Elaprase
Generic Name:
Idursulfase
Brand Names:
Elaprase
Elaprase Description:
Elaprase is a form of enzyme replacement therapy used to treat patients with Hunter syndrome, a genetic metabolic disorder that results from a lack of the enzyme iduronate-2-sulfatase. Elaprase is formulated from idursulfase, a purified form of human iduronate-2-sulfatase that is produced by recombinant DNA technology. Elaprase can only treat the symptoms of Hunter syndrome and is not a cure for the condition.
Elaprase Uses:
Elaprase is used to treat patients with an inherited disease known as Hunter syndrome, or mucopolysaccharidosis type II. Hunter syndrome is a genetic disorder caused by a lack of the enzyme iduronate-2-sulfatase and is usually seen in males. Elaprase can improve the signs and symptoms of Hunter syndrome, especially the walking capacity of patients, by replacing the iduronate-2-sulfatase enzyme.
The absence of the enzyme iduronate-2-sulfatase can result in a build-up of sugar molecules in various body tissues, leading to a toxic condition. Symptoms of Hunter syndrome may become noticeable after the first year of life, and the major symptoms include:
- Developmental delays
- Coarse facial features
- Skeletal irregularities
- Short stature
- Carpal tunnel syndrome
- Frequent ear infections
- Respiratory infections
- Enlarged abdomen
- Breathing problems when sleeping (sleep apnea)
- Joint stiffness
- Cardiovascular disorders
- Vision loss
- Skin lesions on the back and upper arms
- Hearing loss
- Hyperactivity or aggression
- Diarrhea
Hunter syndrome can lead to many other medical conditions such as:
- Chronic ear and sinus infections
- Pneumonia
- Sleep apnea
- Breathing difficulties
- Thickening of the heart valves
- Heart failure
- High blood pressure
- Joint stiffness
- Stunted growth
- Hernias
- Carpal tunnel syndrome
- Seizures
Elaprase Dosage Information:
Typical Dosage Recommendation:
Elaprase dosage recommendations are based on a patient’s body weight. The usual dose is 0.5mg/kg of body weight, received once a week as an intravenous injection. The medication must be given over a period of up to eight hours, but the typical administration lasts one to three hours.
Missing a Dose:
Elaprase must be given in a treatment center by a doctor. There is a minimal chance for missing a dose of this medication, but be sure to keep all medical appointments so as to not miss a weekly dosage.
Overdosing:
There has been no experience with Elaprase overdose in humans.
Elaprase FDA Approval:
July 24, 2006
Elaprase Recall/Black Box Warning Information:
There have been no recalls for Elaprase.
Other Warnings
Anaphylactic Reactions
Elaprase has a Black Box warning regarding its potential to cause life-threatening anaphylactic reactions following intravenous administration. Patients with inadequate respiratory function or acute respiratory disease may be at an increased risk for anaphylactic reaction and should be monitored accordingly.
The symptoms associated with anaphylactic reaction include:
- Anxiety
- Confusion
- Cough
- Diarrhea
- Dizziness
- Fainting
- Hives
- Nausea
- Skin redness
- Slurred speech
- Trouble breathing, wheezing
- Vomiting
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