Idursulfase Solution (Elaprase)
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Idursulfase Solution (Elaprase)

ELAPRASE™
(idursulfase) Solution for intravenous infusion

WARNING

Risk of anaphylaxis.

Life-threatening anaphylactic reactions have been observed in some patients during ELAPRASE (idursulfase solution) infusions. Therefore, appropriate medical support should be readily available when ELAPRASE (idursulfase solution) is administered. Biphasic anaphylactic reactions have also been observed after ELAPRASE (idursulfase solution) administration and patients who have experienced anaphylactic reactions may require prolonged observation. Patients with compromised respiratory function or acute respiratory disease may be at risk of serious acute exacerbation of their respiratory compromise due to infusion reactions, and require additional monitoring.

DRUG DESCRIPTION

ELAPRASE is a formulation of idursulfase, a purified form of human iduronate-2-sulfatase, a lysosomal enzyme. Idursulfase is produced by recombinant DNA technology in a human cell line. Idursulfase is an enzyme that hydrolyzes the 2-sulfate esters of terminal iduronate sulfate residues from the glycosaminoglycans dermatan sulfate and heparan sulfate in the lysosomes of various cell types.

Idursulfase is a 525-amino acid glycoprotein with a molecular weight of approximately 76 kilodaltons. The enzyme contains eight asparagine-linked glycosylation sites occupied by complex oligosaccharide structures. The enzyme activity of idursulfase is dependent onthe post-translational modification of a specific cysteine to formylglycine. Idursulfase has a specific activity ranging from 41 to 77 U/mg of protein (one unit is defined as the amount of enzyme required to hydrolyze 1 µmole of heparin disaccharide substrate per hour under the specified assay conditions).

ELAPRASE (idursulfase solution) is intended for intravenous infusion and is supplied as a sterile, nonpyrogenic clear to slightly opalescent, colorless solution that must be diluted prior to administration in 0.9% Sodium Chloride Injection, USP. Each vial contains an extractable volume of 3.0 mL with an idursulfase concentration of 2.0 mg/mL at a pH of approximately 6, providing 6.0 mg idursulfase, 24.0 mg sodium chloride, 6.75 mg sodium phosphate monobasic monohydrate, 2.97 mg sodium phosphate dibasic heptahydrate, and 0.66 mg polysorbate 20. ELAPRASE (idursulfase solution) does not contain preservatives; vials are for single use only.

What are the possible side effects of idursulfase (Elaprase)?

Some people receiving a idursulfase injection have had a reaction to the infusion (when the medicine is injected into the vein). Tell your caregiver right away if you feel dizzy, light-headed, or have hives, seizure (convulsions), trouble breathing, or swelling of your face, lips, tongue, or throat.

It may still be possible for you to receive idursulfase even after you have had a reaction to it. There are other medications that can be given to you before your idursulfase infusion to help prevent any reaction symptoms.

Call your doctor at once if you have any of these serious...

Read All Potential Side Effects and See Pictures of Elaprase »

Last reviewed on RxList: 11/28/2007
This monograph has been modified to include the generic and brand name in many instances.

INDICATIONS

ELAPRASE (idursulfase solution) is indicated for patients with Hunter syndrome (Mucopolysaccharidosis II, MPS II). ELAPRASE (idursulfase solution) has been shown to improve walking capacity in these patients.

DOSAGE AND ADMINISTRATION

The recommended dosage regimen of ELAPRASE (idursulfase solution) is 0.5 mg/kg of body weight administered every week as an intravenous infusion.

ELAPRASE (idursulfase solution) is a concentrated solution for intravenous infusion and must be diluted in 100 mL of 0.9% Sodium Chloride Injection, USP. Each vial of ELAPRASE contains a 2.0 mg/mL solution of idursulfase protein (6.0 mg) in an extractable volume of 3.0 mL, and is for single use only. Use of an infusion set equipped with a 0.2 micrometer (µm) filter is recommended.

The total volume of infusion may be administered over a period of 1 to 3 hours. Patients may require longer infusion times due to infusion reactions; however, infusion times should not exceed 8 hours (see STORAGE). 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, or discontinued for that visit, based on clinical judgment, if infusion reactions were to occur (see WARNINGS). ELAPRASE (idursulfase solution) should not be infused with other products in the infusion tubing.

Preparation and Administration Instructions: Use Aseptic Techniques

ELAPRASE (idursulfase solution) should be prepared and administered by a health care professional.

  1. Determine the total volume of ELAPRASE (idursulfase solution) to be administered and the number of vials needed based on the patient's weight and the recommended dose of 0.5 mg/kg.
    Patient's weight (kg) × 0.5 mg per kg of ELAPRASE (idursulfase solution) ÷ 2 mg per mL = Total # mL of ELAPRASE (idursulfase solution)
    Total # mL of ELAPRASE (idursulfase solution) ÷ 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 (idursulfase solution) to be administered.
  2. Perform a visual inspection of each vial. ELAPRASE (idursulfase solution) 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 (idursulfase solution) should not be shaken.
  3. Withdraw the calculated volume of ELAPRASE (idursulfase solution) from the appropriate number of vials.
  4. Dilute the total calculated volume of ELAPRASE (idursulfase solution) in 100 mL of 0.9% Sodium Chloride Injection, USP. Once diluted into normal saline, the solution in the infusion bag should be mixed gently, but not shaken. Diluted solution should be discarded if not administered or refrigerated within 8 hours of preparation. Diluted solution may be stored refrigerated for up to 48 hours.
  5. ELAPRASE (idursulfase solution) is supplied in single-use vials. Remaining ELAPRASE (idursulfase solution) left in a vial after withdrawing the patient's calculated dose should be disposed of in accordance with local requirements.

Storage

Store ELAPRASE (idursulfase solution) vials under refrigeration at 2°C to 8°C (36°F to 46°F), and protect from light. Do not freeze or shake. Do not use ELAPRASE (idursulfase solution) after the expiration date on the vial.

This product contains no preservatives. The diluted solution should be used immediately. If immediate use is not possible, the diluted solution can be stored refrigerated at 2°C to 8°C (36°F to 46°F) for up to 48 hours, or must be administered within 8 hours if held at room temperature.

HOW SUPPLIED

ELAPRASE (idursulfase solution) is a sterile, aqueous, clear to slightly opalescent, colorless solution supplied in a 5 mL Type I glass vial. The vials are closed with a butyl rubber stopper with fluororesin coating and an aluminum overseal with a blue flip-off plastic cap.

NDC 54092-700-01

ELAPRASE (idursulfase solution) is manufactured for: Shire Human Genetic Therapies, Inc. 700 Main Street Cambridge, MA 02139 US License Number 1593 OnePathSM phone # 1-866-888-0660 ELAPRASE (idursulfase solution) is a trademark of Shire Human Genetic Therapies, Inc. REV 2 Last revised (October 2007). FDA Rev date: 11/24/2007

Last reviewed on RxList: 11/28/2007
This monograph has been modified to include the generic and brand name in many instances.

SIDE EFFECTS

The most serious infusion-related adverse reactions reported with ELAPRASE (idursulfase solution) were anaphylactic and allergic reactions (see BOXED WARNING and WARNINGS).

In clinical studies, the most frequent serious adverse events related to the use of ELAPRASE (idursulfase solution) were hypoxic episodes. Other notable serious adverse reactions that occurred in the ELAPRASE (idursulfase solution) treated patients but not in the placebo patients included one case each of: cardiac arrhythmia, pulmonary embolism, cyanosis, respiratory failure, infection, and arthralgia.

Adverse reactions were commonly reported in association with infusions. The most common infusion-related reactions were headache, fever, cutaneous reactions (rash, pruritus, erythema, and urticaria), and hypertension. The frequency of infusion-related reactions decreased over time with continued ELAPRASE (idursulfase solution) treatment.

Because clinical trials are conducted under widely varying conditions, adverse reaction rates observed in the clinical trials of a product cannot be directly compared to rates in the clinical trials of another product and may not reflect the rates observed in practice.

Table 3 enumerates those adverse reactions that were reported during the 53-week, placebo- controlled study that occurred in at least 10% of patients treated with ELAPRASE (idursulfase solution) weekly administration, and that occurred more frequently than in the placebo patients. The most common ( > 30%) adverse reactions were pyrexia, headache, and arthralgia.

Table 3 Summary of Adverse Reactions Occurring in at Least 10% of Patients Treated with ELAPRASE (idursulfase solution) Weekly in the 53-week Controlled Trial and Occurring More Frequently than in the Placebo Group

Adverse Event ELAPRASE (idursulfase solution)
0.5 mg/kg Weekly
(n=32)
Placebo
(n=32)
Pyrexia 20 (63%) 19 (59%)
Headache 19 (59%) 14 (44%)
Arthralgia 10 (31%) 9 (28%)
Limb pain 9 (28%) 8 (25%)
Pruritus 9 (28%) 5 (16%)
Hypertension 8 (25%) 7 (22%)
Malaise 7 (22%) 6 (19%)
Visual disturbance 7 (22%) 2 (6%)
Wheezing 6 (19%) 5 (16%)
Abscess 5 (16%) 0 (0%)
Musculoskeletal dysfunction NOS 5 (16%) 3 (9%)
Chest wall musculoskeletal pain 5 (16%) 0 (0%)
Urticaria 5 (16%) 0 (0%)
Superficial injury 4 (13%) 3 (9%)
Anxiety, irritability 4 (13%) 1 (3%)
Atrial abnormality 4 (13%) 3 (9%)
Adverse events resulting from injury 4 (13%) 2 (6%)
Dyspepsia 4 (13%) 0 (0%)
Infusion site edema 4 (13%) 3 (9%)
Skin disorder NOS 4 (13%) 1 (3%)
Pruritic rash 4 (13%) 0 (0%)

Immunogenicity

Fifty-one percent (32 of 63) of patients in the weekly ELAPRASE (idursulfase solution) treatment arm in the clinical study (53-week placebo-controlled study with an open-label extension) developed anti-idursulfase IgG antibodies as assessed by ELISA or conformation specific antibody assay and confirmed by radioimmunoprecipitation assay (RIP). Sera from 4 out of 32 RIP confirmed anti-idursulfase antibody positive patients were found to neutralize idursulfase activity in vitro. The incidence of antibodies that inhibit cellular uptake of idursulfase into cells is currently unknown, and the incidence of IgE antibodies to idursulfase is not known. Patients who developed IgG antibodies at any time had an increased incidence of infusion reactions, including allergic reactions. The reduction of urinary GAG excretion was less in patients in whom circulating anti-idursulfase antibodies were detected. The relationship between the presence of anti-idursulfase antibodies and clinical efficacy outcomes is unknown.

The data reflect the percentage of patients whose test results were positive for antibodies to idursulfase in specific assays, and are highly dependent on the sensitivity and specificity of these assays. Additionally, the observed incidence of antibody positivity in an assay may be influenced by several factors, including sample handling, timing of sample collection, concomitant medication, and underlying disease. For these reasons, comparison of the incidence of antibodies to idursulfase with the incidence of antibodies to other products may be misleading.

Read the Elaprase (idursulfase solution) Side Effects Center for a complete guide to possible side effects »

DRUG INTERACTIONS

No formal drug interaction studies have been conducted with ELAPRASE (idursulfase solution) .

Last reviewed on RxList: 11/28/2007
This monograph has been modified to include the generic and brand name in many instances.

WARNINGS

Anaphylaxis and Allergic Reactions

(see BOXED WARNING)

Life-threatening anaphylactic reactions have been observed in some patients during ELAPRASE (idursulfase solution) infusions. Reactions have included respiratory distress, hypoxia, hypotension, seizure, loss of consciousness, urticaria and/or angioedema of the throat or tongue. Biphasic anaphylactic reactions have also been reported to occur after administration of ELAPRASE (idursulfase solution) approximately 24 hours after treatment and recovery from an initial anaphylactic reaction that occurred during ELAPRASE (idursulfase solution) infusion. Interventions for biphasic reactions have included hospitalization, and treatment with epinephrine, inhaled beta-adrenergic agonists, and corticosteroids.

In clinical trials with ELAPRASE (idursulfase solution) , 16/108 patients (15%) experienced infusion reactions during 26 of 8,274 infusions (0.3%) that involved adverse events in at least two of the following three body systems: cutaneous, respiratory, or cardiovascular. Of these 16 patients, 11 experienced significant allergic reactions during 19 of 8,274 infusions (0.2%). One of these episodes occurred in a patient with a tracheostomy and severe airway disease, who received an ELAPRASE (idursulfase solution) infusion while he had a pre-existing febrile illness, and then experienced respiratory distress, hypoxia, cyanosis, and seizure with loss of consciousness.

Because of the potential for severe infusion reactions, appropriate medical support should be readily available when ELAPRASE (idursulfase solution) is administered. Because of the potential for biphasic anaphylactic reactions after ELAPRASE (idursulfase solution) administration, patients who experience initial severe or refractory reactions may require prolonged observation.

When severe infusion reactions occurred during clinical studies, subsequent infusions were managed by use of antihistamines and/or corticosteroids prior to or during infusions, a slower rate of ELAPRASE (idursulfase solution) administration, and/or early discontinuation of the ELAPRASE (idursulfase solution) infusion if serious symptoms developed. With these measures, no patient discontinued treatment permanently due to an allergic reaction.

Patients with compromised respiratory function or acute respiratory disease may be at higher risk of life-threatening complications from infusion reactions. Consider delaying the ELAPRASE (idursulfase solution) infusion in patients with concomitant acute respiratory and/or febrile illness.

If a severe reaction occurs, immediately suspend the infusion of ELAPRASE (idursulfase solution) and initiate appropriate treatment, depending on the severity of the symptoms. Consider resuming the infusion at a slower rate, or, if the reaction is serious enough to warrant it, discontinue the ELAPRASE (idursulfase solution) infusion for that visit.

PRECAUTIONS

Carcinogenesis, Mutagenesis, Impairment of Fertility

Long-term studies in animals to evaluate carcinogenic potential or studies to evaluate mutagenic potential have not been performed with ELAPRASE (idursulfase solution) .

ELAPRASE (idursulfase solution) at intravenous doses up to 5 mg/kg, administered twice weekly (about 1.6 times the recommended human weekly dose based on body surface area) had no effect on fertility and reproductive performance in male rats.

Pregnancy: Teratogenic Effects: Category C

Reproduction studies in pregnant female animals have not been conducted with ELAPRASE (idursulfase solution) . It is also not known whether ELAPRASE (idursulfase solution) can cause fetal harm when administered to a pregnant woman or can affect reproduction capacity. ELAPRASE (idursulfase solution) should be given to pregnant women only if clearly needed.

Nursing Mothers

It is not known whether this product is excreted in human milk. Because many drugs are excreted in human milk, caution should be exercised when ELAPRASE (idursulfase solution) is administered to a nursing woman.

Pediatric Use

Patients in the clinical studies were age five and older (see CLINICAL STUDIES). Children, adolescents, and adults responded similarly to treatment with ELAPRASE (idursulfase solution) . Safety and efficacy have not been established in pediatric patients less than five years of age.

Geriatric Use

Clinical studies of ELAPRASE (idursulfase solution) did not include patients aged 65 or over. It is not known whether geriatric patients respond differently from younger patients.

Last reviewed on RxList: 11/28/2007
This monograph has been modified to include the generic and brand name in many instances.

OVERDOSE

There is no experience with overdosage of ELAPRASE (idursulfase solution) in humans. Single intravenous doses of idursulfase up to 20 mg/kg were not lethal in male rats and cynomolgus monkeys (approximately 6.5 and 13 times, respectively, of the recommended human dose based on body surface area) and there were no clinical signs of toxicity.

CONTRAINDICATIONS

None.

Last reviewed on RxList: 11/28/2007
This monograph has been modified to include the generic and brand name in many instances.

CLINICAL PHARMACOLOGY

Mechanism of Action

Hunter syndrome (Mucopolysaccharidosis II, MPS II) is an X-linked recessive disease caused by insufficient levels of the lysosomal enzyme iduronate-2-sulfatase. This enzyme cleaves the terminal 2-O-sulfate moieties from the glycosaminoglycans (GAG) dermatan sulfate and heparan sulfate. Due to the missing or defective iduronate-2-sulfatase enzyme in patients with Hunter syndrome, GAG progressively accumulate in the lysosomes of a variety of cells, leading to cellular engorgement, organomegaly, tissue destruction, and organ system dysfunction.

Treatment of Hunter syndrome patients with ELAPRASE (idursulfase solution) provides exogenous enzyme for uptake into cellular lysosomes. Mannose-6-phosphate (M6P) residues on the oligosaccharide chains allow specific binding of the enzyme to the M6P receptors on the cell surface, leading to cellular internalization of the enzyme, targeting to intracellular lysosomes and subsequent catabolism of accumulated GAG.

Pharmacokinetics

The pharmacokinetic characteristics of idursulfase were evaluated in several studies in patients with Hunter syndrome. The serum concentration of idursulfase was quantified using an antigen-specific ELISA assay. The area under the concentration-time curve (AUC) increased in a greater than dose proportional manner as the dose increased from 0.15 mg/kg to 1.5 mg/kg following a single 1-hour infusion of ELAPRASE (idursulfase solution) . The pharmacokinetic parameters at the recommended dose regimen (0.5 mg/kg ELAPRASE (idursulfase solution) administered weekly as a 3-hour infusion) were determined at Week 1 and Week 27 in 10 patients ages 7.7 to 27 years (Table 1). There were no apparent differences in PK parameter values between Week 1 and Week 27.

Table 1 - Pharmacokinetic Parameters (Mean, Standard Deviation)


Pharmacokinetic Parameter Week 1 Week 27
Cmax (µg/mL) 1.5 (0.6) 1.1 (0.3)
AUC (min*µg/mL) 206 (87) 169 (55)
t1/2 (min) 44 (19) 48 (21)
Cl (mL/min/kg) 3.0 (1.2) 3.4 (1.0)
Vss (% BW) 21 (8) 25 (9)

Clinical Studies

The safety and efficacy of ELAPRASE (idursulfase solution) were evaluated in a randomized, double-blind, placebo-controlled clinical study of 96 patients with Hunter syndrome. The study included patients with a documented deficiency in iduronate-2-sulfatase enzyme activity who had a percent predicted forced vital capacity (%-predicted FVC) less than 80%. The patients' ages ranged from 5 to 31 years. Patients who were unable to perform the appropriate pulmonary function testing, or those who could not follow protocol instructions were excluded from the study. Patients received ELAPRASE (idursulfase solution) 0.5 mg/kg every week (n=32), ELAPRASE (idursulfase solution) 0.5 mg/kg every other week (n=32), or placebo (n=32). The study duration was 53 weeks.

The primary efficacy outcome assessment was a two-component composite score based on the sum of the ranks of the change from baseline to Week 53 in distance walked during a six-minute walk test (6-MWT) and the ranks of the change in %-predicted FVC. This two-component composite primary endpoint differed statistically significantly between the three groups, and the difference was greatest between the placebo group and the weekly treatment group (weekly ELAPRASE (idursulfase solution) vs. placebo, p=0.0049).

Examination of the individual components of the composite score showed that, in the adjusted analysis, the weekly ELAPRASE (idursulfase solution) -treated group experienced a 35 meter greater mean increase in the distance walked in six minutes compared to placebo. The changes in %-predicted FVC were not statistically significant (Table 2).

Table 2 - Clinical Study Results


  ELAPRASE (idursulfase solution) Weekly
n=32a
Placebo
n=32a
ELAPRASE (idursulfase solution)
Weekly -
Placebo
Baseline Week 53 Changeb Baseline Week 53 Changeb Difference in
Change
Results from the 6-Minute Walk Test (Meters)
Mean ± SD 392 ± 108 436 ± 138 44 ± 70 393 ± 106 400 ± 106 7 ± 54 37 ± 16c
35 ± 14d
(p=0.01)
Median 397 429 31 403 412 -4
Percentiles (25th, 75th) 316, 488 365, 536 0, 94 341, 469 361, 460 -30, 31
Results from the Forced Vital Capacity Test (% of Predicted)
Mean ± SD 55.3 ± 15.9 58.7 ± 19.3 3.4 ± 10.0 55.6 ± 12.3 56.3 ± 15.7 0.8 ± 9.6 2.7 ± 2.5c
4.3 ± 2.3d
(p=0.07)
Median 54.9 59.2 2.1 57.4 54.6 -2.5
Percentiles (25th, 75th) 43.6, 69.3 44.4, 70.7 -0.8, 9.5 46.9, 64.4 43.8, 67.5 -5.4, 5.0
a One patient in the placebo group and one patient in the ELAPRASE (idursulfase solution) group died before Week 53; imputation wasby last observation carried forward in the intent-to-treat analysis
b Change, calculated as Week 53 minus Baseline
c Observed mean ± SE
d ANCOVA model based mean ± SE, adjusted for baseline disease severity, region, and age.

Measures of bioactivity were urinary GAG levels and changes in liver and spleen size. Urinary GAG levels were elevated in all patients at baseline. Following 53 weeks of treatment, mean urinary GAG levels were markedly reduced in the ELAPRASE (idursulfase solution) weekly group, although GAG levels still remained above the upper limit of normal in half of the ELAPRASE (idursulfase solution) -treated patients. Urinary GAG levels remained elevated and essentially unchanged in the placebo group. Sustained reductions in both liver and spleen volumes were observed in the ELAPRASE (idursulfase solution) weekly group through Week 53 compared to placebo. There were essentially no changes in liver and spleen volumes in the placebo group.

Last reviewed on RxList: 11/28/2007
This monograph has been modified to include the generic and brand name in many instances.

PATIENT INFORMATION

A Hunter Outcome Survey has been established in order to understand better the variability and progression of Hunter syndrome (MPS II) in the population as a whole, and to monitor and evaluate long-term treatment effects of ELAPRASE (idursulfase solution) . Patients and their physicians are encouraged to participate in this program. For more information, visit www.elaprase (idursulfase solution) .com or call OnePathSM at 1-866-888-0660.

Last reviewed on RxList: 11/28/2007
This monograph has been modified to include the generic and brand name in many instances.

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PATIENT INFORMATION

A Hunter Outcome Survey has been established in order to understand better the variability and progression of Hunter syndrome (MPS II) in the population as a whole, and to monitor and evaluate long-term treatment effects of ELAPRASE (idursulfase solution) . Patients and their physicians are encouraged to participate in this program. For more information, visit www.elaprase (idursulfase solution) .com or call OnePathSM at 1-866-888-0660.

Last reviewed on RxList: 11/28/2007
This monograph has been modified to include the generic and brand name in many instances.

ELAPRASE™
(idursulfase) Solution for intravenous infusion

WARNING

Risk of anaphylaxis.

Life-threatening anaphylactic reactions have been observed in some patients during ELAPRASE (idursulfase solution) infusions. Therefore, appropriate medical support should be readily available when ELAPRASE (idursulfase solution) is administered. Biphasic anaphylactic reactions have also been observed after ELAPRASE (idursulfase solution) administration and patients who have experienced anaphylactic reactions may require prolonged observation. Patients with compromised respiratory function or acute respiratory disease may be at risk of serious acute exacerbation of their respiratory compromise due to infusion reactions, and require additional monitoring.

DRUG DESCRIPTION

ELAPRASE is a formulation of idursulfase, a purified form of human iduronate-2-sulfatase, a lysosomal enzyme. Idursulfase is produced by recombinant DNA technology in a human cell line. Idursulfase is an enzyme that hydrolyzes the 2-sulfate esters of terminal iduronate sulfate residues from the glycosaminoglycans dermatan sulfate and heparan sulfate in the lysosomes of various cell types.

Idursulfase is a 525-amino acid glycoprotein with a molecular weight of approximately 76 kilodaltons. The enzyme contains eight asparagine-linked glycosylation sites occupied by complex oligosaccharide structures. The enzyme activity of idursulfase is dependent onthe post-translational modification of a specific cysteine to formylglycine. Idursulfase has a specific activity ranging from 41 to 77 U/mg of protein (one unit is defined as the amount of enzyme required to hydrolyze 1 µmole of heparin disaccharide substrate per hour under the specified assay conditions).

ELAPRASE (idursulfase solution) is intended for intravenous infusion and is supplied as a sterile, nonpyrogenic clear to slightly opalescent, colorless solution that must be diluted prior to administration in 0.9% Sodium Chloride Injection, USP. Each vial contains an extractable volume of 3.0 mL with an idursulfase concentration of 2.0 mg/mL at a pH of approximately 6, providing 6.0 mg idursulfase, 24.0 mg sodium chloride, 6.75 mg sodium phosphate monobasic monohydrate, 2.97 mg sodium phosphate dibasic heptahydrate, and 0.66 mg polysorbate 20. ELAPRASE (idursulfase solution) does not contain preservatives; vials are for single use only.

Last reviewed on RxList: 11/28/2007
This monograph has been modified to include the generic and brand name in many instances.

ELAPRASE™
(idursulfase) Solution for intravenous infusion

WARNING

Risk of anaphylaxis.

Life-threatening anaphylactic reactions have been observed in some patients during ELAPRASE (idursulfase solution) infusions. Therefore, appropriate medical support should be readily available when ELAPRASE (idursulfase solution) is administered. Biphasic anaphylactic reactions have also been observed after ELAPRASE (idursulfase solution) administration and patients who have experienced anaphylactic reactions may require prolonged observation. Patients with compromised respiratory function or acute respiratory disease may be at risk of serious acute exacerbation of their respiratory compromise due to infusion reactions, and require additional monitoring.

DRUG DESCRIPTION

ELAPRASE is a formulation of idursulfase, a purified form of human iduronate-2-sulfatase, a lysosomal enzyme. Idursulfase is produced by recombinant DNA technology in a human cell line. Idursulfase is an enzyme that hydrolyzes the 2-sulfate esters of terminal iduronate sulfate residues from the glycosaminoglycans dermatan sulfate and heparan sulfate in the lysosomes of various cell types.

Idursulfase is a 525-amino acid glycoprotein with a molecular weight of approximately 76 kilodaltons. The enzyme contains eight asparagine-linked glycosylation sites occupied by complex oligosaccharide structures. The enzyme activity of idursulfase is dependent onthe post-translational modification of a specific cysteine to formylglycine. Idursulfase has a specific activity ranging from 41 to 77 U/mg of protein (one unit is defined as the amount of enzyme required to hydrolyze 1 µmole of heparin disaccharide substrate per hour under the specified assay conditions).

ELAPRASE (idursulfase solution) is intended for intravenous infusion and is supplied as a sterile, nonpyrogenic clear to slightly opalescent, colorless solution that must be diluted prior to administration in 0.9% Sodium Chloride Injection, USP. Each vial contains an extractable volume of 3.0 mL with an idursulfase concentration of 2.0 mg/mL at a pH of approximately 6, providing 6.0 mg idursulfase, 24.0 mg sodium chloride, 6.75 mg sodium phosphate monobasic monohydrate, 2.97 mg sodium phosphate dibasic heptahydrate, and 0.66 mg polysorbate 20. ELAPRASE (idursulfase solution) does not contain preservatives; vials are for single use only.

Last reviewed on RxList: 11/28/2007
This monograph has been modified to include the generic and brand name in many instances.

ELAPRASE™
(idursulfase) Solution for intravenous infusion

WARNING

Risk of anaphylaxis.

Life-threatening anaphylactic reactions have been observed in some patients during ELAPRASE (idursulfase solution) infusions. Therefore, appropriate medical support should be readily available when ELAPRASE (idursulfase solution) is administered. Biphasic anaphylactic reactions have also been observed after ELAPRASE (idursulfase solution) administration and patients who have experienced anaphylactic reactions may require prolonged observation. Patients with compromised respiratory function or acute respiratory disease may be at risk of serious acute exacerbation of their respiratory compromise due to infusion reactions, and require additional monitoring.

DRUG DESCRIPTION

ELAPRASE is a formulation of idursulfase, a purified form of human iduronate-2-sulfatase, a lysosomal enzyme. Idursulfase is produced by recombinant DNA technology in a human cell line. Idursulfase is an enzyme that hydrolyzes the 2-sulfate esters of terminal iduronate sulfate residues from the glycosaminoglycans dermatan sulfate and heparan sulfate in the lysosomes of various cell types.

Idursulfase is a 525-amino acid glycoprotein with a molecular weight of approximately 76 kilodaltons. The enzyme contains eight asparagine-linked glycosylation sites occupied by complex oligosaccharide structures. The enzyme activity of idursulfase is dependent onthe post-translational modification of a specific cysteine to formylglycine. Idursulfase has a specific activity ranging from 41 to 77 U/mg of protein (one unit is defined as the amount of enzyme required to hydrolyze 1 µmole of heparin disaccharide substrate per hour under the specified assay conditions).

ELAPRASE (idursulfase solution) is intended for intravenous infusion and is supplied as a sterile, nonpyrogenic clear to slightly opalescent, colorless solution that must be diluted prior to administration in 0.9% Sodium Chloride Injection, USP. Each vial contains an extractable volume of 3.0 mL with an idursulfase concentration of 2.0 mg/mL at a pH of approximately 6, providing 6.0 mg idursulfase, 24.0 mg sodium chloride, 6.75 mg sodium phosphate monobasic monohydrate, 2.97 mg sodium phosphate dibasic heptahydrate, and 0.66 mg polysorbate 20. ELAPRASE (idursulfase solution) does not contain preservatives; vials are for single use only.

Last reviewed on RxList: 11/28/2007
This monograph has been modified to include the generic and brand name in many instances.

Elaprase Patient Information Including Side Effects

Brand Names: Elaprase

Generic Name: idursulfase (Pronunciation: EYE dur SUL fase)

What is idursulfase (Elaprase)?

Idursulfase is used to treat some of the symptoms of a genetic condition called Hunter's syndrome. Hunter syndrome is also called mucopolysaccharidosis (MYOO-koe-pol-ee-SAK-a-rye-DOE-sis).

Hunter syndrome is a metabolic disorder in which the body lacks the enzyme needed to break down certain sugars and proteins. These substances can build up in the body, causing enlarged organs, abnormal bone structure, changes in facial features, breathing problems, heart problems, vision loss, and changes in mental or physical abilities.

Idursulfase may improve walking ability in people with this condition. However, this medication is not a cure for Hunter syndrome.

Idursulfase may also be used for purposes not listed in this medication guide.

What are the possible side effects of idursulfase (Elaprase)?

Some people receiving a idursulfase injection have had a reaction to the infusion (when the medicine is injected into the vein). Tell your caregiver right away if you feel dizzy, light-headed, or have hives, seizure (convulsions), trouble breathing, or swelling of your face, lips, tongue, or throat.

It may still be possible for you to receive idursulfase even after you have had a reaction to it. There are other medications that can be given to you before your idursulfase infusion to help prevent any reaction symptoms.

Call your doctor at once if you have any of these serious side effects:

  • worsened asthma;
  • uneven heartbeats;
  • blue lips or fingernails;
  • fever;
  • vision problems; or
  • increased blood pressure (severe headache, blurred vision, trouble concentrating, chest pain, numbness, seizure).

Less serious side effects may include:

  • joint pain;
  • pain in your arms or legs;
  • headache;
  • itching, mild skin rash; or
  • weakness.

This is not a complete list of side effects and others may occur. Tell your doctor about any unusual or bothersome side effect. You may report side effects to FDA at 1-800-FDA-1088.

Read the Elaprase (idursulfase solution) Side Effects Center for a complete guide to possible side effects »

What is the most important information I should know about idursulfase (Elaprase)?

Idursulfase may improve walking ability in people with Hunter syndrome. However, idursulfase is not a cure for this condition.

Some people receiving an idursulfase injection have had a reaction to the infusion (when the medicine is injected into the vein). Tell your caregiver right away if you feel dizzy, light-headed, or have hives, seizure (convulsions), trouble breathing, or swelling of your face, lips, tongue, or throat.

It may still be possible for you to receive idursulfase even after you have had a reaction to it. There are other medications that can be given to you before your idursulfase infusion to help prevent any reaction symptoms.

You may be more likely to have a reaction to idursulfase if you have a breathing disorder. Tell your doctor if you have asthma or other lung disease.

Your name may need to be listed on a Hunter Outcome Survey while you are using this medication. The purpose of this registry is to track the progression of this disorder and the effects that idursulfase has on long-term treatment of Hunter syndrome.

Side Effects Centers

Elaprase Patient Information including How Should I Take

What should I discuss with my health care provider before receiving idursulfase (Elaprase)?

You should not receive this medication if you are allergic to idursulfase.

Before receiving idursulfase, tell your doctor if you are allergic to any drugs, or if you have asthma or other breathing disorder.

You may be more likely to have a reaction to idursulfase if you have a breathing disorder. You may need to receive other medications to prevent an symptoms of a reaction to idursulfase. Follow your doctor's instructions.

Your name may need to be listed on a Hunter Outcome Survey while you are using this medication. The purpose of this registry is to track the progression of this disorder and the effects that idursulfase has on long-term treatment of Hunter syndrome.

FDA pregnancy category C. This medication may be harmful to an unborn baby. Tell your doctor if you are pregnant or plan to become pregnant during treatment.

It is not known whether idursulfase passes into breast milk or if it could harm a nursing baby. Do not use this medication without telling your doctor if you are breast-feeding a baby.

How is idursulfase given (Elaprase)?

Idursulfase is given as an injection through a needle placed into a vein. You will most likely receive this injection in a clinic or hospital setting. Idursulfase is usually given once per week.

The medicine must be given slowly through an IV infusion, and can take up to 3 hours to complete.

Side Effects Centers

Elaprase Patient Information including If I Miss a Dose

What happens if I miss a dose (Elaprase)?

Call your doctor if you miss an appointment for your idursulfase injection.

What happens if I overdose (Elaprase)?

Seek emergency medical attention if you think you have received too much of this medicine.

Symptoms of an idursulfase overdose are not known.

What should I avoid while receiving idursulfase (Elaprase)?

Follow your doctor's instructions about any restrictions on food, beverages, or activity while you are receiving idursulfase.

What other drugs will affect idursulfase (Elaprase)?

There may be other drugs that can interact with idursulfase. Tell your doctor about all your prescription and over-the-counter medications, vitamins, minerals, herbal products, and drugs prescribed by other doctors. Do not start a new medication without telling your doctor.

Where can I get more information?

Your doctor or pharmacist can provide more information about idursulfase.


Remember, keep this and all other medicines out of the reach of children, never share your medicines with others, and use this medication only for the indication prescribed.

Every effort has been made to ensure that the information provided by Cerner Multum, Inc. ('Multum') is accurate, up-to-date, and complete, but no guarantee is made to that effect. Drug information contained herein may be time sensitive. Multum information has been compiled for use by healthcare practitioners and consumers in the United States and therefore Multum does not warrant that uses outside of the United States are appropriate, unless specifically indicated otherwise. Multum's drug information does not endorse drugs, diagnose patients or recommend therapy. Multum's drug information is an informational resource designed to assist licensed healthcare practitioners in caring for their patients and/or to serve consumers viewing this service as a supplement to, and not a substitute for, the expertise, skill, knowledge and judgment of healthcare practitioners. The absence of a warning for a given drug or drug combination in no way should be construed to indicate that the drug or drug combination is safe, effective or appropriate for any given patient. Multum does not assume any responsibility for any aspect of healthcare administered with the aid of information Multum provides. The information contained herein is not intended to cover all possible uses, directions, precautions, warnings, drug interactions, allergic reactions, or adverse effects. If you have questions about the drugs you are taking, check with your doctor, nurse or pharmacist.

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Side Effects Centers

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