KEVEYIS, Dichlorphenamide Form

Dichlorphenamide (Keveyis®) is an oral carbonic anhydrase inhibitor. FDA Approved Indication(s) Keveyis is indicated for the treatment of primary hyperkalemic periodic paralysis, primary hypokalemic periodic paralysis, and related variants. Policy/Criteria Provider must submit documentation (such as office chart notes, lab results or other clinical information) supporting that member has met all approval criteria.
It is the policy of health plans affiliated with Centene Corporation® that Keveyis is medically necessary when the following criteria are met:
I. Initial Approval Criteria
A. Hyperkalemic/Hypokalemic Periodic Paralysis and Variants (must meet all):

1. Diagnosis of primary hyperkalemic or hypokalemic periodic paralysis, or related variants (i.e., Andersen’s syndrome, paramyotonia congenita);
2. Age ≥ 18 years;
   
   3. Failure of acetazolamide at up to maximally indicated dose, unless contraindicated or clinically significant adverse effects are experienced;
3. If request is for brand Keveyis, member must use generic dichlorphenamide, unless contraindicated or clinically significant adverse effects are experienced;
4. Dose does not exceed 200 mg (4 tablets) per day. Approval duration: 2 months B. Other diagnoses/indications (must meet 1 or 2):

5. If this drug has recently (within the last 6 months) undergone a label change (e.g., newly approved indication, age expansion, new dosing regimen) that is not yet reflected in this policy, refer to one of the following policies (a or b): a. For drugs on the formulary (commercial, health insurance marketplace) or PDL (Medicaid), the no coverage criteria policy for the relevant line of business: CP.CPA.190 for commercial, HIM.PA.33 for health insurance marketplace, and CP.PMN.255 for Medicaid; or b. For drugs NOT on the formulary (commercial, health insurance marketplace) or PDL (Medicaid), the non-formulary policy for the relevant line of business: Page 1 of 5

   CLINICAL POLICY Dichlorphenamide CP.CPA.190 for commercial, HIM.PA.103 for health insurance marketplace, and CP.PMN.16 for Medicaid; or

6. If the requested use (e.g., diagnosis, age, dosing regimen) is NOT specifically listed under section III (Diagnoses/Indications for which coverage is NOT authorized) AND criterion 1 above does not apply, refer to the off-label use policy for the relevant line of business: CP.CPA.09 for commercial, HIM.PA.154 for health insurance marketplace, and CP.PMN.53 for Medicaid. II. Continued Therapy A. Hyperkalemic/Hypokalemic Periodic Paralysis and Variants (must meet all):
7. Member meets one of the following (a or b): a. Currently receiving medication via Centene benefit or member has previously met initial approval criteria; b. Member is currently receiving medication and is enrolled in a state and product with continuity of care regulations (refer to state specific addendums for CC.PHARM.03A and CC.PHARM.03B);
8. Member is responding positively to therapy as evidenced by reduced frequency of paralysis;
9. If request is for brand Keveyis, member must use generic dichlorphenamide, unless contraindicated or clinically significant adverse effects are experienced;
10. If request is for a dose increase, new dose does not exceed 200 mg (4 tablets) per day. Approval duration: 12 months B. Other diagnoses/indications (must meet 1 or 2):
11. If this drug has recently (within the last 6 months) undergone a label change (e.g., newly approved indication, age expansion, new dosing regimen) that is not yet reflected in this policy, refer to one of the following policies (a or b): a. For drugs on the formulary (commercial, health insurance marketplace) or PDL (Medicaid), the no coverage criteria policy for the relevant line of business: CP.CPA.190 for commercial, HIM.PA.33 for health insurance marketplace, and CP.PMN.255 for Medicaid; or b. For drugs NOT on the formulary (commercial, health insurance marketplace) or PDL (Medicaid), the non-formulary policy for the relevant line of business: CP.CPA.190 for commercial, HIM.PA.103 for health insurance marketplace, and CP.PMN.16 for Medicaid; or

12. If the requested use (e.g., diagnosis, age, dosing regimen) is NOT specifically listed under section III (Diagnoses/Indications for which coverage is NOT authorized) AND criterion 1 above does not apply, refer to the off-label use policy for the relevant line of business: CP.CPA.09 for commercial, HIM.PA.154 for health insurance marketplace, and CP.PMN.53 for Medicaid. III. Diagnoses/Indications for which coverage is NOT authorized:
    A. Non-FDA approved indications, which are not addressed in this policy, unless there is sufficient documentation of efficacy and safety according to the off label use policies – CP.CPA.09 for commercial, HIM.PA.154 for health insurance marketplace, and CP.PMN.53 for Medicaid, or evidence of coverage documents.
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    CLINICAL POLICY Dichlorphenamide IV. Appendices/General Information Appendix A: Abbreviation/Acronym Key FDA: Food and Drug Administration Appendix B: Therapeutic Alternatives
    This table provides a listing of preferred alternative therapy recommended in the approval criteria. The drugs listed here may not be a formulary agent for all relevant lines of business and may require prior authorization.
    Drug Name Dosing Regimen acetazolamide (Diamox®) 250 to 1,000 mg/day PO in divided doses Therapeutic alternatives are listed as Brand name® (generic) when the drug is available by brand name only and generic (Brand name®) when the drug is available by both brand and generic. Dose Limit/ Maximum Dose 1,000 mg/day Appendix C: Contraindications/Boxed Warnings • Contraindication(s): hepatic insufficiency, severe pulmonary obstruction, hypersensitivity to dichlorphenamide or other sulfonamides, concomitant use of Keveyis and high dose aspirin • Boxed warning(s): none reported Appendix D: General Information
    • Variants of periodic paralysis include paramyotonia congenita and Andersen syndrome.
    • Per the Keveyis prescribing information: primary hyperkalemic periodic paralysis, primary hypokalemic periodic paralysis, and related variants are a heterogeneous group of conditions, for which the response to Keveyis may vary. Therefore, prescribers should evaluate the patient's response to Keveyis after 2 months of treatment to decide whether Keveyis should be continued. V. Dosage and Administration
    Indication Primary hyperkalemic periodic paralysis, primary hypokalemic periodic paralysis, and related variants VI. Product Availability
    Tablet: 50 mg Dosing Regimen Initial dose of 50 mg PO QD or BID; titrate based on individual response at weekly intervals up to a maximum recommended daily dose of 200 mg Maximum Dose 200 mg/day VII.