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nusinersen (Spinraza)

Indications: - treatment of spinal muscular atrophy Dosage: - intrathecal injection - 12 mg (5 mL) per administration - 5 ML nusinersen 2.4 MG/ML Injection [Spinraza] - 4 loading doses; - the 1st 3 loading doses at 14-day intervals - the 4th loading dose 30 days after the 3rd dose - maintenance dose every 4 months [1] - treatment expected for lifetime [1] Adverse effects: - upper respiratory tract infection - lower respiratory tract infection - constipation Mechanism of action: - SMN2-directed antisense oligonucleotide Notes: - $750,000 for 1st year, $375,000/year thereafter [1] (drug only, not including intrathecal procedure costs)

General

neurologic agent oligonucleotide

Database Correlations

PUBCHEM cid=124037382

References

  1. Gerrity MS, Prasad V, Obley AJ Concerns About the Approval of Nusinersen Sodium by the US Food and Drug Administration. JAMA Intern Med. Published online April 30, 2018 PMID: 29710299 https://jamanetwork.com/journals/jamainternalmedicine/fullarticle/2678835
  2. HIGHLIGHTS OF PRESCRIBING INFORMATION SPINRAZA (nusinersen) injection, for intrathecal use http://www.accessdata.fda.gov/drugsatfda_docs/label/2016/209531lbl.pdf