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Medical Policy Scenesse (afamelanotide) for Treatment of Erythropoietic Protoporphyria (EPP) Table of Contents • Policy: Commercial • Description • Information Pertaining to All Policies
• Authorization Information • Policy History • References
• Coding Information

• Endnotes Policy Number: 077 BCBSA Reference Number: N/A NCD/LCD: N/A Related Policies
Elzonris Scenesse (afamelanotide) for the treatment of Erythropoietic Protoporphyria Prior Authorization Form #160 Policy1 Commercial Members: Managed Care (HMO and POS), PPO, and Indemnity
Medicare HMO BlueSM and Medicare PPO BlueSM Members

Scenesse (afamelanotide) may be MEDICALLY NECESSARY for the treatment of erythropoietic protoporphyria (EPP) in adults 18 and over when all of the following criteria are met:
• History of phototoxic reaction with sun exposure, AND • No evidence of significant liver involvement, AND • Biochemically confirmed diagnosis of protoporphyria (total protoporphyrin level >500ug/dl (generally 1000-5000ug/dl), AND • The prescriber is a specialist in the area of the patient’s diagnosis (eg., dermatology), or the prescriber has consulted with a specialist in the area of the patient’s diagnosis.

Retreatment is evaluated on an annual basis and may be MEDICALLY NECESSARY when all of the following criteria are met: • Increase in pain free time during light/sun exposure, AND • Reduction in number of phototoxic reactions or decrease in severity of phototoxic reactions from pretreatment baseline.

Prior Authorization Information
Inpatient • For services described in this policy, precertification/preauthorization IS REQUIRED for all products if the procedure is performed inpatient.
Outpatient

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• For services described in this policy, see below for products where prior authorization might be required if the procedure is performed outpatient.

Outpatient Commercial Managed Care (HMO and POS) Prior authorization is required. Commercial PPO and Indemnity Prior authorization is required. Medicare HMO BlueSM Prior authorization is required. Medicare PPO BlueSM Prior authorization is required.

Requesting Prior Authorization Using Authorization Manager Providers will need to use Authorization Manager to submit initial authorization requests for services. Authorization Manager, available 24/7, is the quickest way to review authorization requirements, request authorizations, submit clinical documentation, check existing case status, and view/print the decision letter. For commercial members, the requests must meet medical policy guidelines.

To ensure the request is processed accurately and quickly: • Enter the facility’s NPI or provider ID for where services are being performed. • Enter the appropriate surgeon’s NPI or provider ID as the servicing provider, not the billing group.

Authorization Manager Resources • Refer to our Authorization Manager page for tips, guides, and video demonstrations.

Complete Prior Authorization Request Form for Scenesse (160) using Authorization Manager.

For out of network providers: Requests should still be faxed to 888-973-0726.

CPT Codes / HCPCS Codes / ICD Codes
Inclusion or exclusion of a code does not constitute or imply member coverage or provider reimbursement. Please refer to the member’s contract benefits in effect at the time of service to determine coverage or non-coverage as it applies to an individual member.

Providers should report all services using the most up-to-date industry-standard procedure, revenue, and diagnosis codes, including modifiers where applicable.

The following codes are included below for informational purposes only; this is not an all-inclusive list.

The above medical necessity criteria MUST be met for the following codes to be covered for Commercial Members: Managed Care (HMO and POS), PPO, Indemnity, Medicare HMO Blue and Medicare PPO Blue:

HCPCS Codes:
HCPCS codes: Code Description J7352 Afamelanotide implant, 1 mg

ICD-10 Procedure Codes ICD-10-PCS procedure codes: Code Description 0HHPXYZ Insertion of Other Device into Skin, External Approach Description Erythropoietic protoporphyria (EPP) is a rare condition that results in the build up of protoporphyrin in the blood marrow, red blood cells, blood plasma, skin and liver. EPP is caused by a genetic mutation on the

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FECH gene, which is responsible for the normal production of heme. The build-up of protoporphyrin can cause extreme skin reactions to sunlight, including severe pain, burns and blisters, and edema. Protoporphyrin is excreted through the liver putting patients with EPP at risk of chronic liver disease and recurrent gallstones. In extreme cases, patients may experience rapid acute liver failure. EPP affects 75,000-200,000 people worldwide and is often diagnosed in infancy and early childhood after noticeable symptoms due to sun exposure. Genetic testing can provide confirmatory diagnosis.

Patients with EPP are recommended to reduce exposure to sunlight and avoid sun exposure whenever possible. In some patients, the use of tanning creams demonstrated increase tolerance to sun exposure by increasing the amount of pigmentation in the skin. Protoporphyrin levels should be monitored annually to assess for damage to the liver. Patients with severe cases of EPP may require liver and bone marrow transplants if there is significant liver damage. There are currently no oral or topical medications available for the treatment of EPP.

In October of 2019, the FDA approved a subcutaneous injectable medication that increases the pigmentation of the skin and allows patients to have longer periods of sun exposure without reaction. Scenesse is indicated for patients with confirmatory genetic testing and into the torso and is administered every 2 months. For individuals with EPP without significant liver involvement, the evidence includes three randomized controlled trials evaluating the effect of Scenesse in reducing phototoxic reactions and pain. In all of the available studies, patients were administered with either Scenesse or a “vehicle.” In the Scenesse groups, patients reported no pain after spending a median of 6 hours per day outside in direct sunlight. In the control groups, patients reported “no pain” over the median time of .75 hours per day.

Summary
Erythropoietic Protoporphyria is a rare, genetic mutation that causes an abnormal production of protoporphyria in the bone marrow, red blood cells, plasma, skin and liver. As a result, patients experience phototoxic reactions when exposed to the sun. While symptoms vary, patients can experience pain, reddening of skin, blisters and burns to exposed skin and edema. In severe cases, patients might experience disease of the liver. There are currently no oral or topical medications that are approved to treat EPP. The FDA has approved a subcutaneous implant, Scenesse (afamelanotide), that is injected every 2 months in adult patients without significant liver involvement.

The evidence includes three randomized controlled trials. Of the 167 patients included, 86 patients with EPP without significant liver involvement received Scenesse and were evaluated on the primary endpoint of number of hours spent outside. Patients documented degree of pain during sun exposure each day. Over the course of 270 days, patients in the treatment group reported significant improvement over the controlled group for number of hours spent outside without symptom reaction and with reduced pain. The evidence is sufficient to determine that the technology results in a meaningful improvement in the net health outcome.

Policy History Date Action 11/2024 Annual policy review. Description, summary and references reviewed. Policy statements unchanged. 11/1/24. 10/2023 Annual policy review. Description, summary and references reviewed. Policy statements unchanged. 10/1/23. 9/2023 Policy clarified to include prior authorization requests using Authorization Manager.
10/2022 Annual policy review. Description, summary and references reviewed. Policy statements unchanged. 10/1/22.
2/2021 New medically necessary and investigational indications described. Clarified coding information. Effective 2/1/2021.
1/2021 Clarified coding information 5/2020 New medical policy describing investigational indications. Effective 5/1/2020.
Information Pertaining to All Blue Cross Blue Shield Medical Policies

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Click on any of the following terms to access the relevant information: Medical Policy Terms of Use Managed Care Guidelines Indemnity/PPO Guidelines Clinical Exception Process Medical Technology Assessment Guidelines

References

1. Clinuvel Pharmaceuticals, Inc. Prescribing Label: SCENESSE (afamelotide) implant, for subcutaneous use. Initial U.S. Approval: 2019.
2. Scenesse. Clinicaltrial.gov. Accessed on 1/20/2020. Available at: https://clinicaltrials.gov/ct2/show/NCT01097044?cond=scenesse&draw=2&rank=7
3. Scenesse. Clinicaltrial.gov. Accessed on 1/20/2020. Available at: https://clinicaltrials.gov/ct2/show/NCT04053270?cond=scenesse&draw=2&rank=5

4. National Institute of Health. Genetic and Rare Disease Information Center. Erythropoietic Protoporphyria. Accessed 1/3/2020. https://rarediseases.info.nih.gov/diseases/4527/erythropoietic- protoporphyria

   Endnotes

   1 Based on expert opinion