Dosage & Administration
Administration of KANUMA should be supervised by a healthcare provider knowledgeable in the management of hypersensitivity reactions including anaphylaxis. (
See Full Prescribing Information for complete Dosage and Administration Information.
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Kanuma Prescribing Information
In clinical trials, 21 of 106 (20%) KANUMA-treated patients, including 9 of 14 (64%) infants and 12 of 92 (13%) pediatric patients who were 4 years and older and adults, experienced signs and symptoms either consistent with or that may be related to a hypersensitivity reaction. Signs and symptoms of hypersensitivity reactions, occurring in two or more patients, included abdominal pain, agitation, fever, chills, diarrhea, eczema, edema, hypertension, irritability, laryngeal edema, nausea, pallor, pruritus, rash, and vomiting. The majority of reactions occurred during or within 4 hours of the completion of the infusion. Patients were not routinely pre-medicated prior to infusion of KANUMA in these clinical trials.
Consider the risks and benefits of re-administering KANUMA following a severe reaction. Monitor patients, with appropriate resuscitation measures available, if the decision is made to re-administer the product.
WARNING: HYPERSENSITIVITY REACTIONS INCLUDING ANAPHYLAXIS Patients treated with enzyme replacement therapies have experienced life-threatening hypersensitivity reactions, including anaphylaxis. Anaphylaxis has occurred during the early course of enzyme replacement therapy and after extended duration of therapy. Initiate KANUMA in a healthcare setting with appropriate medical monitoring and support measures, including access to cardiopulmonary resuscitation equipment. If a severe hypersensitivity reaction (e.g., anaphylaxis) occurs, discontinue KANUMA and immediately initiate appropriate medical treatment, including use of epinephrine. Inform patients of the symptoms of life-threatening hypersensitivity reactions, including anaphylaxis and to seek immediate medical care should symptoms occur [see Warnings and Precautions (5.1)]. WARNING: HYPERSENSITIVITY REACTIONS INCLUDING ANAPHYLAXIS See full prescribing information for complete boxed warning.
| 7/2024 |
Dosage and Administration (Administration of KANUMA should be supervised by a healthcare provider knowledgeable in the management of hypersensitivity reactions including anaphylaxis [see Warnings and Precautions (5.1)]. Initiate KANUMA in a healthcare setting with appropriate medical monitoring and support measures, including access to cardiopulmonary resuscitation equipment [see Warnings and Precautions (5.1)]. | 7/2024 |
Warnings and Precautions (Life-threatening hypersensitivity reactions, including anaphylaxis, have been reported in patients treated with enzyme replacement therapies, including KANUMA. These reactions in KANUMA-treated patients were based on application of Sampson criteria to identify signs/symptoms consistent with anaphylaxis. In clinical trials, 3 (infants) of 106 (3%) patients treated with KANUMA experienced signs and symptoms consistent with anaphylaxis. These patients experienced reactions during infusion with signs and symptoms including chest discomfort, conjunctival injection, dyspnea, generalized and itchy rash, hyperemia, swelling of eyelids, rhinorrhea, severe respiratory distress, tachycardia, tachypnea, and urticaria. In clinical trials, 21 of 106 (20%) KANUMA-treated patients, including 9 of 14 (64%) infants and 12 of 92 (13%) pediatric patients who were 4 years and older and adults, experienced signs and symptoms either consistent with or that may be related to a hypersensitivity reaction. Signs and symptoms of hypersensitivity reactions, occurring in two or more patients, included abdominal pain, agitation, fever, chills, diarrhea, eczema, edema, hypertension, irritability, laryngeal edema, nausea, pallor, pruritus, rash, and vomiting. The majority of reactions occurred during or within 4 hours of the completion of the infusion. Patients were not routinely pre-medicated prior to infusion of KANUMA in these clinical trials. Anaphylaxis has occurred during the early course of enzyme replacement therapy and after extended duration of therapy. Administration of KANUMA should be supervised by a healthcare provider knowledgeable in the management of hypersensitivity reactions including anaphylaxis. Initiate KANUMA in a healthcare setting with appropriate medical monitoring and support measures, including access to cardiopulmonary resuscitation equipment. Observe patients closely during and after the infusion. The management of hypersensitivity reactions should be based on the severity of the reaction and may include temporarily interrupting the infusion, lowering the infusion rate, and/or treatment with antihistamines, antipyretics, and/or corticosteroids. If a severe hypersensitivity reaction (e.g., anaphylaxis) occurs, discontinue KANUMA and immediately initiate appropriate medical treatment, including use of epinephrine. If interrupted, the infusion may be resumed at a slower rate with increases as tolerated. Pre-treatment with antipyretics and/or antihistamines may prevent subsequent reactions in those cases where symptomatic treatment was required. Inform patients of the symptoms of life-threatening hypersensitivity reactions, including anaphylaxis and to seek immediate medical care should symptoms occur. Consider the risks and benefits of re-administering KANUMA following a severe reaction. Monitor patients, with appropriate resuscitation measures available, if the decision is made to re-administer the product. | 7/2024 |
KANUMA® is indicated for the treatment of patients with a diagnosis of Lysosomal Acid Lipase (LAL) deficiency.
Administration of KANUMA should be supervised by a healthcare provider knowledgeable in the management of hypersensitivity reactions including anaphylaxis. (
- The recommended starting dosage is 1 mg/kg as an intravenous infusion once weekly. ()
2.2 Recommended DosageInfants with Rapidly Progressive LAL Deficiency Presenting within the First 6 Months of Life:
- The recommended starting dosage is 1 mg/kg administered as an intravenous infusion once weekly.
- For patients with a suboptimal clinical response, increase the dosage to 3 mg/kg once weekly.
- For patients with continued suboptimal clinical response on the 3 mg/kg once weekly dosage, further increase the dosage to 5 mg/kg once weekly.
- A suboptimal clinical response is defined as any of the following: poor growth, deteriorating biochemical markers, or persistent or worsening organomegaly.
Pediatric and Adult Patients with LAL Deficiency:- The recommended dosage is 1 mg/kg administered as an intravenous infusion once every other week.
- For patients with a suboptimal clinical response, increase the dosage to 3 mg/kg once every other week.
- A suboptimal clinical response is defined as any of the following: poor growth, deteriorating biochemical markers [e.g., alanine aminotransferase (ALT), aspartate aminotransferase (AST)], and/or parameters of lipid metabolism [e.g., low-density lipoprotein cholesterol (LDL-c), triglycerides (TG)].
- For patients with a suboptimal clinical response, increase the dosage to 3 mg/kg once weekly. ()
2.2 Recommended DosageInfants with Rapidly Progressive LAL Deficiency Presenting within the First 6 Months of Life:
- The recommended starting dosage is 1 mg/kg administered as an intravenous infusion once weekly.
- For patients with a suboptimal clinical response, increase the dosage to 3 mg/kg once weekly.
- For patients with continued suboptimal clinical response on the 3 mg/kg once weekly dosage, further increase the dosage to 5 mg/kg once weekly.
- A suboptimal clinical response is defined as any of the following: poor growth, deteriorating biochemical markers, or persistent or worsening organomegaly.
Pediatric and Adult Patients with LAL Deficiency:- The recommended dosage is 1 mg/kg administered as an intravenous infusion once every other week.
- For patients with a suboptimal clinical response, increase the dosage to 3 mg/kg once every other week.
- A suboptimal clinical response is defined as any of the following: poor growth, deteriorating biochemical markers [e.g., alanine aminotransferase (ALT), aspartate aminotransferase (AST)], and/or parameters of lipid metabolism [e.g., low-density lipoprotein cholesterol (LDL-c), triglycerides (TG)].
- For patients with continued suboptimal clinical response, further increase the dosage to 5 mg/kg once weekly. ()
2.2 Recommended DosageInfants with Rapidly Progressive LAL Deficiency Presenting within the First 6 Months of Life:
- The recommended starting dosage is 1 mg/kg administered as an intravenous infusion once weekly.
- For patients with a suboptimal clinical response, increase the dosage to 3 mg/kg once weekly.
- For patients with continued suboptimal clinical response on the 3 mg/kg once weekly dosage, further increase the dosage to 5 mg/kg once weekly.
- A suboptimal clinical response is defined as any of the following: poor growth, deteriorating biochemical markers, or persistent or worsening organomegaly.
Pediatric and Adult Patients with LAL Deficiency:- The recommended dosage is 1 mg/kg administered as an intravenous infusion once every other week.
- For patients with a suboptimal clinical response, increase the dosage to 3 mg/kg once every other week.
- A suboptimal clinical response is defined as any of the following: poor growth, deteriorating biochemical markers [e.g., alanine aminotransferase (ALT), aspartate aminotransferase (AST)], and/or parameters of lipid metabolism [e.g., low-density lipoprotein cholesterol (LDL-c), triglycerides (TG)].
- The recommended dosage is 1 mg/kg as an intravenous infusion once every other week. ()
2.2 Recommended DosageInfants with Rapidly Progressive LAL Deficiency Presenting within the First 6 Months of Life:
- The recommended starting dosage is 1 mg/kg administered as an intravenous infusion once weekly.
- For patients with a suboptimal clinical response, increase the dosage to 3 mg/kg once weekly.
- For patients with continued suboptimal clinical response on the 3 mg/kg once weekly dosage, further increase the dosage to 5 mg/kg once weekly.
- A suboptimal clinical response is defined as any of the following: poor growth, deteriorating biochemical markers, or persistent or worsening organomegaly.
Pediatric and Adult Patients with LAL Deficiency:- The recommended dosage is 1 mg/kg administered as an intravenous infusion once every other week.
- For patients with a suboptimal clinical response, increase the dosage to 3 mg/kg once every other week.
- A suboptimal clinical response is defined as any of the following: poor growth, deteriorating biochemical markers [e.g., alanine aminotransferase (ALT), aspartate aminotransferase (AST)], and/or parameters of lipid metabolism [e.g., low-density lipoprotein cholesterol (LDL-c), triglycerides (TG)].
- For patients with a suboptimal clinical response, increase the dosage to 3 mg/kg once every other week. ()
2.2 Recommended DosageInfants with Rapidly Progressive LAL Deficiency Presenting within the First 6 Months of Life:
- The recommended starting dosage is 1 mg/kg administered as an intravenous infusion once weekly.
- For patients with a suboptimal clinical response, increase the dosage to 3 mg/kg once weekly.
- For patients with continued suboptimal clinical response on the 3 mg/kg once weekly dosage, further increase the dosage to 5 mg/kg once weekly.
- A suboptimal clinical response is defined as any of the following: poor growth, deteriorating biochemical markers, or persistent or worsening organomegaly.
Pediatric and Adult Patients with LAL Deficiency:- The recommended dosage is 1 mg/kg administered as an intravenous infusion once every other week.
- For patients with a suboptimal clinical response, increase the dosage to 3 mg/kg once every other week.
- A suboptimal clinical response is defined as any of the following: poor growth, deteriorating biochemical markers [e.g., alanine aminotransferase (ALT), aspartate aminotransferase (AST)], and/or parameters of lipid metabolism [e.g., low-density lipoprotein cholesterol (LDL-c), triglycerides (TG)].
See Full Prescribing Information for complete Dosage and Administration Information.
- Infuse over at least 2 hours. ()
2.4 Administration InstructionsAdminister the diluted solution as an intravenous infusion using a low-protein binding infusion set with an in-line, low-protein binding 0.2 micron filter.
Infuse over at least 2 hours. Consider further prolonging the infusion time for patients receiving dosages greater than 1 mg/kg or those who have experienced hypersensitivity reactions
[see Warnings and Precautions (5.1)]. A 1-hour infusion may be considered for those patients receiving the 1 mg/kg dose who tolerate the infusion. - Consider further prolonging the infusion time for patients receiving dosages greater than 1 mg/kg or for those who have experienced a hypersensitivity reaction. ()
2.4 Administration InstructionsAdminister the diluted solution as an intravenous infusion using a low-protein binding infusion set with an in-line, low-protein binding 0.2 micron filter.
Infuse over at least 2 hours. Consider further prolonging the infusion time for patients receiving dosages greater than 1 mg/kg or those who have experienced hypersensitivity reactions
[see Warnings and Precautions (5.1)]. A 1-hour infusion may be considered for those patients receiving the 1 mg/kg dose who tolerate the infusion. - Consider a 1-hour infusion for the 1 mg/kg dose in patients who tolerate the infusion. ()
2.4 Administration InstructionsAdminister the diluted solution as an intravenous infusion using a low-protein binding infusion set with an in-line, low-protein binding 0.2 micron filter.
Infuse over at least 2 hours. Consider further prolonging the infusion time for patients receiving dosages greater than 1 mg/kg or those who have experienced hypersensitivity reactions
[see Warnings and Precautions (5.1)]. A 1-hour infusion may be considered for those patients receiving the 1 mg/kg dose who tolerate the infusion.
Injection: 20 mg/10 mL (2 mg/mL) clear to slightly opalescent, colorless to slightly colored solution in single-dose vials.
Available data with KANUMA use in pregnant women are insufficient to identify a drug-associated risk of major birth defects, miscarriage or other adverse maternal or fetal outcomes. Animal reproductive studies conducted with sebelipase alfa showed no evidence of embryolethality, fetotoxicity, teratogenicity, or abnormal early embryonic development at dosages up to 164 and 526 times the human dosage of 1 mg/kg every other week (based on AUC) in rats and rabbits, respectively.
The background risk of major birth defects and miscarriage for the indicated population is unknown. In the U.S. general population, the estimated background risk of major birth defects and miscarriage in clinically recognized pregnancies is 2 to 4% and 15 to 20%, respectively.