Elapegademase-lvlr injection is prescribed to treat adenosine deaminase (ADA) enzyme deficiency in children and adult patients with adenosine deaminase severe combined immune deficiency (ADA-SCID).
Elapegademase-lvlr is a PEGylated recombinant enzyme that eliminates the usage of enzymes obtained from animal sources and works by providing sufficient amounts of enzyme in deficient patients thereby increasing the levels of ADA enzyme.
ADA-SCID is a rare inherited genetic disorder that occurs in less than one in 100,000 live births globally.
Elapegademase-lvlr should not be used in patients-
• If there is an allergy to elapegademase-lvlr
• With a history of severe thrombocytopenia
Extreme caution is required while treating pregnant women and breastfeeding mothers with elapegademase-lvlr and their status has to be monitored closely.
The dose of elapegademase-lvlr is chosen based on the patients who use the animal source of adenosine deaminase and either who do not use or do not have an idea of what medications have been given to them.
I) Patients who are transferred to elapegademase-lvlr from the animal source of ADA (pegademase bovine)-
The recommended initial dose of elapegademase-lvlr is 0.2 mg/kg given once a week if the patient’s weekly dose of pegademase bovine is not known or if it is at or less than 30 U/kg
If the patient’s weekly dose of pegademase bovine is more than 30 U/kg, the exact dose of elapegademase-lvlr can be calculated by the following conversion formula:
Elapegadeaminase-lvlr dose in mg/kg = Pegademase bovine in U/kg divided by 150
The dose of elapegademase-lvlr can be increased gradually by 0.033 mg/kg per week based on the level of ADA and the clinical response of the patient.
II) Patients who have not used pegademase bovine-
The initial dose of elapegademase-lvlr is 0.4 mg/kg per week prescribed based on the body weight which can be given in two doses as 0.2 mg/kg twice a week for a minimum of 12 to 24 weeks.
The dose can be readjusted and the treatment duration is decided based on the ADA enzyme levels and the clinical status of the patient.
Elapegadeaminase-lvlr comes as an injection and should be given by a trained healthcare provider intramuscularly or directly into the muscle.
The injection site can be changed periodically to avoid repeated injury.
Elapegadeaminase-lvlr injection should be inspected for the presence of any visible particles or cloudy solution before injecting and must be discarded if such things are present.
The injection must be removed from the refrigerator and kept outside for at least 30 minutes before injecting by allowing it to come to room temperature.
The injection must be given immediately once withdrawn from the vial and any unused portion left in the vial must be discarded.
• Patients with thrombocytopenia must be carefully observed as injection site bleeding can occur; do not use if the thrombocytopenia is severe.
• Adequate precautions must be followed in patients with low immunity status to protect against infections until the required immune function has been obtained.
• ADA activity must be monitored every two weeks in patients treated with elapegademase-lvlr for the first time and every four weeks in patients who have previously received pegademase bovine (animal source), during the first 8 to 12 weeks of treatment followed by every 3 to 6 months thereafter.
• Levels of erythrocyte dAXP must be monitored two months after initiating elapegademase-lvlr therapy and should be monitored at least twice a year.
• Every patient treated with elapegademase-lvlr should be continuously monitored for their immune status, and in those were not previously treated with pegademase bovine lymphocytes levels must be checked every 4 to 8 weeks for up to 1 year followed by every 3 to 6 months.
• In patients who were previously treated with pegademase bovine and transferred to elapegademase-lvlr, lymphocyte count must be monitored every 3 to 6 months.
The most common side effects of elapegademase-lvlr are
• Cough and infections of the airways
• Nausea and vomiting
• Injection site reactions such as rashes, itching, pain, and swelling
• Blood disorders (hemolytic anemia, neutropenia, abnormal levels of platelets)
• Lymphomas or cancer of the lymphatic system
Patients or the caregivers must be advised to take the elapegademase-lvlr therapy as suggested by the doctor and not stop or adjust the dose as it may affect the quality and the success of the treatment.
The doctor must be informed if the patient is taking or is planning to take any medications or herbal supplements before prescribing elapegademase-lvlr to avoid unwanted side effects caused by the drugs.
Store elapegadeaminase-lvlr vials in a refrigerator at 2°C to 8°C.
Protect the vials from light by keeping them in their original carton.
Do not shake or freeze the vials.
Keep away from the reach of children.
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