Subject: dichlorphenamide-Keveyis-Ormalvi SGM 2491-A P2025

Drug

KEVEYIS  (dichlorphenamide)

ORMALVI  (dichlorphenamide)

Policy:

Indications

The indications below including FDA-approved indications and compendial uses are considered a covered benefit provided that all the approval criteria are met and the member has no exclusions to the prescribed therapy.

FDA-approved Indications^1-3

Treatment of primary hyperkalemic periodic paralysis, primary hypokalemic periodic paralysis, and related variants.

All other indications are considered experimental/investigational and not medically necessary.

Coverage Criteria

Primary Hypokalemic Periodic Paralysis^1-6

Authorization of 60 days may be granted for treatment of primary hypokalemic periodic paralysis when all of the following criteria are met:

• The diagnosis was supported by at least one of the following:
  • Genetic test results, or
  • Member has a family history of primary hypokalemic periodic paralysis, or
  • Member’s attacks are associated with hypokalemia AND both Andersen-Tawil syndrome and thyrotoxic periodic paralysis have been ruled out.
• Member had a trial and suboptimal response to treatment with acetazolamide.

Primary Hyperkalemic Periodic Paralysis^1-6

Authorization of 60 days may be granted for treatment of primary hyperkalemic periodic paralysis when all of the following criteria are met:

• The diagnosis was supported by at least one of the following:
  • Genetic test results, or
  • Member has a family history of primary hyperkalemic periodic paralysis, or
  • Member’s attacks are associated with hyperkalemia AND Andersen-Tawil syndrome has been ruled out.
• Member had a trial and suboptimal response to treatment with acetazolamide.

Continuation of Therapy

Authorization of 12 months may be granted to members who have demonstrated a response to therapy as evidenced by an improvement in their condition (e.g. decrease in the number or severity of attacks).

Place of Service:

Outpatient

The above policy is based on the following references:

1. Keveyis [package insert]. Chicago, IL: Xeris Pharmaceuticals, Inc.; August 2024.
2. Dichlorphenamide [package insert]. Basking Ridge, NJ: Torrent Pharma Inc.; January 2023.
3. Ormalvi [package insert]. Cambridge, UK: Cycle Pharmaceuticals LTD; February 2024.
4. Levitt JO. Practical aspects in the management of hypokalemic periodic paralysis. J Transl Med. 2008;6:18.
5. Charles G, Zheng C, Lehmann-Horn F, Jurkat-Rott K, Levitt J. Characterization of hyperkalemic periodic paralysis: a survey of genetically diagnosed individuals. J Neurol. 2013;260(10):2606-2613.
6. Statland JM, Fontaine B, Hanna MG, et al. Review of the diagnosis and treatment of periodic paralysis. Muscle Nerve. 2018;57(4):522-530.

Copyright Aetna Inc. All rights reserved. Pharmacy Clinical Policy Bulletins are developed by Aetna to assist in administering plan benefits and constitute neither offers of coverage nor medical advice. This Clinical Policy Bulletin contains only a partial, general description of plan or program benefits and does not constitute a contract. Aetna does not provide health care services and, therefore, cannot guarantee any results or outcomes. Participating providers are independent contractors in private practice and are neither employees nor agents of Aetna or its affiliates. Treating providers are solely responsible for medical advice and treatment of members. This Clinical Policy Bulletin may be updated and therefore is subject to change.

June 07, 2025