In a clinical study of 100 LOPD patients who had not been on treatment before, and who were given either NEXVIAZYME or alglucosidase alfa, NEXVIAZYME helped improve their breathing and walking distance as compared to when they began the study.

After 49 weeks on NEXVIAZYME, people were able to improve their breathing capacity by an average of 2.9 percentage points in a breathing test.* Those who took alglucosidase alfa improved their breathing by an average of 0.5 percentage points.* This resulting in a measurable improvement of 2.4 percentage points for people treated with NEXVIAZYME compared with people taking alglucosidase alfa, although it was not statistically superior.

People on NEXVIAZYME were also able to improve their walking distance by an average of 106 feet during a 6MWT.* The people taking alglucosidase alfa improved their walking distance by and average of 7.2 feet.* People taking NEXVIAZYME walked an average of 98 feet farther than those who were taking alglucosidase alfa. This measurement was not tested to determine statistical superiority of NEXVIAZYME to alglucosidase alfa. ^*Compared to when they began treatment.

What is the most important information about side effects that I should know about NEXVIAZYME (avalglucosidase alfa-ngpt)?

The information shown is highlighted from the Important Safety Information. Please scroll down to see more details.

NEXVIAZYME has caused serious side effects, including:

• Hypersensitivity Reactions Including Anaphylaxis
• Infusion-Associated
  Reactions (IARs)
• Risk of Acute Cardiorespiratory Failure in Susceptible Patients

What are the possible side effects of NEXVIAZYME? Over the 49-week study, people taking NEXVIAZYME had fewer serious side effects than those taking alglucosidase alfa. No one had to stop taking NEXVIAZYME because of side effects, while 4 people had to stop taking alglucosidase alfa.

The most common side effects reported by people receiving NEXVIAZYME were headache, fatigue, diarrhea, nausea, joint pain, dizziness, muscle pain, itching, vomiting, shortness of breath, rash, “pins-and-needles” sensation, and hives.

These are the most common side effects, but there are other possible side effects. You should always tell your doctor about any changes in the way you feel, even if it’s not one of the listed side effects.

You can also report side effects at 1-800-FDA-1088 or www.fda.gov/medwatch.

When you have LOPD, the body does not have enough of the enzyme that breaks down glycogen in your muscle cells, so the glycogen builds up and causes muscle damage.

NEXVIAZYME has something called “high binding affinity” for a receptor in your muscle cell called M6PR. That receptor is like a lock on a door. A molecule called M6P is the key to open that lock. The NEXVIAZYME molecule has “keys” on its surface that fit in the “lock.” In fact, NEXVIAZYME has 15x the number of keys that alglucosidase alfa has. In a clinical study, NEXVIAZYME was not shown to be statistically superior to alglucosidase alfa.

NEXVIAZYME is given every 2 weeks by intravenous (IV) infusion. The recommended dosage of NEXVIAZYME is either 20 mg or 40 mg for each kilogram of body weight—your healthcare provider will calculate the appropriate dosage for you. The infusion usually takes approximately 4-5 hours for those receiving 20 mg/kg and between approximately 5-7 hours for those receiving 40 mg/kg. There could be additional time if you need any pretreatment. Also, infusion times may vary based on your response to therapy and comfort. Your healthcare provider will give you more details about what to expect during and after your infusion as well as how to prepare.

You’ll most likely want to bring a book, work, and/or electronic devices to make the most of your time. If you’re switching to NEXVIAZYME from alglucosidase alfa, your treatment schedule may stay the same.