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Kanuma is a brand name for Sebelipase Alfa. The medicine, its uses, side effects and dosage are the same regardless of brand.
Used for: KANUMA is indicated for long-term enzyme replacement therapy (ERT) in patients of all ages with lysosomal acid lipase (LAL) deficiency.
Verbatim from this product's EMA label. Tap a section to expand.
KANUMA treatment should be supervised by a healthcare professional experienced in the management of patients with LAL deficiency, other metabolic disorders, or chronic liver diseases. KANUMA should be administered by a trained healthcare professional who can manage medical emergencies.
Posology It is important to initiate treatment as early as possible after diagnosis of LAL deficiency. For instructions on the preventive measures and monitoring of hypersensitivity reactions, see section
8. Therefore, appropriate medical support must be readily available when sebelipase alfa is administered. If severe reactions occur, the sebelipase alfa infusion should be immediately stopped and appropriate medical treatment should be initiated.
The risks and benefits of re-administering sebelipase alfa following a severe reaction should be considered. Following the first sebelipase alfa infusion, including the first infusion after a dose escalation, patients should be observed for 1 hour in order to monitor for any signs or symptoms of anaphylaxis or a severe hypersensitivity reaction.
The management of hypersensitivity reactions may include temporarily interrupting the infusion, lowering the infusion rate, and/or treatment with antihistamines, antipyretics, and/or corticosteroids. For patients who have experienced allergic reactions during infusion, caution should be exercised upon re-administration.
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.
In cases of severe infusion reactions and in cases of lack or loss of effect, patients should be tested for the presence of antibodies. This medicinal product may contain traces of egg proteins. 3). Immunogenicity As with all therapeutic proteins, there is potential for immunogenicity.
In the sebelipase alfa clinical program, patients were routinely tested for anti-sebelipase alfa anti-drug antibodies (ADAs) to determine the immunogenicity potential of sebelipase alfa. Patients who tested positive for ADAs were also tested for inhibitory antibody activity.
8). Overall, no conclusion on the relationship between development of ADAs/NAbs and associated hypersensitivity reactions or suboptimal clinical response can be made. 5 In clinical studies, 3 patients homozygous for a deletion affecting both alleles of genes Lipase A, lysosomal acid [LIPA] and Cholesterol 25-Hydroxylase developed inhibitory antibody activity associated with a suboptimal clinical response.
4. 4). 3 Patients with Rapidly Progressive LAL Deficiency Presenting within the First 6 Months of Life The recommended starting dose in infants (< 6 months of age) presenting with rapidly progressive LAL deficiency is either 1 mg/kg or 3 mg/kg administered as an intravenous infusion once weekly, depending on the clinical status of the patient.
A higher starting dose of 3 mg/kg should be considered based on the severity of the disease and rapid disease progression. g. g. gastrointestinal symptoms): - a dose escalation to 3 mg/kg should be considered in case of suboptimal clinical response; - a further dose escalation up to 5 mg/kg should be considered in case of persistent suboptimal clinical response.
Further dose adjustments, as a reduction of the dose or an extension of the dose interval, can be made on an individual basis based on achievement and maintenance of therapeutic goals. 5 mg/kg once weekly. 5 mg/kg have not been studied.
Pediatric and Adult Patients with LAL Deficiency The recommended dose in children and adults who do not present with rapidly progressive LAL deficiency prior to 6 months of age is 1 mg/kg administered as an intravenous infusion once every other week.
, gastrointestinal symptoms). 2). 2). 1). Overweight patients The safety and efficacy of sebelipase alfa in overweight patients have not been thoroughly evaluated and therefore no alternative dose regimens can be recommended for these patients at this time.
Paediatric population Administration of sebelipase alfa to infants with confirmed multiple-organ failure should be at the discretion of the treating physician. Method of administration KANUMA is for intravenous (IV) use only. 4 The total volume of the infusion should be administered over approximately 2 hours.
A 1-hour infusion may be considered for those patients receiving the 1 mg/kg dose after patient tolerability is established. ) The infusion period may be extended in the event of dose escalation. 6). 6. 4). 4 Special warnings and precautions for use Traceability In order to improve the traceability of biological medicinal products, the name and the batch number of the administered product should be clearly recorded.
4).
Not medical advice. Always read the patient information leaflet and follow your prescriber or pharmacist.
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These patients underwent either immunomodulatory therapy alone or in combination with haematopoietic stem cell transplant (HSCT) or bone marrow transplant (BMT), resulting in improved clinical response to sebelipase alfa. 7% of the WHO recommended maximum daily intake of 2 g sodium for an adult.
6). This should be taken into consideration by patients on a controlled sodium diet. 5 Interaction with other medicinal products and other forms of interaction No interaction studies have been performed. Because it is a recombinant human protein, sebelipase alfa is an unlikely candidate for cytochrome P450 mediated or other drug-drug interactions.
6 Fertility, pregnancy and lactation Pregnancy There are no or limited data from the use of sebelipase alfa in pregnant women. 3). As a precautionary measure, it is preferable to avoid use of sebelipase alfa during pregnancy. Breast-feeding There are no data from studies in breast-feeding women.
It is not known whether sebelipase alfa is excreted in human milk. A decision must be made whether to discontinue breast-feeding or to discontinue/abstain from sebelipase alfa therapy taking into account the benefit of breast-feeding for the child and the benefit of therapy for the woman.
Fertility There are no clinical data on the effects of sebelipase alfa on fertility. 3). 7 Effects on ability to drive and use machines KANUMA may have a minor influence on the ability to drive and use machines. 8). 5 months (5 years).
2%) have received sebelipase alfa at a dosage regimen of 1 mg/kg once every other week, with a median duration of exposure of 33 months (6, 59 months). 5 months (1 day to 60 months). The most serious adverse reactions experienced by 4% of patients in clinical studies were signs and symptoms consistent with anaphylaxis.
Signs and symptoms included chest discomfort, conjunctival hyperaemia, dyspnoea, hyperaemia, eyelid oedema, rhinorrhoea, severe respiratory distress, tachycardia, tachypnoea, irritability, flushing, pruritus, urticaria, stridor, hypoxia, pallor and diarrhoea.
Tabulated list of adverse reactions The data in Table 1 describe adverse reactions reported in infants who received sebelipase alfa in clinical studies. The data in Table 2 describe adverse reactions reported in children and adults who received sebelipase alfa in clinical studies.
Adverse reactions are listed by system organ class (SOC) and frequency. Frequencies are defined according to the following convention: very common ( 1/10); common ( 1/100 to < 1/10), uncommon ( 1/1,000 to < 1/100), rare ( 1/10,000 to < 1/1,000), very rare (< 1/10,000) and not known (cannot be estimated from the available data).
Table 1:
Adverse reactions […]
8. Therefore, appropriate medical support must be readily available when sebelipase alfa is administered. If severe reactions occur, the sebelipase alfa infusion should be immediately stopped and appropriate medical treatment should be initiated.
The risks and benefits of re-administering sebelipase alfa following a severe reaction should be considered. Following the first sebelipase alfa infusion, including the first infusion after a dose escalation, patients should be observed for 1 hour in order to monitor for any signs or symptoms of anaphylaxis or a severe hypersensitivity reaction.
The management of hypersensitivity reactions may include temporarily interrupting the infusion, lowering the infusion rate, and/or treatment with antihistamines, antipyretics, and/or corticosteroids. For patients who have experienced allergic reactions during infusion, caution should be exercised upon re-administration.
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.
In cases of severe infusion reactions and in cases of lack or loss of effect, patients should be tested for the presence of antibodies. This medicinal product may contain traces of egg proteins. 3). […]