Enzyme replacement therapy for Fabry disease
- Vials should be stored in the fridge (2 to 8 degrees C)
- Vials should be protected from light
- Let vials stand at room temperature for 30 min prior to reconstitution
- Reconstitute each 5 mg vial by slowly injecting 1.1 mL of sterile water for injection down the inside wall of each vial and not directly on the lyophilized cake. This will yield a 5 mg/mL solution
- Reconstitute each 35 mg vial by slowly injecting 7.2 mL of sterile water for injection down the inside wall of each vial and not directly on the lyophilized cake. This will yield a 5 mg/mL solution
- Do not shake vial. Gently swirl, roll and tilt the vial to mix solution
- Stability: Reconstituted vials must be further diluted immediately following reconstitution.
- Stability: Further diluted solution of agalsidase beta in 0.9% NaCL can be stored in the fridge for up to 24 h.
Available: 5 and 35 mg vials
Compatible only with 0.9% NaCL
(For approved routes of administration by nursing personnel, refer to Policy for the Administration of Intravenous Medications.)
|IV intermittent infusion||
Yes DO NOT SHAKE VIAL OR BAG. Gently invert further diluted solution to mix the solution.
Usual dilution: Further dilution of agalsidase beta is based on patient weight. Refer to table below
Infusion time: initial infusion rate should be no more than 15 mg/hr. With subsequent infusions, the infusion rate may be increased by 3 - 5 mg/hr
For patients weighing less than 30 kg: max infusion rate: 15 mg/hr
For patients weighing greater than or equal to 30 kg: max infusion rate is 1.5 hrs
|IV continuous infusion||No|
(For neonatal dosages, refer to Neonatal IV Drug Manual.)
- 1 mg/kg once every 2 weeks IV
- Dosage adjustments can be made on an individual basis based on achievement and maintenance of therapeutic goals
- Patients in clinical studies have received doses up to 3 mg/kg every 2 weeks IV
- Infusion related reactions: headache, dizziness, hypotension, hypertension, nausea, fatigue and increased body temperature
- Rare infusion related reaction symptoms: chest discomfort, dyspnea, pruritis
- Infusion related reactions can be managed by slowing the infusion rate or stopping and resuming at a slower infusion rate, treatment with medications such as antihistamines, antipyretics etc.
- Please call the physician if a patient experiences any symptoms of an infusion related reaction.
- Agalsidase should be administered through a 0.2 micron filter
- Agitation of the reconstituted solution or diluted bag may increase the chance for precipitation
- Further dilution of agalsidase beta is weight dependent
Patient weight (kg) Minimum total volume of solution to be infused (mL) Less than or equal to 35 kg 50 mL 35.1 - 70 kg 100 mL 70.1 - 100 kg 250 mL Greater than 100 kg 500 mL