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.
(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
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. 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 (SMA)
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.
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 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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