Idursulfase Brand Name– Elaprase
What is Idursulfase
Idursulfase a purified form of human iduronate-2-sulfatase, is for the treatment of Hunter syndrome, which is also called mucopolysaccharidosis (MPS) II.
Patients with the syndrome do not produce enough of a functional lysosomal enzyme called iduronate-2-sulfatase because of a a genetic defect.
The deficiency of iduronate-2-sulfatase production causes an accumulation of glycosaminoglycans in cells throughout the body. The buildup of these carbohydrates produces a variety of physical and neurological problems including abnormal bone and joint growth, respiratory problems, learning disabilities, and hearing loss.
Many people with severe MPS II die during early adolescence, but people with milder forms of the disorder can live well into adulthood. Idursulfase serves as a replacement for the deficient enzyme in people with MPS II.
Idursulfase is produced by recombinant DNA technology in a human cell line and is the first treatment for Hunter syndrome.
A Hunter outcome survey has been established in order to understand better the variability and progression of Hunter syndrome in the population as a whole and to monitor and evaluate long-term treatment effects of idursulfase.
Patients and their physicians are encouraged to participate in this program. For more information, visit the official Elaprase website or call 1—866—888—0660.
Idursulfase was FDA-approved in July 2006.
Indications
- mucopolysaccharidosis II (Hunter Syndrome)
For the treatment of mucopolysaccharidosis II (Hunter syndrome)
NOTE: Idursulfase has been designated as an orphan drug for this indication by the FDA.
Side Effects
- angioedema
- antibody formation
- arthralgia
- bronchospasm
- chills
- cough
- cyanosis
- diarrhea
- dizziness
- edema
- erythema
- fatigue
- fever
- flushing
- headache
- hypotension
- hypoxia
- infection
- infusion-related reactions
- nausea
- pruritus
- pulmonary embolism
- rash
- respiratory arrest
- seizures
- serious hypersensitivity reactions or anaphylaxis
- urticaria
- ventricular tachycardia
- vomiting
- wheezing
Monitoring Parameters
- laboratory monitoring not necessary
Contraindications
- acute bronchospasm
- angioedema
- asthma
- breast-feeding
- cardiac disease
- children
- fever
- geriatric
- heart failure
- hypotension
- infants
- infusion-related reactions
- neonates
- pregnancy
- pulmonary edema
- respiratory depression
- respiratory infection
- respiratory insufficiency
- serious hypersensitivity reactions or anaphylaxis
Interactions
There are no drug interactions associated with Idursulfase products.
