What Nexviazyme is
Nexviazyme contains the active substance Avalglucosidase Alfa.

Avalglucosidase Alfa is hydrolytic lysosomal glycogen- specific enzyme that provide an external source of GAA enzymatic activity.

What Nexviazyme is used for
Nexviazyme is indicated for the treatment of patients 1 year of age and older with late-onset Pompe disease (lysosomal acid alpha-glucosidase [GAA] deficiency).

How Nexviazyme works
Pompe disease (also known as glycogen storage disease type II, acid maltase deficiency, and glycogenosis type II) is an inherited disorder of glycogen metabolism caused by a deficiency of the lysosomal enzyme acid α-glucosidase (GAA), which results in intralysosomal accumulation of glycogen in various tissues.
Avalglucosidase alfa provides an exogenous source of GAA. The M6P on avalglucosidase alfa mediates binding to M6P receptors on the cell surface with high affinity. After binding, it is internalized and transported into lysosomes where it undergoes proteolytic cleavage that results in increased GAA enzymatic activity. Avalglucosidase alfa then exerts enzymatic activity in cleaving glycogen

Contraindications:
Do not use Nexviazyme
• if you are allergic to Nexviazyme alfa or any of the other ingredients of this medicine (listed insection 6).
• if you think you may be allergic, or you are not sure, ask your doctor, pharmacist or nurse for advice before using Nexviazyme

Warnings and precautions
Talk to your doctor, pharmacist or nurse before using Nexviazyme:

Hypersensitivity Reactions Including Anaphylaxis
• Nexviazyme can cause serious side effects including allergic reaction (hypersensitivity), pre-treating with antihistamines, antipyretics and/or corticosteroids should be considered.
• If you noticed any hypersensitivity reactions, including respiratory distress, chest discomfort, flushing, cough, erythema, lip swelling, pruritus, swollen tongue, dysphagia, rash and urticaria, tell your doctor immediately and get medical help.

Infusion-Associated Reactions
Tell your doctor or nurse immediately if you have signs of infusion reactions during or after the infusion of Nexviazyme.
• Before starting a Nexviazyme infusion, you may be given medicines to reduce infusion reactions (see section 3)
• Infusion reactions can happen during the Nexviazyme infusion or after the infusion. The hospital staff will monitor you closely during treatment.

If you get an infusion reaction, your doctor or nurse may give you additional medicines to treat your
symptoms and prevent complications. They may also temporarily stop, slow down, or completely stop
the Nexviazyme infusion.

Risk of Acute Cardiorespiratory Failure in Susceptible Patient
Patients susceptible to fluid volume overload, or those with acute underlying respiratory illness or compromised cardiac or respiratory function for whom fluid restriction is indicated may be at risk of serious exacerbation of their cardiac or respiratory status during the NEXVIAZYME infusion. More frequent monitoring of vitals should be performed during NEXVIAZYME infusion in these patients. Some patients may require prolonged observation times.

Other medicines and Nexviazyme
Tell your doctor or pharmacist if you are using, have recently used, or might use any other medicines.
Pregnancy and breast-feeding
Talk to your doctor before using Nexviazyme if you are pregnant or breastfeeding, think you may be pregnant, or are planning to have a baby. There is no experience with the use of Nexviazyme in pregnant women. Do not take Nexviazyme during pregnancy unless your doctor specifically recommends it. You and your doctor should decide if you should use Nexviazyme if you are breastfeeding.

Driving and using machines
Cautions on driving and using machinery
Take care when driving or using any tools or machines shortly after infusion of Nexviazyme because you may experience dizziness, low blood pressure, and tiredness.

How Nexviazyme is given
Treatment should be initiated by healthcare professionals experienced in the diagnosis and treatment of conditions for which Nexviazyme is indicated.

How much Nexviazyme you will receive
The amount of Nexviazyme given is based on your weight. For patients weighing:
o ≥30 kg, the recommended dosage is 20 mg/kg (of actual body weight) every two weeks.
o <30 kg, the recommended dosage is 40 mg/kg (of actual body weight) every two weeks.

Injecting Nexviazyme
NEXVIAZYME is administered as intravenous infusion. For patients weighing:
o ≥30 kg, the recommended dosage is 20 mg/kg (of actual body weight) every two weeks.
o <30 kg, the recommended dosage is 40 mg/kg (of actual body weight) every two weeks.
The initial recommended infusion rate is 1 mg/kg/hour. Gradually increase the infusion rate every 30 minutes if there are no signs of infusion-associated reactions (IARs)
NEXVIAZYME must be reconstituted and diluted prior to use.

If you given more Nexviazyme than you should
Faster infusion of Nexviazyme may result in occurrence of IARs. If you experience a reaction similar to this, tell your doctor or nurse immediately.

If you forget to use Nexviazyme
If you have missed an infusion, please contact your doctor. If you have any further questions on the use of this medicine, ask your doctor, pharmacist, or nurse.

If you stop using Nexviazyme
If you stop using Nexviazyme Speak to your doctor if you wish to stop Nexviazyme treatment. The symptoms of your disease may worsen if you stop treatment

If you have any further questions on the use of this medicine, ask your doctor, pharmacist or nurse.

Like all medicines, this medicine can cause side effects, although not everybody gets them.
Nexviazyme can cause serious side affects including respiratory distress, chills and pyrexia.

Other side effects:
Common
• Headache
• Abdominal side effects (Diarrhea, vomitting, abdominal pain, nausea
• Fatigue
• Arthralgia
• Myalgia
• Dizziness
• Rash
• Pyrexia
• Pruritus
• Chills
• Cough
• Urticaria
• Dyspnea
• High blood pressure
• Low blood pressure
• Erythema
If any of the above apply to you, or you are not sure, talk to your doctor, pharmacist or nurse
immediately.

To report any side effect(s):
• Saudi Arabia:

Keep this medicine out of the sight and reach of children.
Do not use this medicine after the expiry date which is stated on the label and carton after EXP. The expiry date refers to the last day of that month.
Refrigerate vials of NEXVIAZYME at 36°F to 46°F (2°C to 8°C ).
If immediate use is not possible, the reconstituted solution can be stored up to 24 hours in a refrigerator, 36°F to 46°F (2°C to 8°C). Do not freeze.