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ELAPRASE is a brand name for Idursulfase, supplied as a solution. The medicine, its uses, side effects and dosage are the same regardless of brand.
Used for: ELAPRASE (idursulfase) is indicated for: long-term enzyme replacement therapy in patients with Hunter syndrome (Mucopolysaccharidosis II, MPS II).
Verbatim from this product's HC label. Tap a section to expand.
1 Dosing Considerations ELAPRASE is intended for use under the supervision of a physician or other experienced health care provider. 5 mg/kg body weight every week by intravenous infusion. 3 Administration The total volume of infusion may be administered over a period of 3 hours, which may be Serious Warnings and Precautions Risk of hypersensitivity reactions: Anaphylactoid/anaphylactic reactions, which have the potential to be life threatening, have been observed in some patients treated with ELAPRASE up to several years after initiating treatment.
Patients with compromised respiratory function or acute respiratory disease may be at risk of serious exacerbation of their respiratory dysfunction due to infusion related reactions. These patients require additional monitoring. Late-emergent symptoms and signs of anaphylactoid/anaphylactic reactions have been observed after ELAPRASE administration as long as 24 hours after an initial reaction.
If an anaphylactoid/anaphylactic reaction occurs, the infusion of ELAPRASE should be immediately suspended and appropriate treatment and observation initiated. The current medical standards for emergency treatment are to be followed.
Patients experiencing severe or refractory anaphylactoid/anaphylactic reactions may require prolonged observation times. Due to the potential for severe infusion reactions appropriate medical support measures should be readily available when ELAPRASE is administered.
ELAPRASE® Product Monograph Page 5 of 31 gradually reduced to 1 hour if no infusion-related reactions are observed. Patients may require longer infusion times due to infusion reactions; however, infusion times should not exceed 8 hours.
The initial infusion rate should be 8 mL/hr for the first 15 minutes. If the infusion is well tolerated, the rate may be increased by 8 mL/hr increments at 15 minute intervals in order to administer the full volume within the desired period of time.
However, at no time should the infusion rate exceed 100 mL/hr. The infusion rate may be slowed and/or temporarily stopped, based on clinical judgment, when infusion-related reactions occur. Method of Preparation and Dilution ELAPRASE should be prepared and administered by a healthcare professional.
1. 5 mg/kg. 5 mg per kg of ELAPRASE ÷ 2 mg per mL = Total # mL of ELAPRASE Total # mL of ELAPRASE ÷ 3 mL per vial = Total # of vials Round up to determine the number of whole vials needed from which to withdraw the calculated volume of ELAPRASE to be administered.
1 Adverse Reaction Overview Adverse reactions were commonly reported in association with infusions. The most common infusion-related reactions were cutaneous reactions (rash, pruritus, and urticaria), flushing, hypertension, pyrexia, wheezing, dyspnea, headache, abdominal pain, nausea, dyspepsia, chest pain, and infusion site swelling.
The frequency of infusion-related reactions decreased over time with continued ELAPRASE treatment. Adverse drug reactions (ADRs) that were reported during the 53-week placebo-controlled study were almost all mild to moderate in severity.
2 Clinical Trial Adverse Reactions Because clinical trials are conducted under very specific conditions, the adverse reaction rates ELAPRASE® Product Monograph Page 9 of 31 observed in the clinical trials may not reflect the rates observed in practice and should not be compared to the rates in the clinical trials of another drug.
Adverse reaction information from clinical trials is useful for identifying drug-related adverse events and for approximating rates. 5 mg/kg weekly ELAPRASE compared to patients receiving placebo. Information is presented by system organ class and frequency.
Frequency is given as very common (>1/10) or common (>1/100, <1/10). The occurrence of an event in a single patient is defined as common in view of the small number of patients treated in the trial. Adverse drug reactions were defined in Table 1 as treatment-emergent events with suspected causality and excluded non-serious events that were reported only once in a single patient; treatment emergent events with an excess incidence of at least 9% compared with placebo were also considered as adverse drug reactions.
Adverse reactions occurring only in placebo- treated patients are excluded.
Note:
Please see the Serious Warnings and Precautions Box at the beginning of Part I:
Health Professional Information. General A registry for patients with Hunter syndrome (the Hunter Outcome Survey) has been established in order to better understand the variability and progression of the disease and monitoring and evaluation of treatments.
Patients should be encouraged to participate in the process and advised that their participation may involve a long-term follow-up. Information on the registry program may be obtained by calling 1-800-268-2772.
Carcinogenesis and Mutagenesis See Part II:
Scientific Information, Non-Clinical Toxicology, Mutagenicity and Carcinogenicity Studies, for animal data. Cardiovascular Caution should be exercised when administering ELAPRASE to patients susceptible to fluid overload, or patients with underlying respiratory illness or compromised cardiac and/or respiratory function for whom fluid restriction is indicated.
These patients may be at risk of serious exacerbations of their cardiac or respiratory status during infusion. Hepatic/Biliary/Pancreatic No studies have been performed in patients with hepatic impairment. Route of Administration Dosage Form / Strength/Composition Non-medicinal Ingredients Intravenous (IV) 2 mg/mL concentrate for solution for infusion Polysorbate 20; Sodium chloride; Sodium phosphate dibasic, heptahydrate; Sodium phosphate monobasic, monohydrate; Water for Injection.
ELAPRASE® Product Monograph Page 7 of 31 Immune Hypersensitivity Allergic reactions have included respiratory distress, hypoxia, decreased blood pressure, angioedema, or seizure. If severe allergic or anaphylactoid reactions occur, it is recommended that the administration of ELAPRASE be discontinued immediately and appropriate treatment initiated.
ELAPRASE is contraindicated in Patients who are hypersensitive to this drug or to any ingredient in the formulation, including any non-medicinal ingredient, or component of the container. For complete listing, see Dosage Forms, Strengths, Composition and Packaging.
Any ELAPRASE re-treatment of patients who developed hypersensitivity reactions is at the discretion of the healthcare provider upon successful management of the symptoms.
Not medical advice. Always read the patient information leaflet and follow your prescriber or pharmacist.
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2. Perform a visual inspection of each vial. ELAPRASE is a clear to slightly opalescent, colorless solution. Do not use if the solution in the vials is discolored or particulate matter is present. ELAPRASE should not be shaken. 3. Withdraw the calculated volume of ELAPRASE from the appropriate number of vials.
4. 9% Sodium Chloride Injection, USP. Once diluted into normal saline, the solution in the infusion bag should be mixed gently, but not shaken. Diluted solution stored at room temperature should be discarded if not administered within 8 hours of preparation.
Diluted solution may be stored refrigerated for up to 24 hours (see Storage, Stability, and Disposal). 5. 2 micrometer (μm) filter is recommended. ELAPRASE should not be infused with other products in the infusion tubing. 6. ELAPRASE is supplied in single-use vials.
Remaining ELAPRASE left in a vial after withdrawing the patient’s calculated dose should be disposed of in accordance with local requirements.
All types of rash and all types of urticaria have been combined. 5 mg/kg of ELAPRASE weekly or every other week. Four patients experienced a hypoxic episode during one or several infusions, which necessitated oxygen therapy in 3 patients with severe underlying obstructive airway disease (2 with a tracheostomy).
The most severe episode, which was associated with a short seizure, occurred in a patient who received his infusion while he had a febrile respiratory exacerbation. In this patient, who had less severe underlying disease, spontaneous resolution occurred shortly after the infusion was interrupted.
These events did not recur with subsequent infusions using a slower infusion rate and administration of pre-infusion medication, usually with low-dose steroids, antihistamine, and beta-agonist nebulization. The fifth patient, who had pre-existing cardiopathy, was diagnosed with ventricular premature complexes and pulmonary embolism during the study.
5 mg/kg weekly ELAPRASE® Product Monograph Page 11 of 31 ELAPRASE and placebo treatment groups. Reported frequencies of adverse events have been classified by MedDRA terms. 4) Investigations Alanine aminotransferase […]
The current medical standards for emergency treatment are to be observed. 3%) that involved adverse events in at least two of the following three body systems: cutaneous, respiratory, or cardiovascular. 2%) with symptoms of bronchospasm, cyanosis, dyspnea, erythema, edema (facial and peripheral), flushing, rash, respiratory distress, urticarial, vomiting, and wheezing.
Infusion Reactions In clinical trials with ELAPRASE, the most common infusion-related reactions included cutaneous reactions (rash, pruritus, and urticaria), flushing, hypertension, pyrexia, wheezing, hypoxia, dyspnea, headache, abdominal pain, nausea, dyspepsia, chest pain, and infusion site swelling.
Infusion-related reactions were treated or ameliorated by slowing the infusion rate, interrupting the infusion, or by administration of medicines, such as antihistamines, antipyretics, low-dose corticosteroids (prednisone and methylprednisolone), or beta-agonist nebulization.
Reactions were more severe in patients with compromised respiratory function or respiratory illnesses. No patient discontinued treatment with ELAPRASE due to an infusion reaction during clinical studies. Patients using supplemental oxygen should have this treatment readily available during infusion in the event of an infusion-related reaction.
Monitoring and Laboratory Tests No special laboratory tests are required for patients receiving ELAPRASE, other than the usual tests that are required for monitoring patients with Hunter syndrome. Renal No studies have been conducted in patients with renal impairment.
Respiratory Special care should be taken when administering an infusion in patients with severe underlying airway disease. These patients should be closely monitored and infused in an appropriate clinical setting. Caution must be exercised in the management and treatment of such patients by limitation or careful monitoring of antihistamine and other sedative medicinal product use.
Institution of positive-airway pressure may be necessary in some cases. Delaying the infusion in patients who present with an acute febrile respiratory illness should be considered. Patients using supplemental oxygen should have this treatment readily available during infusion in the event of an infusion-related reaction.
1 Pregnant Women There are no data from studies in pregnant women. Results in animals show that idursulfase is present in the fetal circulation in utero (see Non-Clinical Toxicology, Reproduction and Teratology). ELAPRASE should not be administered during pregnancy except when the indication and need are clear and the potential benefit is judged by the physician to substantially justify the risk.
Caution should be used when giving ELAPRASE to pregnant women after consideration of risks and benefits. 2 Breast-feeding Because of results in animals that show that idursulfase is excreted in the breast milk (see Non- Clinical Toxicology, Reproduction and Teratology), caution should be used when giving ELAPRASE to nursing women after consideration of risks and benefits.
3 Pediatrics Patients in the clinical studies were age 16 months and older. Children, adolescents, and adults responded similarly to treatment with ELAPRASE. 4 Geriatrics Studies in patients over the age of 65 have not been performed.
5 Patients with the Complete Deletion, Large Rearrangement Genotype Pediatric patients with complete deletion, large gene rearrangement genotype have a high probability of developing antibodies, including neutralizing antibodies in response to exposure to ELAPRASE.
Patients with this genotype have a higher probability of developing infusion-related events and tend to show a muted response as assessed by decrease in urine output of glycosaminoglycans, liver size and spleen volume compared to patients with the missense genotype.
Management of patients must be decided on an individual basis.