NEXVIAZYME POWDER FOR CONCENTRATE FOR SOLUTION FOR INFUSION 100MG/VIAL [SIN16989P]

Product Info

NEXVIAZYME POWDER FOR CONCENTRATE FOR SOLUTION FOR INFUSION 100MG/VIAL

[SIN16989P]

4.1 Therapeutic indications

Nexviazyme (avalglucosidase alfa) is indicated for long-term enzyme replacement therapy for the treatment of patients with Pompe disease (acid α-glucosidase deficiency).

4.2 Posology and method of administration

Nexviazyme treatment should be supervised by a physician experienced in the management of patients with Pompe disease or other inherited metabolic or neuromuscular diseases.

Posology

Patients may be pre-treated with antihistamines, antipyretics, and/or corticosteroids to prevent or reduce allergic reactions.

The recommended dose of avalglucosidase alfa is 20 mg/kg of body weight administered once every 2 weeks.

Dose modification for IOPD patients
For IOPD patients who experience lack of improvement or insufficient response in cardiac, respiratory, and/or motor function while receiving 20 mg/kg, a dose increase to 40 mg/kg every other week should be considered in the absence of safety concerns (e.g., severe hypersensitivity, anaphylactic reactions, or risk of fluid overload).

In patients who do not tolerate avalglucosidase alfa at 40 mg/kg every other week (e.g., severe hypersensitivity, anaphylactic reactions, or risk of fluid overload), consider decreasing the dose to 20 mg/kg every other week. (see section 4.4 – please refer to the Product Insert/Patient Information Leaflet published on HSA for the full drug information).

Special populations

Elderly patients
No dose adjustment is required in patients >65 years.

Hepatic impairment
The safety and efficacy of avalglucosidase alfa in patients with hepatic impairment have not been evaluated and no specific dose regimen can be recommended for these patients.

Renal impairment
No dose adjustment is required in patients with mild renal impairment. The safety and efficacy of avalglucosidase alfa in patients with moderate or severe renal impairment have not been evaluated and no specific dose regimen can be recommended for these patients. (see section 5.2 – please refer to the Product Insert/Patient Information Leaflet published on HSA for the full drug information).

Paediatric population (patients 6 months of age and younger)
The safety and efficacy of avalglucosidase alfa in children 6 months of age and younger have not yet been established. There are no data available in patients 6 months of age and younger.

Method of administration

Nexviazyme vials are for single use only and the medicinal product should be administered as an intravenous infusion.

The infusion should be administered incrementally as determined by patient response and comfort. It is recommended that the infusion begins at an initial rate of 1 mg/kg/hour and is gradually increased every 30 minutes if there are no signs of infusion-associated reactions (IARs), in accordance with Table 1. Vital signs should be obtained at each step, before increasing the infusion rate.

In the event of anaphylaxis or severe hypersensitivity reaction or severe IARs, administration of Nexviazyme should be immediately discontinued and appropriate medical treatment should be initiated. In the event of mild to moderate hypersensitivity reactions or IARs, the infusion rate may be slowed or temporarily stopped and/or appropriate medical treatment initiated (see section 4.4 – please refer to the Product Insert/Patient Information Leaflet published on HSA for the full drug information).

Symptoms may persist despite temporarily stopping the infusion; therefore, the treating physician should wait at least 30 minutes for symptoms of the reactions to resolve before deciding to stop the infusion for the remainder of the day. If symptoms subside, infusion rate should be resumed for 30 minutes at half the rate, or less, of the rate at which the reactions occurred, followed by an increase in infusion rate by 50% for 15 to 30 minutes. If symptoms do not recur, the infusion rate should be increased to the rate at which the reactions occurred and consider continuing to increase the rate in a stepwise manner until the maximum rate is achieved.

Home infusion
Infusion of Nexviazyme at home may be considered for patients who are tolerating their infusions well and have no history of moderate or severe IARs for a few months. The decision to have a patient move to home infusion should be made after evaluation and upon recommendation by the treating physician. A patient’s underlying co-morbidities and ability to adhere to the home infusion requirements need to be taken into account when evaluating the patient for eligibility to receive home infusion. The following criteria should be considered:

• The patient must have no ongoing concurrent condition that, in the opinion of the physician, may affect patient’s ability to tolerate the infusion.

• The patient is considered medically stable. A comprehensive evaluation must be completed before the initiation of home infusion.

• The patient must have received Nexviazyme infusions supervised by a physician with expertise in management of Pompe patients for a few months that could be in a hospital or in another appropriate setting of outpatient care. Documentation of a pattern of well-tolerated infusions with no IARs, or mild IARs that have been controlled with premedication, is a prerequisite for the initiation of home infusion.

• The patient must be willing and able to comply with home infusion procedures.

• Home infusion infrastructure, resources, and procedures, including training, must be established and available to the healthcare professional. The healthcare professional should be available at all times during the home infusion and a specified time after infusion, depending on patient’s tolerance prior to starting home infusion.

If the patient experiences adverse reactions during the home infusion, the infusion process should be stopped immediately, and appropriate medical treatment should be initiated (see section 4.4 – please refer to the Product Insert/Patient Information Leaflet published on HSA for the full drug information). Subsequent infusions may need to occur in a hospital or in an appropriate setting of outpatient care until no such adverse reaction is present. Dose and infusion rate must not be changed without consulting the responsible physician.

For instructions on reconstitution and dilution of medicinal product before administration, see section 6.6 – please refer to the Product Insert/Patient Information Leaflet published on HSA for the full drug information.

4.3 Contraindications

Life-threatening hypersensitivity to the active substance or to any of the excipients listed in section 6.1 when re-challenge was unsuccessful – please refer to the Product Insert/Patient Information Leaflet published on HSA for the full drug information. (see sections 4.4 and 4.8 – please refer to the Product Insert/Patient Information Leaflet published on HSA for the full drug information)