The information in this site is intended for U.S. healthcare professionals only.

Visit Patient Site
navigation logo
Doctor measuring a patient's height and weight with caregiver
Committed to MPS II Care

ADMINISTRATION INSTRUCTIONS

  1. ELAPRASE is intended for IV use only. Obtain or assess IV access.1
  1. Administer the diluted ELAPRASE solution to patients using a low-protein-binding infusion set equipped with a low-protein-binding 0.2 micrometer (μm) in-line filter.1
  • ELAPRASE should not be infused with other products in the infusion tubing.
  1. Start the infusion control device to regulate the flow of the ELAPRASE solution at the prescribed infusion rate:1
  • The total volume of infusion should be administered over a period of 3 hours, which may be gradually reduced to 1 hour if no hypersensitivity reactions are observed.
  • The initial infusion rate should be 8 mL per hour for the first 15 minutes.
  • If the infusion is well tolerated, the rate of infusion may be increased by 8 mL per hour increments every 15 minutes.
  • The infusion rate should not exceed 100 mL per hour.
  • Patients may require longer infusion times if hypersensitivity reactions occur; however, infusion times should not exceed 8 hours.
  • The infusion rate may be slowed, temporarily stopped, or discontinued for that visit in the event of hypersensitivity reactions. See Section 5.1 of the Warnings and Precautions section of the Prescribing Information.
  1. After the ELAPRASE solution has been infused, remove the ELAPRASE drug bag from the IV administration set and replace with the bag containing 50 mL of 0.9% sodium chloride injection, USP. Flush through the volume of drug remaining in the IV tubing to ensure full dosing.2

Physicians can consider whether home infusions may be an option for patients that tolerate ELAPRASE infusions well. These administration guidelines are recommendations only. They should be reconciled with appropriate institution policies and procedures, other manufacturers’ guidelines, required regulations, and medical judgment.

Important Safety Information
Important Safety Information

WARNING: RISK OF ANAPHYLAXIS

Life-threatening anaphylactic reactions have occurred in some patients during and up to 24 hours after ELAPRASE infusions. Anaphylaxis, presenting as respiratory distress, hypoxia, hypotension, urticaria and/or angioedema of throat or tongue have been reported to occur during and after ELAPRASE infusions, regardless of duration of the course of treatment. Closely observe patients during and after ELAPRASE administration and be prepared to manage anaphylaxis.

Important Safety Information

WARNING: RISK OF ANAPHYLAXIS

Life-threatening anaphylactic reactions have occurred in some patients during and up to 24 hours after ELAPRASE infusions. Anaphylaxis, presenting as respiratory distress, hypoxia, hypotension, urticaria and/or angioedema of throat or tongue have been reported to occur during and after ELAPRASE infusions, regardless of duration of the course of treatment. Closely observe patients during and after ELAPRASE administration and be prepared to manage anaphylaxis. Inform patients of the signs and symptoms of anaphylaxis and have them seek immediate medical care should symptoms occur. Patients with compromised respiratory function or acute respiratory disease may be at risk of serious acute exacerbation of their respiratory compromise due to hypersensitivity reactions, and require additional monitoring.

Hypersensitivity Reactions Including Anaphylaxis:

Ensure that personnel administering product are adequately trained in cardiopulmonary resuscitative measures, and have ready access to emergency medical services (EMS).

If anaphylactic or other acute reactions occur, immediately discontinue the infusion of ELAPRASE and initiate appropriate medical treatment. Observe patients closely for an appropriate period of time after administration of ELAPRASE, taking into account the time to onset of anaphylaxis seen in premarketing clinical trials and postmarketing reports. Inform patients of the signs and symptoms of anaphylaxis, and instruct them to seek immediate medical care should signs and symptoms occur. When severe reactions have occurred during clinical trials, subsequent infusions were managed with antihistamine and/or corticosteroids prior to or during infusions, a slower rate of ELAPRASE infusion, and/or early discontinuation of the ELAPRASE infusion.

Risk of Hypersensitivity, Serious Adverse Reactions, and Antibody Development in Hunter Syndrome Patients with Severe Genetic Mutations:

Hunter syndrome patients aged 7 years and younger with complete gene deletion, large gene rearrangement, nonsense, frameshift or splice site mutations experienced a higher incidence of hypersensitivity reactions, serious adverse reactions and anti-idursulfase antibody development.

Risk of Acute Respiratory Complications:

Patients with compromised respiratory function or acute febrile or respiratory illness may be at higher risk of life-threatening complications from hypersensitivity reactions. Careful consideration should be given to the patient’s clinical status prior to administration of ELAPRASE and consider delaying the ELAPRASE infusion.

Risk of Acute Cardiorespiratory Failure:

Caution should be exercised when administering ELAPRASE to patients susceptible to fluid overload, or patients with acute underlying respiratory illness or compromised cardiac and/or respiratory function for whom fluid restriction is indicated. These patients may be at risk of serious exacerbation of their cardiac or respiratory status during infusions. Appropriate medical support and monitoring measures should be readily available during ELAPRASE infusion, and some patients may require prolonged observation times that should be based on the individual needs of the patient.

Adverse Reactions:

In clinical trials, the most frequent serious adverse reactions following ELAPRASE treatment were hypoxic episodes. Other notable serious adverse reactions that occurred in the ELAPRASE treated patients but not in the placebo treated patients included one case each of: cardiac arrhythmia, pulmonary embolism, cyanosis, respiratory failure, infection, and arthralgia.

The most common adverse reactions occurring in at least three patients (≥9%) aged five years and older were headache, pruritus, musculoskeletal pain, urticaria, diarrhea, and cough. The most common adverse reactions occurring in at least three patients (≥10%) aged seven years and younger were pyrexia, rash, vomiting, and urticaria. In all clinical trials, the most common adverse reactions requiring medical intervention were hypersensitivity reactions, and included rash, urticaria, pruritus, flushing, pyrexia, and headache.

Immunogenicity:

In clinical trials in patients 5 years and older, 32 of 63 (51%) patients tested positive for anti-idursulfase IgG antibodies (Ab) at least one time. Of the 32 Ab-positive patients, 23 of 32 (72%) tested positive for Ab at three or more different time points (persistent Ab). The incidence of hypersensitivity reactions was higher in patients who tested positive for Ab than those who tested negative.

Thirteen of 32 (41%) Ab-positive patients also tested positive for antibodies that neutralize idursulfase uptake into cells (neutralizing antibodies, NAb) or enzymatic activity at least one time, and 8 (25%) of Ab-positive patients had persistent NAb. There was no clear relationship between the presence of either Ab or NAb and therapeutic response.

In the clinical trial in patients 7 years and younger, 19 of 28 (68%) patients treated with ELAPRASE 0.5 mg/kg once weekly tested Ab-positive, with 16 of 19 (84%) having persistent Ab. In addition, 15 of 19 (79%) Ab-positive patients tested positive for NAb, with 14 of 15 (93%) having persistent NAb.

Postmarketing Experience:

Late-emergent symptoms and signs of anaphylactic reactions have occurred up to 24 hours after initial treatment and recovery from an initial anaphylactic reaction. In addition, patients experienced repeated anaphylaxis over a two to four month period, up to several years after initiating ELAPRASE treatment.

Serious adverse reactions that resulted in death included cardiorespiratory arrest, respiratory failure, respiratory distress, cardiac failure, and pneumonia.

To report SUSPECTED ADVERSE REACTIONS, contact Takeda at 1-877-TAKEDA-7 (1-877-825-3327) or FDA at 1-800-FDA-1088 or www.fda.gov/medwatch
For additional safety information, please click here for Full Prescribing Information, including Boxed Warning.

1. ELAPRASE Prescribing Information.

2. Lilley LL, Raniforth Collins S, Snyder JS. Pharmacology and the Nursing Process (7th ed). Maryland Heights, MO; Elsevier, 2014.

Questions about patient support services?
Contact Takeda Patient Support: 1-866-888-0660

For more information, contact Takeda at 1-877-TAKEDA-7 (1-877-825-3327), or by email at medinfous@takeda.com

©2024 Takeda Pharmaceuticals U.S.A., Inc., 500 Kendall Street, Cambridge, MA 02142. 1-877-TAKEDA-7 (1-877-825-3327). All rights reserved. TAKEDA, , and are trademarks or registered trademarks of Takeda Pharmaceutical Company Limited. ELAPRASE and are registered trademarks of Shire Human Genetic Therapies, Inc. All content of this website including text, images, graphics, audio, and their arrangement, is the property of Takeda and is protected by international copyright laws. All other intellectual property rights are reserved. The content may not be copied for commercial use or distribution, nor may these objects be downloaded, modified, or posted to other sites. This site is intended solely for US residents and is governed solely by US laws and government regulations. Please see our Terms of Use and online Privacy Notice for more information. While Takeda makes reasonable efforts to include accurate, up-to-date information on the site, Takeda makes no warranties or representations as to its accuracy. Takeda assumes no liability for any errors or omissions in the content of the site.
US-IDU-0023v5.0 07/24. US-IDU-0240v1.0 12/24