Criteria
Coverage is subject to the specific terms of the member’s benefit plan.
The use of nusinersen (Spinraza) may be considered medically necessary when ALL of the following criteria are met:
- The individual must have a diagnosis of spinal muscular atrophy (SMA) with the following (as evidenced with submitted documentation):
- Bi-allelic deletions or mutations of SMN1 as confirmed by genetic testing, reported as one of the following:
- Homozygous deletions of exon 7; or
- Compound heterozygous mutations; and
- One of the following:
- The individual has number of SMN2 gene copies ≥ 1 but ≤ 3 as confirmed by genetic testing; or
- The individual is symptomatic (e.g., loss of reflexes, motor delay, motor weakness, abnormal EMG/neuromuscular ultrasound); and
- The medication must be prescribed by or in consultation with a neuromuscular neurologist or neuromuscular physiatrist; and
- The individual must visit with a neuromuscular clinic once per year and clinic name, contact information, and date of last visit must be provided; and
- The individual must not have received gene therapy (i.e., Zolgensma); and
- The individual’s weight and prescribed dose must be provided and within dosing recommendations per the manufacturer label; and
- The provider must submit documentation of the individual’s current motor function, as evidenced by scores from at least two of the following assessments:
- Children’s Hospital of Philadelphia Infant Test of Neuromuscular Disorder (CHOP-INTEND)
- Hammersmith Infant Neurological Examination (HINE) Section 2 motor milestone score
- Hammersmith Functional Motor Scale Expanded (HFMSE)
- Motor Function Measure – 32 items (MFM-32)
- Revised Upper Limb Module (RULM)
- 6-minute walk test (6MWT)
- Forced Vital Capacity (FVC) via Pulmonary Function Test; and
- The individual must not have permanent invasive ventilation; and
- The individual must not have severe contractures or severe scoliosis.
Initial Authorization: 12 months
Reauthorization Criteria
Continuation of therapy with nusinersen (Spinraza) may be considered medically necessary when ALL of the following are met:
- The individual’s weight and prescribed dose must be provided and within dosing recommendations per the manufacturer label; and
- The individual must visit with a Neurology clinic once per year and clinic name, contact information, and date of last visit must be provided; and
- The individual must not have permanent invasive ventilation; and
- The individual must not have severe contractures or severe scoliosis; and
- The provider must submit documentation showing that the individual has experienced clinical benefit (defined as maintenance of baseline motor function or significant slowed rate of decline vs expected natural course of the disease) since starting treatment with Spinraza, as evidenced by documentation of one of the following:
- Current Forced Vital capacity (FVC and FEV1) via Pulmonary Function Test; or
- CHOP-INTEND, HINE, HFMSE, MFM-32, 6MWT, or RULM scores
Continuation Authorization: 12 months
The use of nusinersen (Spinraza) for any other indication is considered experimental/investigational and therefore non-covered due to the lack of scientific evidence to support efficacy and safety.
Procedure Codes
