Alternate Name(s):
KYNDRISA
Classification:
Antisense oligonucleotide
Original Date:
May 2017
Indications:
- Duchenne muscular dystrophy
Reconstitution and Stability:
- Available as 188.7 mg/mL vial - single use only
- Clear, colourless to yellow solution - must be free of visible particles and discolouration
- Vial stable 24 months refrigerated (2-8 C) in the original packaging
- Protect from light
Compatibility:
Solutions Compatible: 0.9% NaCl
Administration:
(For approved routes of administration by nursing personnel, refer to Policy for the Administration of Intravenous Medications.)
| SC | Yes - undiluted |
| IM | NO |
| IV Direct | NO |
| IV Intermittent Infusion | YES Usual Dilution: see mixing instructions below Infusion Time: 83.3 mL/hour over 3 hours |
| IV Continuous Infusion | NO |
Dosage:
(For neonatal dosages, refer to Neonatal IV Drug Manual.)
Children:
- 6 mg/kg/dose once weekly
- Dose should be given on the same day each week. If a dose is missed, give as soon as possible unless it is within 2 days of the next injection date. If it is within 2 days, wait until the next scheduled dose
- Allow medication to come to room temperature before administration
- Dose calculation: body weight (kg) x 6 mg/kg drisapersen = patient dose (mg)
- Dose volume: patient dose (mg) divided by 188.7 mg/mL = volume (mL)
- For subcutaneous use the dose may be split into 2 portions if needed and injected into the same area on any given treatment day
Mixing instructions for IV administration:
- Withdraw 25 mL from a 250 mL 0.9% NaCl bag. This removes the overfill volume from the bag.
- Withdraw the equivalent volume of 0.9% NaCl to the volume of drisapersen that will be added to the bag
- Measure the volume of drisapersen needed and add to the 250 mL bag of 0.9% NaCl.
- Gently shake the bag to mix the contents. Cover with an amber bag to PROTECT FROM LIGHT while infusing.
- Set the pump infusion rate to 83.3 mL/h to run over 3 hours. Flush the line after administration
Potential hazards of parenteral administration:
- Prolonged aPTT
- Thrombocytopenia
- Proteinuria
- Hematuria
- Hypersensitivity reactions
Notes:
- Rotate injection sites with every injection. Sites that may be used include: abdomen (not including the 5 cm area around the navel), front of middle thighs, upper outer areas of buttocks and upper back or upper arm
- Each 0.9 mL vial contains 11.3 mg sodium and each 0.6 mL vial contains 7.5 mg of sodium
- Does not inhibit or induce CYP450 enzymes
Monitoring:
- Platelet count: Baseline and every 2 weeks while on therapy
- Urine protein (dipstick): Baseline and every 2 weeks
- A complete 24 hour collection must be done and dosing withheld for patients with 2 consecutive positive dipsticks of ≥1+ (30-100 mg/dL) or a single positive dipstick of ≥2+ (100-300 mg/dL)
- Patients with nephrotic level proteinuria (>1000 mg/m2 per 24 hours) should discontinue drisapersen permanently
- Patients with a urine protein >300 mg/m2 per 24 hours should interrupt treatment and may resume when urine protein is < 250 mg/m2 per 24 hours OR a urine dipstick is negative or shows trace proteinuria - Serum Cystatin C: Baseline and monthly
- Liver function testing: Baseline and monthly. Monitor ALT, AST, ALP, total bilirubin, GGT
- aPTT: Baseline and monthly
- Patients testing positive for Factor VIII inhibition must immediately discontinue therapy - Injection site reactions: with every administration. Dermatologic assessments are required every 6 months in patients that develop injection site reactions
Contraindications:
- Concomitant use of antiplatelet, thrombolytic or anticoagulants
- Use of nephrotoxic agents (eg. Aminoglycosides, diuretics, NSAIDs or lithium) may increase the risk of renal adverse reactions (proteinuria)