Patient Medication Information: uses, side effects, how to take - Elaprase
What is ELAPRASE used for?
- ELAPRASE is a long-term enzyme replacement therapy in patients with Hunter syndrome. Treatment with ELAPRASE should be supervised by a physician or other experienced health care provider.
How does ELAPRASE work?
Patients with Hunter syndrome do not produce enough of their own enzyme, iduronate-2-sulfatase. The reduced iduronate-2-sulfatase levels in patients result in the accumulation of substances called glycosaminoglycans (GAG) in a number of cell types and tissues. ELAPRASE is an enzyme replacement therapy that is intended to restore sufficient levels of enzyme to assist in the removal of these accumulated substances and to reduce further accumulation.
What are the ingredients in ELAPRASE?
Medicinal ingredients: The active substance in ELAPRASE is idursulfase (2 mg/mL). Idursulfase is a form of the human enzyme iduronate-2-sulfatase. It is produced by recombinant DNA technology.
Non-medicinal ingredients: polysorbate 20, sodium chloride, sodium phosphate dibasic heptahydrate, sodium phosphate monobasic monohydrate and water for injection.
Serious Warnings and Precautions
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ELAPRASE comes in the following dosage forms:
2 mg/mL concentrate for solution for infusion in a clear, glass vial (bottle).
Warnings and Precautions:
To help avoid side effects and ensure proper use, talk to your healthcare professional before you take ELAPRASE. Talk about any health conditions or problems you may have.
If you are treated with ELAPRASE you may experience reactions during or following an infusion. Most infusion reactions are mild or moderate but some may be serious. The most common symptoms are rash, itching, flushing, hypertension, wheezing, cough, headache, abdominal pain, nausea, chest pain, and swelling. Most of the time, you can still be given ELAPRASE even if these symptoms occur. If you experience an allergic side effect following administration of ELAPRASE, you should contact your doctor immediately. You may be given additional medicines such as antihistamines and corticosteroids to treat or help prevent allergic-type reactions.
If severe, allergic-type (hypersensitivity) reactions occur, your doctor may consider stopping the infusion immediately, and should start giving you suitable treatment.
If you have a fever and an acute illness affecting your lungs, your doctor may delay your infusion.
The nature of your genetic mutation may influence your therapeutic response to ELAPRASE, as well as your risk of developing antibodies and infusion-related adverse events; please consult your doctor.
If you are pregnant or breast-feeding, think you may be pregnant or are planning to have a baby, ask your doctor or pharmacist for advice before taking this medicine.
Tell your healthcare professional about all the medicines you take, including any drugs, vitamins, minerals, natural supplements or alternative medicines.
The following may interact with ELAPRASE:
- There is no known interaction of ELAPRASE with other medicines
How to take ELAPRASE:
ELAPRASE has to be diluted in 9 mg/mL (0.9%) sodium chloride solution before use. The usual dose is an infusion of 0.5 mg (half a milligram) for every kg you weigh. This would be about 18 mg or 3 vials (bottles) of ELAPRASE for a 36 kg individual.
After dilution ELAPRASE is given through a vein (drip feed).
The infusion will normally last for 3 hours, which may be gradually reduced to 1 hour if no infusion-related reactions are observed and will be given every week.
Overdose:
One patient with Hunter syndrome, who received ELAPRASE at twice the recommended dosage for one and a half years, experienced two anaphylactic reactions over a 3-month period 4.5 years after initiating ELAPRASE treatment.
| If you think you have taken too much ELAPRASE, contact your health care professional, hospital emergency department or regional poison control centre immediately, even if there are no symptoms. |
What are possible side effects from using ELAPRASE?
These are not all the possible side effects you may feel when taking ELAPRASE. If you experience any side effects not listed here, contact your healthcare professional.
Like all medicines, ELAPRASE can cause side effects, although not everybody gets them. Most side effects are mild to moderate and generally are associated with the infusion; however some side effects may be serious and may need treatment. Over time the number of these infusion-associated reactions generally decreases.
Very common side effects (more than 1 per 10) are:
- Headache
- Increased blood pressure
- Heartburn
- Chest pain
- Hives, rash, itching
- Fever, and infusion site swelling
Common side effects (more than 1 per 100) are:
- Dizziness, tremor
- Teary eyes
- Changes in the way your heart beats, bluish skin
- Decreased blood pressure, flushing (redness)
- Difficulty breathing, wheezing, blood clot in the lung artery, cough, quickened breathing
- Abdominal pain, nausea, diarrhea, swollen tongue
- Facial swelling, skin lesions (redness, eczema)
- Pain in the joints
- Swelling of the extremities
Allergic reactions have included temporary breathing difficulty, decreased blood pressure, or swelling. In a more severe reaction, in a single patient, a seizure occurred because of a drop in blood oxygen level from difficulty in breathing. Inform your doctor immediately if you have any of these side effects. Patients with compromised respiratory function or acute respiratory disease are at greater risk for infusion-related reactions.
If you notice any side effects not mentioned in this leaflet, please inform your doctor.
A registry (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. All patients are encouraged to participate and advised that their participation may involve long-term follow-up. Information on this registry program is available by calling 1-800-268-2772.
Serious side effects and what to do about them
| Symptom / effect | Talk to your healthcare professional | Stop taking drug and get immediate medical help | |
| Only if severe | In all cases | ||
COMMON / VERY COMMON (occurring in ≥5% in controlled clinical studies) Fever | ✓ | ||
| Difficulty breathing | ✓ | ||
| Bluish skin discoloration | ✓ | ||
| Rash or Hives | ✓ | ||
UNCOMMON (occurring in <5% in controlled clinical studies) Seizure, blood clot in the lungs | ✓ | ||
| Missed or extra heartbeats | ✓ | ||
If you have a troublesome symptom or side effect that is not listed here or becomes bad enough to interfere with your daily activities, talk to your healthcare professional.
Reporting Side Effects
You can report any suspected side effects associated with the use of health products to Health Canada by:
NOTE: Contact your health professional if you need information about how to manage your side effects. The Canada Vigilance Program does not provide medical advice. |
Storage:
Store at 2°C to 8°C (in a refrigerator), and protect from light.
Do not freeze or shake.
Keep out of reach and sight of children.
If you want more information about ELAPRASE:
- Talk to your healthcare professional
- Find the full product monograph that is prepared for healthcare professionals and includes this Patient Medication Information by visiting the Health Canada website; the manufacturer’s website www.takeda.com/en-ca, or by calling 1-800-268-2772.
Takeda Canada Inc.
22 Adelaide Street West, Suite 3800
Toronto Ontario M5H 4E3
Last revised:
ELAPRASE® and the ELAPRASE Logo® are registered trademarks of Shire Human Genetic Therapies, Inc. TAKEDA™ and the TAKEDA Logo® are trademarks of Takeda Pharmaceutical Company Limited, used under license.