Usual Adult Dose for Chronic Granulomatous Disease

Body surface area (BSA) 0.5 m2 or less:
1.5 mcg/kg/dose subcutaneously 3 times a week

BSA greater than 0.5 m2:
50 mcg/m2 (1 million International Units/m2) subcutaneously 3 times a week

Comments:

• Prior to the beginning of treatment and at 3-month intervals during treatment the following laboratory tests are recommended for all patients: hematologic tests (including complete blood counts), differential and platelet counts, blood chemistries (including renal and liver function tests), and urinalysis.

Use: For reducing the frequency and severity of serious infections associated with Chronic Granulomatous Disease (CGD)

Usual Adult Dose for Osteopetrosis

Body surface area (BSA) 0.5 m2 or less:
1.5 mcg/kg/dose subcutaneously 3 times a week

BSA greater than 0.5 m2:
50 mcg/m2 (1 million International Units/m2) subcutaneously 3 times a week

Comments:

• Prior to the beginning of treatment and at 3-month intervals during treatment the following laboratory tests are recommended for all patients: hematologic tests (including complete blood counts), differential and platelet counts, blood chemistries (including renal and liver function tests), and urinalysis.

Use: For delaying time to disease progression in patients with severe malignant osteopetrosis (SMO)

Usual Pediatric Dose for Chronic Granulomatous Disease

One year and older:

• Body surface area (BSA) 0.5 m2 or less:

1.5 mcg/kg/dose subcutaneously 3 times a week

• BSA greater than 0.5 m2:

50 mcg/m2 (1 million International Units/m2) subcutaneously 3 times a week

Comments:

• Prior to the beginning of treatment and at 3-month intervals during treatment the following laboratory tests are recommended for all patients: Hematologic tests (including complete blood counts), differential and platelet counts, blood chemistries (including renal and liver function tests) and urinalysis. In patients less than 1 year, liver function tests should be measured monthly.

Use: For reducing the frequency and severity of serious infections associated with Chronic Granulomatous Disease (CGD) in children 1 year and older

Usual Pediatric Dose for Osteopetrosis

One month and older:

• Body surface area (BSA) 0.5 m2 or less:

1.5 mcg/kg/dose subcutaneously 3 times a week

• BSA greater than 0.5 m2:

50 mcg/m2 (1 million International Units/m2) subcutaneously 3 times a week

Comments:

• Prior to the beginning of treatment and at 3-month intervals during treatment the following laboratory tests are recommended for all patients: Hematologic tests (including complete blood counts), differential and platelet counts, blood chemistries (including renal and liver function tests) and urinalysis. In patients less than 1 year, liver function tests should be measured monthly.

Use: For delaying time to disease progression in patients with severe malignant osteopetrosis (SMO) in children 1 month and older

Renal Dose Adjustments

Dose adjustment(s) may be required; however, no specific guidelines have been suggested. Caution recommended.

Liver Dose Adjustments

Reduce dose or discontinue therapy to reverse severe elevations of aspartate transaminase (AST) and/or alanine transaminase (ALT). Monitor liver function monthly in patients less than 1 year old.

Dose Adjustments

Dose Modification:

• If severe reactions occur, the dose should be reduced by 50% or therapy should be interrupted until the adverse reaction abates.
• Safety and efficacy have not been established for this drug given in doses greater or less than the recommended dose of 50 mcg/m2. Higher doses (i.e., greater than 50 mcg/m2) are not recommended. The minimum effective dose has not been established.

Precautions

CONTRAINDICATIONS:

• Hypersensitivity to the active component or any of the ingredients
• Hypersensitivity to interferon gamma or E. coli derived products

Safety and efficacy have not been established in patients younger than 1 year for chronic granulomatous disease (CGD).
Safety and efficacy have not been established in patients younger than 1 month for severe malignant osteopetrosis (SMO).

Consult WARNINGS section for additional precautions.

Dialysis

Data not available

Other Comments

Administration advice:

• This drug can be administered by a physician, nurse, family member, or patient when counseled in the administration of subcutaneous injections.
• The optimum sites of subcutaneous injection are the right and left deltoid and anterior thigh.
• Parenteral drug products should be inspected visually for particulate matter and discoloration prior to administration, whenever solution and container permit.
• This drug is for a single use only.
• Discard any unused portion.
• Do not mix other drugs with this drug in the same syringe.
• Administer this drug using either sterilized glass or plastic disposable syringes.

Storage requirements:

• Store vials in the refrigerator at 2C to 8C (36F to 46F).
• Do not freeze.
• Avoid excessive or vigorous agitation. Do not shake.
• An unused vial can be stored at room temperature up to 12 hours prior to use.
• Discard vials if not used within a 12-hour period. Do not return to the refrigerator.

Monitoring:

• When administering this drug in combination with other potentially myelosuppressive agents, monitor neutrophil and platelet counts.