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Medication Policy Manual Policy No: dru299 Topic: Tecfidera, dimethyl fumarate Date of Origin: May 16, 2013 Committee Approval Date: December 12, 2014 Next Review Date: December 2015 Effective Date: January 1, 2015 IMPORTANT REMINDER This Medical Policy has been developed through consideration of medical necessity, generally accepted standards of medical practice, and review of medical literature and government approval status. Benefit determinations should be based in all cases on the applicable contract language. To the extent there are any conflicts between these guidelines and the contract language, the contract language will control. The purpose of medical policy is to provide a guide to coverage. Medical Policy is not intended to dictate to providers how to practice medicine. Providers are expected to exercise their medical judgment in providing the most appropriate care. Description Dimethyl fumarate (Tecfidera) is an oral medication used to prevent or lessen the severity of multiple sclerosis attacks. It works by helping to prevent nerve cells from inflammation and damage. dru299.3 Page 1 of 7

Policy/Criteria I. Most contracts require prior authorization approval of dimethyl fumarate prior to coverage. Dimethyl fumarate may be considered medically necessary when all of the following criteria A, B, and C are met: A. A definitive diagnosis of a relapsing form of multiple sclerosis (relapsingremitting or secondary progressive multiple sclerosis) that has been established by a specialist in neurology or multiple sclerosis. AND B. Dimethyl fumarate is prescribed by, or in consultation with, a specialist in neurology or multiple sclerosis. AND C. An interferon beta product (see Appendix A) and one additional preferred disease modifying therapy [either glatiramer acetate (Copaxone) or natalizumab (Tysabri)] were documented in clinical notes to be ineffective, contraindicated, or not tolerated. Ineffectiveness is defined as meeting at least two of the following three criteria (1, 2, or 3) during treatment with one of these medications. 1. The patient continues to have clinical relapses (at least two relapses within the past 12 months). 2. The patient continues to have CNS lesion progression as measured by MRI. 3. The patient continues to have worsening disability. Examples of worsening disability include, but are not limited to, decreased mobility, decreased ability to perform activities of daily living due to disease progression or an EDSS score >3.5. II. Administration, Quantity Limitations, and Authorization Period A. RegenceRx considers dimethyl fumarate to be a self-administered medication. B. When prior authorization is approved, dimethyl fumarate may be authorized in quantities of 60 capsules per month. C. Authorization may be reviewed at least annually to confirm that current medical necessity criteria are met and that the medication is effective. III. Dimethyl fumarate is considered investigational when used concomitantly with other disease-modifying multiple sclerosis therapies (see Appendix A). dru299.3 Page 2 of 7

IV. Dimethyl fumarate is considered investigational when used for all other conditions, including but not limited to: A. Primary progressive multiple sclerosis (PPMS). B. Progressive-relapsing multiple sclerosis (PRMS). B. Psoriasis. Position Statement - Dimethyl fumarate is an oral disease-modifying therapy used in the treatment of relapsing forms (relapsing-remitting and secondary progressing) of multiple sclerosis (MS) where it was found to decrease the frequency of MS attacks relative to placebo. - There is no reliable evidence that dimethyl fumarate is safer or more effective than other disease-modifying medications used in the treatment of MS. Published clinical trials have only directly compared it with placebo. - The safety and effectiveness of dimethyl fumarate when used in combination with other disease-modifying MS medications has not been established. - Interferon beta-1a products (Avonex and Rebif) currently provide the best value in treating MS for Regence members. - The most common adverse effects associated with dimethyl fumarate include flushing and gastrointestinal effects such as nausea and diarrhea. - Routine monitoring of blood counts (CBC) is recommended before starting and periodically during therapy because there is a small chance that it may lower WBC count. - Dimethyl fumarate is approved in doses of 240 mg taken twice daily. Higher doses have not been found to be more effective. Clinical Efficacy DIMETHYL FUMARATE IN RELAPSING FORMS OF MULTIPLE SCLEROSIS There is moderate certainty in the evidence that dimethyl fumarate lowers the rate of MS attacks relative to placebo. However, there is no evidence directly comparing it with other diseasemodifying MS therapies, including interferon beta-1a products (Avonex or Rebif) which are less costly. - Two, large randomized controlled studies compared dimethyl fumarate with placebo in patients with relapsing-remitting multiple sclerosis (RRMS). [1,2] * Sixty to seventy percent of patients enrolled in the studies were naïve to prior disease-modifying MS therapies. dru299.3 Page 3 of 7

* The studies employed dimethyl fumarate in doses of 240 mg orally twice a day and 240 mg orally three times per day. * The primary endpoint of the studies was relapse rate at 2 years, which is a standard, clinically relevant outcome. Time to confirmed disease progression, also a clinically relevant outcome, was followed as a secondary endpoint. * There was a 44% to 53% reduction in annualized relapse rate reported with the 240 mg twice daily dose of dimethyl fumarate. Efficacy was similar in the 240 mg three times daily dosing arm. * Only one of the two studies reported a statistically significant difference in slowing of progression of disability. [2] * Glatiramer acetate was employed as an active comparator in one of the studies. Although there were numerically fewer relapses in the dimethyl fumarate treatment arm, there was no statistical comparison made between these two treatment groups. [2] - Dimethyl fumarate has not been directly compared with other disease-modifying MS therapies. - Dimethyl fumarate has not been incorporated into national MS treatment guidelines. Use of Dimethyl Fumarate in Other Conditions - Dimethyl fumarate is being studied in plaque psoriasis; however, there are currently no well-conducted, published clinical trials evaluating its efficacy in this condition. [3] - Dimethyl fumarate has not been studied in primary progressive MS, or progressiverelapsing MS. Background on Multiple Sclerosis (MS) - There are four clinical courses of multiple sclerosis (characterized in Table 1 below). [4] - Relapsing-remitting multiple sclerosis accounts for up to 85% of cases. [4] dru299.3 Page 4 of 7

Table 1: Multiple Sclerosis Forms/Clinical Course Definitions [4] Relapsing-remitting (RRMS) Secondary progressive (SPMS) Primary progressive (PPMS) Progressive relapsing (PRMS) Characterized by acute relapses that are followed by some degree of recovery; patients do not develop worsening of disability between relapses. The American Academy of Neurology (AAN) defines RRMS as the first clinical course of MS and is characterized by selflimited attacks of neurologic dysfunction. These attacks develop acutely, evolving over days to weeks. Over the next several weeks to months, most patients experience a recovery of function that is often (but not always) complete. Between attacks the patient is neurologically and symptomatically stable. Defined as sustained progression of physical disability occurring separately from relapses, in patients who previously had RRMS. The AAN defines SPMS as the second clinical course which begins as RRMS, but at some point the attack rate is reduced and the course becomes characterized by a steady deterioration in function unrelated to acute attacks. Defined as progression of disability from onset without superimposed relapses. The AAN defines PPMS as the third clinical type characterized by a steady decline in function from the beginning without acute attacks. Defined as primary progressive patients who develop acute relapses well after disease onset. The AAN defines PRMS as the fourth clinical type which also begins with a progressive course although these patients also experience occasional attacks. Safety [5] - The most common (incidence > 10% and > 2% versus placebo) adverse effects associated with dimethyl fumarate incidence: flushing, abdominal pain, diarrhea, and nausea. - The incidence and severity of flushing and gastrointestinal symptoms typically decreased over time. - Approximately 6% of patients taking dimethyl fumarate in clinical studies experienced abnormally low (< 0.5x 10 9 /L) white blood cell counts (WBC) while on therapy. However, there was no increased risk of serious infections observed in these patients. - There are no boxed warnings associated with the labeling for dimethyl fumarate. Dosing and Administration - Dimethyl fumarate delayed-release capsules are dose as 120 mg orally twice daily for one week, then 240 mg orally twice daily, thereafter. [5] - They can be taken with or without food. The capsules should not be crushed, chewed, or sprinkled on food. [5] dru299.3 Page 5 of 7

- Higher doses of dimethyl fumarate were not associated with additional benefit, but may be associated with more side effects. [1,2] - Complete blood counts (CBC) are recommended before starting therapy, annually, and when clinically indicated because some patients may experience a decrease in white blood cell count while taking dimethyl fumarate. [5] - Dimethyl fumarate is available through Specialty pharmacies. Appendix A: Disease-Modifying Agents Used in the Treatment of Multiple Sclerosis (MS) Alemtuzumab (Lemtrada TM ) Dimethyl fumarate (Tecfidera ) Fingolimod (Gilenya ) Glatiramer acetate (Copaxone ) Interferon beta-1a (Avonex, Rebif ) Interferon beta-1b (Betaseron, Extavia ) Mitoxantrone (Novantrone ) Natalizumab (Tysabri ) Peginterferon beta-1a (Plegridy TM ) Teriflunomide (Aubagio ) Cross References Aubagio, teriflunomide, RegenceRx Medication Policy Manual, Policy No. 283 Betaseron /Extavia, interferon beta-1b, RegenceRx Medication Manual, Policy No. 108 Gilenya, fingolimod, RegenceRx Medication Manual, Policy No. 229 Lemtrada TM, alemtuzumab, RegenceRx Medication Manual, Policy No. 381 Plegridy TM, peginterferon beta-1a, RegenceRx Medication Policy Manual, policy No.376 Tysabri, natalizumab, RegenceRx Medication Manual, Policy No. 111 dru299.3 Page 6 of 7

Codes Number Description HCPCS J8499 Prescription drug, oral, non-chemotherapeutic, Not Otherwise Specified HCPCS J2323 Injection, natalizumab, 1 mg HCPCS J3590 Unclassified Biologics References 1. Gold, R, Kappos, L, Arnold, DL, et al. Placebo-controlled phase 3 study of oral BG-12 for relapsing multiple sclerosis. N Engl J Med. 2012 Sep 20;367(12):1098-107. PMID: 22992073 2. Fox, RJ, Miller, DH, Phillips, JT, et al. Placebo-controlled phase 3 study of oral BG-12 or glatiramer in multiple sclerosis. N Engl J Med. 2012 Sep 20;367(12):1087-97. PMID: 22992072 3. National Institutes of Health, Clinicaltrials.gov. [cited 4/2/2013]; Available from: www.clinicaltrials.gov 4. Goodin, DS, Frohman, EM, Garmany, GP, Jr., et al. Disease modifying therapies in multiple sclerosis: report of the Therapeutics and Technology Assessment Subcommittee of the American Academy of Neurology and the MS Council for Clinical Practice Guidelines. Neurology. 2002 Jan 22;58(2):169-78. PMID: 11805241 5. Tecfidera [package insert]. Cambridge, MA: Biogen Idec; March 2013. dru299.3 Page 7 of 7