Version May 2024
Spinal muscular atrophy: Intrathecal: Loading dose: 12 mg once every 14 days for 3 doses; then 12 mg as a single dose 30 days after the third dose, followed by maintenance dosing. Maintenance: 12 mg once every 4 months.
Missed doses:
US labeling:
Loading dose (any of the 4): Administer the missed loading dose as soon possible and adjust the date for the subsequent doses to maintain the recommended interval between doses.
Maintenance dose:
<8 months from the last dose: Administer the missed maintenance dose as soon as possible and then administer the next maintenance dose per the original scheduled date, as long as the 2 doses are administered 14 days apart.
≥8 months and <16 months from the last dose: Administer the missed maintenance dose as soon as possible, followed by one additional dose 14 days later, and then administer the next maintenance dose 4 months thereafter.
≥16 months and <40 months from the last dose: Administer the missed maintenance dose as soon as possible, followed by 2 additional doses 14 days apart, and then administer the next maintenance dose 4 months thereafter.
≥40 months from the last dose: Restart dosing from the beginning.
Canadian labeling:
Loading dose:
Second and third dose: Administer the missed loading dose as soon possible, with at least 14 days between doses, then continue with subsequent doses at the prescribed intervals from the last dose. If the third loading dose is administered ≥8 months from the last dose, follow same recommendation as for the maintenance dose for the same duration of dose interruption. If a loading dose is delayed ≥40 months from the last dose, restart from the beginning.
Fourth dose:
≤5 months from the third loading dose: Administer the missed loading dose as soon as possible and then administer the first maintenance dose 4 months later and then subsequent maintenance doses every 4 months.
>5 months to <8 months from the third loading dose: Administer the missed loading dose as soon as possible, then administer the first maintenance dose 8 months from the third loading dose, with at least 14 days between dose, and then subsequent maintenance doses every 4 months after the last dose.
≥8 months to <16 months from the third loading dose: Administer the missed loading dose as soon as possible, followed by 2 additional doses 14 days apart, and then subsequent maintenance doses every 4 months thereafter.
≥16 months to <40 months from the last dose: Administer the missed loading dose as soon as possible, followed by 2 additional doses 14 days apart, and then subsequent maintenance doses every 4 months thereafter.
≥40 months from the third loading dose: Restart dosing from the beginning with a loading regimen and subsequent maintenance doses.
Maintenance dose:
>4 months to <8 months from the last dose: Administer the missed maintenance dose as soon as possible and then administer the next maintenance dose per the original scheduled date, as long as the 2 doses are administered 14 days apart and then administer the next maintenance dose every 4 months thereafter.
≥8 months and <16 months from the last dose: Administer the missed maintenance dose as soon as possible, followed by 1 additional dose 14 days later, and then administer the next maintenance dose every 4 months thereafter.
≥16 months and <40 months from the last dose: Administer the missed maintenance dose as soon as possible, followed by 2 additional doses 14 days apart, and then administer the next maintenance dose every 4 months thereafter.
≥40 months from the last dose: Restart dosing from the beginning with a loading regimen and subsequent maintenance doses.
There are no dosage adjustments provided in the manufacturer's labeling.
There are no dosage adjustments provided in the manufacturer's labeling.
(For additional information see "Nusinersen: Pediatric drug information")
Spinal muscular atrophy: Infants, Children, and Adolescents:
Loading dose: Intrathecal: 12 mg once every 14 days for 3 doses; then 12 mg once 30 days after the third dose.
Maintenance dose: Intrathecal: 12 mg once every 4 months.
Missed doses:
Loading dose (any of the 4): Administer the missed loading dose as soon as possible and adjust the date for the subsequent doses to maintain the recommended interval between doses.
Maintenance dose:
<8 months from the last dose: Administer the missed maintenance dose as soon as possible and then administer the next maintenance dose per the original scheduled date, as long as the 2 doses are administered 14 days apart.
≥8 months and <16 months from the last dose: Administer the missed maintenance dose as soon as possible, followed by one additional dose 14 days later, and then administer the next maintenance dose 4 months thereafter.
≥16 months and <40 months from the last dose: Administer the missed maintenance dose as soon as possible, followed by 2 additional doses 14 days apart, and then administer the next maintenance dose 4 months thereafter.
≥40 months from the last dose: Restart dosing from the beginning.
There are no dosage adjustments provided in the manufacturer’s labeling.
There are no dosage adjustments provided in the manufacturer’s labeling.
The following adverse drug reactions and incidences are derived from product labeling unless otherwise specified.
>10%:
Central nervous system: Headache (29%)
Gastrointestinal: Constipation (35%), vomiting (29%), period of tooth development (18%)
Genitourinary: Proteinuria (58%)
Hematologic & oncologic: Thrombocytopenia (16%)
Neuromuscular & skeletal: Back pain (25%)
Respiratory: Lower respiratory tract infection (55%), atelectasis (18%)
Miscellaneous: Fever (43%)
1% to 10%:
Central nervous system: Fall (5%)
Endocrine & metabolic: Weight loss (5%)
Gastrointestinal: Flatulence (5%)
Genitourinary: Urinary tract infection (9%)
Hypersensitivity: Seasonal allergy (5%)
Immunologic: Antibody development (6%)
Otic: Otic infection (6%)
Respiratory: Respiratory congestion (5%; upper tract: 8%), epistaxis (7%)
<1%, postmarketing, and/or case reports: Angioedema, aseptic meningitis, hydrocephalus, hypersensitivity reaction, maculopapular rash, meningitis, serious infection, skin rash
There are no contraindications listed in the manufacturer's US labeling.
Canadian labeling: Known or suspected hypersensitivity to nusinersen or any component of the formulation.
Concerns related to adverse effects:
• Hematologic effects: Coagulation abnormalities and thrombocytopenia (including acute severe thrombocytopenia) have been observed with some antisense oligonucleotides; increased risk of bleeding complications may occur.
• Renal toxicity: Renal toxicity, including potentially fatal glomerulonephritis, has been observed with some antisense oligonucleotides. For urinary protein concentration >0.2 g/L, consider repeat testing and further evaluation.
Excipient information presented when available (limited, particularly for generics); consult specific product labeling.
Solution, Intrathecal [preservative free]:
Spinraza: 12 mg/5 mL (5 mL)
No
Solution (Spinraza Intrathecal)
12 mg/5 mL (per mL): $33,096.19
Disclaimer: A representative AWP (Average Wholesale Price) price or price range is provided as reference price only. A range is provided when more than one manufacturer's AWP price is available and uses the low and high price reported by the manufacturers to determine the range. The pricing data should be used for benchmarking purposes only, and as such should not be used alone to set or adjudicate any prices for reimbursement or purchasing functions or considered to be an exact price for a single product and/or manufacturer. Medi-Span expressly disclaims all warranties of any kind or nature, whether express or implied, and assumes no liability with respect to accuracy of price or price range data published in its solutions. In no event shall Medi-Span be liable for special, indirect, incidental, or consequential damages arising from use of price or price range data. Pricing data is updated monthly.
Excipient information presented when available (limited, particularly for generics); consult specific product labeling.
Solution, Intrathecal:
Spinraza: 12 mg/5 mL (5 mL)
For intrathecal use only. Allow vial to warm to room temperature before use; do not use external heat sources. Prior to administration, remove 5 mL of cerebrospinal fluid. Administer as an intrathecal bolus injection over 1 to 3 minutes. Do not administer in areas with signs of infection or inflammation.
For intrathecal use only. Allow vial to warm to room temperature before use; do not use external heat sources. Prior to administration, remove 5 mL of cerebrospinal fluid. Administer as an intrathecal bolus injection over 1 to 3 minutes using spinal anesthesia needle. Do not administer in areas with signs of infection or inflammation.
Spinal muscular atrophy: Treatment of spinal muscular atrophy.
The Institute for Safe Medication Practices (ISMP) includes this medication (intrathecal administration) among its list of drugs that have a heightened risk of causing significant patient harm when used in error.
None known.
There are no known significant interactions.
Adverse events have been observed in some animal reproduction studies.
It is not known if nusinersen is present in breast milk.
According to the manufacturer, the decision to continue or discontinue breastfeeding during therapy should take into account the risk of infant exposure, the benefits of breastfeeding to the infant, and benefits of treatment to the mother.
Platelet count, coagulation tests (prothrombin time; activated partial thromboplastin time), and quantitative spot urine protein testing (preferably first morning urine) at baseline, prior to each dose, and as clinically indicated (eg, repeat testing if urinary protein concentration >0.2 g/L).
Treats spinal muscular atrophy caused by mutations in chromosome 5q that lead to survival motor neuron (SMN) protein deficiency by binding to a specific sequence in the intron downstream of exon 7 of the SMN2 messenger ribonucleic acid (mRNA) transcript and increasing production of full-length SMN protein.
Distribution: Within the CNS and peripheral tissues
Metabolism: Via exonuclease (3’- and 5’)-mediated hydrolysis
Half-life elimination: Terminal (mean range): CSF: 135 to 177 days; Plasma: 63 to 87 days
Time to peak (median range): 1.7 to 6 hours
Excretion: Urine
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