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Zolgensma Dosage

Generic name: ADENO-ASSOCIATED VIRUS 200000000000001 in 1mL; ISOPROPYL ALCOHOL 0.7mL in 1mL
Dosage form: intravenous infusion, kit

Medically reviewed by Drugs.com. Last updated on May 24, 2019.

For single-dose intravenous infusion only.

Dose and Administration

The recommended dose of ZOLGENSMA is 1.1 × 1014 vector genomes per kilogram (vg/kg) of body weight.

Table 1: Dosing
Patient Weight Range (kg) Dose Volumea (mL)

2.6 – 3.0

16.5

3.1 – 3.5

19.3

3.6 – 4.0

22.0

4.1 – 4.5

24.8

4.6 – 5.0

27.5

5.1 – 5.5

30.3

5.6 – 6.0

33.0

6.1 – 6.5

35.8

6.6 – 7.0

38.5

7.1 – 7.5

41.3

7.6 – 8.0

44.0

8.1 – 8.5

46.8

8.6 – 9.0

49.5

9.1 – 9.5

52.3

9.6 – 10.0

55.0

10.1 – 10.5

57.8

10.6 – 11.0

60.5

11.1 – 11.5

63.3

11.6 – 12.0

66.0

12.1 – 12.5

68.8

12.6 – 13.0

71.5

13.1 – 13.5b

74.3

a Dose volume is calculated using the upper limit of the patient weight range for pediatric patients less than 2 years of age between 2.6 kg and 13.5 kg

b Dose volume for pediatric patients less than 2 years of age weighing equal to or greater than 13.6 kg will require a combination of ZOLGENSMA kits.

Prior to ZOLGENSMA infusion
o
Assess liver function [see Boxed Warning, Laboratory Testing and Monitoring to Assess Safety (2.3), Warnings and Precautions (5.1) and Use in Specific Populations (8.6)].
o
Measure platelet counts and troponin-I [see Laboratory Testing and Monitoring to Assess Safety (2.3), Warnings and Precautions (5.2)(5.3)]
o
Perform baseline testing for the presence of anti-AAV9 antibodies [see Laboratory Testing and Monitoring to Assess Safety (2.3), Adverse Reactions (6.2)].
One day prior to ZOLGENSMA infusion, begin administration of systemic corticosteroids equivalent to oral prednisolone at 1 milligram per kilogram of body weight per day (mg/kg/day) for a total of 30 days.
Administer ZOLGENSMA as a single‑dose intravenous infusion through a venous catheter.

Follow the steps below for infusion:

1.
Place a primary catheter into a vein (generally a peripheral vein in the arm or leg). Insertion of a back-up catheter is recommended.
2.
Program syringe pump for saline priming, or prime tubing manually with saline.
3.
Administer ZOLGENSMA as a slow infusion over 60 minutes. DO NOT INFUSE AS AN INTRAVENOUS PUSH OR BOLUS.
4.
Flush line with saline following completion of infusion.
Monitor liver function by clinical examination and by laboratory testing on a regular basis [see Laboratory Testing and Monitoring to Assess Safety (2.3)].
o
At the end of the 30-day period of systemic corticosteroid treatment, check liver status clinically and by assessing ALT, AST, total bilirubin, and prothrombin time.
o
For patients with unremarkable findings (normal clinical exam, total bilirubin, and prothrombin time, and ALT and AST levels below 2 × upper limit of normal (ULN)), taper the corticosteroid dose over the next 28 days [see Warnings and Precautions (5.1)].
o
If liver function abnormalities persist, continue systemic corticosteroids (equivalent to oral prednisolone at 1 mg/kg/day) until AST and ALT values are both below 2 × ULN and all other assessments return to normal range, and then taper the corticosteroid dose over the next 28 days.
o
Consult expert(s) if patients do not respond adequately to the equivalent of 1 mg/kg/day oral prednisolone.

Preparation

Thaw ZOLGENSMA before use. The contents of the ZOLGENSMA kit will thaw in approximately 12 hours if placed in a refrigerator, or in approximately 4 hours if placed at room temperature. If thawed in a refrigerator, remove from refrigerator on day of dosing.
When thawed, ZOLGENSMA is a clear to slightly opaque, colorless to faint white liquid, free of particles. Visually inspect vials for particulate matter and discoloration prior to infusion. Do not use vials if particulates or discoloration are present.
DO NOT SHAKE.
Draw the appropriate dose volume from all vials into a syringe, remove air from the syringe, cap the syringe, and deliver the syringe at room temperature to the patient infusion location.
Use ZOLGENSMA within 8 hours of drawing into syringe. Discard the vector-containing syringe if the drug is not infused within the 8‑hour timeframe.
DO NOT REFREEZE.

Laboratory Testing and Monitoring to Assess Safety

Perform baseline anti-AAV9 antibody testing prior to ZOLGENSMA infusion. Retesting may be performed if anti-AAV9 antibody titers are reported as >1:50 [see Dose and Administration (2.1)].

Conduct the following tests at baseline and as directed below [see Warnings and Precautions (5.1, 5.2, 5.3)]:

Liver function (clinical exam, AST, ALT, total bilirubin, prothrombin time) weekly for the first month; every other week for the second and third months, until results are unremarkable (normal clinical exam, total bilirubin, and prothrombin results, and ALT and AST levels below 2 × ULN).
Platelet counts weekly for the first month, and then every other week for the second and third months, until platelet counts return to baseline.
Troponin‑I weekly for the first month, and then monthly for the second and third months, until troponin-I level returns to baseline.

Further information

Always consult your healthcare provider to ensure the information displayed on this page applies to your personal circumstances.

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