WARNING: HYPERSENSITIVITY REACTIONS INCLUDING ANAPHYLAXIS

Life-threatening hypersensitivity reactions, including anaphylaxis, have occurred in some ELELYSO-treated patients. Administration of ELELYSO should be supervised by a healthcare provider knowledgeable in the management of hypersensitivity reactions, including anaphylaxis.  Anaphylaxis has occurred during the early course of enzyme replacement therapy and after extended duration of therapy. Initiate ELELYSO in a healthcare setting with appropriate medical monitoring and support measures, including access to cardiopulmonary resuscitation equipment. Observe patients closely for 3 hours after the start of each infusion. If a severe hypersensitivity reaction occurs, discontinue ELELYSO and immediately initiate appropriate medical treatment, including use of epinephrine.  

In clinical trials, (patients were not routinely pretreated with antihistamines and/or corticosteroids prior to ELELYSO infusions during the clinical trials):

2 of 72 (3.0%) ELELYSO-treated patients experienced signs and symptoms of anaphylaxis, including urticaria, hypotension, flushing, wheezing, chest tightness, nausea, vomiting, and dizziness. These reactions have occurred during ELELYSO infusion.

21 of 72 (29%) ELELYSO-treated patients experienced hypersensitivity reactions, including the 2 ELELYSO-treated patients who experienced signs and symptoms consistent with anaphylaxis. This included pruritus, angioedema, flushing, erythema, rash, nausea, vomiting, cough, chest tightness, and throat irritation. These reactions occurred during ELELYSO infusion and up to 3 hours after the start of infusion.

ELELYSO-treated patients who developed anti-taliglucerase alfa antibodies (referred to as anti-drug antibodies (ADA)) generally had a greater frequency of hypersensitivity reactions compared to those who did not develop ADA. Closely monitor for hypersensitivity reactions in patients who develop ADA.

Management of hypersensitivity reactions should be based on the severity of the reaction and includes slowing or temporary interruption of the infusion and/or administration of antihistamines, antipyretics, and/or corticosteroids for mild reactions. To reduce the risk of hypersensitivity reactions, consider pretreatment with antihistamines and/or corticosteroids. If a severe hypersensitivity reaction (e.g. anaphylaxis occurs), discontinue ELELYSO and immediately initiate appropriate medical treatment, including use of epinephrine. Inform patients of the signs and symptoms of anaphylaxis and instruct them to seek immediate medical care should signs and symptoms occur.

Consider the risks and benefits of re-administering ELELYSO in patients who have experienced a severe hypersensitivity reaction associated with ELELYSO. Caution should be exercised upon rechallenge.

The most common adverse reactions (≥5%) in treatment-naïve adults treated with ELELYSO were headache, arthralgia, fatigue, nausea, dizziness, abdominal pain, pruritus, flushing, vomiting, and urticaria.

The most common adverse reaction (≥10%) for treatment-naïve pediatric patients treated with ELELYSO was vomiting.

The most common adverse reactions (≥10%) in patients who switched from imiglucerase to ELELYSO were arthralgia, headache, and pain in extremity.

Clinical trials (Trials 1, 2 and 3) evaluated ELELYSO enzyme replacement therapy (ERT)-naïve and ERT- experienced adult and pediatric patients with Gaucher disease. In patients with Type 1 Gaucher disease, hypersensitivity reactions occurred in 36% (9/25) of ELELYSO treated patients who developed ADA during the treatment period and in 15% (6/41) of ELELYSO-treated patients who did not develop ADA during the treatment period. Of the 9 ELELYSO-treated patients who tested positive for ADA and who developed hypersensitivity reactions, 2 patients had anaphylaxis and 1 additional patient discontinued ELELYSO due to hypersensitivity reactions.

The recommended dosage for treatment-naïve adult and pediatric patients 4 years of age and older is 60 units per kg of body weight administered once every 2 weeks as a 60- to 120-minute intravenous infusion.

If it is acceptable to switch from a stable imiglucerase dosage to ELELYSO, initiate ELELYSO intravenous treatment (60- to 120-minute infusion) with the same units/kg imiglucerase dosage and subsequently administer ELELYSO every other week. Dose adjustments can be made based on achievement and maintenance of each patient’s therapeutic goals.

ELELYSO is supplied as 200 units per vial and is available by prescription only.

Please click here for Full Prescribing Information, including BOXED WARNING. 

Please click here for Full Prescribing Information, including BOXED WARNING.