ELAPRASE (idursulfase) is an enzyme replacement therapy from Shire Human Genetic Therapies. ELAPRASE is designed to replace iduronate-2-sulfatase (I2S), the enzyme that is deficient or absent in people with Hunter syndrome.
Indication
ELAPRASE® (idursulfase) is indicated for patients with Hunter syndrome (Mucopolysaccharidosis II, MPS II). ELAPRASE has been shown to improve walking capacity in these patients.
Important Safety Information
Some patients in the clinical trials experienced life-threatening immediate allergic reactions to ELAPRASE (idursulfase) infusion. Patients who have experienced severe allergic reactions may experience another allergic reaction approximately 24 hours after the initial reaction and may require prolonged observation. Patients with compromised respiratory function or acute respiratory disease may have a higher risk of life-threatening reactions to ELAPRASE infusion and require additional monitoring.
Life-threatening allergic reactions have been seen in some patients during ELAPRASE infusions. Reactions included difficulty breathing, lack of oxygen, seizure or loss of consciousness, hives, and/or swelling of the throat or tongue. Patients received an antihistamine to reverse the allergic reaction or corticosteroid to decrease inflammation prior to or during later infusions. ELAPRASE was given more slowly or was stopped early if patients had serious reactions. With these measures, no patients stopped treatment permanently due to an infusion reaction.
Because of the potential for life-threatening complications, the ELAPRASE infusion may have to be delayed for patients who have respiratory illness and/or fever.
The most common side effects that required intervention had to do with infusions. These included headache, fever, rash, hives, itching and increased blood pressure. These side effects decreased over time with continued ELAPRASE treatment.
In clinical trials, patients given ELAPRASE once a week for 52 weeks had the following side effects at a rate greater than placebo and at least 10%. Approximately:
About half of the patients in clinical studies produced antibodies to treatment with ELAPRASE and these patients had an increase in infusion reactions. The presence of antibodies on the effectiveness of ELAPRASE is unknown.
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