Olipudase alfa parenteral
- Drugs List
- Therapeutic Indications
- Dosage
- Administration
- Contraindications
- Precautions and Warnings
- Pregnancy and Lactation
- Side Effects
- Monograph
Presentation
Infusions of olipudase alfa.
Olipudase alfa is a recombinant form of human acid sphingomyelinase and is produced by recombinant DNA technology using Chinese Hamster Ovary (CHO) cell lines.
Drugs List
Therapeutic Indications
Uses
Acid sphingomyelinase deficiency
Treatment of non central nervous system manifestations of acid sphingomyelinase deficiency in paediatric and adult patients with type A/B or type B.
Dosage
Olipudase alfa dose is based on the actual body weight for patient with a body mass index (BMI) less than or equal to 30 or an optimal body weight for patient with a BMI of greater than 30.
Adults
Patients with a BMI less than or equal to 30
Initial: 0.1mg/kg body weight.
The dose should be administered every 2 weeks according to the dose escalation regimen:
Week 2: 0.3mg/kg
Week 4: 0.3mg/kg
Week 6: 0.6mg/kg
Week 8: 0.6mg/kg
Week 10: 1mg/kg
Week 12: 2mg/kg
Week 14: 3mg/kg
Maintenance dose: 3mg/kg every 2 weeks.
Patients with a BMI greater than 30
The dose is estimated via the following method:
Body weight (kg) to be used for dose calculation = 30 x (actual height in metres) squared.
Children
Patients with a BMI less than or equal to 30
Initial: 0.03mg/kg body weight.
The dose should be administered every 2 weeks according to the dose escalation regimen:
Week 2: 0.1mg/kg
Week 4: 0.3mg/kg
Week 6: 0.3mg/kg
Week 8: 0.6mg/kg
Week 10: 0.6mg/kg
Week 12: 1mg/kg
Week 14: 2mg/kg
Week 16: 3mg/kg
Maintenance dose: 3mg/kg every 2 weeks.
Patients with a BMI greater than 30
The dose is estimated via the following method:
Body weight (kg) to be used for dose calculation = 30 x (actual height in metres) squared.
Additional Dosage Information
A dose is defined as missed if it is not administered within 3 days of the scheduled date. Dose escalation phase
If 1 dose is missed: the last tolerated dose should be administered and then the dose escalation should be resumed according to the appropriate regimen.
If 2 consecutive doses are missed: 1 dose level lower than the last tolerated dose (a minimal dose of 0.3mg/kg) should be administered and then the dose escalation should be resumed according to the appropriate regimen.
If 3 or more consecutive doses are missed: the dose escalation should be resumed at 0.3mg/kg according to the appropriate regimen. If a missed dose occurred and the next planned dose is 0.3mg/kg or 0.6mg/kg than that dose should be administered twice as specified in the appropriate regimen. Dose maintenance phase
If 1 dose is missed: the maintenance dose should be administered and the schedule of next dose adjusted accordingly. If 2 consecutive doses are missed: 1 dose below the maintenance dose should be administered and then for next planned administration the maintenance dose should be resumed according to the appropriate regimen every 2 weeks. If 3 or more consecutive doses are missed: the dose escalation should be resumed at 0.3mg/kg according to the appropriate regimen.
Administration
For intravenous infusion after reconstitution and dilution.
Infusions should be administered in a stepwise manner preferably using an infusion pump.
Contraindications
Breastfeeding
Pregnancy
Precautions and Warnings
Females of childbearing potential
Advise patient hypotension may affect ability to drive or operate machinery
Premedication with corticosteroid, antihistamine & antipyretic recommended
Treatment to be initiated and supervised by a specialist
Record name and batch number of administered product
Resuscitation facilities must be immediately available
Monitor serum transaminases before treatment
Monitor transaminases 1 month before and during any dose escalation
Monitor patient for infusion-associated reactions (IARs)
Interrupt therapy/reduce infusion rate if infusion-related reactions occur
Discontinue if serious allergic or anaphylactic reaction occurs
Female: Ensure adequate contraception during treatment
IgE antidrug antibodies (ADA) testing may be considered for patients who experienced a severe hypersensitivity reaction to olipudase alfa.
IgG ADA testing may be considered in case of loss of response to therapy.
During dose escalation or when resuming treatment following missed doses, transaminase levels should be obtained within 72 hours prior to next scheduled olipudase alfa infusion. If either the baseline or a pre-infusion transaminase level is greater than 2 times the upper limit of normal during dose escalation, then additional transaminase levels should be obtained within 72 hours after the end of the infusion. If the pre-infusion transaminase levels are elevated above baseline and greater than 2 times the upper limit of normal, the olipudase alfa dose can be adjusted (prior dose repeated or reduced) or treatment can be temporarily stopped in line with the degree of transaminase elevation.
Transaminase testing can be performed as part of routine clinical management once the recommended maintenance dose is reached.
Home infusion can be considered if the patient is on a maintenance dose and tolerating the infusions well.
Pregnancy and Lactation
Pregnancy
Olipudase alfa is contraindicated during pregnancy.
The manufacturer advises that olipudase alfa should be avoided during pregnancy and in women of childbearing potential not using contraception unless the potential benefit to the mother outweighs the potential risk to the fetus. At the time of writing there is no experience with the use of olipudase alfa in pregnant women. Risks are unknown.
Lactation
Olipudase alfa is contraindicated during breastfeeding.
The manufacturer does not recommend breastfeeding whilst taking olipudase alfa and advises that the patient discontinues olipudase alfa or discontinues breastfeeding. It is unknown if olipudase alfa is excreted in human milk. A risk to neonates cannot be excluded.
Side Effects
Abdominal discomfort
Abdominal pain
Alanine aminotransferase increased
Anaphylaxis
Angioedema
Arthralgia
Aspartate aminotransferase increased
Asthenia
Back pain
Bone pain
Catheter site pain
Catheter site pruritus
Catheter site related reactions
Catheter site swelling
Chills
Diarrhoea
Dyspnoea
Erythema
Erythematous rash
Eye pruritus
Fatigue
Fixed drug eruption
Flushing
Gastro-intestinal pain
Headache
Hepatic pain
Hot flushes
Hypersensitivity reactions
Hypotension
Increased serum ferritin
Macular rash
Maculopapular rash
Morbilliform eruption
Myalgia
Nausea
Ocular discomfort
Ocular hyperaemia
Pain
Palpitations
Papular eruption
Papules
Pharyngeal oedema
Pruritic rash
Pruritus
Pyrexia
Raised C-reactive protein
Rash
Tachycardia
Throat irritation
Throat swelling
Throat tightness
Upper abdominal pain
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: January 2023
Reference Sources
Summary of Product Characteristics: Xenpozyme 20mg powder for concentrate for solution for infusion. Aventis Pharma Ltd. Revised August 2022.
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