CurrentCignaPolicy N/A

Dichlorphenamide Prior Authorization Policy

Prior authorization policy for dichlorphenamide (Keveyis and generic) for treatment of primary hypokalemic and hyperkalemic periodic paralysis and related variants within Cigna-administered health benefit plans. Specifies initial and continuation approval criteria, required prescriber specialty, exclusions, and approval durations.

Policy Summary

PayerCigna

PolicyDichlorphenamide Prior Authorization Policy

Policy CodePolicy N/A

Change Typeclarified

Effective Date

Next Review Date

Key ActionPrior Authorization is recommended for prescription benefit coverage of dichlorphenamide; approvals provided for specified durations (initial 2 months or continuation 1 year).

POLICY UPDATE CHANGES

Annual Revision, Summary of Changes = No criteria changes.

2Covered Indications (HypoPP, HyperPP)

2 monthsInitial approval duration

1 yearContinuation approval duration

experimentalNot covered for other uses

Coverage Summary

This prior authorization policy covers dichlorphenamide (Keveyis and generics) for the treatment of primary hypokalemic periodic paralysis (HypoPP) and primary hyperkalemic periodic paralysis (HyperPP) and related variants. Coverage stance: covered_with_criteria, with initial and continuation approval criteria and specified durations. Per the policy background, acetazolamide has historically been used and should be trialed prior to initiating dichlorphenamide because dichlorphenamide is more potent and may exacerbate symptoms in some patients.

Hypokalemic Periodic Paralysis — Initial and Continuation Criteria

Hypokalemic Periodic Paralysis (HypoPP) and Related Variants

Approve for the duration noted if the patient meets ONE of the following (A or B):

A) Initial Therapy (approve for 2 months)

ANY of the following
- Patient has had a serum potassium concentration of less than 3.5 mEq/L during a paralytic attack< 3.5 mEq/L
- Patient has a family history of the condition
- Patient has a genetically confirmed skeletal muscle calcium or sodium channel mutation
The prescriber has excluded other reasons for acquired hypokalemia
Examples include renal, adrenal, or thyroid dysfunction; renal tubular acidosis; and diuretic or laxative abuse.
Patient has had improvements in paralysis attack symptoms with potassium intake
Patient has tried oral acetazolamide therapy
According to the prescriber, acetazolamide therapy did not worsen the paralytic attack frequency or severity in the patient
The medication is prescribed by or in consultation with a neurologist or a physician who specializes in the care of patients with primary periodic paralysis (e.g., muscle disease specialist, physiatrist)

B) Currently Receiving Dichlorphenamide (approve for 1 year)

Patient is currently receiving dichlorphenamide
Patient has responded to dichlorphenamide (e.g., decrease in the frequency or severity of paralytic attacks) as determined by the prescriber

Hyperkalemic Periodic Paralysis — Initial and Continuation Criteria

Hyperkalemic Periodic Paralysis (HyperPP) and Related Variants

Approve for the duration noted if the patient meets ONE of the following (A or B):

A) Initial Therapy (approve for 2 months)

ANY of the following
- Patient has had an increase from baseline in serum potassium concentration of greater than or equal to 1.5 mEq/L during a paralytic attack>= 1.5 mEq/L
- Patient has had a serum potassium concentration during a paralytic attack of greater than 5.0 mEq/L> 5.0 mEq/L
- Patient has a family history of the condition
- Patient has a genetically confirmed skeletal muscle sodium channel mutation
The prescriber has excluded other reasons for acquired hyperkalemia
Examples include drug abuse, renal dysfunction, and adrenal dysfunction.
Patient has tried oral acetazolamide therapy
According to the prescriber, acetazolamide therapy did not worsen the paralytic attack frequency or severity in the patient
The medication is prescribed by or in consultation with a neurologist or a physician who specializes in the care of patients with primary periodic paralysis (e.g., muscle disease specialist, physiatrist)

B) Currently Receiving Dichlorphenamide (approve for 1 year)

Patient is currently receiving dichlorphenamide
Patient has responded to dichlorphenamide (e.g., decrease in the frequency or severity of paralytic attacks) as determined by the prescriber

Prior Authorization & Documentation Requirements

Prior Authorization

Prior authorization required

Prior Authorization is recommended for prescription benefit coverage of dichlorphenamide; approvals are provided for specified durations (initial 2 months or continuation 1 year).

Documentation Required

Diagnostic and treatment documentation required

For initial therapy, document diagnostic criteria (examples: serum potassium levels during an attack, family history, or genetically confirmed calcium or sodium channel mutation), exclusion of other causes of acquired dyskalemia (e.g., renal, adrenal, thyroid dysfunction, renal tubular acidosis, drug or laxative/diuretic abuse), response to potassium for HypoPP, a prior trial of oral acetazolamide with documentation that acetazolamide did not worsen attack frequency or severity, and that the medication is prescribed by or in consultation with a neurologist or a physician who specializes in primary periodic paralysis (e.g., muscle disease specialist, physiatrist).

Documentation Required

Response documentation for continuation

For patients currently receiving dichlorphenamide, document clinical response (e.g., decreased frequency or severity of paralytic attacks) as determined by the prescriber to justify 1-year approval.

Documentation Required

Specialist prescribing requirement

Initial approval requires dichlorphenamide to be prescribed by or in consultation with a neurologist or a physician who specializes in the care of patients with primary periodic paralysis (e.g., muscle disease specialist, physiatrist).

Background

Dichlorphenamide is a carbonic anhydrase inhibitor indicated for primary HyperPP, primary HypoPP, and related variants. Genetic testing is recommended as the first diagnostic step for periodic paralyses. Acetazolamide has been used historically and should be trialed prior to dichlorphenamide because dichlorphenamide is reported to be 30 times more potent in vitro and may exacerbate symptoms in some patients; therefore clinicians should verify whether acetazolamide caused exacerbation before initiating dichlorphenamide.

Definitions

Initial approvalApproval for 2 months (1 month = 30 days) when initial criteria are met.

Continuation approvalApproval for 1 year for patients currently receiving dichlorphenamide who have demonstrated response.

Revision History

01/03/2024clarified

Annual Revision, Summary of Changes = No criteria changes.; Review dates updated; no clinical policy statement changes per history.

01/08/2025clarifiedLatest

Annual Revision, Summary of Changes = No criteria changes.; Review dates updated; no clinical policy statement changes per history.

01/08/2025last_reviewLatest

Policy review date recorded as 01/08/2025.

Policy Summary

PayerCigna

PolicyDichlorphenamide Prior Authorization Policy

Policy CodePolicy N/A

Change Typeclarified

Effective Date

Next Review Date

Key ActionPrior Authorization is recommended for prescription benefit coverage of dichlorphenamide; approvals provided for specified durations (initial 2 months or continuation 1 year).

On This Page

OpenPayer

Dichlorphenamide Prior Authorization Policy

Coverage Summary

Hypokalemic Periodic Paralysis — Initial and Continuation Criteria

Hyperkalemic Periodic Paralysis — Initial and Continuation Criteria

Prior Authorization & Documentation Requirements

Applicable Codes

Clinical Evidence & References

Background

Revision History