- Prime the intravenous access line with normal saline solution.
- Infuse the diluted EXONDYS 51 solution over 35 to 60 minutes via an in-line 0.2 micron filter.
- If a hypersensitivity reaction occurs, consider slowing the infusion or interrupting the EXONDYS 51 therapy.
- Do not mix other medications with EXONDYS 51 or infuse other medications concomitantly via the same intravenous access line with EXONDYS 51.
- After completion of the infusion, flush the intravenous access line with sodium chloride 0.9% injection, USP, to allow the entire dose, including the contents of the intravenous access line, to be administered.
- In cases where EXONDYS 51 is administered into a venous access port, after administration of the drug and flushing with normal saline, the port may be flushed with heparin prior to de-access.
- Once you have completed the infusion, discard any unused product.
- If a dose of EXONDYS 51 is missed, it may be administered as soon as possible after the scheduled time.
- EXONDYS 51 has been infused at a treatment center (medical office, stand-alone infusion center, hospital) or at home.
- Based on physician, patient, and/or caregiver discretion, a port (implanted venous access device) may be considered for treatment administration.
- Application of a topical anesthetic cream to the infusion site prior to administration of EXONDYS may be considered.
SYMPTOMS OF HYPERSENSITIVITY.
Advise patients and/or caregivers that symptoms of hypersensitivity, including bronchospasm, chest pain, cough, tachycardia and urticaria, can occur with EXONDYS 51. Instruct them to seek immediate medical care should they experience signs and symptoms of hypersensitivity.
REPORTING ADVERSE REACTIONS.
To report suspected adverse reactions, contact Sarepta Therapeutics, Inc. at 1-888-SAREPTA (1-888-727-3782) or FDA at 1-800-FDA-1088 or www.fda.gov/medwatch
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EXONDYS 51 (eteplirsen) is indicated for the treatment of Duchenne muscular dystrophy (DMD) in patients who have a confirmed mutation of the DMD gene that is amenable to exon 51 skipping. This indication is approved under accelerated approval based on an increase in dystrophin in skeletal muscle observed in some patients treated with EXONDYS 51. Continued approval for this indication may be contingent upon verification of a clinical benefit in confirmatory trials.
IMPORTANT SAFETY INFORMATION
Hypersensitivity reactions, including bronchospasm, chest pain, cough, tachycardia and urticaria, have occurred in patients who were treated with EXONDYS 51. If a hypersensitivity reaction occurs, institute appropriate medical treatment and consider slowing the infusion or interrupting the EXONDYS 51 therapy.
Adverse reactions in DMD patients (N=8) treated with EXONDYS 51 30 or 50 mg/kg/week by intravenous (IV) infusion with an incidence of at least 25% more than placebo (N=4) (Study 1, 24 weeks) were (EXONDYS 51, placebo): balance disorder (38%, 0%), vomiting (38%, 0%) and contact dermatitis (25%, 0%). The most common adverse reactions were balance disorder and vomiting. Because of the small numbers of patients, these represent crude frequencies that may not reflect the frequencies observed in practice. The 50 mg/kg once weekly dosing regimen of EXONDYS 51 is not recommended.
The most common adverse reactions from observational clinical studies (N=163) seen in greater than 10% of patients were headache, cough, rash, and vomiting.
Please see the full Prescribing Information for EXONDYS 51 (eteplirsen).
EXONDYS 51 [package insert]. Cambridge, MA: Sarepta Therapeutics Inc.
Data on file.
Infusion Nurses Society. Infusion Therapy Standards of Practice 2016, Online edition. Journal of Infusion Nursing. Available at: http://ins.tizrapublisher.com/hai13r/. Accessed February 15, 2022.