Adenosine deaminase severe combined immune deficiency (ADA-SCID).
Replaces adenosine deaminase. Without this enzyme, adenosine accumulates, which leads to apoptosis, lymphopenia, and opportunistic infections.
Increased lymphocyte concentrations.
Metabolism and Excretion: Unknown.
TIME/ACTION PROFILE (plasma concentrations)
- Severe thrombocytopenia.
Use Cautiously in:
- OB: Safety not established in pregnancy;
- Lactation: Use while breastfeeding only if potential maternal benefit justifies potential risk to infant;
- Geri: Safety and effectiveness not established in older adults.
Adverse Reactions/Side Effects
* CAPITALS indicate life-threatening.
Underline indicate most frequent.
IM (Adults and Children): Transitioning from pegademase bovine weekly dose ≤30 units/kg or unknown– 0.2 mg/kg once weekly; may ↑ dose by increments of 0.033 mg/kg if trough adenosine deaminase activity <30 mmol/hr/L, trough deoxyadenosine nucleotides >0.02 mmol/L, and immune reconstitution not achieved. Transitioning from pegademase bovine weekly dose >30 units/kg– elapegademase dose (in mg/kg) (given once weekly) = pegademase bovine dose (in units/kg) /150; may ↑ dose by increments of 0.033 mg/kg if trough adenosine deaminase activity <30 mmol/hr/L, trough deoxyadenosine nucleotides >0.02 mmol/L, and immune reconstitution not achieved. Pegademase bovine-naïve– 0.2 mg/kg (based on ideal body weight or actual body weight, whichever is greater) twice weekly for ≥12–24 wk until immune reconstitution achieved, then ↓ dose to maintain trough adenosine deaminase activity >30 mmol/hr/L, trough deoxyadenosine nucleotides <0.02 mmol/L, and immune reconstitution.
Solution for injection: 1.6 mg/mL
- Monitor for signs and symptoms of infection during therapy and if decline in trough plasma ADA activity occurs.
Lab Test Considerations:
Monitor trough plasma ADA activity, trough dAXP levels, and/or total lymphocyte counts every 2 wk for patients new to therapy and every 4 wk for patient previously receiving therapy, during 1st 8–12 wk of therapy for up to 1 yr and every 3–6 mo thereafter. Monitor more frequent if therapy was interrupted or if an enhanced rate of clearance of plasma ADA activity develops. Collect blood samples for the analysis of trough plasma ADA activity and trough dAXP level prior to the first administration of elapegademase for the week. Target trough plasma ADA activity at least 30 mmol/hr/L. Monitor trough erythrocyte dAXP levels at least twice yearly and maintain at <0.02 mmol/L.
- Risk for infection (Indications)
- IM Do not dilute or mix with other agents. Solution is clear and colorless; do not inject solutions that are cloudy, discolored, or contain particulate matter. Allow to warm to room temperature for 30 min; do not freeze. Administer using polypropylene syringes. Draw solution from the vial with a 25- gauge needle or larger. Change needle to a size and gauge appropriate for patient's intramuscular administration. Administer immediately after preparation. Discard any remaining medication. Administer IM; rotate sites periodically.
- Explain purpose of medication to patient and caregiver. Emphasize importance of adherence to therapy.
- Advise patient to notify health care professional of all Rx or OTC medications, vitamins, or herbal products being taken and to consult health care professional before taking any new medications.
- Advise patient to notify health care professional if pregnancy is planned or suspected or if breastfeeding.
Trough erythrocyte dAXP levels <0.02 mmol/L.