Subject: dichlorphenamide-Keveyis-Ormalvi 2491-A SGM P2024

Drug

KEVEYIS  (dichlorphenamide)

ORMALVI  (dichlorphenamide )

dichlorphenamide

Policy:

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

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


    II.  CRITERIA FOR INITIAL APPROVAL

          A.  Primary Hypokalemic Periodic Paralysis
               Authorization of 60 days may be granted for treatment of primary hypokalemic periodic
               paralysis when the following criteria are met:
               1.  The diagnosis was supported by at least one of the following:
                    i.     Genetic test results, or
                    ii.    Member has a family history of primary hypokalemic periodic paralysis, or
                    iii.   Member’s attacks are associated with hypokalemia AND both Andersen-Tawil
                          syndrome and thyrotoxic periodic paralysis have been ruled out.
               2.  Trial with suboptimal response to treatment with acetazolamide

          B.  Primary Hyperkalemic Periodic Paralysis
               Authorization of 60 days may be granted for treatment of primary hyperkalemic periodic
               paralysis when the following criteria are met:
               1.  The diagnosis was supported by at least one of the following:
                    i.     Genetic test results, or
                    ii.    Member has a family history of primary hyperkalemic periodic paralysis, or
                    iii.   Member’s attacks are associated with hyperkalemia AND Andersen-Tawil syndrome
                           has been ruled out.
             2.  Trial with suboptimal response to treatment with acetazolamide


   III.  CONTINUATION OF THERAPY

          Authorization of 12 months may be granted to members who have demonstrated a response to
          therapy as demonstrated 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.; May 2023.
2. Dichlorphenamide [package insert]. Chicago, IL: Xeris Pharmaceuticals, Inc.; December 2022.
3. Ormalvi [package insert]. Cambridge, UK: Cycle Pharmaceuticals LTD; February 2024.
4. Levitt JO. Practical aspects in the management of hypokalemic periodic paralysis. Journal of Translational Medicine 2008; 6:18.
5. Charles G, Zheng C, Lehmann-Horn F, et al. Characterization of hyperkalemic periodic paralysis: a survey of genetically diagnosed individuals. JNeurol 2013; 260:2606-2613.
6. Statland JM, Fontaine, B, Hanna MG, et al. A review of the diagnosis and treatment of periodic paralysis. Muscle & Nerve 2018; 57:522-530.

 

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 09, 2024