Criteria
Coverage is subject to the specific terms of the member's benefit plan.
Federal Employee Program members (FEP) should check with their Retail Pharmacy Program to determine if prior approval is required by calling the Retail Pharmacy Program at 1-800-624-5060 (TTY: 1-800-624-5077). FEP members can also obtain the list through the www.fepblue.org website.
Nusinersen (Spinraza) may be considered medically necessary for individuals with SMA type I, II, or III caused by mutations in chromosome 5q when the following criteria are met:
-
Individual has had SMA diagnostic test results confirming zero (0) copies of SMN1;
or
-
Molecular genetic testing of 5q SMA for any of the following:
-
Homozygous gene deletion;
or
-
Homozygous conversion mutation;
or
-
Compound heterozygote;
and
-
Individual is symptomatic;
and
-
Individual has two (2), three (3), or four (4) copies of SMN2;
and
-
Individual is not reliant on ventilator support (may use non-invasive ventilator support for naps and nighttime sleep);
and
-
Individual is not dependent on a tracheostomy;
or
-
Individual is asymptomatic;
and
-
Individual has two (2) or three (3) copies of SMN2;
and
-
For type I or II, the prescriber has provided documentation indicating the patient had onset of SMA symptoms at or before 21 months of age;
or
-
For type III, the prescriber has provided documentation indicating the patient's onset of SMA symptoms occurred after 18 months of age;
and
-
Nusinersen (Spinraza) is prescribed by or in consultation with a neuromuscular specialist or neurologist with experience and expertise in the treatment and ongoing management individuals with SMA;
and
-
Baseline documentation of individual's functional abilities by
ANY ONE
of the following:
-
For individuals two (2)-24 months old Hammersmith Infant Neurologic Exam (HINE) Section one (1) and Section two (2);
or
-
For individuals four (4) months to four (4) years of age: Children's Hospital of Philadelphia Infant Test of Neuromuscular Disorders (CHOP-INTEND);
or
-
Hammersmith Functional Motor Scale-Expanded (HFMSE), or 6-Minute Walk Test (6MWT);
or
-
For individuals who are non-ambulatory: Upper Limb Module (ULM), Revised Upper Limb Module (RULM);
and
-
Individual has not previously received gene replacement therapy (e.g., Zolgensma) for the treatment of SMA;
or
-
Individual has received gene replacement therapy (e.g., Zolgensma) and individual has experienced a declination in clinical status since receipt of gene replacement therapy as demonstrated by a decline in documentation on individual's functional ability score(s);
and
-
Individual will not be receiving risdiplam
(
Evrysdi) concomitantly;
and
-
Individual is not and will not be concurrently enrolled in a clinical trial for any experimental therapy for SMA;
and
- Initial authorization will be for a period of six (6) months.
Reauthorization Criteria
Continuation of treatment with nusinersen (Spinraza) beyond six (6) months after initiation of therapy may be considered medically necessary for the treatment of spinal muscular atrophy when individuals meet
ALL
of the following:
-
When initial therapy was determined to meet the above criteria;
and
-
When there is documentation demonstrating individual is stable or shows clinically significant improvement in SMA-associated symptoms as demonstrated by stable or improved functional abilities test results compared to baseline or previous functional abilities test whichever is most recent. The current test and the comparator test being utilized for reauthorization purposes must be at least four (4) months apart (HINE, CHOP-INTEND, HFMSE, 6MWT, ULM, RULM, etc.).
and
-
Individual will not be receiving risdiplam
(
Evrysdi) concomitantly;
and
- The individual is not and will not be concurrently enrolled in a clinical trial for any experimental therapy for SMA
The use of nusinersen (Spinraza) for all other indications not listed in this policy is considered experimental/investigational and therefore non-covered because the safety and/or effectiveness cannot be established by the available published peer-reviewed literature.
Note:
There is a lack of robust clinical evidence to support concomitant use of Nusinersen (Spinraza) with other therapies for the treatment of SMA (e.g., gene therapy or investigational treatments).
Procedure Codes
J2326
