Spinal muscular atrophy:
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
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
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: Drug information")
Spinal muscular atrophy: Intrathecal: Loading dose: 12 mg once every 14 days for 3 doses; then 12 mg once 30 days after the third dose. Maintenance: 12 mg once every 4 months.
There are no dosage adjustments provided in the manufacturer’s labeling.
There are no dosage adjustments provided in the manufacturer’s labeling.
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
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 using spinal anesthesia needle. 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. Do not administer in areas with signs of infection or inflammation.
Store intact vials between 2°C to 8°C (36°F to 46°F) in original carton, protected from light. Do not freeze. May store intact vials at or below 30°C (86°F) for up to 14 days in original carton, protected from light. Intact vials in original carton may be removed from and returned to the refrigerator, if necessary; if removed from the original carton, the total combined time out of refrigeration should not exceed 30 hours at a temperature not exceeding 25°C (77°F). Following removal from vial, administer solution within 4 hours; discard unused contents of the vial.
Treatment of spinal muscular atrophy (SMA) (FDA approved in all ages)
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.
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 labeling.
Canadian labeling: Additional contraindications (not in US 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. Perform a platelet count and coagulation testing at baseline, prior to each dose and as clinically needed.
• Renal toxicity: Renal toxicity, including potentially fatal glomerulonephritis, has been observed with some antisense oligonucleotides. Conduct quantitative spot urine protein testing (preferably using first morning urine) at baseline and prior to each dose. For urinary protein concentration >0.2 g/L, consider repeat testing and further evaluation.
None known.
Note: Interacting drugs may not be individually listed below if they are part of a group interaction (eg, individual drugs within “CYP3A4 Inducers [Strong]” are NOT listed). For a complete list of drug interactions by individual drug name and detailed management recommendations, use the Lexicomp drug interactions program
There are no known significant interactions.
Adverse events have been observed in some animal reproduction studies.
Platelet count, coagulation tests (prothrombin time; activated partial thromboplastin time), and quantitative spot urine protein testing at baseline, prior to each dose, and as clinically indicated
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 increase 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
Solution (Spinraza Intrathecal)
12 mg/5 mL (per mL): $31,977.00
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