(al GLOO ser ase)
Replacement therapy for Gauchers disease (type 1)
Hypersensitivity to any component of the formulation
Gauchers disease: IV: Initial: 30-60 units/kg every 2 weeks; dosing is individualized based on disease severity; average dose: 60 units/kg every 2 weeks. Range: 2.5 units/kg 3 times/week to 60 units/kg once weekly to every 4 weeks. Once patient response is well established, dose may be reduced every 3-6 months to determine maintenance therapy.
Refer to adult dosing.
Refer to adult dosing.
Dilute with NS to a final volume ≤200 mL. Do not shake.
IV: Infuse IV over 1-2 hours. Use of an in-line filter is recommended. Do not shake solution as it denatures the enzyme.
Refrigerate (4 ‚ °C), do not freeze. Contains no preservatives. Do not store opened vials for future use. Once diluted, 100 mL and 200 mL solutions for infusion are stable for 18 hours when stored at 2 ‚ °C to 8 ‚ °C.
Excipient information presented when available (limited, particularly for generics); consult specific product labeling. [DSC] = Discontinued product
Injection, solution [preservative free]:
Ceredase ‚ ®: 80 units/mL (5 mL [DSC]) [contains albumin (human)]
Stable in NS; do not mix with any other additives.
There are no known significant interactions.
CBC, platelets, liver function tests, IgG antibody formation, acid phosphatase (AP); MRI or CT of liver and spleen, skeletal x-rays, physical exam every 6-12 months
False-positive pregnancy tests
Frequency not defined.
Cardiovascular: Peripheral edema
Central nervous system: Chills, fatigue, fever, headache, lightheadedness
Endocrine & metabolic: Early virilization (males <10 years), hot flashes, menstrual abnormalities
Gastrointestinal: Abdominal discomfort, diarrhea, nausea, oral ulcerations, vomiting
Local: Injection site: Abscess, burning, discomfort, pruritus, swelling
Neuromuscular & skeletal: Backache, weakness
Miscellaneous: Dysosmia; hypersensitivity reactions (abdominal cramping, angioedema, chest discomfort, flushing, hypotension, nausea, pruritus, respiratory symptoms, urticaria); IgG antibody formation (~13%)
Concerns related to adverse effects:
- Hypersensitivity reactions: Patients who develop IgG antibodies may be at a higher risk for developing hypersensitivity. Use with caution in patients with prior allergies to hCG.
Disease-related concerns:
- Androgen-sensitive malignancies: Use with caution in patients with androgen-sensitive malignancies.
Special populations:
- Pediatrics: Safety and efficacy have not been established in children <2 years of age (limited experience). May cause early virilization in males <10 years of age.
Dosage form specific issues:
- Placental tissue: Prepared from pooled human placental tissue that may contain the causative agents of some viral diseases.
C
Animal studies have not been conducted.
Alglucerase is a modified form of glucocerebrosidase; it is prepared from human placental tissue. Glucocerebrosidase is an enzyme deficient in Gauchers disease. It is needed to catalyze the hydrolysis of glucocerebroside to glucose and ceramide.
~3-11 minutes
This medication will not cure Gauchers disease, but may help control it. Treatment is life-long. May cause abdominal discomfort, nausea, or vomiting; these symptoms should go away with continued use. Inform prescriber if pain, swelling, or redness occurs at injection site or if GI symptoms persist.