimulators (also known as BurstDR spinal cord stimulators), Cord Coverage Rationale are proven and medically necessary for treating the following indications: Complex regional pain syndrome (CRPS) Replaced language indicating Failed back surgery syndrome “implanted electrical stimulators for

spinal cord, including high- For medical necessity clinical coverage criteria, refer to the InterQual® 2020, Apr. frequency dorsal column 2020 Release, CP: Procedures, Spinal Cord Stimulator (SCS) Insertion. stimulators (also known as BurstDR

spinal cord stimulators), are proven Click here to view the InterQual® criteria. and medically necessary in certain

circumstances” with “implanted Implanted electrical stimulators for spinal cord, are unproven and not medically electrical stimulators for spinal necessary for treating the following indications: cord, including high-frequency Diabetic Neuropathy dorsal column stimulators (also Refractory angina pectoris known as BurstDR spinal cord

stimulators), are proven and Note: medically necessary for treating the Coverage of a replacement battery/generator for a previously implanted [listed] indications” electrical stimulator is appropriate when the individual’s existing Added list of proven and medically battery/generator is malfunctioning, cannot be repaired, and is no longer indications: under warranty. Complex regional pain syndrome For Dorsal Root Ganglion (DRG) stimulation, please refer to the Medical (CRPS) Policy titled Electrical Stimulation for the Treatment of Pain and Muscle Failed back surgery syndrome Rehabilitation (for Kentucky Only). Removed list of services addressed ® in the referenced InterQual criteria Added language to indicate implanted electrical stimulators for

spinal cord are unproven and not

medically necessary for treating the

following indications:

o Diabetic neuropathy Refractory angina pectoris o Removed Documentation

Page 8 of 65 UnitedHealthcare Community Plan of Kentucky Medical Policy Update Bulletin: June 2021

Medical Policy Updates

Revised Policy Title Effective Date Summary of Changes Coverage Rationale Implanted Electrical Jul. 1, 2021 Requirements Stimulator for Spinal Applicable Codes Cord Removed CPT code 95972 (continued) Removed HCPCS code L8683 Lower Extremity Jul. 1, 2021 Template Update Note: This policy does not apply to upper extremities. Invasive Diagnostic Removed CMS section and Endovascular Coverage Rationale Diagnosis Procedures Diagnosis Lower extremity vascular angiography is proven and medically necessary for Replaced language indicating: evaluating arterial disease, aneurysms, perivascular masses and trauma related injuries of the lower extremity under certain circumstances. o “Lower extremity vascular

angiography is proven and medically necessary for Angiography for known chronic lower extremity arterial disease (peripheral

evaluating arterial disease, vascular disease, occlusion or stenosis of arteries of the leg, claudication and limb ischemia) requires all of the following: aneurysms, and trauma related injuries of the lower extremity” Abnormal ankle brachial index (ABI) or an abnormal computed tomography

with “lower extremity vascular angiography (CTA), magnetic resonance angiography (MRA) or duplex angiography is proven and ultrasound; and medically necessary for One of the following: evaluating arterial disease, o Claudication – as evidenced with an ABI ≤0.90; or Limb Ischemia – as evidenced with an ABI <0.40 aneurysms, perivascular o masses, and trauma related ® injuries of the lower extremity For additional medical necessity clinical coverage criteria, refer to the InterQual 2021, April. 2021 Release, CP: Imaging, Imaging, Peripheral Vascular for: under certain circumstances” “Angiography for known lower Angiogram, X-ray, Extremity, Bilateral o extremity arterial disease Angiogram, X-ray, Extremity, Unilateral

(peripheral vascular disease, occlusion or stenosis of Click here to view the InterQual® criteria. arteries of the leg, claudication and limb ischemia) requires all Treatment of the [listed criteria]” with Endovascular revascularization procedures (e.g., stents, angioplasty and/or “angiography for known atherectomy) for treating lower extremity ischemia are proven and medically

chronic lower extremity arterial necessary in individuals who meet the following indication-specific criteria:

disease (peripheral vascular Claudication due to atherosclerotic disease of the aortoiliac and/or

Page 9 of 65 UnitedHealthcare Community Plan of Kentucky Medical Policy Update Bulletin: June 2021

Medical Policy Updates

Revised Policy Title Effective Date Summary of Changes Coverage Rationale Lower Extremity Jul. 1, 2021 disease, occlusion or stenosis femoropopliteal arteries when all of the following criteria are met: Invasive Diagnostic of arteries of the leg, o Impaired ability to work and/or perform activities of daily living (ADL) and Endovascular claudication and limb o All of the following conservative therapies have been tried and failed: Procedures ischemia) requires all of the . At least twelve (12) weeks of a Supervised or Structured Exercise (continued) [listed criteria]” Program

Removed language indicating . Pharmacologic therapy

angiography [is proven and . Smoking cessation, if applicable

medically necessary] for trauma o Moderate to severe ischemic peripheral artery disease with ankle- related injuries including, but not brachial index (ABI) ≤0.69

limited to, one of the following Imaging results show anatomic location and severity of occlusion o findings: (stenosis ≥ 50%) (e.g., duplex ultrasound, CTA, MRA or invasive

o Clinically significant hematoma angiography)

o Knee dislocation (confirmed by Chronic limb-threatening ischemia (CLTI) when all of the following criteria x-ray) are met:

Need for localization of the One or more of the following: o o source of bleeding and . Pain at rest

vascular injury found on duplex . Nonhealing wound or ulcer due to ischemia ultrasound . Gangrene

Updated language pertaining to Moderate to severe ischemic peripheral artery disease and any of the o additional medical necessity following:

coverage criteria; replaced . Ankle-Brachial Index (ABI) ≤0.69 or ® reference to “InterQual 2020” with . Ankle pressure <50 mmHg or ® . “InterQual 2021” Toe-Brachial Index ≤ 0.70 or . Toe pressure <30 mmHg or Supporting Information . Transcutaneous Oxygen Pressure (TcPO2) <25 mmHg Updated Description of Services, Clinical Evidence, and References o Imaging results show anatomic location and severity of occlusion

sections to reflect the most current (stenosis ≥ 50%) (e.g., duplex ultrasound, CTA, MRA or invasive angiography) information

Due to insufficient evidence of efficacy, endovascular revascularization

procedures (e.g., stents, angioplasty and/or atherectomy) for treating lower

extremity ischemia are unproven and not medically necessary in the following circumstances:

Page 10 of 65 UnitedHealthcare Community Plan of Kentucky Medical Policy Update Bulletin: June 2021

Medical Policy Updates

Revised Policy Title Effective Date Summary of Changes Coverage Rationale Lower Extremity Jul. 1, 2021 Claudication due to isolated infrapopliteal (e.g., anterior tibial, posterior tibial Invasive Diagnostic or peroneal) artery disease and Endovascular To prevent the progression of claudication to CLTI Procedures Individual is asymptomatic (continued) Treatment of a nonviable limb

Replaced/Retired Policy Title Effective Date Summary of Changes Femoroacetabular Jun. 1, 2021 Policy replaced; refer to the policy; refer to the policy titled Surgery of the Hip Impingement Syndrome Otoacoustic Emissions Jun. 1, 2021 Policy retired; refer to the Kentucky Hearing Services Manual for coverage guidelines for otoacoustic emissions testing Testing

Page 11 of 65 UnitedHealthcare Community Plan of Kentucky Medical Policy Update Bulletin: June 2021

Medical Benefit Drug Policy Updates

New Policy Title Effective Date Coverage Rationale Long-Acting Injectable Jul. 1, 2021 Cabenuva (cabotegravir/rilpivirine) has been added to the Review at Launch program. Some members may not be Antiretroviral Agents eligible for coverage of this medication at this time. Refer to the policy titled Review at Launch for New to Market for HIV Medications for additional details.

This policy refers to the following long-acting injectable antiretroviral products:

Cabenuva (cabotegravir/rilpivirine)

Cabenuva (cabotegravir/rilpivirine) is proven for the treatment of a human immunodeficiency virus type-1 (HIV-1) in patients who are virologically suppressed (HIV-1 RNA less than 50 copies per mL). Cabenuva is medically necessary when the following additional criteria are met:

For initial therapy, all of the following: Diagnosis of HIV-1 infection; and o Patient has no prior virologic failures or baseline resistance to either cabotegravir or rilpivirine; and o Patient is currently on a stable antiretroviral regimen; and o Submission of medical records (e.g., chart notes, laboratory results) showing viral suppression (HIV-1 RNA less o than 50 copies per mL) for at least 6 months prior to initiation of Cabenuva; and

o Provider attests that patient demonstrates treatment readiness by both of the following: . Patient understands the risks of missed doses of Cabenuva . Patient has the ability to adhere to the required monthly injection appointments; and o Provider confirms that tolerability will be assessed using a 28-day oral lead-in of Vocabria (cabotegravir) and Edurant® (rilpivirine) tablets prior to the first injection of Cabenuva; and

Dosing is in accordance with the United States Food and Drug Administration approved labeling; and o Initial authorization is for no more than 12 months. o

For continuation therapy, all of the following:

o Patient has previously received treatment with Cabenuva; and

o Physician confirms that the patient has achieved and maintained viral suppresion (HIV-1 RNA less than 50 copies per mL) while on Cabenuva therapy; and

o Dosing is in accordance with the United States Food and Drug Administration approved labeling; and

o Authorization is for no more than 12 months.

Page 12 of 65 UnitedHealthcare Community Plan of Kentucky Medical Policy Update Bulletin: June 2021

Medical Benefit Drug Policy Updates

New Policy Title Effective Date Coverage Rationale Long-Acting Injectable Jul. 1, 2021 Cabenuva is unproven and not medically necessary for the treatment of human immunodeficiency virus type-1 (HIV-1) Antiretroviral Agents in patients who are not currently virally suppressed (HIV-1 RNA less than 50 copies per mL). for HIV (continued) Documentation Requirements Benefit coverage for health services is determined by the member specific benefit plan document and applicable laws that may require coverage for a specific service. The documentation requirements outlined below are used to assess whether the member meets the clinical criteria for coverage, but do not guarantee coverage of the service requested.

HCPCS Code Required Clinical Information Cabenuva C9399 For initial therapy requests, medical notes or laboratory results documenting viral suppression (HIV-1 J3490 RNA less than 50 copies per mL) for at least 6 months prior to initiation of Cabenuva.

. Oxlumo™ (Lumasiran) Jul. 1, 2021 Oxlumo™ (lumasiran) has been added to the Review at Launch program. Some members may not be eligible for coverage of this medication at this time. Refer to the policy titled Review at Launch for New to Market Medications for additional details.

Oxlumo is proven and medically necessary for the treatment of CAD when all the following criteria are met:

Initial Therapy

Diagnosis of PH1 by, or in consultation with, a specialist (e.g., geneticist, nephrologist, urologist) with expertise in the diagnosis of PH1; and Confirmation of the PH1 diagnosis based on both of the following:

o Metabolic testing demonstration one of the following: . Increased urinary oxalate excretion ( e.g., greater than 1 mmol/1.73 m2 per day [90 mg/1.73 m2 per day], increased urinary oxalate:creatinine ratio relative to normative values for age); or . Increased plasma oxalate and glyoxylate concentrations and Genetic testing has confirmed a mutation in the alanine:glyoxylate aminotransferase (AGT or AGXT) gene o and

Patient has not received a liver transplant; and Oxlumo is prescribed by, or in consultation with, a specialist (e.g., geneticist, nephrologist, urologist) with expertise in

Page 13 of 65 UnitedHealthcare Community Plan of Kentucky Medical Policy Update Bulletin: June 2021

Medical Benefit Drug Policy Updates

New Policy Title Effective Date Coverage Rationale Oxlumo™ (Lumasiran) Jul. 1, 2021 the treatment of PH1; and (continued) Oxlumo dosing is in accordance with the United States Food and Drug Administration approved labeling; and Initial authorization will be for no more than 6 months.

Continuation Therapy • Submission of medical records (e.g., chart notes, laboratory values) documenting a positive clinical response to therapy from pre-treatment baseline (e.g., decreased urinary oxalate concentrations, decreased urinary oxalate: creatinine ratio, decreased plasma oxalate concentrations); and

• Patient has not received a liver transplant; and

• Oxlumo is prescribed by, or in consultation with, a specialist (e.g., geneticist, nephrologist, urologist) with expertise in the treatment of PH1; and • Oxlumo dosing is in accordance with the United States Food and Drug Administration approved labeling; and Reauthorization will be for no more than 12 months.

Revised Policy Title Effective Date Summary of Changes Coverage Rationale Antiemetics for Jul. 1, 2021 Title Change This policy refers to the following products used as antiemetics for oncology Oncology Previously titled Intravenous Anti- use: ® Emetics Akynzeo (palonosetron/fosnetupitant) injection Aloxi® (palonosetron) injection Coverage Rationale Cinvanti™ (aprepitant emulsion) injectable emulsion Revised list of antiemetic products ® for oncology use to reflect/ Emend (fosaprepitant) injection ® include: Sustol (granisetron extended release) injection ® Akynzeo® (palonosetron/ Kytril (granisetron) injection o ® fosnetupitant) injection Zofran (ondansetron) injection ® o Aloxi (palonosetron) injection ™ Neurokinin 1 Receptor Antagonist (NK1 RA) o Cinvanti (aprepitant) injectable emulsion Preferred Product Non-Preferred Product ® o Emend (fosaprepitant) Emend injection Cinvanti injectable emulsion injection ® o Sustol (granisetron extended release) injection ® o Kytril (granisetron) injection

Page 14 of 65 UnitedHealthcare Community Plan of Kentucky Medical Policy Update Bulletin: June 2021

Medical Benefit Drug Policy Updates

Revised Policy Title Effective Date Summary of Changes Coverage Rationale ® Antiemetics for Jul. 1, 2021 o Zofran (ondansetron) injection 5-Hydroxytryptamine Receptor Antagonist (5HT3 RA) Oncology Added list of preferred and non- Preferred Product Non-Preferred Product (continued) preferred antiemetic products for Kytril injection Aloxi injection oncology use Zofran injection Sustol injection Preferred Product

Revised language to indicate coverage for Cinvanti, Aloxi, Sustol, NK1 RA/5HT3 RA combination

and NK1 RA/5HT3 RA combination Preferred Product Non-Preferred Product

(Akynzeo) will be provided Akynzeo injection contingent on the criteria in the

Preferred Product and Diagnosis- Coverage for antiemetics will be provided contingent on the coverage criteria in Specific Criteria section of the the Diagnosis-Specific Criteria section. policy

Preferred Product Criteria Preferred Product Added language to indicate Coverage for Cinvanti will be provided contingent on the criteria in this section treatment with non-preferred NK1 and the coverage criteria in the Diagnosis-Specific Criteria section. RA, 5HT3 RA, or NK1 RA/5HT3 RA

combination product is medically Coverage of Aloxi and Sustol will be provided contingent on the criteria in this necessary for the indications section and the coverage criteria in the Diagnosis-Specific Criteria section. specified in the policy when one of

the following is met: NK1 RA/5HT3 RA combination (Akynzeo): Coverage of Akynzeo will be Both of the following: o provided contingent on the criteria in this section and the coverage criteria in the . History of a trial of Diagnosis-Specific Criteria section. adequate dose and

duration of preferred NK1 Preferred Product Criteria RA or 5HT3 RA product, Treatment with non-preferred NK1 RA, 5HT3 RA, or NK1 RA/5HT3 RA resulting in minimal clinical response; and combination product is medically necessary for the indications specified in

. Physician attests that, in the policy when one of the following is met:

their clinical opinion, the Both of the following:

clinical response would be o History of a trial of adequate dose and duration of preferred NK1 RA or expected to be superior 5HT3 RA product, resulting in minimal clinical response; and with non-preferred NK1 o Physician attests that, in their clinical opinion, the clinical response

Page 15 of 65 UnitedHealthcare Community Plan of Kentucky Medical Policy Update Bulletin: June 2021

Medical Benefit Drug Policy Updates

Revised Policy Title Effective Date Summary of Changes Coverage Rationale Antiemetics for Jul. 1, 2021 RA, 5HT3 RA, or NK1 would be expected to be superior with non-preferred NK1 RA, 5HT3 RA, Oncology RA/5HT3 RA combination or NK1 RA/5HT3 RA combination product, than experienced with (continued) product, than experienced preferred NK1 RA or 5HT3 RA; with preferred NK1 RA or or 5HT3 RA Both of the following: or o History of intolerance, contraindication, or adverse event to preferred o Both of the following: NK1 RA or 5HT3 RA; and . History of intolerance, o Physician attests that, in their clinical opinion, the same intolerance, contraindication, or contraindication, or adverse event would not be expected to occur with adverse event to preferred non-preferred NK1 RA, 5HT3 RA, or NK1 RA/5HT3 RA combination NK1 RA or 5HT3 RA; and product . Physician attests that, in their clinical opinion, the Diagnosis-Specific Criteria same intolerance, For the coverage criteria below, in absence of specified drug products, the term contraindication, or “antiemetics” will be used in this policy where the coverage criteria apply to all adverse event would not products listed above. be expected to occur with non-preferred NK1 RA, Antiemetics are proven and medically necessary for the following indications: 5HT3 RA, or NK1 RA/5HT3 RA combination NK1 RA (Emend, Cinvanti) may be indicated when one of following are product present: Diagnosis-Specific Criteria Both of the following: Added language to indicate, in o Prevention of chemotherapy-induced nausea and vomiting due to high absence of specified drug emetic risk parenteral anticancer agents; and products, the term “antiemetics” o In combination with a 5HT3 RA; will be used in this policy where the or coverage criteria apply to all All of the following: products listed [in this policy] o Prevention of chemotherapy-induced nausea and vomiting due to Revised coverage criteria to moderate emetic risk parenteral anticancer agents; and indicate antiemetics are proven o In combination with a 5HT3 RA; and and medically necessary for the o One of the risk factors for anticancer-agent induced nausea/vomiting: following indications: . Younger age ( < 55 years)

o NK1 RA (Emend, Cinvanti) . Female sex

Page 16 of 65 UnitedHealthcare Community Plan of Kentucky Medical Policy Update Bulletin: June 2021

Medical Benefit Drug Policy Updates

Revised Policy Title Effective Date Summary of Changes Coverage Rationale Antiemetics for Jul. 1, 2021 may be indicated when one of . Previous history of chemotherapy induced nausea or vomiting Oncology following are present: . Little or no previous alcohol use (continued) . Both of the following: . History of motion sickness or morning sickness during pregnancy Prevention of . High anxiety chemotherapy- induced nausea and 5HT3 RA (Aloxi, Kytril, Sustol, Zofran) may be indicated when one of the vomiting due to high following are present: emetic risk parenteral Both of the following: anticancer agents; and o Prevention of chemotherapy-induced nausea and vomiting due to high In combination with a emetic risk parenteral anticancer agents; and 5HT3 RA o In combination with a NK1 RA or or . All of the following: Prevention of chemotherapy-induced nausea and vomiting due to moderate Prevention of emetic risk parenteral anticancer agents; or chemotherapy- All of the following: induced nausea and o Prevention of chemotherapy-induced nausea and vomiting due to vomiting due to moderate emetic risk parenteral anticancer agents11; and moderate emetic risk o In combination with a NK1 RA; and parenteral anticancer o One of the risk factors for anticancer-agent induced nausea/vomiting: agents; and . Younger age ( < 55 years) In combination with a . Female sex 5HT3 RA; and . Previous history of chemotherapy induced nausea or vomiting One of the risk factors . Little or no previous alcohol use for anticancer-agent . History of motion sickness or morning sickness during pregnancy induced . High anxiety nausea/vomiting: or • Younger age ( < . Treatment of breakthrough nausea and/or vomiting due to 55 years) anticancer agent(s) • Female sex • Previous history of NK1 RA / 5HT3 RA combination product (Akynzeo) may be indicated when chemotherapy one of the following are present: induced nausea or Prevention of chemotherapy-induced nausea and vomiting due to high vomiting emetic risk parenteral anticancer agents;

Page 17 of 65 UnitedHealthcare Community Plan of Kentucky Medical Policy Update Bulletin: June 2021

Medical Benefit Drug Policy Updates

Revised Policy Title Effective Date Summary of Changes Coverage Rationale Antiemetics for Jul. 1, 2021 • Little or no or Oncology previous alcohol Both of the following: (continued) use o Prevention of chemotherapy-induced nausea and vomiting due to • History of motion moderate emetic risk parenteral anticancer agents; and sickness or o One of the risk factors for anticancer-agent induced nausea/vomiting: morning sickness . Younger age ( < 55 years) during pregnancy . Female sex • High anxiety . Previous history of chemotherapy induced nausea or vomiting o 5HT3 RA (Aloxi, Kytril, Sustol, . Little or no previous alcohol use Zofran) may be indicated when . History of motion sickness or morning sickness during pregnancy one of the following are . High anxiety present: . Both of the following: Prevention of chemotherapy- induced nausea and vomiting due to high emetic risk parenteral anticancer agents; and In combination with a NK1 RA or . Prevention of chemotherapy-induced nausea and vomiting due to moderate emetic risk parenteral anticancer agents or . All of the following: Prevention of chemotherapy- induced nausea and

Page 18 of 65 UnitedHealthcare Community Plan of Kentucky Medical Policy Update Bulletin: June 2021

Medical Benefit Drug Policy Updates

Revised Policy Title Effective Date Summary of Changes Coverage Rationale Antiemetics for Jul. 1, 2021 vomiting due to Oncology moderate emetic risk (continued) parenteral anticancer agents; and In combination with NK1 RA; and

One of the risk factors

for anticancer-agent

induced nausea/vomiting: Younger age ( < 55 years) Female sex Previous history of chemotherapy

induced nausea or

vomiting

Little or no previous alcohol use History of motion sickness or morning sickness during pregnancy High anxiety or

. Treatment of breakthrough

nausea and/or vomiting due to anticancer agent(s)

NK1 RA/5HT3 RA o combination product

(Akynzeo) may be indicated when one of the following are present:

Page 19 of 65 UnitedHealthcare Community Plan of Kentucky Medical Policy Update Bulletin: June 2021

Medical Benefit Drug Policy Updates

Revised Policy Title Effective Date Summary of Changes Coverage Rationale Antiemetics for Jul. 1, 2021 . Prevention of Oncology chemotherapy-induced (continued) nausea and vomiting due to high emetic risk parenteral anticancer agents12; or

. Both of the following:

Prevention of

chemotherapy- induced nausea and vomiting due to moderate emetic risk parenteral anticancer agents11; and One of the risk factors

for anticancer-agent

induced

nausea/vomiting: • Younger age (< 55 years) • Female sex • Previous history of chemotherapy induced nausea or vomiting

• Little or no

previous alcohol use • History of motion sickness or morning sickness during pregnancy • High anxiety

Page 20 of 65 UnitedHealthcare Community Plan of Kentucky Medical Policy Update Bulletin: June 2021

Medical Benefit Drug Policy Updates

Revised Policy Title Effective Date Summary of Changes Coverage Rationale Antiemetics for Jul. 1, 2021 Definitions Oncology Added definition of: (continued) o Low Emetic Risk o Minimal Emetic Risk Applicable Codes Added HCPCS code J2405 Added ICD-10 diagnosis code V58.11 Removed ICD-10 diagnosis codes R11.11, R11.12, R11.13, and R11.14 Supporting Information Removed CMS section Updated Background, Clinical Evidence, FDA, and References sections Benlysta® (Belimumab) Jul. 1, 2021 Coverage Rationale This policy refers only to Benlysta (belimumab) injection for intravenous infusion Added language to indicate: for the treatment of systemic lupus erythematosus (SLE) and active lupus nephritis (LN). Benlysta (belimumab) for self-administered subcutaneous o This policy refers only to Benlysta (belimumab) injection injection is obtained under the pharmacy benefit and is indicated for systemic for intravenous infusion for the lupus erythematosus and active lupus nephritis. treatment of active lupus nephritis (LN) Benlysta (belimumab) is proven and medically necessary for the treatment of systemic lupus erythematosus when all of the following criteria are met: o Benlysta (belimumab) for self- administered subcutaneous For initial therapy, all of the following: injection is obtained under the o Diagnosis of active systemic lupus erythematosus, without severe active pharmacy benefit and is central nervous system lupus; and indicated for active lupus o Laboratory testing has documented the presence of autoantibodies (e.g., ANA, Anti-dsDNA, Anti-Sm, Anti-Ro/SSA, Anti-La/SSB); and nephritis Benlysta (belimumab) is Currently receiving at least one standard of care treatment for active o o proven and medically systemic lupus erythematosus (e.g., antimalarials, corticosteroids, or necessary for the treatment of immunosuppressants); that is not a biologic; and

active lupus nephritis when all o Benlysta is initiated and titrated according to U.S. Food and Drug

Page 21 of 65 UnitedHealthcare Community Plan of Kentucky Medical Policy Update Bulletin: June 2021

Medical Benefit Drug Policy Updates

Revised Policy Title Effective Date Summary of Changes Coverage Rationale Benlysta® (Belimumab) Jul. 1, 2021 of the following criteria are Administration labeled dosing for SLE; and (continued) met: o Initial authorization is for no more than 12 months. Initial Therapy For continuation of therapy, all of the following: . Diagnosis of active lupus o Patient has previously received Benlysta injection for intravenous nephritis without severe infusion; and active central nervous o Documentation of positive clinical response; and system lupus; and o Currently receiving at least one standard of care treatment for active . Currently receiving at least systemic lupus erythematosus (e.g., antimalarials, corticosteroids, or one standard of care immunosuppressants); that is not a biologic; and treatment for active o Benlysta is dosed according to U.S. Food and Drug Administration

systemic lupus labeled dosing for SLE; and erythematosus (e.g., o Authorization is for no more than 12 months. antimalarials,

corticosteroids, or Benlysta (belimumab) is proven and medically necessary for the treatment of immunosuppressants) that active lupus nephritis when all of the following criteria are met: is not a biologic; and For initial therapy, all of the following: . Benlysta is initiated and o Diagnosis of active lupus nephritis, without severe active central titrated according to US nervous system lupus; and FDA labeled dosing; and o Currently receiving at least one standard of care treatment for active . Initial authorization is for systemic lupus erythematosus (e.g., antimalarials, corticosteroids, or no more than 12 months immunosuppressants) that is not a biologic; and Continuation of Therapy o Benlysta is initiated and titrated according to US Food and Drug Administration labeled dosing; and . Patient has previously Initial authorization is for no more than 12 months. received Benlysta injection o For continuation of therapy, all of the following: for intravenous infusion; Patient has previously received Benlysta injection for intravenous and o infusion; and . Documentation of positive Documentation of positive clinical response; and clinical response; and o Currently receiving at least one standard of care treatment for active . Currently receiving at least o systemic lupus erythematosus (e.g., antimalarials, corticosteroids, or one standard of care immunosuppressants; that is not a biologic; and treatment for active Benlysta is dosed according to US Food and Drug Administration systemic lupus o labeled dosing; and erythematosus (e.g.,

Page 22 of 65 UnitedHealthcare Community Plan of Kentucky Medical Policy Update Bulletin: June 2021

Medical Benefit Drug Policy Updates

Revised Policy Title Effective Date Summary of Changes Coverage Rationale ® Benlysta (Belimumab) Jul. 1, 2021 antimalarials, o Reauthorization is for no more than 12 months. (continued) corticosteroids, or immunosuppressants) that Benlysta is unproven and not medically necessary for: is not a biologic; and Antineutrophil cytoplasmic antibody-associated vasculitis . Benlysta is dosed Severe active central nervous system (CNS) lupus according to US FDA Use in combination with other biologics labeled dosing; and Waldenström macroglobulinemia . Reauthorization is for no Sjögren's syndrome more than 12 months Rheumatoid arthritis Revised coverage criteria for systemic lupus erythematosus (SLE): Initial Therapy

o Replaced criterion requiring “diagnosis of active SLE without severe active lupus nephritis or severe active central nervous system lupus” with “diagnosis of active SLE without severe active central nervous system lupus”

o Removed criterion requiring the patient is currently receiving at least one standard of care treatment for active systemic lupus erythematosus (e.g., antimalarials, corticosteroids, or immunosuppressants) that is not an intravenous cyclophosphamide

o Removed specific dosage requirements; refer to the US

Page 23 of 65 UnitedHealthcare Community Plan of Kentucky Medical Policy Update Bulletin: June 2021

Medical Benefit Drug Policy Updates

Revised Policy Title Effective Date Summary of Changes Coverage Rationale Benlysta® (Belimumab) Jul. 1, 2021 Food and Drug Administration (continued) (FDA) labeled dosing for SLE Continuation of Therapy o Removed criterion requiring the patient is currently receiving at least one standard of care treatment for active

systemic lupus erythematosus

(e.g., antimalarials, corticosteroids, or immunosuppressants) that is not an intravenous cyclophosphamide o Removed specific dosage requirements; refer to the US Food and Drug Administration

(FDA) labeled dosing for SLE

Revised list of unproven and not

medically necessary indications; removed:

o Severe active lupus nephritis o Use in combination with intravenous cyclophosphamide Supporting Information Updated Clinical Evidence, FDA, and References sections to reflect the most current information Complement Inhibitors Jul. 1, 2021 Coverage Criteria This policy refers to the following complement inhibitor drug products: (Soliris® & Ultomiris®) Revised coverage criteria for Soliris (eculizumab) treatment of generalized Ultomiris (ravulizumab-cwvz)

myasthenia gravis; replaced Soliris and Ultomiris are proven and medically necessary for the treatment of criterion requiring “patient is currently on a stable therapeutic atypical Hemolytic Uremic Syndrome (aHUS) when all of the following criteria

Page 24 of 65 UnitedHealthcare Community Plan of Kentucky Medical Policy Update Bulletin: June 2021

Medical Benefit Drug Policy Updates

Revised Policy Title Effective Date Summary of Changes Coverage Rationale Complement Inhibitors Jul. 1, 2021 dose (at least 3 to 6 months) of are met: (Soliris® & Ultomiris®) immunosuppressive therapy” with Initial Therapy: (continued) “patient is currently on a stable o Documentation supporting the diagnosis of aHUS by ruling out both of dose (at least 2 months) of the following: immunosuppressive therapy” . Shiga toxin E. coli related hemolytic uremic syndrome (STEC-HUS) Applicable Codes . Thrombotic thrombocytopenia purpura (TTP) (e.g., rule out Maximum Allowed Quantities by ADAMTS13 deficiency) National Drug Code (NDC) Units and Laboratory results, signs, and/or symptoms attributed to aHUS (e.g., Revised list of applicable NDCs for o thrombocytopenia, microangiopathic hemolysis, thrombotic Ultomiris; added: microangiopathy, acute renal failure, etc.); and 25682-0025-01 o Patient is treatment naïve with both Soliris and Ultomiris; and . How supplied: 300 mg/3 o Soliris or Ultomiris are dosed according to the U.S. FDA labeled dosing mL solution in vials o for aHUS; and . Maximum allowed: 36 mL Prescribed by, or in consultation with, a hematologist or nephrologist; 25682-0028-01 o o and . How supplied: 1,100 Initial authorization will be for no more than 6 months mg/11 mL solution in vials o Continuation of Therapy: . Maximum allowed: 36 mL Patient has previously been treated with Soliris or Ultomiris; and Supporting Information o o Documentation demonstrating a positive clinical response from baseline Updated References section to (e.g., reduction of plasma exchanges, reduction of dialysis, increased reflect the most current information platelet count, reduction of hemolysis); and o Soliris or Ultomiris are dosed according to the U.S. FDA labeled dosing for aHUS; and o Prescribed by, or in consultation with, a hematologist or nephrologist; and o Reauthorization will be for no more than 12 months

Soliris and Ultomiris are unproven and not medically necessary for the treatment of Shiga toxin E. coli-related Hemolytic Uremic Syndrome (STEC- HUS).

Soliris and Ultomiris are proven and medically necessary for the treatment of

Page 25 of 65 UnitedHealthcare Community Plan of Kentucky Medical Policy Update Bulletin: June 2021

Medical Benefit Drug Policy Updates

Revised Policy Title Effective Date Summary of Changes Coverage Rationale Complement Inhibitors Jul. 1, 2021 paroxysmal nocturnal hemoglobinuria (PNH) when all of the following criteria (Soliris® & Ultomiris®) are met: (continued) Initial Therapy: o Documentation supporting the diagnosis of PNH that includes both of the following: . Flow cytometry analysis confirming presence of PNH clones . Laboratory results, signs, and/or symptoms attributed to PNH (e.g., abdominal pain, anemia, dyspnea, extreme fatigue, smooth muscle dystonia, unexplained/unusual thrombosis, hemolysis/hemoglobinuria, kidney disease, pulmonary hypertension, etc.) and o Patient is treatment naïve with both Soliris and Ultomiris; and o Soliris or Ultomiris is dosed according to the U.S. FDA labeled dosing for PNH; and o Prescribed by, or in consultation with, a hematologist or oncologist; and o Initial authorization will be for no more than 6 months Continuation of Therapy: o Patient has previously been treated with Soliris or Ultomiris; and o Documentation demonstrating a positive clinical response from baseline (e.g., increased or stabilization of hemogloblin levels, reduction in transfusions, improvement in hemolysis, decrease in LDH, increased reticulocyte count, etc.); and o Soliris or Ultomiris is dosed according to the U.S. FDA labeled dosing for PNH; and o Prescribed by, or in consultation with, a hematologist or oncologist; and o Reauthorization will be for no more than 12 months

Soliris is proven and medically necessary for the treatment of generalized myasthenia gravis when all of the following criteria are met: Initial Therapy: o Submission of medical records (e.g., chart notes, laboratory values, etc.) to support the diagnosis of generalized myasthenia gravis (gMG) by a

Page 26 of 65 UnitedHealthcare Community Plan of Kentucky Medical Policy Update Bulletin: June 2021

Medical Benefit Drug Policy Updates

Revised Policy Title Effective Date Summary of Changes Coverage Rationale Complement Inhibitors Jul. 1, 2021 neurologist or in consultation with a neurologist confirming all of the (Soliris® & Ultomiris®) following: (continued) . Patient has not failed a previous course of Soliris therapy; and . Positive serologic test for anti-AChR antibodies; and . One of the following: History of abnormal neuromuscular transmission test demonstrated by single-fiber electromyography (SFEMG) or repetitive nerve stimulation History of positive anticholinesterase test, e.g., edrophonium chloride test Patient has demonstrated improvement in MG signs on oral cholinesterase inhibitors, as assessed by the treating neurologist and . Patient has a Myasthenia Gravis Foundation of America (MGFA) Clinical Classification of class II, III, or IV at initiation of therapy; and . Patient has a Myasthenia Gravis-specific Activities of Daily Living scale (MG-ADL) total score ≥ 6 at initiation of therapy and o Both of the following: . History of failure of at least two immunosuppressive agents over the course of at least 12 months (e.g., azathioprine, methotrexate, cyclosporine, mycophenylate, etc.); and . Patient has required two or more courses of plasmapheresis/plasma exchanges and/or intravenous immune globulin for at least 12 months without symptom control; and o Patient is currently on a stable dose (at least 2 months) of immunosuppressive therapy; and o Soliris is initiated and titrated according to the U.S. FDA labeled dosing for gMG: up to a maximum of 1200 mg every 2 weeks; and o Prescribed by, or in consultation with, a neurologist; and o Initial authorization will be for no more than 6 months

Page 27 of 65 UnitedHealthcare Community Plan of Kentucky Medical Policy Update Bulletin: June 2021

Medical Benefit Drug Policy Updates

Revised Policy Title Effective Date Summary of Changes Coverage Rationale Complement Inhibitors Jul. 1, 2021 Continuation of Therapy: ® ® (Soliris & Ultomiris ) o Patient has previously been treated with Soliris; and (continued) o Submission of medical records (e.g., chart notes, laboratory tests) to demonstrate a positive clinical response from baseline as demonstrated by at least all of the following: . Improvement and/or maintenance of at least a 3 point improvement (reduction in score) in the MG-ADL score from pre-treatment baseline . Reduction in signs and symptoms of myasthenia gravis . Maintenance, reduction, or discontinuation of dose(s) of baseline immunosuppressive therapy (IST) prior to starting *Soliris (*Note: Add on, dose escalation of IST, or additional rescue therapy from baseline to treat myasthenia gravis or exacerbation of symptoms while on Soliris therapy will be considered as treatment failure); and o Soliris is dosed according to the U.S. FDA labeled dosing for gMG: up to a maximum of 1200 mg every 2 weeks; and o Prescribed by, or in consultation with, a neurologist; and o Reauthorization will be for no more than 12 months

Soliris is proven and medically necessary for the treatment of neuromyelitis optica spectrum disorder (NMOSD) when all of the following criteria are met: Initial Therapy: o Submission of medical records (e.g., chart notes, laboratory values, etc.) to support the diagnosis of neuromyelitis optica spectrum disorder (NMOSD) by a neurologist confirming all of the following: . Past medical history of one of the following: Optic neuritis Acute myelitis Area postrema syndrome: Episode of otherwise unexplained hiccups or nausea and vomiting Acute brainstem syndrome Symptomatic narcolepsy or acute diencephalic clinical

Page 28 of 65 UnitedHealthcare Community Plan of Kentucky Medical Policy Update Bulletin: June 2021

Medical Benefit Drug Policy Updates

Revised Policy Title Effective Date Summary of Changes Coverage Rationale Complement Inhibitors Jul. 1, 2021 syndrome with NMOSD-typical diencephalic MRI lesions (Soliris® & Ultomiris®) Symptomatic cerebral syndrome with NMOSD-typical brain (continued) lesions and . Positive serologic test for anti-aquaporin-4 immunoglobulin G (AQP4-IgG)/NMO-IgG antibodies; and . Diagnosis of multiple sclerosis or other diagnoses have been ruled out and o Patient has not failed a previous course of Soliris therapy; and o History of failure of, contraindication, or intolerance to rituximab therapy; and o One of the following: . History of at least two relapses during the previous 12 months prior to initiating Soliris . History of at least three relapses during the previous 24 months, at least one relapse occurring within the past 12 months prior to initiating Soliris; and o Soliris is initiated and titrated according to the U.S. FDA labeled dosing for NMOSD, up to a maximum of 1200 mg every 2 weeks; and o Prescribed by, or in consultation with, a neurologist; and o Patient is not receiving Soliris in combination with any of the following: . Disease modifying therapies for the treatment of multiple sclerosis [e.g., Gilenya (fingolimod), Tecfidera (dimethyl fumarate), Ocrevus (ocrelizumab), etc.] . Anti-IL6 therapy [e.g., Actemra (tocilizumab), Enspryng (satralizumab)] . Rituximab and o Initial authorization will be for no more than 6 months Continuation of Therapy: o Patient has previously been treated with Soliris; and

Page 29 of 65 UnitedHealthcare Community Plan of Kentucky Medical Policy Update Bulletin: June 2021

Medical Benefit Drug Policy Updates

Revised Policy Title Effective Date Summary of Changes Coverage Rationale Complement Inhibitors Jul. 1, 2021 o Submission of medical records (e.g., chart notes, laboratory tests) to (Soliris® & Ultomiris®) demonstrate a positive clinical response from baseline as demonstrated (continued) by at least both of the following: . Reduction in the number and/or severity of relapses or signs and symptoms of NMOSD . Maintenance, reduction, or discontinuation of dose(s) of any baseline immunosuppressive therapy (IST) prior to starting Soliris. Note: Add on, dose escalation of IST, or additional rescue therapy from baseline to treat NMOSD or exacerbation of symptoms while on Soliris therapy will be considered as treatment failure and o Soliris is dosed according to the U.S. FDA labeled dosing for NMOSD: up to a maximum of 1200 mg every 2 weeks; and o Prescribed by, or in consultation with, a neurologist; and o Patient is not receiving Soliris in combination with any of the following: . Disease modifying therapies for the treatment of multiple sclerosis [e.g., Gilenya (fingolimod), Tecfidera (dimethyl fumarate), Ocrevus (ocrelizumab), etc.] . Anti-IL6 therapy [e.g., Actemra (tocilizumab), Enspryng (satralizumab)] . Rituximab; and o Reauthorization will be for no more than 12 months Gonadotropin Jul. 1, 2021 Coverage Rationale Please refer to the Medical Benefit Drug Policy titled Oncology Medication Releasing Hormone Revised list of applicable Clinical Coverage for updated information based on the National Comprehensive ® Analogs gonadotropin releasing hormone Cancer Network (NCCN) Drugs & Biologics Compendium (NCCN Compendium®) for oncology indications. analog (GnRH analog) drug products; added “Fensolvi

(leuprolide acetate)” This policy refers to the following gonadotropin releasing hormone analog

Central Precocious Puberty (GnRH analog) drug products:

(Fensolvi, Lupron Depot-Ped, Fensolvi (leuprolide acetate) Firmagon (degarelix) Supprelin LA, Triptodur, Vantas) Lupaneta Pack (leuprolide acetate injection & norethindrone acetate tablets) Added language to indicate:

Page 30 of 65 UnitedHealthcare Community Plan of Kentucky Medical Policy Update Bulletin: June 2021

Medical Benefit Drug Policy Updates

Revised Policy Title Effective Date Summary of Changes Coverage Rationale Gonadotropin Jul. 1, 2021 o Fensolvi is proven and Lupron Depot (leuprolide acetate) Releasing Hormone medically necessary for the Lupron Depot-Ped (leuprolide acetate) Analogs treatment of central precocious Supprelin LA (histrelin acetate) (continued) puberty when all of the [listed] Trelstar (triptorelin pamoate) criteria are met Triptodur (triptorelin)

Fensolvi should be Vantas (histrelin acetate) o discontinued at the appropriate Zoladex (goserelin acetate)

age of onset of puberty at the

discretion of the physician; For the coverage criteria below, in absence of specified drug products, the term give consideration to “GnRH analogs” will be used in this policy where the coverage criteria apply to discontinuing treatment before all products listed above. 11 years of age in girls and 12 years of age in boys Refer to the policy for complete details. Uterine Leiomyomata (Fibroids) (Lupron Depot) Updated language to clarify Lupron Depot is medically necessary for

the treatment of uterine

leiomyomata when both of the

following criteria are met:

One of the following: o . All of the following: For the treatment of uterine leiomyomata related anemia; and Patient did not respond to iron

therapy of one month duration; and

For use prior to surgery or . For use prior to surgery to

Page 31 of 65 UnitedHealthcare Community Plan of Kentucky Medical Policy Update Bulletin: June 2021

Medical Benefit Drug Policy Updates

Revised Policy Title Effective Date Summary of Changes Coverage Rationale Gonadotropin Jul. 1, 2021 reduce the size of fibroids Releasing Hormone to facilitate a surgical Analogs procedure (e.g., (continued) myomectomy, hysterectomy) and o Authorization will be for no more than 3 months Supporting Information Updated Background, FDA, and References sections to reflect the most current information Infliximab (Remicade®) Jul. 1, 2021 Title Change This policy refers to the following infliximab products: Previously titled Infliximab (Avsola™, Remicade® (infliximab) Inflectra®, Remicade®, & Renflexis®) (for Kentucky Only) Refer to the policy for complete details. Coverage Rationale Revised list of applicable infliximab products; removed: ™ o Avsola ® o Inflectra ® o Renflexis Removed language pertaining to preferred products Added coverage criteria for continuation of therapy for the treatment of immune checkpoint inhibitor-related toxicities to require:

Documentation of positive o clinical response; and

o Patient continues to experience toxicities from treatment with

Page 32 of 65 UnitedHealthcare Community Plan of Kentucky Medical Policy Update Bulletin: June 2021

Medical Benefit Drug Policy Updates

Revised Policy Title Effective Date Summary of Changes Coverage Rationale Infliximab (Remicade®) Jul. 1, 2021 immune checkpoint inhibitor (continued) therapy; and o One of the following: . Patient is receiving infliximab in combination with systemic corticosteroids . Patient is intolerant to systemic corticosteroid therapy and o Reauthorization is for no more than 4 doses Applicable Codes Removed HCPCS codes Q5103, Q5104, Q5109, and Q5121 Added ICD-10 diagnosis codes M05.7A, M06.0A, and M06.8A Supporting Information Updated FDA and References sections to reflect the most current information Intravenous Jul. 1, 2021 Title Change This policy refers to the following intravenous bisphosphonates/bone Bisphosphonates/ Previously titled Zoledronic Acid resorption inhibitors: Bone Resorption (for Kentucky Only) Zoledronic acid Inhibitors (Zoledronic Coverage Rationale Pamidronate disodium Acid & Pamidronate Added langauge to indicate: Disodium) Zoledronic acid is proven and medically necessary for the treatment of bone o This policy refers to the following intravenous loss disorders (Osteoporosis, Paget’s Disease). For medical necessity clinical ® bisphosphonate/bone coverage criteria, refer to the the InterQual 2020, Apr. 2020 Release, CP: resorption inhibitors: Specialty Rx Non-Oncology, Zoledronic acid.

. Zoledronic acid Click here to view the InterQual® criteria. . Pamidronate disodium

Page 33 of 65 UnitedHealthcare Community Plan of Kentucky Medical Policy Update Bulletin: June 2021

Medical Benefit Drug Policy Updates

Revised Policy Title Effective Date Summary of Changes Coverage Rationale Intravenous Jul. 1, 2021 o Pamidronate disodium is Zoledronic acid is proven and medically necessary for the treatment of Bisphosphonates/ proven and medically cancer-related bone conditions. For medical necessity clinical coverage Bone Resorption necessary when all of the criteria, refer to the the InterQual® 2020, Apr. 2020 Release, CP: Specialty Rx Inhibitors (Zoledronic following criteria are met: Oncology, Zoledronic acid (Oncology). Acid & Pamidronate . Used for treatment of one ® Disodium) of the following: Click here to view the InterQual criteria. (continued) ­ Diagnosis of moderate or severe Pamidronate disodium is proven and medically necessary when all of the hypercalcemia following criteria are met: associated with Used for treatment of one of the following: malignancy Diagnosis of moderate or severe hypercalcemia associated with o Diagnosis of moderate malignancy ­ to severe Paget’s Diagnosis of moderate to severe Paget’s disease of bone o disease of bone Treatment of osteolytic bone metastases of breast cancer and given in o Treatment of osteolytic conjunction with standard antineoplastic therapy ­ bone metastases of Treatment of osteolytic lesions of multiple myeloma and given in o breast cancer and conjunction with standard antineoplastic therapy

given in conjunction Dose does not exceed 90 mg per treatment

with standard Authorization is for no more than 12 months

antineoplastic therapy

Treatment of osteolytic ­ lesions of multiple

myeloma and given in

conjunction with

standard

antineoplastic therapy

. Dose does not exceed 90

mg per treatment

. Authorization is for no

more than 12 months

Applicable Codes Added HCPCS code J2430 Added ICD-10 diagnosis codes

Page 34 of 65 UnitedHealthcare Community Plan of Kentucky Medical Policy Update Bulletin: June 2021

Medical Benefit Drug Policy Updates

Revised Policy Title Effective Date Summary of Changes Coverage Rationale Intravenous Jul. 1, 2021 E83.59 and M89.9 Bisphosphonates/ Supporting Information Bone Resorption Updated FDA and References Inhibitors (Zoledronic sections to reflect the most current Acid & Pamidronate information Disodium) (continued) Rituximab (Riabni™, Jul. 1, 2021 Coverage Rationale This policy refers only to the following drug products, rituximab injections for Rituxan®, Ruxience®, & Revised list of applicable rituximab intravenous infusion for non-oncology conditions: Truxima®) drug products for intravenous Riabni™ (rituximab-arrx) infusion for non-oncology Rituxan® (rituximab) conditions; added: Rituxan Hycela® (rituximab and hyaluronidase human) ™ Ruxience™ (rituximab-pvvr) o Riabni (rituximab-arrx) Truxima® (rituximab-abbs) o Any FDA-approved rituximab biosimilar product not listed Any FDA-approved rituximab biosimilar product not listed here Added language to indicate: “Rituximab” will be used to refer to all rituximab products without hyaluronidase. o Truxima (rituximab-abbs) and Ruxience (rituximab-pvvr) are the preferred rituximab Refer to the policy for complete details. products; coverage will be provided for Truxima and Ruxience contingent on the coverage criteria in the Diagnosis-Specific Criteria section [of the policy] o Any U.S. Food and Drug Administration approved and launched rituximab biosimilar product not listed by name in this policy will be considered non-preferred until reviewed by UnitedHealthcare

o Coverage for Rituxan, Riabni

Page 35 of 65 UnitedHealthcare Community Plan of Kentucky Medical Policy Update Bulletin: June 2021

Medical Benefit Drug Policy Updates

Revised Policy Title Effective Date Summary of Changes Coverage Rationale Rituximab (Riabni™, Jul. 1, 2021 (rituximab-arrx), or other Rituxan®, Ruxience®, & rituximab product will be Truxima®) provided contingent on the (continued) criteria in the Preferred Product Criteria section and the coverage criteria in the Diagnosis-Specific Criteria section [of the policy] Preferred Product Criteria

o Treatment with Rituxan, Riabni, or other rituximab product is medically necessary for the indications specified in the policy when both of the following criteria are met: . One of the following: All of the following: • History of a trial of Truxima resulting in minimal clinical response to therapy and residual disease activity; and • History of a trial of Ruxience resulting in minimal clinical response to therapy and residual disease activity; and • Physician attests that, in their

Page 36 of 65 UnitedHealthcare Community Plan of Kentucky Medical Policy Update Bulletin: June 2021

Medical Benefit Drug Policy Updates

Revised Policy Title Effective Date Summary of Changes Coverage Rationale Rituximab (Riabni™, Jul. 1, 2021 clinical opinion, Rituxan®, Ruxience®, & the clinical Truxima®) response would (continued) be expected to be superior with Rituxan, Riabni, or other rituximab products, than experienced with Truxima and Ruxience or All of the following: • History of intolerance, contraindication, or adverse event to Truxima; and • History of intolerance, contraindication, or adverse event to Ruxience; and • Physician attests that, in their clinical opinion, the same intolerance, contraindication, or serious adverse event would not be expected to occur with

Page 37 of 65 UnitedHealthcare Community Plan of Kentucky Medical Policy Update Bulletin: June 2021

Medical Benefit Drug Policy Updates

Revised Policy Title Effective Date Summary of Changes Coverage Rationale Rituximab (Riabni™, Jul. 1, 2021 Rituxan, Riabni, or Rituxan®, Ruxience®, & other rituximab Truxima®) (continued) products and . Patient has not had a loss of favorable response after established maintenance therapy with Rituxan, Riabni, or other rituximab products Diagnosis-Specific Criteria

o In absence of specified drug products, the term “Rituximab” will be used in this policy where the coverage criteria apply to all products listed [in the policy]

Revised coverage criteria for:

Immune Thrombocytopenic

Purpura (ITP) o Updated criteria for initial therapy: . Replaced criterion requiring: “Documented platelet 9 count < 50 x 10 /L” with “documented

platelet count < 30 x 9 10 /L” “Rituximab is dosed up to a maximum of 1,225 mg per dose” with “rituximab is

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Medical Benefit Drug Policy Updates

Revised Policy Title Effective Date Summary of Changes Coverage Rationale Rituximab (Riabni™, Jul. 1, 2021 dosed up to a Rituxan®, Ruxience®, & maximum of 375 Truxima®) (continued) mg/m2 once weekly for 4 doses” “Authorization will be for no more than 6 months” with “authorization will be for no more than 3 months” . Added criterion requiring: History of failure, contraindication, or intolerance to thrombopoietin receptor agonist (TPO- RA) (e.g., Promacta [eltrombopag], Nplate [romiplostim]) Rituximab is dosed up to 1,000 mg on days 1 and 15

o Revised criteria for continuation of therapy to require: . Diagnosis of immune thrombocytopenic purpura (ITP); and . Documented platelet count < 30 x 109 / L; and . History of failure, contraindication, or intolerance to one of the

Page 39 of 65 UnitedHealthcare Community Plan of Kentucky Medical Policy Update Bulletin: June 2021

Medical Benefit Drug Policy Updates

Revised Policy Title Effective Date Summary of Changes Coverage Rationale Rituximab (Riabni™, Jul. 1, 2021 following: Rituxan®, Ruxience®, & Anti-D immunoglobulin Truxima®) (continued) Corticosteroids Immune globulin Thrombopoietin receptor agonist (TPO- RA) (e.g., Promacta [eltrombopag], Nplate [romiplostim]) Splenectomy and . One of the following: Rituximab is dosed up to a maximum of 375 mg/m2 once weekly for 4 doses Rituximab is dosed up to 1,000 mg on days 1 and 15 and . Authorization will be for no more than 3 months Wegener’s Granulomatosis or Microscopic Polyangiitis

o Added criterion for initial therapy requiring rituximab is dosed up to a maximum of two 1000 mg intravenous infusions separated by 2 weeks

Neuromyelitis Optica

Removed criterion for initial o therapy requiring history of

failure, contraindication, or

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Medical Benefit Drug Policy Updates

Revised Policy Title Effective Date Summary of Changes Coverage Rationale Rituximab (Riabni™, Jul. 1, 2021 intolerance to at least two of Rituxan®, Ruxience®, & the following: Truxima®) (continued) . Azathioprine . Corticosteroids . Mycophenolate mofetil Applicable Codes Added HCPCS codes J3590 and J9999 Supporting Information Updated Clinical Evidence, FDA, and References sections to reflect the most current information Spinraza® (Nusinersen) Jul. 1, 2021 Coverage Rationale Spinraza® (nusinersen) is proven and medically necessary for the treatment Revised coverage criteria; replaced of spinal muscular atrophy (SMA) in patients who meet all of the following criterion requiring “diagnosis of criteria: spinal muscular atrophy type I, II, or III by, or in consultation with, a For initial therapy, all of the following: neurologist with expertise in the o Diagnosis of spinal muscular atrophy by, or in consultation with, a diagnosis of SMA” with “diagnosis neurologist with expertise in the diagnosis of SMA; and of spinal muscular atrophy by, or in o Submission of medical records (e.g., chart notes, laboratory values) consultation with, a neurologist confirming the following: with expertise in the diagnosis of . The mutation or deletion of genes in chromosome 5q resulting in SMA” one of the following: Removed specific dosage Homozygous gene deletion or mutation (e.g., homozygous requirements for Spinraza; refer to deletion of exon 7 at locus 5q13); or the applicable US FDA approved Compound heterozygous mutation (e.g., deletion of SMN1 labeling exon 7 [allele 1] and mutation of SMN1 [allele 2]) Supporting Information and Patient is not dependent on either of the following: Updated Clinical Evidence and o References sections to reflect the . Invasive ventilation or tracheostomy . most current information Use of non-invasive ventilation beyond use for naps and nightime sleep and

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Medical Benefit Drug Policy Updates

Revised Policy Title Effective Date Summary of Changes Coverage Rationale ® Spinraza (Nusinersen) Jul. 1, 2021 o Submission of medical records (e.g., chart notes, laboratory values) of (continued) the baseline exam of at least one of the following exams (based on patient age and motor ability) to establish baseline motor ability:* *Baseline assessments for patients less than 2 months of age are not necessary in order to not delay access to initial therapy in recently diagnosed infants. Initial assessments shortly post-therapy can serve as baseline with respect to efficacy reauthorization assessment. . Hammersmith Infant Neurological Exam Part 2 (HINE-2) (infant to early childhood) . Hammersmith Functional Motor Scale Expanded (HFMSE) . Upper Limb Module (ULM) Test (Non ambulatory) . Children’s Hospital of Philadelphia Infant Test of Neuromuscular Disorders (CHOP INTEND) and o Spinraza is prescribed by, or in consultation with, a neurologist with expertise in the treatment of SMA; and o Patient has not previously received gene replacement therapy for the treatment of SMA; and o Patient is not receiving concomitant chronic survival motor neuron (SMN) modifying therapy [e.g., Evrysdi (risdiplam)]; and o Spinraza is to be administered intrathecally by, or under the direction of, healthcare professionals experienced in performing lumbar punctures; and o Spinraza dosing for SMA is within accordance with the United States Food and Drug Administration (FDA) approved labeling; and o Initial authorization will be for no more than 4 loading doses

For continuation of therapy, all of the following: o Diagnosis of spinal muscular atrophy by, or in consultation with, a neurologist with expertise in the diagnosis of SMA; and o Patient has previously received Spinraza therapy; and o Patient is not dependent on either of the following: . Invasive ventilation or tracheostomy

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Medical Benefit Drug Policy Updates

Revised Policy Title Effective Date Summary of Changes Coverage Rationale Spinraza® (Nusinersen) Jul. 1, 2021 . Use of non-invasive ventilation beyond use for naps and nightime (continued) sleep and o Patient has not previously received gene replacement therapy for the treatment of SMA; and o Patient is not receiving concomitant chronic survival motor neuron (SMN) modifying therapy [e.g., Evrysdi (risdiplam)]; and o Submission of medical records (e.g., chart notes, laboratory values) with the most recent results (< 1 month prior to request) documenting a positive clinical response from pretreatment baseline status to Spinraza therapy as demonstrated by at least one of the following exams: . HINE-2 milestones: One of the following: • Improvement or maintenance of previous improvement of at least 2 point (or maximal score) increase in ability to kick • Improvement or maintenance of previous improvement of at least 1 point increase in any other HINE-2 milestone (e.g., head control, rolling, sitting, crawling, etc.), excluding voluntary grasp and One of the following: • The patient exhibited improvement or maintenance of previous improvement in more HINE motor milestones than worsening, from pretreatment baseline (net positive improvement) • Achieved and maintained any new motor milestones when they would otherwise be unexpected to do so (e.g., sit unassisted, stand, walk) or . HFMSE: One of the following: Improvement or maintenance of previous improvement of at least a 3 point increase in score from pretreatment baseline Patient has achieved and maintained any new motor milestone

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Medical Benefit Drug Policy Updates

Revised Policy Title Effective Date Summary of Changes Coverage Rationale Spinraza® (Nusinersen) Jul. 1, 2021 from pretreatment baseline when they would otherwise be (continued) unexpected to do so or . ULM: One of the following: Improvement or maintenance of previous improvement of at least a 2 point increase in score from pretreatment baseline Patient has achieved and maintained any new motor milestone from pretreatment baseline when they would otherwise be unexpected to do so or . CHOP INTEND: One of the following: Improvement or maintenance of previous improvement of at least a 4 point increase in score from pretreatment baseline Patient has achieved and maintained any new motor milestone from pretreatment baseline when they would otherwise be unexpected to do so and o Spinraza is prescribed by, or in consultation with, a neurologist with expertise in the treatment of SMA; and o Spinraza is to be administered intrathecally by, or under the direction of, healthcare professionals experienced in performing lumbar punctures; and o Spinraza dosing for SMA is within accordance with the United States FDA approved labeling; and o Reauthorization will be for no more than 3 maintenance doses (12 months)

Unproven Spinraza is not proven or medically necessary for: Spinal muscular atrophy without chromosome 5q mutations or deletions Concomitant treatment of SMA in patients who have previously received gene replacement therapy Concomitant treatment of SMA in patients receiving Evyrsdi (risdiplam)

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Medical Benefit Drug Policy Updates

Revised Policy Title Effective Date Summary of Changes Coverage Rationale White Blood Cell Jul. 1, 2021 Coverage Rationale This policy refers to the following white blood cell colony stimulating factors Colony Stimulating Revised list of applicable long- (CSFs): Factors acting pegfilgrastim agents; Long-acting pegfilgrastim agents: ® ® removed Ziextenzo (pegfilgrastim- o Fulphila (pegfilgrastim-jmdb) ® bmez) o Neulasta (pegfilgrastim) ® Removed language pertaining to o Udenyca (pegfilgrastim-cbqv) preferred products Short-acting filgrastim agents: ® Removed language indicating o Granix (tbo-filgrastim) ® Ziextenzo is proven and medically o Neupogen (filgrastim) ® necessary for: o Nivestym (filgrastim-aafi) Zarxio® (filgrastim-sndz) o Primary Prophylaxis of o Chemotherapy-Induced Febrile Leukine® (sargramostim) (see Diagnosis-Specific Criteria) Neutropenia (FN) o Secondary Prophylaxis of Diagnosis-Specific Criteria Febrile Neutropenia (FN) For the coverage criteria below, in absence of specified drug products, the term o Treatment of Febrile “colony stimulating factors” or “CSFs” will be used in this policy where the Neutropenia (FN) coverage criteria apply to all products listed above. o Hematopoietic Syndrome of Acute Radiation Syndrome Bone Marrow/Stem Cell Transplant (Leukine, Neupogen, Nivestym, Zarxio) Applicable Codes Leukine, Neupogen, Nivestym, and Zarxio are proven and medically Removed HCPCS code Q5120 necessary when all of the following criteria are met:

Supporting Information o One of the following:

Updated FDA and References . Patient has non-myeloid malignancies and is undergoing

sections to reflect the most current myeloablative chemotherapy followed by autologous or allogeneic

information bone marrow transplant (BMT); or . Used for mobilization of hematopoietic progenitor cells into the peripheral blood for collection by leukapheresis; or . Patient has had a peripheral stem cell transplant (PSCT) and has received myeloablative chemotherapy and

o Medication is dosed in accordance with the United States Food and Drug Administration (FDA) approved labeling; and Prescribed by or in consultation with a hematologist or oncologist o

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Medical Benefit Drug Policy Updates

Revised Policy Title Effective Date Summary of Changes Coverage Rationale White Blood Cell Jul. 1, 2021 Acute Myeloid Leukemia (AML) Induction or Consolidation Therapy Colony Stimulating (Leukine, Neupogen, Nivestym, Zarxio) Factors Leukine, Neupogen, Nivestym, and Zarxio are proven and medically (continued) necessary when all of the following criteria are met: o Diagnosis of AML; and o Patient has completed either induction or consolidation chemotherapy

Primary Prophylaxis of Chemotherapy-Induced Febrile Neutropenia (FN) (Fulphila, Granix, Leukine, Neulasta, Neupogen, Nivestym, Udenyca, Zarxio) White blood cell colony stimulating factors are proven and medically necessary when all of the following criteria are met: o One of the following: . Patient is receiving dose dense MVAC (methotrexate, vinblastine, doxorubicin, cisplatin) for bladder cancer; or . Patient is receiving dose dense AC (doxorubicin, cyclophosphamide) followed by dose-dense paclitaxel for breast cancer; or . Patient is receiving chemotherapy regimen(s) associated with > 20% incidence of FN or o Both of the following: . Patient is receiving chemotherapy regimen(s) associated with 10- 20% incidence of FN; and . Patient has one or more risk factors for chemotherapy-induced febrile neutropenia: Persistent neutropenia due to prior chemotherapy, radiation therapy or bone marrow involvement by tumor (ANC <1500 neutrophils/mcL50) Liver dysfunction (bilirubin > 2.0) Renal dysfunction (creatinine clearance < 50) Age > 65 years receiving full chemotherapy dose intensity

Note: Chemotherapy regimen associated incidence of febrile neutropenia

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Medical Benefit Drug Policy Updates

Revised Policy Title Effective Date Summary of Changes Coverage Rationale White Blood Cell Jul. 1, 2021 will be based on the clinical trial(s) with the highest level of evidence Colony Stimulating according to the GRADE criteria. Factors (continued) Secondary Prophylaxis of Febrile Neutropenia (FN) (Fulphila, Granix, Leukine, Neulasta, Neupogen, Nivestym, Udenyca, Zarxio) White blood cell colony stimulating factors are proven and medically necessary when all of the following criteria are met: o Patient is receiving myelosuppressive anticancer drugs associated with neutropenia (ANC ≤ 1500 neutrophils/mcL); and o A documented history of febrile neutropenia during a previous course of chemotherapy

Treatment of Febrile Neutropenia (FN) (Fulphila, Leukine, Neulasta, Neupogen, Nivestym, Udenyca, Zarxio) [Off-Label] Fulphila, Leukine, Neulasta, Neupogen, Nivestym, Udenyca, and Zarxio are proven and medically necessary when all of the following criteria are met: o Diagnosis of febrile neutropenia; and o Patient is considered high risk for infection-associated complications (e.g., hypotension, acute renal, respiratory, heart failure) with the score of < 21 on Multinational Association of Supportive Care in Cancer (MASCC) Scoring system in patients with cancer and febrile neutropenia

Severe Chronic Neutropenia (SCN) (Neupogen, Nivestym, Zarxio) Neupogen, Nivestym, and Zarxio are proven and medically necessary when all of the following criteria are met: o Diagnosis of SCN (i.e., congenital, cyclic, and idiopathic neutropenias with chronic ANC ≤ 500 neutrophils/mcL); and o Medication is dosed in accordance with the U.S. FDA approved labeling; and o Prescribed by or in consultation with a hematologist or oncologist

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Medical Benefit Drug Policy Updates

Revised Policy Title Effective Date Summary of Changes Coverage Rationale White Blood Cell Jul. 1, 2021 Hematopoietic Syndrome of Acute Radiation Syndrome (Fulphila, Leukine, Colony Stimulating Neulasta, Neupogen, Nivestym, Udenyca, Zarxio) Factors (continued) Fulphila, Leukine, Neulasta, Neupogen, Nivestym, Udenyca, and Zarxio are proven and medically necessary when all of the following criteria are met: o Patient has been acutely exposed to myelosuppressive doses of radiation; and o Medication is dosed in accordance with the U.S. FDA approved labeling; and o Prescribed by or in consultation with a hematologist or oncologist Xiaflex® (Collagenase, Jul. 1, 2021 Coverage Rationale Xiaflex® is proven and medically necessary for the treatment of: Clostridium, Revised coverage criteria for Histolyticum) Dupuytren’s contracture: Dupuytren’s contracture when all of the following criteria are met: Initial Therapy o For initial therapy all of the following: . Patient has diagnosis of Dupuytren’s contracture with a palpable o Replaced criterion requiring: . “Documented contracture cord; and of at least 40 degrees . Patient is 18 years of age or older; and flexion for a . Xiaflex is prescribed and administered by a healthcare provider metacarpophalangeal (MP) experienced in injection procedures of the hand and in the joint contracture or at least treatment of Dupuytren’s contracture; and 20 degrees flexion for a . Documented contracture of at least 20 degrees flexion for a proximal interphalangeal metacarpophalangeal (MP) joint contracture or proximal (PIP) joint contracture” interphalangeal (PIP) joint contracture; and with “documented . Documentation that the flexion deformity meets one of the contracture of at least 20 following: degrees flexion for a – Function is impaired; or – Contracture is progressing; or metacarpophalangeal (MP) – Severe disabling deformity joint contracture or proximal interphalangeal and . (PIP) joint contracture” If two injections (two vials) are requested, they are for one of the following: . “Documentation that the

flexion deformity results in – One cord affecting two joints in the same finger; or

functional limitations” with – Two cords affecting two joints in the same hand

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Medical Benefit Drug Policy Updates

Revised Policy Title Effective Date Summary of Changes Coverage Rationale Xiaflex® (Collagenase, Jul. 1, 2021 “documentation that the and Clostridium, flexion deformity meets . Xiaflex dosing is in accordance with the U.S. Food and Drug Histolyticum) one of the following: Administration (FDA) approved labeling: 0.58 mg per injection into a (continued) function is impaired or palpable cord; and contracture is progressing . The total number of injections does not exceed 3 injections per or severe disabling cord at approximately 4-week intervals; and deformity” . Authorization is for no more than 2 injections in the same hand o Removed criterion requiring o For continuation of therapy, all of the following: patient has not received . Patient has previously received Xiaflex; and surgical treatment on the . Documentation of positive clinical response to Xiaflex; and selected primary joint within . Treatment request is for at least one of the following: the last 90 days – Metacarpophalangeal (MP) or proximal interphalangeal (PIP) Continuation of Therapy contracture remains in affected cord since previous injection and the contracture is > 5 degrees o Removed criterion requiring patient has not received – A different MP or PIP contracture will be injected surgical treatment (e.g., and fasciectomy, fasciotomy) on . If two injections (two vials) are requested, use is for one of the the selected primary joint following: within the last 90 days – One cord affecting two joints in the same finger; or Supporting Information – Two cords affecting two joints in the same hand and • Updated Clinical Evidence, FDA, . The previous treatment was at least 4 weeks ago; and and References sections to reflect . Xiaflex dosing is in accordance with the U.S. Food and Drug the most current information Administration (FDA) approved labeling: 0.58 mg per injection into a palpable cord; and . The total number of injections does not exceed 3 injections per cord at approximately 4-week intervals; and . Authorization is for no more than 2 injections in the same hand

Peyronie’s disease when all of the following criteria are met: o For initial therapy all of the following: . Patient has diagnosis of Peyronie’s disease with both of the following:

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Medical Benefit Drug Policy Updates

Revised Policy Title Effective Date Summary of Changes Coverage Rationale Xiaflex® (Collagenase, Jul. 1, 2021 – Palpable plaque; and Clostridium, – Curvature deformity of greater than or equal to 30 degrees at Histolyticum) the start of therapy (continued) and . Patient is 18 years of age or older; and . Xiaflex is prescribed and administered by a healthcare provider experienced in the treatment of male urological diseases; and . Xiaflex dosing is in accordance with the U.S. Food and Drug Administration (FDA) approved labeling: 0.58 mg per injection into a Peyronie’s plaque; and . Authorization is for no more than 2 injections o For continuation of therapy, all of the following: . Patient has previously received Xiaflex; and . Documentation of positive clinical response to Xiaflex; and . Last treatment was at least 6 weeks ago; and . Documented curvature deformity of ≥ 15 degrees remaining since last treatment cycle; and . Patient has received less than 4 treatment cycles (i.e. less than 8 injections [2 injections per cycle]); and . Xiaflex dosing is in accordance with the U.S. Food and Drug Administration (FDA) approved labeling: 0.58 mg per injection into a Peyronie’s plaque; and . Authorization is for no more than 2 injections

Xiaflex is considered unproven and not medically necessary for any other uses. Xolair® (Omalizumab) Jul. 1, 2021 Coverage Rationale Xolair (omalizumab) for subcutaneous use is proven for: Added language for Nasal Polyps to indicate: Patients with moderate to severe persistent asthma who meet all of the following criteria: o Xolair (omalizumab) for subcutaneous use is proven for o Have a positive skin test or in vitro reactivity to a perennial aeroallergen patients with nasal polyps with o Symptoms inadequately controlled with inhaled corticosteroids inadequate response to nasal o Have a baseline plasma immunoglobulin E (IgE) level greater than or

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Medical Benefit Drug Policy Updates

Revised Policy Title Effective Date Summary of Changes Coverage Rationale Xolair® (Omalizumab) Jul. 1, 2021 corticosteroids, as add-on equal to 30 IU/mL and less than or equal to 1500 IU/mL (continued) maintenance treatment o Xolair is medically necessary Additional Information to Support Medical Necessity Review Where when all of the following Applicable criteria are met: Xolair is medically necessary when all of the following criteria are met: . Diagnosis of nasal polyps; o Diagnosis of moderate or severe asthma; and and o Classification of asthma as uncontrolled or inadequately controlled as . Patient remains defined by at least one of the following: symptomatic despite at . Poor symptom control (e.g., Asthma Control Questionnaire [ACQ] least a 2-week trial of or score consistently greater than 1.5 or Asthma Control Test [ACT] history of contraindication score consistently less than 20); or or intolerance to nasal . Two or more bursts of systemic corticosteroids for at least 3 days corticosteroids [e.g., each in the previous 12 months; or Flonase (fluticasone), . Asthma-related emergency treatment (e.g., emergency room visit, Rhinocort (budesonide), hospital admission, or unscheduled physician’s office visit for Nasonex (mometasone)]; nebulizer or other urgent treatment); or and . Airflow limitation (e.g., after appropriate bronchodilator withhold . Patient is currently on and forced expiratory volume in 1 second [FEV1] less than 80% will continue current predicted [in the face of reduced FEV1/forced vital capacity [FVC] maintenance therapy; and defined as less than the lower limit of normal]); or . Patient has bilateral polyps . Patient is currently dependent on maintenance therapy with oral as determined by a nasal corticosteroids for the treatment of asthma polyp score (NPS) ≥ 5 with and NPS ≥ 2 in each nostril; o One of the following: and . Baseline (pre-omalizumab treatment) serum total IgE level greater . Patient has a weekly than or equal to 30 IU/mL and less than or equal to 1500 IU/mL; or average of nasal . Patient is currently dependent on maintenance therapy with oral congestion score (NCS) > corticosteroids for the treatment of asthma 1; and and . Xolair dosing for nasal o Positive skin test or in vitro reactivity to a perennial aeroallergen; and polyps is in accordance o Used in combination with one of the following: with the United States . One maximally dosed (appropriately adjusted for age) combination Food and Drug inhaled corticosteroid (ICS)/long-acting beta2-agonist (LABA)

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Medical Benefit Drug Policy Updates

Revised Policy Title Effective Date Summary of Changes Coverage Rationale Xolair® (Omalizumab) Jul. 1, 2021 Administration approved product [e.g., Advair/AirDuo Respiclick (fluticasone (continued) labeling; and propionate/salmeterol), Symbicort (budesonide/formoterol), Breo . Prescribed by or in Ellipta (fluticasone furoate/vilanterol)]; or consultation with an . Combination therapy including both of the following: allergist/immunologist/EN One high-dose (appropriately adjusted for age) ICS product T/ pulmonologist; and [e.g., ciclesonide (Alvesco®), mometasone furoate (Asmanex®), . Initial authorization will be beclomethasone dipropionate (QVAR®)]; and for no more than 6 months One additional asthma controller medication [e.g., LABA - ® ® o For patients currently on Xolair olodaterol (Striverdi ) or indacaterol (Arcapta ); leukotriene for the treatment of nasal receptor antagonist – montelukast (Singulair®); theophylline] polyps, authorization for and continued use will be approved o Patient is not receiving Xolair in combination with either of the following: based on all of the following . Anti-interleukin 4 therapy [e.g., Dupixent (dupilumab)] criteria: . Anti-interleukin 5 therapy [e.g., Nucala (mepolizumab), Cinqair . Documentation of positive (resilizumab), Fasenra (benralizumab)] clinical response (e.g., and reduction in polyp size, o Xolair dosing for moderate to severe persistent asthma is in accordance decreased congestion, with the United States Food and Drug Administration approved labeling; improved sense of smell); and and o Prescribed by or in consultation with an allergist/immunologist or . Xolair dosing for nasal pulmonologist; and polyps is in accordance o Initial authorization will be for no more than 6 months with the United States Food and Drug Reauthorization/Continuation of Care Criteria Administration approved For patients currently on Xolair for the treatment of moderate to severe labeling; and persistent asthma, authorization for continued use will be approved . Prescribed by or in based on all of the following criteria: consultation with an o Documentation of positive clinical response as demonstrated by at least allergist/immunologist/EN one of the following: T/ pulmonologist; and . Reduction in the frequency of exacerbations . Reauthorization will be for . Decreased utilization of rescue medications no more than 12 months . Increase in percent predicted FEV1 from pretreatment baseline Applicable Codes . Reduction in severity of frequency of asthma-related symptoms

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Medical Benefit Drug Policy Updates

Revised Policy Title Effective Date Summary of Changes Coverage Rationale Xolair® (Omalizumab) Jul. 1, 2021 Added ICD-10 diagnosis codes (e.g., wheezing, shortness of breath, coughing, etc.) (continued) J33.0, J33.1, J33.8, and J33.9 and Added Maximum Allowed o Used in combination with an ICS-containing controller medication; and Quantities by HCPCS Units for the diagnosis of nasal polyps: o Patient is not receiving Xolair in combination with either of the following: . o Maximum dosage per Anti-interleukin 4 therapy [e.g., Dupixent (dupilumab)] administration: 600 mg . Anti-interleukin 5 therapy [e.g., Nucala (mepolizumab), Cinqair o HCPCS code: J2357 (resilizumab), Fasenra (benralizumab)] o Maximum allowed: 120 HCPCS and units (5 mg per unit) o Xolair dosing for moderate to severe persistent asthma is in accordance Added Maximum Allowed with the United States Food and Drug Administration approved labeling; Quantities by National Drug Code and (NDC) Units for the diagnosis of o Prescribed by or in consultation with allergist/immunologist or nasal polyps: pulmonologist; and Reauthorization will be for no more than 12 months o How supplied: 150 mg vials o

o National Drug Code (NDC): 50242-0040-62 Patients with chronic urticaria who continue to remain symptomatic despite H1 antihistamine [e.g., cetirizine (Zyrtec), fexofenadine (Allegra)] o Maximum allowed: 4 vials Supporting Information treatment3 Updated Clinical Evidence, FDA, and References sections to reflect Additional Information to Support Medical Necessity Review Where the most current information Applicable Xolair is medically necessary when all of the following criteria are met:

o Diagnosis of chronic urticarial; and

o One of the following: . Patient remains symptomatic despite at least a 2-week trial of, or history of contraindication or intolerance to, two H1-antihistamines [e.g., Allegra (fexofenadine), Benadryl (diphenhydramine), Claritin (loratadine)];* or . Patient remains symptomatic despite at least a 2-week trial of, or history of contraindication or intolerance to both of the following taken in combination: A second generation H1-antihistamine [e.g., Allegra

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Medical Benefit Drug Policy Updates

Revised Policy Title Effective Date Summary of Changes Coverage Rationale Xolair® (Omalizumab) Jul. 1, 2021 (fexofenadine), Claritin (loratadine), Zyrtec (cetirizine)]; and (continued) One of the following: • Different second generation H1-antihistamine [e.g., Allegra (fexofenadine), Claritin (loratadine), Zyrtec (cetirizine)] • First generation H1-antihistamine [e.g., Benadryl (diphenhydramine), Chlor-Trimeton (chlorpheniramine), Vistaril (hydroxyzine)]* • H2-antihistamine [e.g., Pepcid (famotidine), Tagamet HB (cimetidine), Zantac (ranitidine)] • Leukotriene modifier [e.g., Singulair (montelukast)] and o Xolair dosing for chronic urticaria is in accordance with the United States Food and Drug Administration approved labeling; and o Prescribed by or in consultation with an allergist/immunologist or dermatologist; and o Initial authorization will be for no more than 6 months

Reauthorization/Continuation of Care Criteria For patients currently on Xolair for the treatment of chronic urticaria, authorization for continued use will be approved based on all of the following criteria:

o Documentation of positive clinical response (e.g., reduction in exacerbations, itch severity, hives); and

o Xolair dosing for chronic urticaria is in accordance with the United States Food and Drug Administration approved labeling; and

o Prescribed by or in consultation with allergist/immunologist or dermatologist; and

o Reauthorization will be for no more than 12 months

*Note: Patients 65 years of age and older in whom first generation H1- antihistamines are considered high risk medications to be avoided (e.g., Beers criteria, HEDIS) should be directed to try alternatives that are not considered high risk.

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Medical Benefit Drug Policy Updates

Revised Policy Title Effective Date Summary of Changes Coverage Rationale Xolair® (Omalizumab) Jul. 1, 2021 Patients with nasal polyps with inadequate response to nasal (continued) corticosteroids, as add-on maintenance treatment.

Additional Information to Support Medical Necessity Review Where Applicable Xolair is medically necessary when all of the following criteria are met: o Diagnosis of nasal polyps; and o Patient remains symptomatic despite at least a 2-week trial of, or history of contraindication or intolerance to nasal corticosteroids [e.g., Flonase (fluticasone), Rhinocort (budesonide), Nasonex (mometasone)]; and o Patient currently on and will continue maintenance therapy; and o Patient has bilateral polyps as determined by a nasal polyp score (NPS) ≥ 5 with NPS ≥ 2 in each nostril; and o Patient has a weekly average of nasal congestion score (NCS) > 1; and o Xolair dosing for nasal polyps is in accordance with the United States Food and Drug Administration approved labeling; and o Prescribed by or in consultation with an allergist/immunologist/ENT/pulmonologist; and o Initial authorization will be for no more than 6 months.

Reauthorization/Continuation of Care Criteria For patients currently on Xolair for the treatment of nasal polyps, authorization for continued use will be approved based on all of the following criteria: o Documentation of positive clinical response (e.g., reduction in polyp size, decreased congestion, improved sense of smell); and o Xolair dosing for nasal polyps is in accordance with the United States Food and Drug Administration approved labeling; and o Prescribed by or in consultation with allergist/immunologist/ENT/pulmonologist; and o Reauthorization will be for no more than 12 months.

Xolair is unproven and not medically necessary in the following:

Page 55 of 65 UnitedHealthcare Community Plan of Kentucky Medical Policy Update Bulletin: June 2021

Medical Benefit Drug Policy Updates

Revised Policy Title Effective Date Summary of Changes Coverage Rationale Xolair® (Omalizumab) Jul. 1, 2021 Seasonal allergic rhinitis (continued) Perennial allergic rhinitis Atopic dermatitis Peanut allergy Acute bronchospasm or status asthmaticus Zolgensma® Jul. 1, 2021 Coverage Rationale Zolgensma is proven and medically necessary for one treatment per lifetime (Onasemnogene Revised list of Additional for the treatment of spinal muscular atrophy (SMA) in patients who meet all Abeparvovec-Xioi) Information Relevant to the Review of the following criteria: Process but Not Impacting the Submission of medical records (e.g., chart notes, laboratory values) Determination of Medical Necessity confirming the mutation or deletion of genes in chromosome 5q resulting in one of the following: o Removed/replaced: . Physician attests that the o Homozygous gene deletion or mutation of SMN1 gene (e.g., homozygous deletion of exon 7 at locus 5q13); or patient will be assessed via Compound heterozygous mutation of SMN1 gene (e.g., deletion of an age appropriate o validated exam scale SMN1 exon 7 [allele 1] and mutation of SMN1 [allele 2])

within two weeks of and

Zolgensma administration One of the following: to establish a baseline o Diagnosis of symptomatic SMA by a neurologist with expertise in the functional assessment diagnosis of SMA; or Both of the following: o Added: o . Physician attests that the . Diagnosis of SMA based on the results of SMA newborn screening; and patient will be assessed via . Submission of medical records (e.g., chart notes, laboratory values) the CHOP INTEND scale to establish a baseline confirming that patient has 4 copies or less of SMN2 gene

functional assessment and

within the following For use in a neonatal patient born prematurely, the full-term gestational age timelines: has been reached; and For patients greater One of the following: than 2 months of age o Both of the following: at the time of . Patient is less than or equal to 6 months of age . Zolgensma Patient does not have advanced SMA at baseline (e.g., complete paralysis of limbs) administration, a or baseline CHOP

Page 56 of 65 UnitedHealthcare Community Plan of Kentucky Medical Policy Update Bulletin: June 2021

Medical Benefit Drug Policy Updates

Revised Policy Title Effective Date Summary of Changes Coverage Rationale ® Zolgensma Jul. 1, 2021 INTEND score will be o All of the following: (Onasemnogene assessed within the 2 . Patient is greater than 6 months of age, but less than 2 years of age; Abeparvovec-Xioi) weeks prior to and (continued) Zolgensma . One of the following: administration Both of the following: For patients less than • Patient has previously received SMN modifying therapy or equal to 2 months [e.g., Spinraza (nusinersen), Evrysdi (risdiplam)] for the of age at the time of treatment of SMA before 6 months of age with positive Zolgensma clinical response; and administration, a • Submission of medical records (e.g., chart notes, baseline CHOP laboratory values) confirming patient does not have INTEND score will be advanced SMA as defined by the fact that the patient has assessed within the 2 not shown evidence of clinical decline while receiving SMN weeks prior to or the 2 modifying therapy [e.g., Spinraza (nusinersen), Evrysdi weeks following (risdiplam)] Zolgensma or administration Both of the following: Supporting Information • Patient has previously received SMN modifying therapy Updated Clinical Evidence and [e.g., Spinraza (nusinersen), Evrysdi (risdiplam)] for the References sections to reflect the treatment of later-onset SMA before 2 years of age with most current information positive clinical response; and • Submission of medical records (e.g., chart notes, laboratory values) confirming patient does not have advanced SMA as defined by the fact that the patient has not shown evidence of clinical decline while receiving SMN modifying therapy [e.g., Spinraza (nusinersen), Evrysdi (risdiplam)] or Patient has recently been diagnosed with symptomatic later- onset SMA within the previous 6 months and Patient is not dependent on either of the following: o Invasive ventilation or tracheostomy

Page 57 of 65 UnitedHealthcare Community Plan of Kentucky Medical Policy Update Bulletin: June 2021

Medical Benefit Drug Policy Updates

Revised Policy Title Effective Date Summary of Changes Coverage Rationale ® Zolgensma Jul. 1, 2021 o Use of non-invasive ventilation beyond use for naps and nighttime sleep (Onasemnogene and Abeparvovec-Xioi) Zolgensma is prescribed by a neurologist with expertise in the treatment of (continued) SMA; and Patient is not to receive routine concomitant SMN modifying therapy [e.g., Spinraza (nusinersen), Evrysdi (risdiplam)] (patient’s medical record will be reviewed and any current authorizations for SMN modifying therapy will be terminated upon Zolgensma approval); and Patient does not have an elevated anti-AAV9 antibody titer above 1:50*; and Patient is less than 13.5 kg; and Patient will receive prophylactic prednisolone (or glucocorticoid equivalent) prior to and following receipt of Zolgensma in accordance with the United States Food and Drug Administration (FDA) approved Zolgensma labeling; and Patient will receive Zolgensma intravenously in accordance with the FDA approved labeling, 1.1 x 10 vector genomes (vg) per kg of body weight; and Patient has never received Zolgensma treatment in their lifetime; and Authorization will be for no longer than 14 days from approval or until 2 years of age, whichever is first

Additional Information Relevant to the Review Process but Not Impacting the Determination of Medical Necessity 1. Physician attests that the patient, while under the care of the physician, will be assessed by one of the following exam scales during subsequent office visits*† o Children’s Hospital of Philadelphia Infant Test of Neuromuscular Disorders (CHOP INTEND) scale during subsequent office visits while the patient is 2 to 3 years of age or younger; or o Hammersmith Functional Motor Scale Expanded (HFMSE) during subsequent office visits while the patient is 2 to 3 years of age or older and

Page 58 of 65 UnitedHealthcare Community Plan of Kentucky Medical Policy Update Bulletin: June 2021

Medical Benefit Drug Policy Updates

Revised Policy Title Effective Date Summary of Changes Coverage Rationale Zolgensma® Jul. 1, 2021 Physician attests that the patient will be assessed via the CHOP INTEND (Onasemnogene scale to establish a baseline functional assessment within the following Abeparvovec-Xioi) timelines:*† (continued) o For patients greater than 2 months of age at the time of Zolgensma administration, a baseline CHOP INTEND score will be assessed within the 2 weeks prior to Zolgensma administration; or o For patients less than or equal to 2 months of age at the time of Zolgensma administration, a baseline CHOP INTEND score will be assessed within the 2 weeks prior to, or the 2 weeks following Zolgensma administration and Physician acknowledges that UnitedHealthcare may request documentation, not more frequently than biannually, and not for a period to exceed 3 years, of follow-up patient assessment(s) including, but not necessarily limited to, serial CHOP INTEND or HFMSE assessments while the patient is under the care of the physician*†

*UnitedHealthcare has established an internal registry where this is a potential outcome measure. This registry has been established to prioritize early access to an SMA therapy with promising early clinical evidence, yet with unique commercialization characteristics, while ensuring appropriate management as clinical data matures.

†For quality purposes only, this information will not be considered as part of the individual coverage decision.

Zolgensma is not proven or medically necessary for: The treatment of pre-symptomatic patients diagnosed by newborn screening who have more than 4 copies of the SMN2 gene; or The treatment of symptomatic later-onset SMA older than 2 years of age; or SMA without chromosome 5q mutations or deletions; or The routine combination treatment of SMA with concomitant SMN modifying therapy

Page 59 of 65 UnitedHealthcare Community Plan of Kentucky Medical Policy Update Bulletin: June 2021

Coverage Determination Guideline Updates

Updated Policy Title Effective Date Summary of Changes Breast Reconstruction Jun. 1, 2021 Applicable Codes Post Mastectomy and Added CPT codes 15771 and 15772

Poland Syndrome Rhinoplasty and Other Jun. 1, 2021 Coverage Rationale Nasal Surgeries Added language to clarify Rhinophyma Excision (CPT Code 30120) is considered reconstructive and medically necessary when all of the [listed] criteria are present Definitions • Updated definition of “Rhinitis Medicamentosa”

Revised Policy Title Effective Date Summary of Changes Coverage Rationale Ambulance Services Jul. 1, 2021 Coverage Rationale Indications for Coverage Emergency Air Ambulance Emergency Ambulance (Ground, Water, or Air) Revised coverage criteria for Coverage includes Medically Necessary Emergency Ambulance Transportation Emergency air ambulance (including wait time and treatment at the scene) by a licensed ambulance service transportation; added criterion from the location of the sudden illness or injury, to the nearest hospital in the requiring: Medical Service Area where Emergency Health Care Services can be performed. Inaccessibility to ground o ambulance transport or The following Emergency ambulance services are covered: extended length of time To the nearest hospital that can provide services appropriate to the covered required to transport the person's illness or injury. member via ground ambulance To the nearest neonatal special care unit for newborn infants treatment of transportation could endanger illness, injuries, congenital birth defects, or complications of premature birth the member that require that level of care. Weather or traffic conditions o Ground ambulance transportation requiring basic life support or advanced make ground ambulance life support transportation impractical, Supplies that are needed for advanced life support or basic life support to impossible, or overly time stabilize a member’s medical condition consuming Treatment at the scene (paramedic services) without ambulance Non-Emergency Ambulance transportation (Ground or Air) Between Faciliites Wait time associated with covered Ambulance Transportation Updated language to indicate Transportation to a hospital that provides a required higher level of care that coverage guidelines apply to non-

Page 60 of 65 UnitedHealthcare Community Plan of Kentucky Medical Policy Update Bulletin: June 2021

Coverage Determination Guideline Updates

Revised Policy Title Effective Date Summary of Changes Coverage Rationale Ambulance Services Jul. 1, 2021 Emergency ambulance was not available at the original hospital (continued) transportation by a licensed ambulance service (either ground Emergency Air Ambulance or air ambulance, as As a general guideline, when it would take a ground ambulance 30-60 minutes UnitedHealthcare determines or more to transport a member whose medical condition at the time of pick-up appropriate) between facilities required immediate and rapid transport due to the nature and/or severity of the member’s illness/injury, air transportation may be appropriate.

Emergency Air Ambulance Transportation should meet the following criteria:

Those situations in which the covered person is in a location that cannot be reached by ground ambulance.

The member’s destination is an acute care hospital; and

The member’s condition is such that the ground ambulance (basic or advanced life support) would endanger the member’s life or health; or

Emergency Air ambulance transportation requiring advanced life support; or

Inaccessibility to ground ambulance transport or extended length of time

required to transport the member via ground ambulance transportation

could endanger the member; or

Weather or traffic conditions make ground ambulance transportation

impractical, impossible, or overly time consuming

Non-Emergency Ambulance (Ground or Air) Between Facilities Coverage includes non-Emergency ambulance transportation by a licensed ambulance service (either ground or air ambulance, as UnitedHealthcare determines appropriate) between facilities only when the transport meets one of the following: A non-Emergency ambulance service to a provider within the Medical Service Area shall be covered if: o The member’s medical condition warrants transport by stretcher; o The member is traveling to or from a Medicaid-covered service, exclusive of a pharmary service; and o The service is the least expensive available transportation for the member’s need.

Page 61 of 65 UnitedHealthcare Community Plan of Kentucky Medical Policy Update Bulletin: June 2021

Coverage Determination Guideline Updates

Revised Policy Title Effective Date Summary of Changes Coverage Rationale Ambulance Services Jul. 1, 2021 A non-Emergency ambulance service to a provider outside the Medical (continued) Service Area shall be covered if o The criteria specificed above are satisfied; o The medical service required by the member is not available in the Medical Service Area; and o The member is referred by a physician.

Coverage Limitations and Exclusions The following services are not eligible for coverage:

Ambulance services from providers that are not properly licensed to be performing the ambulance services rendered.

Air Ambulance Transportation that does not meet the covered indications in the Emergency Air Ambulance Transportation criteria listed above.

Air Ambulance services:

Outside of the 50 United States and the District of Columbia; or o o From a country or territory outside the United States to a location within the 50 United States or the District of Columbia; or

o From a location within the 50 United States or the District of Columbia to a country or territory outside the United States.

Ambulance Transportation to a home, residential, domiciliary or custodial facility that does not meet the Non-Emergency Ambulance criteria above is not covered..

Ambulance Transportation for member convenience or other miscellaneous reasons for member and/or family. Examples include but are not limited to:

o Member wants to be at a certain hospital or facility for personal/preference reasons

o Member is in foreign country, or out of state, and wants to come home for a surgical procedure or treatment (this includes those recently discharged from inpatient care)

o Member is going for a routine service and is medically able to use another mode of transportation

o Member is deceased and family wants transportation to the coroner’s

Page 62 of 65 UnitedHealthcare Community Plan of Kentucky Medical Policy Update Bulletin: June 2021

Coverage Determination Guideline Updates

Revised Policy Title Effective Date Summary of Changes Coverage Rationale Ambulance Services Jul. 1, 2021 office or mortuary (continued) Ambulance Transportation deemed not appropriate that do not meet above Indications of Coverage criteria. Examples include but are not limited to: o Hospital to home o Home to physician’s office o Home (e.g., residence, nursing home, domiciliary or custodial facility) to a hospital for a scheduled service If the member is at a Skilled Nursing Facility/Inpatient Rehabilitation Facility and has met the annual day/visit limit on Skilled Nursing Facility/Inpatient Rehabilitation Facility Services, nonemergent ambulance transports (during the non-covered days) are not eligible.

For nonemergency medical transportation, please refer to Kentucky Administrative Rules 3:066.

Documentation Requirements for Determination of Coverage

For Emergency Ambulance Services:

Documentation must be maintained for post payment review to indicate

immediate emergency medical attention was provided in the emergency

room.

An Emergency ambulance service to an Appropriate Medical Facility or

Provider other than a hospital emergency room shall require documentation from the attending physician of:

Medical Necessity; o o Absence of a hospital emergency room in the Medical Service Area; and

Delivery of emergency care to the patient. o

The necessity for an Ambulance Transportation service shall be:

Determined by The Plan; and

Based upon a statement of medical necessity by an attending physician which shall: o Be maintained on file by the transportation provider for a period of five

Page 63 of 65 UnitedHealthcare Community Plan of Kentucky Medical Policy Update Bulletin: June 2021

Coverage Determination Guideline Updates

Revised Policy Title Effective Date Summary of Changes Coverage Rationale Ambulance Services Jul. 1, 2021 (5) years; and (continued) o Include the following information: . Verification by the provider of the: Date of ambulance service; Patient's name; Patient's Medicaid identification number; Patient's address; Origin of ambulance service; and Destination of ambulance service; and . A signed and dated statement by the attending physician, or other medical professional carrying out the orders of the attending physician, which verifies the patient's diagnosis and whether or not the patient: Received treatment in an outpatient setting following transport; Required admission to the hospital following transport; Transferred from one (1) medical facility to another; Was confined to bed before and after transport; Required movement by stretcher; or Had a medical condition which contraindicated transportation by means other than an ambulance Chiropractic Services Jul. 1, 2021 Coverage Rationale Indications for Coverage Removed reference to “Medical Refer to the Kentucky Administrative Regulations 907 KAR 3:125 for Necessary criteria” from Indications Chiropractic Services. for Coverage Applicable Codes Coverage Limitations and Exclusions Physical Therapy Chiropractic Services limited to twenty-six (26) visits per recipient per twelve (12) • Added CPT code 97014 month period. • Removed HCPCS codes G0281 and G0283 Evaluation and Treatment • Revised description for CPT codes 99204, 99211, and 99212

Page 64 of 65 UnitedHealthcare Community Plan of Kentucky Medical Policy Update Bulletin: June 2021

General Information

The inclusion of a health service (e.g., test, drug, device or procedure) in this Policy Update Classifications bulletin indicates only that UnitedHealthcare is adopting a new policy and/or New updated, revised, replaced or retired an existing policy; it does not imply that UnitedHealthcare provides coverage for the health service. Note that most New clinical coverage criteria have been adopted for a health service (e.g., test, benefit plan documents exclude from benefit coverage health services identified drug, device or procedure) as investigational or unproven/not medically necessary. Physicians and other health care professionals may not seek or collect payment from a member for Updated services not covered by the applicable benefit plan unless first obtaining the An existing policy has been reviewed and changes have not been made to the member’s written consent, acknowledging that the service is not covered by the clinical coverage criteria; however, items such as the clinical evidence, FDA benefit plan and that they will be billed directly for the service. information, and/or list(s) of applicable codes may have been updated

Note: The absence of a policy does not automatically indicate or imply coverage. Revised As always, coverage for a health service must be determined in accordance with An existing policy has been reviewed and revisions have been made to the the member’s benefit plan and any applicable federal or state regulatory clinical coverage criteria requirements. Additionally, UnitedHealthcare reserves the right to review the clinical evidence supporting the safety and effectiveness of a medical technology prior to rendering a coverage determination. Replaced An existing policy has been replaced with a new or different policy UnitedHealthcare respects the expertise of the physicians, health care professionals, and their staff who participate in our network. Our goal is to Retired support you and your patients in making the most informed decisions regarding The health service(s) addressed in the policy are no longer being managed or the choice of quality and cost-effective care, and to support practice staff with a are considered to be proven/medically necessary and are therefore not simple and predictable administrative experience. The Medical Policy Update excluded as unproven/not medically necessary services, unless coverage Bulletin was developed to share important information regarding guidelines or criteria are otherwise documented in another policy UnitedHealthcare Community Plan of Kentucky Medical Policy, Medical Benefit Drug Policy, Coverage Determination Guideline, and Utilization Review Guideline updates. When information in this bulletin conflicts with applicable state and/or federal law, UnitedHealthcare follows such applicable federal and/or state law.

The complete library of UnitedHealthcare Community Plan of Kentucky Medical Policies, Medical Benefit Drug Policies, Coverage Determination Guidelines, and Utilization Review Guidelines is available at UHCprovider.com/Kentucky > Medicaid (Community Plan) > Current Policies and Clinical Guidelines > UnitedHealthcare Community Plan of Kentucky Medical & Drug Policies and Coverage Determination Guidelines.

Page 65 of 65 UnitedHealthcare Community Plan of Kentucky Medical Policy Update Bulletin: June 2021