CurrentCignaPolicy IP0204

Dichlorphenamide (Keveyis, Ormalvi) for periodic paralysis — Coverage Criteria

Cigna coverage policy governing medical necessity, prior authorization, and reauthorization of dichlorphenamide for primary hyperkalemic and hypokalemic periodic paralysis and related variants for members covered under Cigna-administered health benefit plans.

Policy Summary

PayerCigna

PolicyDichlorphenamide (Keveyis, Ormalvi) for periodic paralysis — Coverage Criteria

Policy CodePolicy IP0204

Change TypeNo material change

Effective DateApr 1, 2025

Next Review DateN/A

Key ActionObtain prior authorization and document trial and failure, contraindication, or intolerance to acetazolamide (or current dichlorphenamide use) to support coverage.

SourceLink

POLICY UPDATE CHANGES

No material clinical or coverage changes in this revision.

3 moinitial auth

12 morenewal auth

Keveyis/Ormalviproducts covered

Acetazolamide priorstep therapy

4/1/2025effective

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Coverage and Medical Necessity Criteria

Initial Medical Necessity Criteria

Covered when BOTH of the following are met for primary HyperPP, primary HypoPP, or related variants:

Overall: Patient meets BOTH A and B

A. Prior therapy or current use: ONE of the following: i. Documented failure, contraindication, or intolerance to acetazolamide capsules or tablets; OR ii. Is currently receiving dichlorphenamide.

B. Preferred product criteria: Preferred product criteria is met for the product(s) as listed in the employer plans table (e.g., trial of generic dichlorphenamide required for coverage of brand unless formulation difference causes significant allergy or serious adverse reaction per prescriber).

See employer plan criteria for Keveyis and Ormalvi specifics.

Reauthorization

Reauthorization criteria:

Reauthorization: Initial criteria are met AND there is documentation of beneficial response to dichlorphenamide.

Any use of dichlorphenamide outside of primary hyperkalemic periodic paralysis, primary hypokalemic periodic paralysis, or the related variants described in this policy is not supported by this coverage guideline and is considered experimental, investigational, or unproven. Such uses may be denied.

Provider Actions, Prior Authorization, and Documentation

Prior Authorization

Prior Authorization and Duration

Prior authorization may be required. Initial approval is up to 3 months; reauthorization may be approved for up to 12 months when medical necessity criteria are met.

Initial approval: up to 3 months
Reauthorization: up to 12 months when medical necessity criteria are met

Step Therapy

Step Therapy Requirement

Step therapy requires a trial of acetazolamide with documented failure, contraindication, or intolerance prior to approval of dichlorphenamide.

Documented trial of acetazolamide with failure, contraindication, or intolerance required before dichlorphenamide

Documentation Required

Required Documentation

Documentation must include evidence of prior trial and failure, contraindication, or intolerance to acetazolamide OR documentation that the member is currently using dichlorphenamide. Receipt of sample product does not satisfy documentation requirements.

Evidence of prior trial and failure, contraindication, or intolerance to acetazolamide OR documentation of current dichlorphenamide use
Receipt of sample product does not satisfy criteria

Denial Risk

Conditions Not Covered

Use of dichlorphenamide for any indication other than primary Hyperkalemic Periodic Paralysis (HyperPP), primary Hypokalemic Periodic Paralysis (HypoPP), and related variants (e.g., Andersen-Tawil syndrome, paramyotonia congenita) is considered experimental, investigational, or unproven and is not covered.

Not covered for indications other than primary HyperPP, primary HypoPP, and related variants

Background

Dichlorphenamide is a carbonic anhydrase inhibitor indicated for the treatment of primary hyperkalemic periodic paralysis (HyperPP), primary hypokalemic periodic paralysis (HypoPP), and related variants. Primary periodic paralyses are rare, typically autosomal dominant ion channel disorders that cause episodic muscle weakness or paralysis; genetic testing is recommended as the initial diagnostic step and other causes of potassium abnormalities should be excluded. Historically, acetazolamide has been used and about half of patients with HypoPP benefit, while dichlorphenamide is more potent and clinical response should be evaluated after approximately 2 months of therapy.

Related Variants and Definitions

Related variants

DefinitionRelated variants — disorders considered under this policy include Andersen-Tawil syndrome and paramyotonia congenita.

Clinical contextThese related variants are part of the group of primary periodic paralyses treated with dichlorphenamide.

Evaluation noteResponse to dichlorphenamide may vary; prescribers should evaluate patient response after ~2 months to determine continued use.

Diagnostic considerationGenetic testing is recommended as the first diagnostic step; a heterozygous pathogenic mutation is identified in ~60–70% of cases.

Treatment cautionVerify prior acetazolamide response before initiating dichlorphenamide, as dichlorphenamide is more potent and may exacerbate symptoms in some patients.