eteplirsen

General

Genetic Implications: Genetic Implications

Pronunciation:
e-tep-lir-sen

Trade Name(s)

  • Exondys 51

Ther. Class.
none assigned

Pharm. Class.
temporary class antisense oligonucleotides

Indications

Genetic implication Treatment of Duchenne muscular dystrophy (DMD) in patients who have a confirmed mutation of the DMD gene that is amenable to exon 51 skipping.

Action

Binds to exon 51 of dystrophin pre-messenger RNA (mRNA), resulting in exclusion of this exon during mRNA processing. Exon skipping allows for production of an internally truncated dystrophin protein.

Therapeutic Effect(s):

Increased dystrophin production.

Pharmacokinetics

Absorption: IV administration results in complete bioavailability.

Distribution: Widely distributed.

Metabolism and Excretion: Not hepatically metabolized; excreted renally.

Half-life: 3–4 hr.

TIME/ACTION PROFILE (plasma concentrations)

ROUTEONSETPEAKDURATION
IVunknown1 hrunknown

Contraindication/Precautions

Contraindicated in:

  • None.

Use Cautiously in:

OB: Lactation: Safety and effectiveness not established

Adverse Reactions/Side Effects

Derm: rash

GI: vomiting

Neuro: balance disorder

MS: arthralgia

Resp: upper respiratory tract infection

Misc: HYPERSENSITIVITY REACTIONS (INCLUDING RASH, URTICARIA, PYREXIA, FLUSHING, DYSPNEA, BRONCHOSPASM, AND HYPOTENSION)

* CAPITALS indicate life-threatening.
Underline indicate most frequent.

Interactions

Drug-Drug

None noted.

Route/Dosage

IV Children 30 mg/kg once weekly.

Availability

Solution for IV infusion (must be diluted): 50 mg/mL

Assessment

  • Monitor for signs and symptoms of hypersensitivity reactions (rash, urticaria, pyrexia, flushing, cough, dyspnea, bronchospasm, hypotension) during infusion. Treat symptomatically. Consider slowing or interrupting infusion.

Potential Diagnoses

Implementation

IV Administration

  • Apply topical anesthetic cream to infusion site prior to infusion. Flush IV line with 0.9% NaCl before and after infusion.
  • Intermittent Infusion: Allow vials to reach room temperature before using; mix by inverting 2–3 times, do not shake. Solution is clear and colorless with some opalescence; do not administer solutions that are discolored or contain particulate matter. Diluent: Withdraw dose from vials and dilute with 0.9% NaCl for a total volume of 100–150 mL. Complete infusion within 4 hrs. Diluted solution stable for up to 24 hrs if refrigerated. Do not freeze.
  • Rate:Infuse over 35 to 60 min.
  • Y-Site Incompatibility: Do not infuse other medication concomitantly in same IV access line with eteplirsen.

Patient/Family Teaching

Instruct patient and parents to notify health care professional immediately if signs and symptoms of hypersensitivity reactions occur.

Evaluation/Desired Outcomes

Increase in dystrophin levels in muscle tissues.

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