Keveyis – NC Standard

Restricted Product(s)

Restriction applies to brand and generic

• Keveyis® (dichlorphenamide)

FDA Approved Use

• For the treatment of primary hyperkalemic periodic paralysis, primary hypokalemic periodic paralysis, and related variants.

Criteria for Approval of Restricted Product(s)

Initial Coverage Criteria

1. The patient is 18 years of age or older; AND 
2. The patient has a diagnosis of ONE of the following (medical record documentation required): 
   1. Primary hypo-kalemic periodic paralysis; OR 
   2. Primary hyper-kalemic periodic paralysis; OR 
   3. A related variant of familial periodic paralysis (e.g. congenital myasthenic syndrome, Andersen-Tawil syndrome, paramyotonia congenita, potassium-associated myotonia); AND 
3. The patient experiences one or more episodes of muscle weakness or paralysis per week (medical record documentation required); AND 
4. The patient has implemented and maintained dietary and lifestyle changes to help prevent episodes; AND 
5. The patient has tried and failed, is intolerant to, or has a clinical contraindication to acetazolamide (medical record documentation required); AND 
6. If the request is for brand Keveyis, 
   1. The patient has tried and failed, is intolerant to, or has a clinical contraindication to generic dichlorphenamide (medical record documentation required); AND 
7. The patient is being managed by or in consultation with a specialist in the area of the patient’s diagnosis (e.g., neurologist or Congenital and Genetic Disease Specialist); AND 
8. Indications outside of FDA labeling will be subject to medical necessity review in accordance with specific strong endorsement or support by nationally recognized compendia, when such recommendation is based on strong/high levels of evidence, and/or uniform consensus of clinical appropriateness has been reached. Medical records and references / evidence must be provided; AND 
9. For formularies that exclude (non-formulary) the requested medication, Non-formulary Exception Criteria applies.
   
   Duration of Approval: 90 days (3 months)

Continuation Coverage Criteria

1. The patient has been previously approved with Blue Cross and Blue Shield of North Carolina (Blue Cross NC) or would have met initial criteria for approval upon the start of therapy; AND 
2. The patient has continued to maintain dietary and lifestyle changes to help prevent episodes; AND 
3. The patient has had clinical benefit with the requested agent (e.g., reduction in severity of and number of attacks of muscle weakness per week) (medical record documentation required); AND 
4. If the request is for brand Keveyis, 
   1. The patient has tried and failed, is intolerant to, or has a clinical contraindication to generic dichlorphenamide (medical record documentation required).
      
      Duration of Approval: 365 days (1 year)

Quantity Limit Exception Criteria

1. The quantity (dose) requested is for documented titration purposes at the initiation of therapy (authorization for a 90 day titration period); AND 
2. The prescribed dose cannot be achieved using a lesser quantity of a higher strength; AND 
3. The quantity (dose) requested does not exceed the maximum FDA labeled dose, when specified, or to the safest studied dose per the manufacturer’s product insert; OR 
4. If the quantity (dose) requested exceeds the maximum FDA labeled dose, when specified, or to the safest studied dose per the manufacturer’s product insert, then the prescriber must submit documentation in support of therapy with a higher dose for the intended diagnosis (submitted documentation may include medical records OR fax form which reflects medical record documentation that shows the length of time the requested dose has been used, and what other medications and doses have been tried and failed).
   
   Duration of Approval: 365 days (1 year)

References

All information referenced is from FDA package insert unless otherwise noted below.

Policy Implementation/Update Information

July 2023: Criteria change: Added age requirement for initial criteria. Added patient experiences one or more episode of muscle weakness in initial criteria with requirement for clinical benefit in continuation criteria. Added the patient is being managed by or in consultation with a specialist in the area of the patient’s diagnosis. Added medical records required for t/f of acetazolamide.
February 2023: Criteria change: New generic dichlorphenamide 50 mg tablets available. Require usage of generic medication unless patient has tried and failed, is intolerant to, or has a clinical contraindication to generic medication. Criteria review and formatting changes. Added duration of approval to quantity limit exception criteria.
April 2021: Original utilization management criteria issued.