Your first 90 days
Your first 90 days on Nexviazyme for late-onset Pompe disease
What happens before the first fill, the days that matter, the label's rules and who to call, from the label, the drugmaker's own program and one insurer's written rules.
Nexviazyme is a long-term schedule of IV infusions every 2 weeks, with a dose your care team works out from your weight. The first 90 days hold 7 infusions. This page lays out that stretch from the FDA label, Sanofi's CareConnectPSS program and one insurer's written rules.
What has to happen before the first bottle ships
Nexviazyme has no REMS, so the first infusion waits on a confirmed diagnosis, your weight, an insurance approval and an infusion site ready to treat a severe allergic reaction. These are the 6 things in the way, in order.
A confirmed diagnosis
The Cigna policy shown below asks for a lab test showing low acid alpha-glucosidase (GAA) enzyme activity, or a genetic test showing changes in both copies of the GAA gene.Cigna policy
Late-onset Pompe disease
The label covers late-onset Pompe disease in people 1 year and older.
- It says Nexviazyme is not approved for infantile-onset Pompe disease.FDA label
Your current weight
The dose is counted per kilogram of your actual weight.
- Weight also sets the size of the infusion bag, from 100mL for a small child to 700mL for a large adult.FDA label
An infusion site ready for reactions
The label asks that medical monitoring and equipment to restart breathing and the heart be ready during every infusion, because severe allergic reactions can happen.FDA label
Signing up with CareConnectPSS
Your doctor can send you a link to a digital consent form, or you can call a Case Manager.
- Sanofi says the program is free, voluntary and confidential.CareConnect for HCPs
The insurance check, or QuickStart
CareConnectPSS can help verify insurance.
- If you have commercial insurance, your doctor can apply for QuickStart, which may give Nexviazyme at no charge while the plan reviews coverage.Starting Nexviazyme (HCP)
The days that matter
Counted from the first infusion, on the every 2 weeks schedule the label sets for everyone who takes Nexviazyme. Type your first-dose date and the tiles become real dates you can add to your calendar.
The tiles below count days from a first dose. Enter the date of yours, or the date you expect, and each tile becomes a real date on your calendar.
The date stays on your device. It is not sent to Trial Friend or anyone else. The calendar file works with Apple, Google and Outlook calendars.Enrollment and the insurance check
You enroll in CareConnectPSS, and a Case Manager helps check your insurance.
- No source sets how long this takes, because each insurer works on its own timeline.
Approval, then booking the first infusion
Once the plan approves, or QuickStart is in place, the first infusion can be booked.
- Sanofi says your doctor's office must send the QuickStart application.
First infusion
The infusion starts at 1mg/kg per hour and steps up every 30 minutes if there are no reactions.
- It takes about 4 to 5 hours at 20mg/kg and about 7 hours at 40mg/kg.
Infusion 2
For children under 30kg (about 66 lb), the label lets later infusions use a faster 5-step schedule that takes about 5 hours.
- Your care team decides whether to use it.
Infusion 3
If an infusion is missed, the label says to restart as soon as possible and keep 2 weeks between infusions after that.
- Call your infusion site to rebook.
Infusion 4
Sanofi's Product Services team can send infusion and refill reminders so each shipment arrives on time.
- QuickStart usually covers up to 4 infusions, sometimes more, until insurance takes over.
Infusion 5
Most people form antibodies to Nexviazyme, usually around 8 weeks in.
- People with high antibody levels had more infusion reactions in studies, so tell your team about any new symptoms.
Infusion 6
The label invites you to join the Pompe Registry, which follows how the disease and treatment go over time.
- Joining is voluntary.
Infusion 7
This is the seventh infusion counted from day 1.
- In the main trial, breathing and walking were measured at week 49, well past this point.
The label, revision by revision
The FDA lists which sections changed in each revision of a label. These are the revisions the current Nexviazyme label records, dated by the FDA, with a plain note on what each one meant for a patient. Older references and handouts can lag these by months.
Current label dated June 5, 2025, read from the FDA's label database today. FDA label
- June 2025
DailyMed posted version 4 of the label. Its recent changes list still dates the last major changes to September 2023.FDA label
- September 2023
The FDA added clearer steps for slowing or pausing an infusion after a reaction, a missed-dose rule and longer trial results. Vials are now rounded up to the next whole vial.FDA letter
- August 2021
The FDA approved Nexviazyme for late-onset Pompe disease in people 1 year and older, with its boxed warning from the start.FDA letter
How the label says to take it
The parts of the prescribing information and the patient leaflet that come up in the first 3 months, in plain words.
- A dose set by weight
People who weigh 30kg (about 66 lb) or more get 20mg/kg every 2 weeks.
- People under 30kg get 40mg/kg every 2 weeks.FDA label
- How the dose is mixed
Each vial holds 100mg of powder, mixed with sterile water and then added to a sugar water (dextrose) bag.
- The number of vials is rounded up to the next whole vial.FDA label
- Infusion size goes with weight
The bag holds 100mL to 300mL for people under 30kg (about 66 lb).
- For heavier people it holds 200mL to 700mL, depending on weight band.FDA label
- Infusion speed
Every infusion starts at 1mg/kg per hour and goes up every 30 minutes, to 7mg/kg per hour.
- Children on 40mg/kg may later step up to 10mg/kg per hour.FDA label
- Medicines before an infusion
The label tells doctors to consider allergy, fever or steroid medicines before each infusion to lower the risk of reactions.
- Reactions can still happen after these medicines.FDA label
- If a mild or moderate reaction happens
Staff may pause the infusion for 30 minutes or cut the speed in half, and treat the symptoms.
- If symptoms last longer than 30 minutes, they may stop for the day.FDA label
- If a severe reaction happens
The label says to stop Nexviazyme right away and treat the reaction.
- Your doctor then weighs whether to try again, possibly at a lower dose and slower speed.FDA label
- If an infusion is missed
The label says to restart as soon as possible, then keep a 2 week gap between infusions after that.FDA label
- Pregnancy
Data on Nexviazyme in pregnancy are too limited to judge the risk.
- Untreated Pompe disease can worsen breathing and muscle symptoms in pregnancy, so the label says the choice should fit each person.FDA label
- Breastfeeding
There are no data on Nexviazyme in breast milk.
- If you are pregnant or breastfeeding while on Nexviazyme, the label asks you to report it at 1-800-633-1610, option 1.FDA label
What the label says to act on
The symptoms the label singles out, and what it tells patients and prescribers to do about each. If any of these happen, the label's instruction is the one to follow, with your care team.
Signs of a severe allergic reaction
Chest discomfort, redness, swelling all over, a swollen tongue, hives, rash, trouble breathing, low oxygen or low blood pressure.
- The label says to get medical care right away.FDA label
Infusion reactions
These can happen during the infusion or within a few hours after it, and are more likely at faster speeds.
- In studies, 34% of people on Nexviazyme had one.FDA label
Heart or lung strain from the fluid
People with weak heart or lung function, or who hold on to fluid, may get worse during an infusion.
- The label calls for closer checks of vital signs in these people.FDA label
Being sick on infusion day
People with a new illness at the time of an infusion seem to be at greater risk of reactions.
- Tell your infusion team if you feel unwell before an infusion starts.FDA label
Other side effects
In the main trial, the most common were headache (22%), tiredness (18%), diarrhea (12%), nausea (12%), joint pain, dizziness and muscle pain (10% each).FDA label
Why the pharmacy or the insurer says no, and what fixes it
Most first-fill delays are one of these. Knowing which one tells you who to call.
- Prior authorization pending or deniedThe Cigna policy below asks for a lab report showing low GAA enzyme activity in blood, skin cells or muscle, or a genetic test showing changes in both GAA genes.Cigna policy
- No record of late-onset diseaseCigna asks for documents showing the late-onset form, in a patient 1 year or older. The label does not cover infantile-onset Pompe disease.Cigna policy
- The doctor is not a specialistCigna asks that Nexviazyme be prescribed by, or with advice from, a geneticist, neurologist, metabolic specialist or lysosomal storage disorder specialist.Cigna policy
- A dose outside the plan's limitCigna approves 20mg/kg every 2 weeks at 30kg or more and 40mg/kg every 2 weeks under 30kg. Other doses are reviewed case by case.Cigna policy
- Waiting on a commercial planQuickStart may give eligible commercially insured patients Nexviazyme at no charge during the review, or after a delay or denial. Your doctor's office sends the application.Starting Nexviazyme (HCP)
- A copay you cannot payCommercial insurance: the Co-Pay Program pays 100% of eligible out-of-pocket drug costs up to a program maximum. Medicare, Medicaid, VA, TRICARE and other government coverage are excluded.Starting Nexviazyme (HCP)
- Costs the copay program skipsSanofi says the Co-Pay Program does not cover office visits, nursing or observation time, bloodwork, premedications or transportation. Infusion costs are not covered in Massachusetts or Rhode Island.Starting Nexviazyme (HCP)
- A new calendar yearThe Co-Pay Program runs from January 1 through December 31. Sanofi says you may need to re-enroll each year to confirm you still qualify.Co-Pay Program application
- No insurance, or the plan will not cover itThe CareConnectPSS Patient Assistance Program may provide Nexviazyme at no cost. Sanofi calls it a temporary solution until coverage can be secured. Call 1-800-745-4447, option 3.CareConnect for HCPs
Cigna prior authorization policy, August 15, 2026
- Patient is 1 year of age or older
- Late-onset Pompe disease, with documents
- Diagnosis shown by low acid alpha-glucosidase activity in blood, skin cells or muscle, or by disease-causing variants in both GAA genes
- Prescribed by, or with advice from, a geneticist, neurologist, metabolic disorder specialist or lysosomal storage disorder specialist
- 20mg/kg by IV every 2 weeks at 30kg or more, or 40mg/kg by IV every 2 weeks under 30kg
Approval length. One year.
This policy (Drug Coverage Policy IP0279) added documentation requirements on August 15, 2026. It cites the September 2023 label, which is still the latest FDA-approved text. It is here as an example of what a plan asks for, not as a rule. Cigna policy
Who to call, and what they can do
The drugmaker's program handles most of the first-fill problems above. Charity funds and foundation help are on the help paying page.
- A Case Manager for insurance, financial help, care coordination and life changes such as travel, moving or new insurance. For Spanish, press 7
- A Patient Education Liaison for disease, treatment and inheritance education, in person, by phone or online
- QuickStart: Nexviazyme at no charge for eligible commercially insured patients while coverage is reviewed, delayed or denied, usually up to 4 infusions Starting Nexviazyme (HCP)
- The Co-Pay Program for commercial insurance, covering 100% of eligible drug costs and certain infusion-related costs up to a program maximum, with questions answered at 1-855-448-6520 Co-Pay Program application
- Co-pay help is not open to Medicare, Medicaid, VA, Department of Defense or TRICARE patients, under the program's terms Starting Nexviazyme (HCP)
- The Patient Assistance Program, certain Sanofi therapies at no cost for eligible people without insurance or whose plan will not cover them
- Help finding treatment centers and home infusion resources when you travel or move
Details as the drugmaker publishes them. CareConnect for HCPs
Five questions for the pharmacist or the support line
- Who orders my Nexviazyme for each infusion, and where is it shipped?
- Is my dose based on my current weight, and who do I tell if my weight or my child's weight changes?
- How many vials make up my dose, and how long will my infusion run?
- Which medicines will I get before my infusion, and why?
- If I am switching from Lumizyme, can I keep the same schedule and infusion center?
- Has my Co-Pay Program been applied to both the medicine and the infusion costs?
Questions people ask in the first weeks
How long does a Nexviazyme infusion take?
About 4 to 5 hours at the 20mg/kg dose. At 40mg/kg, the first infusion takes about 7 hours, and later ones can be cut to about 5 hours with a faster step schedule.
How is my dose worked out?
By weight. People who weigh 30kg (about 66 lb) or more get 20mg/kg, and people under 30kg get 40mg/kg. Both are given every 2 weeks.
Will I get medicine before my infusion?
Possibly. The label tells doctors to consider allergy, fever or steroid medicines before each infusion to lower the risk of reactions. Your doctor decides what you get.
Does Nexviazyme have a boxed warning?
Yes, since its approval in August 2021. It covers severe allergic reactions including anaphylaxis, severe infusion reactions, and sudden heart or lung failure in people at risk from the extra fluid.
Can I switch from Lumizyme to Nexviazyme?
Sanofi says people switching from alglucosidase alfa (Lumizyme) need no break between the last Lumizyme dose and the first Nexviazyme dose. Your doctor decides whether a switch fits you.
Can young children receive Nexviazyme?
Yes, from age 1, for late-onset Pompe disease. Children under 30kg (about 66 lb) get the higher 40mg/kg dose. Nexviazyme is not approved for infantile-onset Pompe disease.
What if I miss an infusion?
The label says to restart as soon as possible, then keep 2 weeks between infusions after that. Call your infusion site to rebook.
How long does it take to start Nexviazyme after it is prescribed?
There is no fixed number of days. The diagnosis paperwork, prior authorization and booking each take their own time. With commercial insurance, QuickStart may let you begin while the plan reviews.
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Sources
- NEXVIAZYME (avalglucosidase alfa-ngpt) for injection, for intravenous use, prescribing information, Revised 9/2023 (SPL version 4, effective June 5, 2025), Genzyme Corporation, via openFDA and DailyMed, June 5, 2025.
- NEXVIAZYME (avalglucosidase alfa-ngpt) for injection, for intravenous use, prescribing information, Revised: 9/2023, FDA, Drugs@FDA, September 15, 2023.
- NEXVIAZYME (avalglucosidase alfa-ngpt) for injection, for intravenous use, prescribing information, Revised: 8/2021, FDA, Drugs@FDA, August 6, 2021.
- NEXVIAZYME® (avalglucosidase alfa-ngpt) Dosing & Infusion for LOPD Patients, Sanofi, April 1, 2025.
- Support for LOPD Patients | NEXVIAZYME® (avalglucosidase alfa-ngpt), Sanofi, April 1, 2025.
- Starting NEXVIAZYME® (avalglucosidase alfa-ngpt), Sanofi, March 1, 2026.
- Dosing and Administration | NEXVIAZYME® (avalglucosidase alfa-ngpt), Sanofi, March 1, 2026.
- CareConnect for Healthcare Professionals, Sanofi, November 1, 2024.
- CareConnect Personalized Support Services Co-Pay Program Application, Enzyme Replacement Therapies (MAT-US-2506611-v1.0-06/2025), Sanofi, June 1, 2025.
- Pompe Disease – Enzyme Replacement Therapy – Nexviazyme, Drug Coverage Policy IP0279, Cigna, August 15, 2026.
- BLA 761194/S-005 supplement approval letter, additional clinical data and dosage and administration information, FDA, September 15, 2023.
- BLA 761194 approval letter, Nexviazyme (avalglucosidase alfa-ngpt), FDA, August 6, 2021.
This page explains what the FDA label, the drugmaker's support program and an insurer policy say. It is not medical advice and does not tell anyone to start, stop or change a medicine. Dosing, test timing and restarts after a gap are decisions for the prescribing neurologist. Found an error? Use the report link in the footer and it will be checked against the source.