- Enzyme replacement therapy in patients with Mucopolysaccharidosis 1 (MPS 1) to treat non central nervous system manifestations of the disease
- Available as 0.58 mg/mL in 5 mL vial (2.9 mg/5 mL)
- Refrigerate vials
- DO NOT FREEZE or SHAKE
- Remove required vials from refrigerator and allow them to reach room temperature
- Solution is slightly opalescent and colorless to pale yellow. A few translucent particles may be present. DO NOT use if solution is discoloured or has particulate matter.
- Total Infusion Volume: 100 mL for patients 20 kg or less ( volume drug & 0.9% Sodium Chloride = 100 mL)
250 mL for patients greater than 20 kg ( volume drug & 0.9% Sodium Chloride = 250 mL) - Slowly withdraw laronidase from vials avoiding excessive agitation. DO NOT use a filter needle as this may cause agitation rendering it biologically inactive
- Slowly add laronidase to bag & gently rotate bag. DO NOT SHAKE
- Use within 3 hours once diluted in bag.
(manufacturer states that although not recommended the diluted solution may to stored up to 36 hours in the refrigerator)
- Solutions Compatible: 0.9% Sodium Chloride
- Additives/Above Cassette Compatible: no information
- Y-site Compatible: no information
- Incompatible: DO NOT infuse with any other medications
(For approved routes of administration by nursing personnel, refer to Policy for the Administration of Intravenous Medications.)
| SC | NO |
| IM | NO |
| IV Direct | NO |
| IV Intermittent Infusion |
YES: Large Volume Pump ONLY -administer with a PVC infusion set with an in-line, low-protein binding 0.2 micrometer filter |
| IV Continuous Infutions |
NO |
(For neonatal dosages, refer to Neonatal IV Drug Manual.)
Pediatric/Adult:
- 0.58 mg/kg/dose once weekly given as a single IV infusion. Round up dose to the nearest whole vial of 5 mL
- Hypersensitivity reactions (during or within 2 hours after infusion): urticaria, dyspnea, hypotension
- Flushing, fever, headache,rash
- Chills, vomiting, nausea, arthralgia, diarrhea, tachycardia, abdominal pain, increased blood pressure, decreased oxygen saturation, erythema, feeling cold, cyanosis
- Premedication with acetaminophen and diphenhydramine administered 30 minutes prior to infusion
- Monitor vital signs pre-infusion, prior to each rate increase (& increase only if vitals stable), and 1 hour post-infusion
- Have epinephrine, diphenhydramine and hydrocortisone available at bedside
INFUSION GUIDELINES:
For patients weighing 20 kg or Less:
Total Volume of Aldurazyme Infusion = 100 mL
| 2 mL/hr x 15 minutes | obtain vital signs, if stable then increase the rate to |
| 4 mL/hr x 15 minutes | obtain vital signs, if stable then increase the rate to |
| 8 mL/hr x 15 minutes | obtain vital signs, if stable then increase the rate to |
| 16 mL/hr x 15 minutes | obtain vital signs, if stable then increase the rate to |
| 32 mL/hr x about 3 hours | For the remainder of the infusion |
For patients weighing Greater than 20 kg:
Total Volume of Aldurazyme Infusion = 250 mL
| 5 mL/hr x 15 minutes | obtain vital signs, if stable then increase the rate to |
| 10 mL/hr x 15 minutes | obtain vital signs, if stable then increase the rate to |
| 20 mL/hr x 15 minutes | obtain vital signs, if stable then increase the rate to |
| 40 mL/hr x 15 minutes | obtain vital signs, if stable then increase the rate to |
| 80 mL/hr x about 3 hours | For the remainder of the infusion |
ANAPHYLAXIS PRECAUTIONS:
- Dosage guidelines (Administer in sequence listed):
- Epinephrine (Adrenalin®) (1:1,000 is 1 mg/mL)______ mg = _______mL(0.01 mg/kg/dose = 0.01 mL/kg/dose, Max. 0.5 mg = 0.5 mL) IM in the thigh or alternative large muscle q15 minutes x 2, then Q4H PRN.
- Diphenhydramine [Benadryl®] __________ mg (1 mg/kg/dose, Max. 50 mg) I.V. x 1, then Q6H PRN.
- Hydrocortisone [Solu-cortef®] __________ mg (2.5 - 5 mg/kg/dose) I.V. x 1, then Q4H PRN