• Home
  • A-B index
  • Pharmacological Index
  • Drug Classes
  • Active Ingredients
  • Companies
  • News
  • Strensiq
    / Alexion


    Active Ingredient
    Asfotase Alfa 40 mg/ml, 100 mg/ml

    Status in Israel
    RX

    Presentation and Status in Health Basket

    Presentation Basket Yarpa Pharmasoft

    Vial

    1 X 12 mg / 0.3 ml

    partial basket chart 62117

    Vial

    1 X 18 mg / 0.45 ml

    partial basket chart 62118

    Vial

    1 X 28 mg / 0.7 ml

    partial basket chart 62119

    Vial

    1 X 40 mg/ml

    partial basket chart 62120

    Vial

    1 X 80 mg / 0.8ml

    partial basket chart 62121

    Related information


    Dosage

    Treatment should be initiated by a physician experienced in the management of patients with metabolic or bone disorders.
    Recommended dosage regimen of asfotase alfa is 2 mg/kg of body weight administered subcutaneously three times per week, or a dosage regimen of 1 mg/kg of body weight administered subcutaneously six times per week.
    Refer to the dosing chart at the attached doctor’s leaflet for more details.
    Renal and hepatic impairment: The safety and efficacy of Strensiq in patients with renal or hepatic impairment have not been evaluated and no specific dose regimen can be recommended for these patients.
    Adult patients: Efficacy and safety data in patients with hypophosphatasia >18 years old are limited.
    Elderly: There is no evidence for special considerations when Strensiq is administered to elderly patients.
    Method of administration: Strensiq is for subcutaneous use only. It is not intended for intravenous or intramuscular injection.
    The maximum volume of medicinal product per injection should not exceed 1 ml. If more than 1 ml is required, multiple injections may be administered at the same time.
    Strensiq should be administered using sterile disposable syringes and injection needles. The syringes should be of small enough volume that the prescribed dose can be withdrawn from the vial with reasonable accuracy.
    Injections sites should be rotated and carefully monitored for signs of potential reactions.
    Patients can self-inject only if they have properly been trained on administration procedures.


    Indications

    Strensiq is indicated for long-term enzyme replacement therapy in patients with paediatric-onset hypophosphatasia to treat the bone manifestations of the disease.


    Contra-Indications

    Severe or life-threatening hypersensitivity to the active substance or to any of the excipients if hypersensitivity is not controllable.


    Special Precautions

    Hypersensitivity: Hypersensitivity reactions including signs and symptoms consistent with anaphylaxis have been reported in patients treated with asfotase alfa. These symptoms included difficulty breathing, choking sensation, nausea, periorbital edema, and dizziness. The reactions have occurred within minutes after subcutaneous administration of Strensiq and can occur in patients on treatment for more than one year. Other hypersensitivity reactions included vomiting, fever, headache, flushing, irritability, chills, skin erythema, rash, pruritus, and oral hypaesthesia. If these reactions occur, immediate discontinuation of treatment is recommended and appropriate medical treatment should be initiated. The current medical standards for emergency treatment should be observed.
    Consider the risks and benefits of re-administering Strensiq to individual patients following a severe reaction, taking other factors into account that may contribute to the risk of a hypersensitivity reaction, such as concurrent infection and/ or use of antibiotics. If the decision is made to re-administer the product, the re-challenge should be made under medical supervision and consideration may be given to use of appropriate pre-medication. Patients should be monitored for recurrence of signs and symptoms of a severe hypersensitivity reaction.
    The need for supervision for subsequent administrations and need for emergency treatment for home care should be at the discretion of the treating physician.
    Severe or potentially life-threatening hypersensitivity is a contraindication to re-challenge, if hypersensitivity is not controllable.
    Injection reaction: Administration of asfotase alfa may result in local injection site reactions (including, but not limited to, erythema, rash, discoloration, pruritus, pain, papule, nodule, atrophy) defined as any related adverse event occurring during the injection or until the end of the injection day.
    Rotation of injection sites usually helps to effectively manage these reactions. These have been generally assessed as non-serious, mild to moderate in severity and self-limiting.
    Strensiq administration should be interrupted in any patient experiencing severe injection reactions and appropriate medical therapy administered.
    Craniosynostosis: In asfotase alfa clinical studies adverse events of craniosynostosis (associated with increased intracranial pressure), including worsening of pre-existing craniosynostosis have been reported in hypophosphatasia patients < 5 years of age. There are insufficient data to establish a causal relationship between exposure to Strensiq and progression of craniosynostosis. Craniosynostosis as a manifestation of hypophosphatasia is documented in published literature and occurred in 61.3% of patients between birth and 5 years of age in a natural history study of untreated infantile-onset hypophosphatasia patients. Craniosynostosis can lead to increased intracranial pressure. Periodic monitoring (including fundoscopy for signs of papilloedema) and prompt intervention for increased intracranial pressure is recommended in hypophosphatasia patients below 5 years of age.
    Ectopic calcification: In asfotase alfa clinical studies ophthalmic (conjunctival and corneal) calcification and nephrocalcinosis have been reported in patients with hypophosphatasia. There are insufficient data to establish a causal relationship between exposure to Strensiq and ectopic calcification. Ophthalmic
    (conjunctival and corneal) calcification and nephrocalcinosis as manifestations of hypophosphatasia are documented in published literature. Nephrocalcinosis occurred in 51.6% of patients between birth and 5 years of age in a natural history study of untreated infantile-onset hypophosphatasia patients.
    Periodic ophthalmology examination and renal ultrasounds are recommended in hypophosphatasia patients.
    Serum Parathyroid Hormone and Calcium: Serum parathyroid hormone concentration may increase in hypophosphatasia patients administered asfotase alfa, most notably during the first 12 weeks of treatment. It is recommended that serum parathyroid hormone and calcium be monitored in patients treated with asfotase alfa. Supplements of calcium and oral vitamin D may be required.
    Disproportionate weight gain: Patients may display disproportionate weight increase. Dietary supervision is recommended.
    Excipients: This medicinal product contains less than 1 mmol sodium (23 mg) per vial, i.e. the product is essentially ‘sodium-free’.


    Side Effects

    The most common adverse reactions observed were injection site reactions and injection-associated adverse reactions. Most of these reactions were non-serious, mild to moderate in intensity. Serious injection-associated reactions were reported in 2 patients with no discontinuation of asfotase alfa treatment: 1 patient with infantile-onset hypophosphatasia recorded fever and chills, and in 1 patient with juvenile-onset hypophosphatasia recorded hypoaesthesia oral, pain in extremity, chills, and headache.
    See prescribing information for full details.


    Drug interactions

    No interaction studies have been performed with asfotase alfa. Based on its structure and pharmacokinetics, asfotase alfa is unlikely to affect Cytochrome P-450 related metabolism.
    Asfotase alfa contains a catalytic domain of tissue non-specific alkaline phosphatase. Administration of asfotase alfa will interfere with routine measurement of serum alkaline phosphatase by hospital laboratories resulting in serum alkaline phosphatase activity measurements of several thousand units
    per litre. Asfotase alfa activity results must not be interpreted as the same measure as serum alkaline phosphatase activity owing to differences in enzyme characteristics.
    Alkaline Phosphatase (ALP) is used as the detection reagent in many routine laboratory assays. If asfotase alfa is present in clinical laboratory samples, aberrant values could be reported.
    The treating physician should inform the testing lab that the patient is treated with medication affecting the ALP levels. Alternative assays (i.e. not utilizing an ALP-conjugated reporter system) may be considered in patients treated with Strensiq.


    Pregnancy and Lactation

    Pregnancy: There are no data from the use of asfotase alfa in pregnant women.
    Following repeated subcutaneous administration to pregnant mice in the therapeutic dose range (>0.5 mg/kg), asfotase alfa levels were quantifiable in fetuses at all doses tested, suggesting cross-placental transport of asfotase alfa. Animal studies are insufficient with respect to reproductive toxicity. Asfotase alfa is not recommended during pregnancy and in women of childbearing potential not using contraception.
    Lactation: There is insufficient information on the excretion of asfotase alfa in human milk. A risk to the newborns/infants cannot be excluded.
    Breast-feeding should be discontinued during treatment with asfotase alfa.


    Overdose

    There is no experience  with overdose of asfotase alfa.


    Important notes

    Incompatibilities: In the absence of compatibility studies, this medicinal product must not be mixed with other medicinal products.
    Shelf life: 2 years. Chemical and physical in-use stability has been demonstrated for up to 1 hour at a temperature between 23°C to 27°C.
    Storage: Store in a refrigerator (2°C – 8°C). Do not freeze.


    Manufacturer
    Alexion Pharma GmbH, Switzerland
    CLOSE