Lumizyme (alglucosidase alfa)
Metabolic Disease Enzyme Replacement, Pompe Disease Intravenous Vial (EA)
Last reviewed · Source: First Databank (FDB) drug data
Overview
Alglucosidase alfa is a hydrolytic lysosomal glycogen-specific recombinant human acid alpha-glucosidase (GAA), an enzyme that catalyzes the hydrolysis of alpha-1,4- and alpha-1,6-glycosidic linkages of lysosomal glycogen.
Alglucosidase alfa, a recombinant form of acid alpha-glucosidase (GAA), is a lysosomal enzyme that catalyzes the hydrolysis of alpha-1,4- and alpha-1,6-glycosidic linkages of lysosomal glycogen. Alglucosidase alfa is prepared from cultures of genetically modified Chinese hamster ovary cells using recombinant DNA technology.
Pompe disease is an inherited disorder of glycogen metabolism caused by the absence or marked deficiency of the lysosomal enzyme GAA. In patients with Pompe disease, alglucosidase alfa provides an exogenous source of GAA. After IV administration, alglucosidase alfa is internalized and transported into lysosomes via cell membrane mannose-6-phosphate receptors on the cell surface.
In the lysosomes, alglucosidase alfa undergoes proteolytic cleavage resulting in increased enzymatic activity.
Plasma concentrations of alglucosidase alfa increase in an approximate dose-proportional manner between the dose range of 20 to 40 mg/kg. The half-life of the drug has been reported to be about 2.3 hours. Increased clearance has been observed in patients who develop anti-alglucosidase alfa antibody titers.
USES: This medication is used by people with a certain inherited condition (Pompe disease) to reduce high levels of a certain substance (glycogen) that builds up in the body. High levels of glycogen in the body may cause muscle weakness, heart problems, and breathing problems. Alglucosidase alfa works by breaking down glycogen.
Generic name: alglucosidase alfa
Pronunciation: (AL-gloo-KOE-si-dase AL-fa)
Drug class: Metabolic Disease Enzyme Replacement, Pompe Disease
Therapeutic class: Metabolic Disease Enzyme Replacement Agents
Reference product & biosimilars
No reference, biosimilar or interchangeable associated with this drug.
Today’s intelligence on Lumizyme
Indications
The following indications are listed for Lumizyme:
- Lysosomal Alpha-1,4-Glucosidase Deficiency
Also known as: Acid Maltase Deficiency, Glycogen Storage Disease Type 2, Glycogen Storage Disease Type Ii, Glycogenosis Type Ii, Pompe's Disease.
Dosing
Alglucosidase alfa has a specific activity of 3.6 to 5.4 units/mg (one unit is defined as that amount of activity that results in the hydrolysis of 1 micromole of synthetic substrate per minute under specified assay conditions).
Alglucosidase alfa is administered by IV infusion only. The drug is commercially available as lyophilized cake or powder that must be reconstituted and diluted prior to administration.
Alglucosidase alfa injection should be refrigerated at 2-8degreesC. Alglucosidase alfa injection contains no preservatives. Aseptic technique must be observed during preparation, and filter needles should not be used. The vials are intended for single use only, and any unused portion of the drug should be discarded.
Alglucosidase alfa should be administered using an infusion pump and an in-line low protein binding 0.2-micron filter.
Do not infuse alglucosidase alfa simultaneously through the same IV line with other products.
| Drug label | Dosing type | Instructions |
|---|---|---|
| Lumizyme 50 Mg Vial | Maintenance | Infuse 20 mg/kg over 4 hour(s) by intravenous route every 2 weeks at a rate of 1 mg/kg/hr for 30 minutes may increase to a max of 7 mg/kg/hr. |
No dosing information is available for this tab.
Drug interactions
No formal drug interaction studies have been performed to date.
There are no contraindication interactions for this drug.
There are no severe interactions for this drug.
There are no moderate interactions for this drug.
Contraindications
*None.
There are no contraindication contraindications for this drug.
There are no severe contraindications for this drug.
- Cardiopulmonary impairment
- Chronic heart failure
- Hypervolemia
Adverse reactions
The most common adverse reactions (>=5%) reported with alglucosidase alfa in clinical trials were hypersensitivity reactions (e.g., anaphylaxis, rash, pyrexia, flushing/feeling hot, urticaria, headache, hyperhidrosis, nausea, cough, decreased oxygen saturation, tachycardia, tachypnea, chest discomfort, dizziness, muscle twitching, agitation, cyanosis, erythema, hypertension/increased blood pressure, pallor, rigors, tremor, vomiting, fatigue, myalgia).
More Frequent
- Anaphylaxis
- Angioedema
- Bronchospastic pulmonary disease
- Cyanosis
- Hypertension
- Hypoxia
- Infection
- Tachypnea
- Urticaria
Less Frequent
- Anemia
- Apnea
- Bradycardia
- Hypoventilation
- Seizure disorder
- Tachycardia
- Throat constriction
Rare or Very Rare
- Acute respiratory failure
- Bacterial sepsis
- Cardiac arrest
- Cerebrovascular accident
- Dermal necrosis
- Dissection of aorta
- Dyspnea
- Glomerulonephritis
- Heart failure
- Hypersensitivity drug reaction
- Nephrotic syndrome
- Pharyngeal edema
- Pneumonia
- Pneumothorax
- Severe dyspnea
- Ventricular premature beats
More Frequent
- Agitation
- Chest pain
- Cough
- Diarrhea
- Dizziness
- Erythema
- Fatigue
- Fever
- Flushing
- Headache disorder
- Hypotension
- Malaise
- Myalgia
- Nausea
- Periorbital edema
- Pharyngitis
- Rhinitis
- Skin rash
- Upper abdominal pain
- Vomiting
- Wheezing
Less Frequent
- Back pain
- Constipation
- Gastroenteritis
- Hyperhidrosis
- Pallor
- Paresthesia
- Pruritus of skin
- Tremor
Rare or Very Rare
- Acute abdominal pain
- Arthralgia
- Chills
- Conjunctivitis
- Drowsy
- Eye tearing
- Flu-like symptoms
- Livedo reticularis
- Muscle spasm
- Nervousness
- Pain
- Peripheral edema
- Proteinuria
- Skin inflammation
- Syncope
Precautions
No enhanced messaging available.
Safety and efficacy of alglucosidase alfa have been established in pediatric patients with Pompe disease. Evidence supporting the use of alglucosidase alfa in pediatric patients is derived from an adequate and well-controlled trial in 57 treatment-naive pediatric patients with infantile-onset Pompe disease who were 0.2 months to 3.5 years of age at first infusion and from a randomized, double-blind, placebo-controlled study in patients with late-onset Pompe disease, which included 2 patients who were 16 years of age or younger.
The available data from postmarketing and published case reports in pregnant women receiving alglucosidase alfa have not identified a drug-associated risk of major birth defects, miscarriage, or adverse maternal or fetal outcomes. The decision to use alglucosidase alfa during pregnancy should be individualized. Untreated Pompe disease may result in worsening respiratory and musculoskeletal symptoms in pregnant women. No adverse embryofetal effects have been observed in animal reproduction studies.
A pregnancy exposure registry has been established for alglucosidase alfa to monitor pregnancy outcomes in women exposed to the drug during pregnancy. Pregnant patients and females of reproductive potential should be encouraged to enroll in the registry. For additional information, visit https://www.registrynxt.com/ or call 1-800-745-4447, extension 15500.
Available data suggest that alglucosidase alfa is distributed into human milk. Adverse effects of alglucosidase alfa on the breastfed infant have not been reported. It is not known whether the drug affects milk production.
To minimize drug exposure to a breastfed infant, the manufacturer states that a lactating woman may consider interrupting breastfeeding, pumping and discarding breast milk during treatment and for 24 hours after administration of alglucosidase alfa. The developmental and health benefits of breastfeeding should be considered along with the mother's clinical need for alglucosidase alfa and any potential adverse effects on the breastfed child from the drug or from the underlying maternal condition.
Lactating women with Pompe disease treated with alglucosidase alfa should be encouraged to enroll in the Pompe disease registry.
Clinical studies of alglucosidase alfa did not include a sufficient number of patients 65 years of age and older to determine whether they respond differently from younger adult patients.
ICD codes
| Code | Type | Description |
|---|---|---|
| Lysosomal alpha-1,4-glucosidase deficiency | ||
| E74.02 | ICD10CM | Pompe disease |
ICD code information from First Databank (FDB).
Formulary status
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Last reviewed · Source: First Databank (FDB) drug data
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