Spinraza

Name: Spinraza

Spinraza Interactions

No drug interactions have been determined by the manufacturer. However, you should tell your doctor about all the medicines you take including prescription and non-prescription medicines, vitamins, and herbal supplements. Not all drug interactions are known or reported and new drug interactions are continually being reported. 

Spinraza Food Interactions

No known food interactions

Medications can interact with certain foods. In some cases, this may be harmful and your doctor may advise you to avoid certain foods. In the case of Spinraza, there are no specific foods that you must exclude from your diet when receiving this medication. 

Spinraza Usage

Spinraza is given through a procedure called intrathecal injection by a trained healthcare provider. This delivers medication through the lower back via a lumbar puncture targeting the central nervous system.

Uses for Spinraza

Nusinersen sodium has the following uses:

Nusinersen sodium is indicated for the treatment of spinal muscular atrophy (SMA) in pediatric and adult patients.1

Spinraza Dosage and Administration

General

Nusinersen sodium is available in the following dosage form(s) and strength(s):

Injection: 12 mg/5 mL (2.4 mg/mL) in a single-dose vial.1

Dosage

It is essential that the manufacturer's labeling be consulted for more detailed information on dosage and administration of this drug. Dosage summary:

Nusinersen sodium is administered intrathecally.1

    Dosing Information1
  • Available as nusinersen sodium; dosage expressed in terms of nusinersen.1

  • The recommended dosage is 12 mg (5 mL) per administration.1

  • Initiate nusinersen sodium treatment with 4 loading doses; the first three loading doses should be administered at 14-day intervals; the 4th loading dose should be administered 30 days after the 3rd dose; a maintenance dose should be administered once every 4 months thereafter.1

    Important Preparation and Administration Instructions1
  • Allow to warm to room temperature prior to administration. 1

  • Administer within 4 hours of removal from vial. 1

  • Prior to administration, remove 5 mL of cerebrospinal fluid. 1

  • Administer as intrathecal bolus injection over 1 to 3 minutes. 1

    Laboratory Testing and Monitoring to Assess Safety1
  • At baseline and prior to each dose, obtain a platelet count, coagulation laboratory testing, and quantitative spot urine protein testing.1

Additional Information

AHFS First Release. For additional information until a more detailed monograph is developed and published, the manufacturer's labeling should be consulted. It is essential that the manufacturer's labeling be consulted for more detailed information on usual uses, dosage and administration, cautions, precautions, contraindications, potential drug interactions, laboratory test interferences, and acute toxicity.

Uses For Spinraza

Nusinersen injection is used to treat children and adults with spinal muscular atrophy (SMA).

This medicine is to be given only by or under the direct supervision of your doctor.

Spinraza Dosage and Administration

Dosing Information

Spinraza is administered intrathecally by, or under the direction of, healthcare professionals experienced in performing lumbar punctures.

Recommended Dosage

The recommended dosage is 12 mg (5 mL) per administration.

Initiate Spinraza treatment with 4 loading doses. The first three loading doses should be administered at 14-day intervals. The 4th loading dose should be administered 30 days after the 3rd dose. A maintenance dose should be administered once every 4 months thereafter.

Missed Dose

If a loading dose is delayed or missed, administer Spinraza as soon as possible, with at least 14-days between doses and continue dosing as prescribed. If a maintenance dose is delayed or missed, administer Spinraza as soon as possible and continue dosing every 4 months.

Important Preparation and Administration Instructions

Spinraza is for intrathecal use only.

Prepare and use Spinraza according to the following steps using aseptic technique. Each vial is intended for single dose only.

Preparation

  • Store Spinraza in the carton in a refrigerator until time of use.
  • Allow the Spinraza vial to warm to room temperature (25° C/77° F) prior to administration. Do not use external heat sources.
  • Inspect the Spinraza vial for particulate matter and discoloration prior to administration. Do not administer Spinraza if visible particulates are observed or if the liquid in the vial is discolored.
  • Withdraw 12 mg (5 mL) of Spinraza from the single-dose vial into a syringe and discard unused contents of the vial.
  • Administer Spinraza within 4 hours of removal from vial.

Administration

  • Consider sedation as indicated by the clinical condition of the patient.
  • Consider ultrasound or other imaging techniques to guide intrathecal administration of Spinraza, particularly in younger patients.
  • Prior to administration, remove 5 mL of cerebrospinal fluid.
  • Administer Spinraza as an intrathecal bolus injection over 1 to 3 minutes using a spinal anesthesia needle [see Dosage and Administration (2.1)]. Do not administer Spinraza in areas of the skin where there are signs of infection or inflammation.

Laboratory Testing and Monitoring to Assess Safety

Conduct the following laboratory tests at baseline and prior to each dose of Spinraza and as clinically needed [see Warnings and Precautions (5.1, 5.2)]:

  • Platelet count
  • Prothrombin time; activated partial thromboplastin time
  • Quantitative spot urine protein testing

Spinraza Description

Spinraza contains nusinersen, which is a modified antisense oligonucleotide, where the 2'-hydroxy groups of the ribofuranosyl rings are replaced with 2'-O-2-methoxyethyl groups and the phosphate linkages are replaced with phosphorothioate linkages. Nusinersen binds to a specific sequence in the intron downstream of exon 7 of the SMN2 transcript. The structural formula is:

Spinraza is supplied as a sterile, preservative-free, colorless solution for intrathecal use in a single-dose glass vial. Each 1 mL solution contains 2.4 mg of nusinersen (equivalent to 2.53 mg of nusinersen sodium salt). Each 1 mL also contains calcium chloride dihydrate (0.21 mg) USP, magnesium chloride hexahydrate (0.16 mg) USP, potassium chloride (0.22 mg) USP, sodium chloride (8.77 mg) USP, sodium phosphate dibasic anhydrous (0.10 mg) USP, sodium phosphate monobasic dihydrate (0.05 mg) USP, and Water for Injection USP. The product may contain hydrochloric acid or sodium hydroxide to adjust pH. The pH is ~7.2.

The molecular formula of Spinraza is C234H323N61O128P17S17Na17 and the molecular weight is 7501.0 daltons.

Nonclinical Toxicology

Carcinogenesis, Mutagenesis, Impairment of Fertility

Carcinogenesis

Long-term studies in animals to evaluate the carcinogenic potential of nusinersen have not been performed.

Mutagenesis

Nusinersen demonstrated no evidence of genotoxicity in in vitro (Ames and chromosomal aberration in CHO cells) and in vivo (mouse micronucleus) assays.

Impairment of Fertility

When nusinersen (0, 3, 10, or 25 mg/kg) was administered by subcutaneous injection to mice every other day prior to and during mating and continuing in females throughout organogenesis, no adverse effects on male or female fertility were observed.

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