Special Warning : אזהרת שימוש

4.4   Special warnings and precautions for use

Infusion-related reactions
Patients treated with idursulfase may develop infusion-related reactions (see section 4.8). During clinical trials, the most common infusion-related reactions included cutaneous reactions (rash, pruritus, urticaria), pyrexia, headache, hypertension, and flushing. Infusion-related reactions were treated or ameliorated by slowing the infusion rate, interrupting the infusion, or by administration of medicinal products, such as antihistamines, antipyretics, low-dose corticosteroids (prednisone and methylprednisolone), or beta-agonist nebulisation. 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. 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.

Anaphylactoid/anaphylactic reactions
Anaphylactoid/anaphylactic reactions, which have the potential to be life threatening, 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.
The current medical standards for emergency treatment are to be observed. Patients experiencing severe or refractory anaphylactoid/anaphylactic reactions may require prolonged clinical monitoring. 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 (see section 4.3).

Patients with the complete deletion/large rearrangement genotype
Paediatric patients with the complete deletion/large rearrangement 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 as assessed by decrease in urinary 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 (see section 4.8).

Sodium
This medicinal product contains 0.482 mmol sodium (or 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.

Effects on Driving

4.7   Effects on ability to drive and use machines
Idursulfase has no or negligible influence on the ability to drive and use machines.