nusinersen

IT (Adults and Children ): Loading dose: 12 mg every 14 days for 3 doses; then 12 mg given 30 days after the third dose; Maintenance dose: 12 mg every 4 mo.

Spinraza

Increases exon 7 inclusion in survival motor neuron-2 messenger ribonucleic acid (mRNA) transcripts, which results in increased production of full-length survival motor neuron protein.

Therapeutic effects:

Reduction in risk of death or permanent ventilation and improvement in motor skills.

Therapeutic Classification: none assigned

Pharmacologic Classification: antisense oligonucleotides

Absorption: Delivered via intrathecal injection into cerebrospinal fluid (CSF).

Distribution: Distributed within CNS and peripheral tissues (skeletal muscle, liver, and kidney).

Metabolism/Excretion: Metabolized via exonuclease (3’- and 5’)-mediated hydrolysis; primarily excreted in urine.

Half-Life: 135–177 days (CSF); 63–87 days (plasma).

(plasma concentrations)

ROUTE	ONSET	PEAK	DURATION
IT	unknown	1.7–6 hr	unknown

Explain purpose of nusinersen to patient and caregiver. Emphasize importance of dosing schedule. If a loading dose is missed, administer missed dose as soon as possible and adjust the date for all subsequent doses to maintain recommended interval between doses. If maintenance dose missed <8 mo from last dose, administer missed dose as soon as possible; administer next maintenance dose according to originally scheduled date as long as this dose and missed dose are administered ≥14 days apart. If maintenance dose missed ≥8 mo but <16 mo from last dose, administer missed dose as soon as possible followed by another dose 14 days later; administer next maintenance dose 4 mo thereafter. If maintenance dose missed ≥16 mo but <40 mo from last dose, administer missed dose as soon as possible followed by two additional doses 14 days apart; administer next maintenance dose 4 mo thereafter. If maintenance dose missed ≥40 mo from last dose, restart dosing recommended in Route/Dosage section.
Advise patient and caregiver to notify health care professional immediately if signs and symptoms of bleeding (unexpected bleeding, blood in stools or urine) or renal toxicity (edema, dyspnea) occur.
Rep: Advise females of reproductive potential to notify health care professional if pregnancy is planned or suspected or if breastfeeding.

nue-si-NER-sen