Version May 2024
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.
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.
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.
(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 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.
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
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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.
None known.
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 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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