Elapegademase Dosage

Medically reviewed by Drugs.com. Last updated on Jan 1, 2024.

Applies to the following strengths: lvlr 1.6 mg/mL

Usual Adult Dose for Adenosine Deaminase Deficiency

Patients transitioning from pegademase bovine to this drug:

If the weekly pegademase bovine dose is unknown, or is 30 U/kg or lower, the minimum starting dose of this drug is 0.2 mg/kg IM once a week.
If the weekly pegademase bovine dose is above 30 U/kg, an equivalent weekly dose (mg/kg) of this drug should be calculated using the following conversion formula: Elapegademase dose (mg/kg) = pegademase bovine dose (U/kg) divided by 150.
Subsequent doses may be increased by increments of 0.033 mg/kg weekly if trough ADA activity is under 30 mmol/hr/L, trough deoxyadenosine nucleotides (dAXP) are above 0.02 mmol/L, and/or the immune reconstitution is inadequate based on assessment of the patient.
The total weekly dose may be divided into multiple IM administrations during a week.

Pegademase bovine naive patients:
0.2 mg/kg IM 2 times a week for a minimum of 12 to 24 weeks until immune
reconstitution is achieved, then the dose may be gradually adjusted down to maintain trough ADA activity over 30 mmol/hr/L, trough dAXP level under 0.02 mmol/L, and/or to maintain adequate immune reconstitution based on assessment of the patient.

Comments:

Doses should be based on ideal body weight.
The long-term dose and schedule of administration should be established by the physician for each patient individually and may be adjusted based on the laboratory values for trough ADA activity, trough dAXP level, and/or on assessment of the patient.

Use: For the treatment of adenosine deaminase severe combined immune
deficiency (ADA-SCID)

Usual Pediatric Dose for Adenosine Deaminase Deficiency

Patients transitioning from pegademase bovine to this drug:

If the weekly pegademase bovine dose is unknown, or is 30 U/kg or lower, the minimum starting dose of this drug is 0.2 mg/kg IM once a week.
If the weekly pegademase bovine dose is above 30 U/kg, an equivalent weekly dose (mg/kg) of this drug should be calculated using the following conversion formula: Elapegademase dose (mg/kg) = pegademase bovine dose (U/kg) divided by 150.
Subsequent doses may be increased by increments of 0.033 mg/kg weekly if trough ADA activity is under 30 mmol/hr/L, trough deoxyadenosine nucleotides (dAXP) are above 0.02 mmol/L, and/or the immune reconstitution is inadequate based on assessment of the patient.
The total weekly dose may be divided into multiple IM administrations during a week.

Doses should be based on ideal body weight.
The long-term dose and schedule of administration should be established by the physician for each patient individually and may be adjusted based on the laboratory values for trough ADA activity, trough dAXP level, and/or on assessment of the patient.

Use: For the treatment of adenosine deaminase severe combined immune
deficiency (ADA-SCID)

Renal Dose Adjustments

Data not available

Liver Dose Adjustments

Data not available

Precautions

CONTRAINDICATIONS:

None

Consult WARNINGS section for additional precautions.

Dialysis

Data not available

Other Comments

Administration advice:

For IM injection only.
Base choice of needle gauge/length/site of administration on patient age/anatomy.
Do not inject into or near an artery or nerve.
Alternate injection site periodically.
Do not use in patients with severe thrombocytopenia.

Storage requirements:

Store in refrigerator at 2C to 8C (36F to 46F) in the original carton to protect from light; do not freeze.

Reconstitution/preparation techniques:

Do not dilute or mix with any other drug prior to administration.
This drug should be a clear, colorless solution; discard if discolored, cloudy or contains particulate matter.
Do not use if container was frozen.
Do not shake.
Remove from refrigerator; let container stand at room temperature for 30 minutes before using.
Administer using polypropylene syringes. Draw solution from vial with

a 25-gauge needle or larger.

Change needle to size/gauge appropriate for the patient.
Administer immediately after preparation.
Discard remaining medication.

General:

Consult the manufacturer product information for further instructions on administration and monitoring.
This drug can cause immunogenicity; therefore, monitoring for changes in ADA levels during therapy is recommended.