Idursulfase parenteral
- Drugs List
- Therapeutic Indications
- Dosage
- Contraindications
- Precautions and Warnings
- Pregnancy and Lactation
- Side Effects
- Monograph
Presentation
Infusions of idursulfase.
Drugs List
Therapeutic Indications
Uses
Mucopolysaccharidosis II (Hunter syndrome): long term treatment
Dosage
Adults
500 micrograms/kg body weight once weekly by intravenous infusion over 3 hours.
The infusion duration may be reduced gradually to 1 hour if no infusion-associated adverse reactions occur.
Children
Children aged 16 months to 18 years
500 micrograms/kg body weight once weekly by intravenous infusion over 3 hours.
The infusion duration may be reduced gradually to 1 hour if no infusion-associated adverse reactions occur.
Additional Dosage Information
Home treatment with idursulfase may be considered for patients who have received several months of treatment in the clinic and who are tolerating their infusions well. These should be performed under the surveillance of a physician or other healthcare professional.
Contraindications
Breastfeeding
Pregnancy
Precautions and Warnings
Children under 16 months
Restricted sodium intake
Severe respiratory disease
Sodium content of formulation may be significant
Consider pre-medication with antihistamines and/or antipyretics
Treatment to be initiated and supervised by a specialist
Delay treatment if acute febrile illness
Delay treatment if respiratory illness
Dilute and use as an infusion
If adverse reactions occur, reduce rate or temporarily stop infusion
Record name and batch number of administered product
Resuscitation facilities must be immediately available
Monitor patient for infusion-associated reactions (IARs)
Monitor patients with risk factors for respiratory symptoms
May cause anaphylactic / anaphylactoid reactions
Discontinue if serious allergic or anaphylactic reaction occurs
In patients who present with an acute febrile respiratory illness, consider delaying the infusion. Patients receiving supplemental oxygen should have their oxygen readily available during the infusion, in case of an infusion-related reaction.
Paediatric patients with the complete deletion or large rearrangement genotype have a high probability of developing antibodies, including neutralising antibodies, in response to exposure to idursulfase. Patients with this genotype have a higher probability of developing infusion-related adverse effects 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.
Anaphylactoid/anaphylatic reactions, have been observed in some patients up to several years after initiating treatment. Late emergent symptoms and signs of anaphylactoid/anaphylatic reactions have been observed as long as 24 hours after an initial reaction.
Pregnancy and Lactation
Pregnancy
Idursulfase is contraindicated during pregnancy.
The manufacturer does not recommend using idursulfase during pregnancy. At the time of writing there is limited published information regarding the use of idursulfase during pregnancy. Animal studies do not indicate direct or indirect harmful effects with respect to pregnancy, embryonal/foetal development, parturition or postnatal development. The potential risk is unknown.
Lactation
Idursulfase is contraindicated during breastfeeding.
The manufacturer does not recommend breastfeeding whilst taking idursulfase. It is not known whether idursulfase is excreted in human breast milk. Available data in animals have shown excretion of idursulfase in breast milk. A risk to the newborn/infant can not be excluded.
Side Effects
Abdominal pain
Anaphylactoid reaction
Anaphylaxis
Antibody formation
Arrhythmias
Arthralgia
Bronchospasm
Chest pain
Cough
Cyanosis
Diarrhoea
Dizziness
Dyspepsia
Dyspnoea
Erythema
Facial oedema
Flushing
Headache
Hypertension
Hypotension
Hypoxia
Infusion-related symptoms
Nausea
Peripheral oedema
Pruritus
Pulmonary embolism
Pyrexia
Rash
Swelling (injection site)
Tachycardia
Tachypnoea
Tongue swelling
Tremor
Urticaria
Vomiting
Wheezing
Overdosage
It is strongly recommended that the UK National Poisons Information Service be consulted on cases of suspected or actual overdose where there is doubt over the degree of risk or about appropriate management.
The following number will direct the caller to the relevant local centre (0844) 892 0111
Information may be obtained if you have access to ToxBase the primary clinical toxicology database of the National Poisons Information Service. This is available via password on the internet ( www.toxbase.org ) or if this is unavailable at the backup site ( www.toxbasebackup.org ).
Further Information
Last Full Review Date: June 2020
Reference Sources
Summary of Product Characteristics: Elaprase 2mg/ml concentrate for solution for infusion. Shire Pharmaceuticals Limited. Revised October 2019.
NICE Evidence Services Available at: www.nice.org.uk Last accessed: 12 June 2020
Medscape UK | Univadis prescription drug monographs & interactions are based on FDB Multilex Content
FDB Disclaimer : FDB Multilex is intended for the use of healthcare professionals and is provided on the basis that the healthcare professionals will retain FULL and SOLE responsibility for deciding what treatment to prescribe or dispense for any particular patient or circumstance.