Imiglucerase parenteral
- Drugs List
- Therapeutic Indications
- Dosage
- Administration
- Contraindications
- Precautions and Warnings
- Pregnancy and Lactation
- Side Effects
- Monograph
Presentation
Parenteral formulations of imiglucerase.
Drugs List
Therapeutic Indications
Uses
Enzyme replacement therapy in patients with Type 1 Gaucher disease
Enzyme replacement therapy in patients with Type III Gaucher disease
For long-term enzyme replacement therapy in patients with a confirmed diagnosis of non-neuronopathic (Type 1) or chronic neuronopathic (Type 3) Gaucher disease and who exhibit clinically significant non-neurological manifestations of the disease.
Such manifestations include one or more of the following conditions:
Anaemia after exclusion of other causes, such as iron deficiency.
Thrombocytopenia.
Bone disease after exclusion of other causes, such as vitamin D deficiency.
Hepatomegaly or splenomegaly.
Dosage
Dosage should be individualised for each patient.
Adults
Initial dose: 60units/kg once every 2 weeks given by intravenous infusion at a rate not exceeding 0.5 unit/kg/minute. At subsequent administrations, infusion rate may be increased but should not exceed 1 unit/kg/minute.
Improvement in haematological and visceral parameters has been shown within 6 months of therapy and continued use has either stopped progression or improved bone disease. Doses of 15units/kg once every 2 weeks has been shown to improve haematological parameters and organomegaly, but not bone parameters.
After the patient response is well established, dosages may be adjusted up or down for continued effective therapy. Usual monitoring intervals range from 6 to 12 months.
Medical or healthcare professionals are encouraged to register Gaucher patients, including those with chronic neuronopathic manifestation of the disease, in the "ICGG Gaucher Registry",
Children
(See Dosage; Adult)
The following alternative dosing schedule may be suitable:
Gaucher's Disease Type 1
Children aged 1 month to 18 years: 30units/kg to 60units/kg once every 2 weeks, adjusted according to response.
Gaucher's Disease Type 3
Children aged 1 month to 18 years: 60units/kg to 120units/kg once every 2 weeks, adjusted according to response.
Neonates
(See Dosage; Adult)
The following alternative dosing schedule may be suitable:
Gaucher's Disease Type 1
30units/kg to 60units/kg once every 2 weeks, adjusted according to response.
Gaucher's Disease Type 3
60units/kg to 120units/kg once every 2 weeks, adjusted according to response.
Administration
For intravenous infusion.
Contraindications
None known
Precautions and Warnings
Restricted sodium intake
Breastfeeding
Pregnancy
Sodium content of formulation may be significant
Treatment to be initiated and supervised by a specialist
Do not use if solution is discoloured or particulates are apparent
Resuscitation facilities must be immediately available
Evaluate patients with respiratory symptoms for pulmonary hypertension
Monitor haematological parameters periodically
Monitor periodically for IgG antibody formation
Monitor visceral parameters periodically
Advise patient to stop therapy & contact Dr if hypersensitivity signs occur
Discontinue if serious allergic or anaphylactic reaction occurs
Patients may develop antibodies to imiglucerase (usually during first 6 to 12 months), these patients are more likely to develop hypersensitivity reactions and may develop a decreased response to treatment. Patients displaying hypersensitivity or decreased response should be monitored for IgG antibody formation.
Treatment should be discontinued immediately if severe allergic or anaphylactic reactions occur. Initiate appropriate treatment in accordance with the current medical standards.
Evaluate patients with respiratory symptoms for pulmonary hypertension. Patients diagnosed with pulmonary hypertension should receive adequate doses of imiglucerase to ensure control of underlying Gaucher disease as well as be evaluated for the need of additional pulmonary hypertension specific treatments.
Pregnancy and Lactation
Pregnancy
Use imiglucerase with caution during pregnancy.
The manufacturer recommends that treatment naive women should consider commencing treatment prior to conception to optimise health. For women already receiving treatment prior to pregnancy, continuation throughout pregnancy should be considered. Women with Gaucher's disease who become pregnant may experience increased disease activity. Close monitoring thoughout pregnancy is recommended to individualise the dose required. Pregnancy is known to stress maternal calcium homeostasis and accelerate bone turnover. Animal studies have not been performed. It is unknown if imiglucerase crosses the human placenta and therefore potential risks are unknown.
Lactation
Use imiglucerase with caution during breastfeeding.
The manufacturer advises that the enzyme is likely to be digested in the child's gastrointestinal tract. It is unknown if imiglucerase is excreted into human breast milk and therefore the effects on exposed infants are unknown.
Side Effects
Abdominal cramps
Abscess formation (injection site)
Anaphylactoid reaction
Angioedema
Arthralgia
Backache
Burning sensation (local)
Chest discomfort
Cough
Cyanosis
Diarrhoea
Dizziness
Dyspnoea
Fatigue
Fever
Flushing
Headache
Hypersensitivity reactions
Hypotension
Local pain (injection site)
Nausea
Paraesthesia
Pruritus
Rash
Respiratory symptoms
Rigors
Swelling (injection site)
Tachycardia
Urticaria
Vomiting
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: December 2019
Reference Sources
Summary of Product Characteristics: Cerezyme 400 units powder for concentrate for solution for infusion. Genzyme Therapeutics. Revised December 2018.
NICE Evidence Services Available at: www.nice.org.uk Last accessed: 08 November 2019.
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.