Version May 2024
Adenosine deaminase severe combined immune deficiency (ADA-SCID): Note: Optimal long-term dose and administration schedule should be individualized based on laboratory values (eg, adenosine deaminase [ADA] activity, erythrocyte [red blood cell] deoxyadenosine triphosphate [dATP] concentration or total ATP) and clinical response.
Initial: IM: 10 units/kg once, then 7 days later 15 units/kg once for the second dose, and then 7 days later 20 units/kg for the third dose; then begin maintenance dosing
Maintenance: IM: 20 units/kg/week administered once weekly; may increase dose by 5 units/kg/week if necessary. Adjust dose to maintain plasma ADA activity (trough) 15 to 35 µmol/hour/mL and a decline in erythrocyte (red blood cell) dATP to ≤0.005 to 0.015 µmol/mL or ≤1% of total erythrocyte adenine nucleotide (ATP + dATP) content; maximum single dose: 30 units/kg/dose
Adenosine deaminase severe combined immune deficiency (ADA-SCID): Note: Optimal long-term dose and administration schedule should be individualized based on laboratory values (eg, adenosine deaminase [ADA] activity, erythrocyte [red blood cell] deoxyadenosine triphosphate [dATP] concentration or total ATP) and clinical response. Infants, Children, and Adolescents:
Initial: IM: 10 units/kg once, then 7 days later 15 units/kg once for the second dose, and then 7 days later 20 units/kg for the third dose; then begin maintenance dosing
Maintenance: IM: 20 units/kg/week administered once weekly; may increase dose by 5 units/kg/week if necessary. Adjust dose to maintain plasma ADA activity (trough) 15 to 35 µmol/hour/mL and a decline in erythrocyte (red blood cell) dATP to ≤0.005 to 0.015 µmol/mL or ≤1% of total erythrocyte adenine nucleotide (ATP + dATP) content; maximum single dose: 30 units/kg/dose
There are no dosage adjustments provided in the manufacturer’s labeling.
There are no dosage adjustments provided in the manufacturer’s labeling.
The following adverse drug reactions and incidences are derived from product labeling unless otherwise specified.
1% to 10%: Immunologic: Antibody development (enhanced rate of clearance of plasma ADA activity; 8%)
<1%, postmarketing, and/or case reports: Autoimmune hemolytic anemia, erythema at injection site, headache, hemolytic anemia, immune thrombocytopenia, malignant lymphoma, pain at injection site, thrombocythemia, thrombocytopenia, urticaria
As preparatory or support therapy for bone marrow transplantation; severe thrombocytopenia
Concerns related to adverse effects:
• Antibody formation: Development of antibodies has been reported in patients resulting in more rapid clearance of pegademase bovine. In patients who experience a persistent decrease in preinjection levels of plasma ADA to <10 micromole/hour/mL and have had other causes ruled out (eg, improper storage of vials, improper handling of plasma sample), antibody formation should be considered and a specific assay for antibody ADA should be performed. Dosage adjustments may be required in patients developing antibodies.
Disease-related concerns:
• Immune status: Failure to maintain adequate levels of plasma ADA activity will increase patients risk for infection.
• Thrombocytopenia: Use with caution in patients with thrombocytopenia; should not be used in patients with severe thrombocytopenia.
Other warnings/precautions:
• Appropriate use: Not a substitute for bone marrow transplant; should be used in conjunction with continued close monitoring and appropriate diagnostic tests and therapy. Therapy is not a cure for SCID and must be continued.
Adagen is being discontinued due to the permanent shortage of the active ingredient. Existing inventory is anticipated to remain available until March 2019.
Yes
Solution (Adagen Intramuscular)
250 units/mL (per mL): $4,165.60
Disclaimer: A representative AWP (Average Wholesale Price) price or price range is provided as reference price only. A range is provided when more than one manufacturer's AWP price is available and uses the low and high price reported by the manufacturers to determine the range. The pricing data should be used for benchmarking purposes only, and as such should not be used alone to set or adjudicate any prices for reimbursement or purchasing functions or considered to be an exact price for a single product and/or manufacturer. Medi-Span expressly disclaims all warranties of any kind or nature, whether express or implied, and assumes no liability with respect to accuracy of price or price range data published in its solutions. In no event shall Medi-Span be liable for special, indirect, incidental, or consequential damages arising from use of price or price range data. Pricing data is updated monthly.
IM: Administer intramuscularly; do not administer IV or SubQ. Do not dilute or mix with other medications prior to administration.
Refrigerate at 2°C to 8°C (36°F to 46°F); do not freeze. Discard unused portions; do not save for further use. Do not use if previously frozen.
Enzyme replacement therapy for treatment of adenosine deaminase severe combined immunodeficiency disease (ADA-SCID) who are not suitable candidates for or who have failed bone marrow transplantation (FDA approved in pediatric patients from birth and up)
None known.
Note: Interacting drugs may not be individually listed below if they are part of a group interaction (eg, individual drugs within “CYP3A4 Inducers [Strong]” are NOT listed). For a complete list of drug interactions by individual drug name and detailed management recommendations, use the Lexicomp drug interactions program by clicking on the “Launch drug interactions program” link above.
Pegloticase: May diminish the therapeutic effect of PEGylated Drug Products. Risk C: Monitor therapy
Pegvaliase: PEGylated Drug Products may enhance the adverse/toxic effect of Pegvaliase. Specifically, the risk of anaphylaxis or hypersensitivity reactions may be increased. Risk C: Monitor therapy
Pentostatin: Pegademase Bovine may diminish the therapeutic effect of Pentostatin. Pentostatin may diminish the therapeutic effect of Pegademase Bovine. Risk C: Monitor therapy
Animal reproduction studies have not been conducted. Pegademase bovine should be given to a pregnant woman only if clearly needed.
Plasma adenosine deaminase (ADA) activity (trough): Baseline, then every 1 to 2 weeks for the first 8 to 12 weeks of therapy, then twice a month for the next 3 to 9 months, then monthly until after 18 to 24 months. After 2 years of therapy, monitor every 2 to 4 months. More frequent monitoring may be necessary if therapy interrupted or clearance increases.
Erythrocyte (red blood cell) deoxyadenosine triphosphate (dATP) concentrations (trough): Baseline, then after 2 months at maintenance treatment, then 2 to 4 times for first year and 2 to 3 times a year thereafter. More frequent monitoring may be necessary if therapy interrupted or clearance increases.
Immune function
Target concentrations:
Plasma adenosine deaminase (ADA) activity: Trough: 15 to 35 µmol/hour/mL
Erythrocyte (red blood cell) deoxyadenosine triphosphate (dATP): Trough: ≤0.005 to 0.015 µmol/mL
Adenosine deaminase is an enzyme that catalyzes the deamination of both adenosine and deoxyadenosine. Hereditary lack of adenosine deaminase activity results in severe combined immunodeficiency disease, a fatal disorder of infancy characterized by profound defects of both cellular and humoral immunity. It is estimated that 25% of patients with the autosomal recessive form of severe combined immunodeficiency lack adenosine deaminase. Pegademase bovine is a (modified) enzyme replacement for adenosine deaminase deficiency.
Half-life elimination: Pediatric patients (6 weeks to 12 years): Plasma adenosine deaminase (ADA) half-life (following administration): Range: 3 to >6 days
Time to peak: Pediatric patients (6 weeks to 12 years): Plasma ADA activity: 2 to 3 days
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