ELAPRASE

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Safety information

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Brief Safety Statement

Please consult the ELAPRASE® Summary Product Characteristics (SmPC) before prescribing.

ELAPRASE treatment should be supervised by a physician or other healthcare professional experienced in the management of patients with MPS II or other inherited metabolic disorders.

Contraindication
Severe or life-threatening hypersensitivity to the active substance or to any of the excipients if hypersensitivity is not controllable.

The most commonly observed adverse events with ELAPRASE treatment were infusion-related reactions, which included cutaneous reactions (rash, pruritus, urticarial, and erythema), pyrexia, flushing, wheezing, dyspnea, headache, vomiting, abdominal pain, nausea, and chest pain.

Very common adverse reactions (frequency ≥1/10) included headache, flushing, wheezing, dyspnoea, abdominal pain, nausea, diarrhoea, vomiting, urticaria, rash, pruritus, erythema, pyrexia, chest pain, infusion-related reactions.

Detailed Safety Statement

Please consult the ELAPRASE Summary Product Characteristics (SmPC) before prescribing, ELAPRASE treatment should be supervised by a physician or other healthcare professional experienced in the management of patients with MPS II or other inherited metabolic disorders.

Contraindication
Severe or life-threatening hypersensitivity to the active substance or to any of the excipients if hypersensivity is not controllable.

Warnings and Precautions

Infusion-related reactions: Patients treated with idursulfase may develop infusion-related reactions. No patient discontinued treatment due to an infusion reaction during clinical studies. Special care should be taken when administering an infusion in patients with severe underlying airway disease. 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.

Anaphylactoid/anaphylactic reactions: These have the potential to be life-threatening and have been observed in some patients treated with idursulfase up to several years after initiating treatment. Late emergent symptoms and signs of anaphylactoid/ anaphylactic reactions have been observed as long as 24 hours after an initial reaction. If an anaphylactoid/ anaphylactic reaction occurs, the infusion should be immediately suspended and appropriate treatment and observation initiated. Patients who have experienced anaphylactoid/ anaphylactic reactions should be treated with caution when re-administering idursulfase, appropriately trained personnel and equipment for emergency resuscitation (including epinephrine) should be available during infusions. Severe or potentially life-threatening hypersensitivity is a contraindication to rechallenge, if hypersensitivity is not controllable.

Patients with the complete deletion/large rearrangement genotype: Paediatric patients with this genotype have a high probability of developing antibodies, including neutralizing antibodies, in response to exposure to idursulfase. Patients with this genotype have a higher probability of developing infusion-related adverse events and tend to show a muted response compared to patients with the missense genotype.

Sodium: ELAPRASE contains 0.482 mmol sodium (11.1 mg) per vial. This is equivalent to 0.6% of the WHO recommended maximum daily intake of 2 g sodium for an adult.

Traceability: In order to improve the traceability of biological medicinal products, the name and batch number of the administered product should be clearly recorded.

Immunogenicity
Across 4 clinical studies 53/107 patients (50%) developed anti-idursulfase IgG antibodies at some point. The overall neutralizing antibody rate was 26/107 patients (24%).

Adverse Reactions

The most commonly observed adverse events with ELAPRASE treatment were infusion-related reactions, which included cutaneous reactions (rash, pruritus, urticarial, and erythema), pyrexia, flushing, wheezing, dyspnea, headache, vomiting, abdominal pain, nausea, and chest pain.

Very common
(frequency ≥1/10):

Headache, flushing, wheezing, dyspnoea, abdominal pain, nausea, diarrhoea, vomiting, urticaria, rash, pruritus, pyrexia, erythema, chest pain, infusion-related reactions

Common
(≥1/100 to <1/10):

Dizziness, tremor, cyanosis, arrhythmia, tachycardia, hypertension, hypotension, hypoxia, bronchospasm, cough, swollen tongue, dyspepsia, arthralgia, infusion-site swelling, face oedema, oedema peripheral

Uncommon
(≥1/1000 to <1/100):

Tachypnoea

Frequency not known

Anaphylactoid/anaphylactic reaction

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