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Keveyis Prior Authorization with Quantity Limit Program Summary
Policy Number: PH-1053
This program applies to Blue Partner, Commercial, GenPlus, NetResults A series, SourceRx and Health Insurance Marketplace formularies.
POLICY REVIEW CYCLE
Effective Date |
Date of Origin |
7/1/2023 |
|
FDA APPROVED INDICATIONS AND DOSAGE
Agent(s) |
FDA Indication(s) |
Notes |
Ref# |
Keveyis® (dichlorphenamide)* Tablets |
Treatment of primary hyperkalemic periodic paralysis, primary hypokalemic periodic paralysis, and related variants
|
* generic available |
1 |
See package insert for FDA prescribing information: https://dailymed.nlm.nih.gov/dailymed/index.cfm
CLINICAL RATIONALE
Primary Periodic Paralyses |
Primary periodic paralyses are rare autosomal dominant conditions characterized by episodes of flaccid paralysis secondary to abnormal sarcolemmal excitability. All forms are associated with mutations in the skeletal muscle sodium, calcium, and potassium channels. These conditions are rarely life threatening, but a number of patients develop permanent weakness and muscle degeneration with age.(3) The two most common forms are hypokalemic periodic paralysis and hyperkalemic periodic paralysis.(2) There are related variants that have overlapping features with hyper- and hypokalemic periodic paralysis, including paramyotonia congenita, potassium aggravated myotonia, and congenital myasthenic syndrome. Treatment options include trigger avoidance, potassium supplementation, and carbonic anhydrase inhibitors.(2,3) Treatment and prevention varies by the form of periodic paralysis diagnosed:
|
Efficacy(1) |
Dichlorphenamide for hypokalemic and hyperkalemic periodic paralysis was studied in two clinical studies. Study 1 was a 9-week, double-blind, placebo-controlled multi-center study examining the average number of self-reported attacks of muscle weakness per week with 44 hypokalemic periodic paralysis patients and 21 hyperkalemic periodic paralysis patients. Hypokalemic patients experienced 2.2 fewer episodes per week vs. placebo (p=0.02). Hyperkalemic patients experienced 3.9 fewer episodes per week vs. placebo (p=0.08). Study 2 was a 35-week, double-blind, placebo-controlled, multi-center, two-period crossover study with 42 hypokalemic periodic paralysis patients and 31 hyperkalemic periodic paralysis patients. In hypokalemic periodic paralysis patients, the primary endpoint was the incidence of acute intolerable worsening necessitating withdrawal. Two hypokalemic periodic paralysis patients on dichlorphenamide and 11 patients on placebo observed intolerable worsening (p=0.02). In hyperkalemic periodic paralysis patients, the primary endpoint was average number of self-reported episodes per week. Patients on dichlorphenamide had 2.3 fewer attacks per week compared to patients on placebo (p=0.006). For both studies, patients naïve to therapy were started at 50 mg twice daily, patients previously on dichlorphenamide were continued on the same dose, and patients previously on acetazolamide were given a dichlorphenamide dose at 20% of the acetazolamide dose. Primary hyperkalemic periodic paralysis, primary hypokalemic periodic paralysis, and related variants are a heterogeneous group of conditions, for which the response to dichlorphenamide may vary. Therefore, prescribers should evaluate the patient's response to dichlorphenamide after 2 months of treatment to decide whether dichlorphenamide should be continued. |
Safety(1) |
Dichlorphenamide has the following contraindications:
|
REFERENCES
Number |
Reference |
1 |
Keveyis prescribing information. Taro Pharmaceuticals USA, Inc. November 2019. |
2 |
Venance SL, Cannon SC, Fialho D, et al. CINCH Investigators. The Primary Periodic Paralyses: Diagnosis, Pathogenesis and Treatment. Brain. 2006;129:8–17. |
3 |
Statland JM, Fontaine B, Hanna MG, et al. Review of the Diagnosis and Treatment of Periodic Paralysis. Muscle Nerve. 2017;57(4):522-530. |
POLICY AGENT SUMMARY PRIOR AUTHORIZATION
Target Brand Agent(s) |
Target Generic Agent(s) |
Strength |
Targeted MSC |
Available MSC |
Preferred Status |
Effective Date |
|
||||||
Keveyis |
dichlorphenamide tab |
50 MG |
M ; N ; O ; Y |
N ; O ; Y |
|
|
POLICY AGENT SUMMARY QUANTITY LIMIT
Target Brand Agent Name(s) |
Target Generic Agent Name(s) |
Strength |
QL Amount |
Dose Form |
Day Supply |
Duration |
Addtl QL Info |
Allowed Exceptions |
Targeted NDCs When Exclusions Exist |
Effective Date |
|
||||||||||
Keveyis |
dichlorphenamide tab |
50 MG |
120 |
TABS |
30 |
Days |
|
|
|
|
CLIENT SUMMARY – PRIOR AUTHORIZATION
Target Brand Agent Name(s) |
Target Generic Agent Name(s) |
Strength |
Client Formulary |
Keveyis |
dichlorphenamide tab |
50 MG |
Blue Partner ; Commercial ; GenPlus ; Health Insurance Marketplace ; NetResults A Series ; SourceRx |
CLIENT SUMMARY – QUANTITY LIMITS
Target Brand Agent Name(s) |
Target Generic Agent Name(s) |
Strength |
Client Formulary |
Keveyis |
dichlorphenamide tab |
50 MG |
Blue Partner ; Commercial ; GenPlus ; Health Insurance Marketplace ; NetResults A Series ; SourceRx |
PRIOR AUTHORIZATION CLINICAL CRITERIA FOR APPROVAL
Module |
Clinical Criteria for Approval |
|
Initial Evaluation Target Agent(s) will be approved when BOTH of the following are met:
Length of Approval: 3 months NOTE: If Quantity Limit applies, please refer to Quantity Limit Criteria.
Renewal Evaluation Target Agent(s) will be approved when ALL of the following are met:
Length of Approval: 12 months NOTE: If Quantity Limit applies, please refer to Quantity Limit Criteria. |
QUANTITY LIMIT CLINICAL CRITERIA FOR APPROVAL
Module |
Clinical Criteria for Approval |
QL with PA |
Evaluation Target Agent(s) will be approved when ONE of the following is met:
Length of Approval: Initial approval - 3 months, Renewal approval - 12 months |
This pharmacy policy is not an authorization, certification, explanation of benefits or a contract. Eligibility and benefits are determined on a case-by-case basis according to the terms of the member’s plan in effect as of the date services are rendered. All pharmacy policies are based on (i) information in FDA approved package inserts (and black box warning, alerts, or other information disseminated by the FDA as applicable); (ii) research of current medical and pharmacy literature; and/or (iii) review of common medical practices in the treatment and diagnosis of disease as of the date hereof. Physicians and other providers are solely responsible for all aspects of medical care and treatment, including the type, quality, and levels of care and treatment.
The purpose of Blue Cross and Blue Shield of Alabama’s pharmacy policies are to provide a guide to coverage. Pharmacy policies are not intended to dictate to physicians how to practice medicine. Physicians should exercise their medical judgment in providing the care they feel is most appropriate for their patients.
Neither this policy, nor the successful adjudication of a pharmacy claim, is guarantee of payment.
Commercial _ PS _ Keveyis Prior Authorization with Quantity Limit _ProgSum_ 7/1/2023