This program applies to Blue Partner, Commercial, GenPlus, NetResults A series, and Health Insurance Marketplace.

TARGET AGENT(S)

Exservan™ (riluzole)

Rilutek^® (riluzole)^a

Tiglutik^® (riluzole)

1. generic available and is not a target in the program

1. generic available and is not a target in the program

PRIOR AUTHORIZATION CRITERIA FOR APPROVAL

Evaluation

Target Agent(s) will be approved when ALL of the following are met:

1. ONE of the following:
   1. The patient has a diagnosis of amyotrophic lateral sclerosis (ALS)

OR

1. 2. The patient has another FDA approved indication for the requested agent

AND

2. ONE of the following:
   1. The patient has tried and had an inadequate response to generic riluzole tablets

OR

1. 2. The patient has an intolerance or hypersensitivity to generic riluzole tablets that is not expected to occur with the requested agent

OR

1. 3. The patient has an FDA labeled contraindication to generic riluzole tablets that is not expected to occur with the requested agent

AND

3. The patient does NOT have any FDA labeled contraindications to the requested agent

AND

4. ONE of the following:
   1. The requested quantity (dose) does NOT exceed the program quantity limit

OR

1. 2. ALL of the following:
      1. The requested quantity (dose) is greater than the program quantity limit

AND

1. 1. 2. The requested quantity (dose) does NOT exceed the maximum FDA labeled dose for the requested indication

AND

1. 1. 3. The requested quantity (dose) cannot be achieved with a lower quantity of a higher strength that does not exceed the program quantity limit

OR

1. 3. ALL of the following:
      1. The requested quantity (dose) is greater than the program quantity limit

AND

1. 1. 2. The requested quantity (dose) is greater than the maximum FDA labeled dose for the requested indication

AND

1. 1. 3. The prescriber has provided information in support of therapy with a higher dose for the requested indication

Length of Approval: 12 months

FDA APPROVED INDICATIONS AND DOSAGE^1-3

1. generic equivalent available

CLINICAL RATIONALE

Amyotrophic Lateral Sclerosis (ALS)

Amyotrophic lateral sclerosis (ALS) is an idiopathic, fatal neurodegenerative disease.⁴ It is characterized by loss of motor neurons in the spinal cord, brainstem, and motor cortex.⁵ Age of onset is between 58-63 years for sporadic disease and 47-52 years for familial disease, with rapidly decreased incidence after 80 years. The clinical hallmark of ALS is the presence of upper and lower motor neuron (UMN and LMN) features involving brainstem and multiple spinal cord regions of innervation.⁴

ALS is a rapidly progressive disease with 50% of patients dying within 30 months of symptom onset, and about 20% of patients survive between 5 years and 10 years after symptom onset. Older age at symptom onset, early respiratory muscle dysfunction, and bulbar-onset disease are associated with reduced survival, whereas limb-onset disease, younger age at presentation, and longer diagnostic delay are independent predictors of prolonged survival. Dysphagia develops in most patients, with consequent weight loss and malnutrition. Respiratory compromise eventually develops in most cases, leading to exertional dyspnea, orthopnea, hypoventilation with resultant hypercapnia, and early morning headaches. Death becomes imminent once patients develop dyspnea at rest.⁴

Symptomatic treatments remain the cornerstone of management for patients with ALS. Riluzole, an inhibitor of glutamate release, is a disease modifying (neuroprotective) therapy for patients with ALS.⁴ The American Academy of Neurology (AAN) notes that riluzole has shown modest benefit (prolonging survival by 2-3 months), and recommends that riluzole be offered to slow disease progression in patients with ALS.⁵  

Efficacy^1-3

The efficacy of Tiglutik (riluzole) and Exservan (riluzole) is based on bioavailability studies comparing oral riluzole tablets to Tiglutik oral suspension and Exservan oral film.

The efficacy of riluzole was demonstrated in two clinical trials. Both studies included patients with familial or sporadic ALS, with disease duration of less than 5 years, and a baseline FVC of greater than or equal to 60%. The clinical outcome for both studies was time to tracheostomy or death. Both studies showed longer time to clinical outcome in patients receiving riluzole compared to placebo, with the median survivals between riluzole and placebo groups being 90 days (Study 1) and 60 days (Study 2).

References

1. Exservan prescribing information. Mitsubishi Tanabe Pharma America, Inc. April 2021.
2. Tiglutik prescribing information. ITF Pharma, Inc. March 2020.
3. Rilutek prescribing information. Covis Pharmaceuticals Inc. March 2020.

4. Kiernan M. C., Vucic S., Cheah B. C., Turner M. R., Eisen A., Hardiman O., et al. (2011). Amyotrophic lateral sclerosis. Lancet 377 942–955. 10.1016/S0140-6736(10)61156-7.
5. Miller R.G., Jackson C.E., Kasarskis E.J., England J.D., Forshew D., Johnston W., Kalra S., Katz J.S., Mitsumoto H., Rosenfeld J., et al. Practice parameter update: The care of the patient with amyotrophic lateral sclerosis: Multidisciplinary care, symptom management, and cognitive/behavioral impairment (an evidence-based review): Report of the Quality Standards Subcommittee of the American of Neurology. Neurology. 2009;73:1227–1233.

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. 
 

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