BlueCross BlueShield of Tennessee Medical Policy Manual

Nusinersen

NDC CODE(S)

64406-0058-XX Spinraza 12 MG/5ML Solution (BIOGEN IDEC)

DESCRIPTION

Nusinersen (Spinraza®) is a modified antisense oligonucleotide (ASO) designed to increase exon 7 inclusion in SMN2 messenger RNA (mRNA) transcripts and increase production of full-length survival motor neuron (SMN) protein. Deficiency of full-length SMN1 protein may be caused by mutations in chromosome 5q. Nusinersen bypasses this particular 5q mutation in the treatment of SMA.

POLICY

MEDICAL APPROPRIATENESS

INITIAL APPROVAL

RENEWAL CRITERIA

INDICATION(S)

DOSAGE & ADMINISTRATION

Spinal muscular atrophy

12 mg administered, as an intrathecal bolus injection over 1 to 3 minutes using a spinal anesthesia needle, per administration. Prior to administration, 5 mL of cerebrospinal fluid should be removed. Imaging guidance and sedation may be required for administration.

 

Initiation:

Four loading doses: the first three loading doses should be administered at 14-day intervals. The 4th loading dose should be administered 30 days after the 3rd dose.

 

Maintenance

One dose every 112 days thereafter

LENGTH OF AUTHORIZATION

Coverage will be provided annually and may be renewed

Refer to DOSAGE LIMITS below

APPLICABLE TENNESSEE STATE MANDATE REQUIREMENTS

BlueCross BlueShield of Tennessee’s Medical Policy complies with Tennessee Code Annotated Section 56-7-2352 regarding coverage of off-label indications of Food and Drug Administration (FDA) approved drugs when the off-label use is recognized in one of the statutorily recognized standard reference compendia or in the published peer-reviewed medical literature.

IMPORTANT REMINDER

We develop Medical Policies to provide guidance to Members and Providers.  This Medical Policy relates only to the services or supplies described in it.  The existence of a Medical Policy is not an authorization, certification, explanation of benefits or a contract for the service (or supply) that is referenced in the Medical Policy.  For a determination of the benefits that a Member is entitled to receive under his or her health plan, the Member's health plan must be reviewed.  If there is a conflict between the Medical Policy and a health plan, the express terms of the health plan will govern.

ADDITIONAL INFORMATION 

SMA phenotype 1 (aka Werdnig-Hoffman disease) has a natural history characterized by onset of symptoms (i.e. severe weakness) prior to 6 months of age, inability to sit without support, and an average life span of less than 2 years (in patients without prior therapy to increase SMN protein). SMA phenotype 2 is characterized by symptom onset between 6 and 18 months of age and failure to walk independently. SMA phenotype 3 is characterized by symptom onset after 18 months of age and an ability to walk independently at some point. Deficiency of SMN protein, due to homozygous deletion/mutation in the SMN1 gene, results in loss of motor neurons in the spinal cord and brain stem manifesting clinically as atrophy and weakness. Copy number of the SMN2 gene, which produces approximated 5-10% functional SMN protein, are positively correlated with milder phenotype.

·         Approximately 80% of patients with SMA1 have 1 or 2 copies of the SMN2 gene; approximately 20% have 3 copies (estimated percentages vary)

·         The c.859G>C single base substitution modification in exon 7 of the SMN2 gene is predictive of a milder phenotype

SOURCES

Glanzman, A. M., Mazzone, E., Main, M., Pelliccioni, M., Wood, J., Swoboda, K. J.et al. (2010). The Children’s Hospital of Philadelphia Infant Test of Neuromuscular Disorders (CHOP INTEND): Test development and reliability. Neuromuscular Disorders, 20(3), 155–161.

Lexicomp Online. (2020, March). AHFS DI. Nusinersen. Retrieved May 27, 2020 from Lexicomp Online with AHFS.

MICROMEDEX Healthcare Series. Drugdex Evaluations. (2019, December). Nusinersen. Retrieved May 27, 2020 from MICROMEDEX Healthcare Series.

Prior, T.W., Leach, M.E., Finanger, E. (2000, February [Updated 2019, November]). Spinal Muscular Atrophy. In: Adam MP, Ardinger HH, Pagon RA, et al., editors. GeneReviews® [Internet]. Seattle (WA): University of Washington, Seattle; 1993-2020. Retrieved May 28, 2020 from https://www.ncbi.nlm.nih.gov/books/NBK1352/.

U. S. Food and Drug Administration. (2019, June). Center for Drug Evaluation and Research. Spinraza® (nusinersen) injection, for intrathecal use. Retrieved May 27, 2020 from

https://www.accessdata.fda.gov/drugsatfda_docs/label/2019/209531s007s008lbl.pdf.pdf.

ORIGINAL EFFECTIVE DATE:  3/30/2017

MOST RECENT REVIEW DATE:  9/30/2020

ID_MRx

Policies included in the Medical Policy Manual are not intended to certify coverage availability. They are medical determinations about a particular technology, service, drug, etc. While a policy or technology may be medically necessary, it could be excluded in a member's benefit plan. Please check with the appropriate claims department to determine if the service in question is a covered service under a particular benefit plan. Use of the Medical Policy Manual is not intended to replace independent medical judgment for treatment of individuals. The content on this Web site is not intended to be a substitute for professional medical advice in any way. Always seek the advice of your physician or other qualified health care provider if you have questions regarding a medical condition or treatment.

This document has been classified as public information.

 

 

 

DOSAGE LIMITS

Maximum billable units per dose and over time by indication as a Medical Benefit; 1 billable unit = 0.1 mg

DIAGNOSIS

MAXIMUM UNITS

Spinal Muscular Atrophy

Loading: 120 billable units on D1, D15, D29, and D59

Maintenance: 120 billable units every 112 days