Subject: Keveyis 2491-A SGM P2023

Drug

KEVEYIS  (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 Indication¹
          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^1-5
               Authorization of 60 days may be granted to members who
               are initiating Keveyis therapy when the following criteria is
               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^1-5
          Authorization of 60 days may be granted to members who are
          initiating Keveyis therapy when the following criteria is 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 Keveyis 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]. Trevose, PA: Strongbridge Biopharma; November 2019.
2. Dichlorphenamide [package insert]. Chicago, IL: Xeris Pharmaceuticals, Inc.; December 2022.
3. Levitt JO. Practical aspects in the management of hypokalemic periodic paralysis. Journal of Translational Medicine 2008; 6:18.
4. 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.
5. 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 11, 2023