Cerliponase alfa parenteral
- Drugs List
- Therapeutic Indications
- Precautions and Warnings
- Pregnancy and Lactation
- Side Effects
Infusions of cerliponase alfa.
Neuronal ceroid lipofuscinosis type 2
A flushing solution should be administered after the infusion is complete.
Children aged 2 years and older
300mg once every other week.
Children aged 1 to under 2 years
200mg once every other week for the first 4 doses and then 300mg once every other week for subsequent doses.
Children aged 6 months to under 1 year
150mg once every other week.
Children under 6 months
100mg once every other week.
Additional Dosage Information
Dose adjustment may be necessary in patients who cannot tolerate the infusion. The dose may be reduced by 50% and/or the infusion rate decreased to a slower rate.
If the infusion is stopped because of hypersensitivity reactions, it should be restarted at approximately one half the initial infusion rate at which the hypersensitivity reaction occurred.
The infusion should be interrupted and/or the rate slowed in patients who the treating physician believes may have a possible increase in intracranial pressure as suggested by symptoms such as headache, nausea, vomiting, or decreased mental state. These precautions are of particular importance in patients below 3 years of age.
For intracerebroventricular infusion only.
The infusion rate for cerliponase alfa and the flushing solution is 2.5ml per hour.
Administration site infection
Precautions and Warnings
Children under 2 years
Cardiac conduction defects
Structural cardiac disorder
Pre-medicate with antihistamines with or without antipyretics
Treatment to be initiated and supervised by a specialist
Aseptic technique should be used throughout
For single use only
Resuscitation facilities must be immediately available
Perform ECG before and during treatment
During long term use make clinical examinations at regular intervals
Monitor cerebrospinal fluid for signs of infection
Monitor for increased intracranial pressure regularly
Monitor for intracerebroventricular access device complications
Monitor patient for infusion-associated reactions (IARs)
Monitor vital signs, respiration & cardiac function
Interrupt therapy/reduce infusion rate if infusion-related reactions occur
Discontinue if serious allergic or anaphylactic reaction occurs
Consider interrupting treatment if infection occurs
Infusion related reactions can be minimised by pre-treatment with antihistamines and/or antipyretics from 30 to 60 minutes prior to infusion.
The scalp should be inspected for skin integrity and to detect intracerebroventricular access device leakage and/or failure prior to the start of the infusion.
Limited data is available for children aged 2 years and no clinical data is available in children less than 2 years of age. The safety and efficacy in children less than 3 years has not yet been established. There is limited data in patients aged over 8 years.
Electrocardiogram (ECG) monitoring should be performed during the infusion in patients with a history of bradycardia, conduction disorders, or with structural heart disease, as some patients with neuronal lipofuscinosis type 2 disease may develop conduction disorders or heart disease. In patients with no previous cardiac problems, an ECG should be performed every 6 months.
Continuation of long term treatment with cerliponase alfa should be subject to regular clinical evaluation, and on whether the benefits outweigh the potential risks to individual patients.
Pregnancy and Lactation
Use cerliponase alfa with caution during pregnancy.
The manufacturer recommends that cerliponase alfa should only be given to pregnant women if clearly needed. Animal studies have not been carried out using cerliponase alfa. At the time of writing, there is limited data on the use of cerliponase alfa during human pregnancy. Potential risks are unknown.
Cerliponase alfa is contraindicated during breastfeeding.
The manufacturer recommends that the patient should stop breastfeeding when cerliponase alfa is used. At the time of writing, there is limited data regarding the use of cerliponase alfa during breastfeeding. Effects on exposed infants are unknown.
Cerebrospinal fluid pleocytosis
Cerebrospinal fluid protein abnormalities
Complications associated with the administration device
Dropped head syndrome
Local infection at injection site
Oral mucosal blistering
Upper respiratory tract infection
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 ).
Last Full Review Date: February 2020
Summary of Product Characteristics: Brineura 150mg solution for infusion. Biomarin International Limited. Revised May 2017.
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