Prescribing guide
Nexviazyme (avalglucosidase alfa-ngpt)Weight-based dosing, infusion rate steps, reaction management and prior authorization criteria for late-onset Pompe diseaseFor prescribers and their offices treating pompe disease: what the pharmacy needs before it ships, what the prior authorization wants documented, the titration and the monitoring the label requires.
Dispensing gate
No REMS. Boxed warning: CPR equipment and monitoring readily available at every infusion. Prior authorization, or QuickStart for eligible commercial patients, and a site of care before the first infusion.
Example PA threshold
Cigna example: age 1 year or older; documented LOPD; deficient GAA activity in blood, fibroblasts or muscle, or biallelic pathogenic or likely pathogenic GAA variants; specialist prescriber; 20mg/kg (30kg or more) or 40mg/kg (under 30kg) every 2 weeks.
Monitoring
Supervised infusion with anaphylaxis readiness at every dose; consider premedication; more frequent vitals in patients prone to fluid overload or with compromised cardiorespiratory function; higher ADA titers linked to more IARs.
Support line
CareConnectPSS Case Managers 1-800-745-4447, option 3 (Monday to Friday 8 AM to 6 PM EST); QuickStart for eligible commercial patients; Co-Pay Program 1-855-448-6520; Patient Assistance Program for uninsured or denied patients.
Give your patient their plan
A one-page handout for starting Nexviazyme, in plain language: what the first fill waits on, the first dates, who will call and the symptoms to report, with blank lines for the start date and your office's number. The link opens the patient's full first 90 days page, where their dates become calendar entries.
trialfriend.com/p/nexviazymeBefore the first fill: REMS, labs and enrollment
The steps that the pharmacy, the REMS or the drugmaker's program will wait on, in the order they usually need to happen. A first fill of a specialty drug most often stalls on one of these rather than on the plan.
| Step | What it requires | Source |
|---|---|---|
| Enroll in CareConnectPSS | Send the patient a digital consent link, or have them call a Case Manager at 1-800-745-4447 (English option 3, Spanish option 7). Lines are open Monday to Friday, 8 AM to 6 PM EST; or email info@CareConnectPSS.com. | CareConnect for HCPs |
| QuickStart | Nexviazyme at no charge for eligible commercially insured patients while authorization is reviewed or after coverage delays or denials. The provider must send the application; up to 4 infusions, sometimes extended. | Starting Nexviazyme (HCP) |
| Ordering and delivery | Purchase through authorized distributors, specialty pharmacies or directly from Sanofi. Rare Disease Product Services, 1-800-745-4447 option 1 or CO@Sanofi.com, sends infusion and refill reminders and has after-hours on-call service. | CareConnect for HCPs |
| Calculate vials | 100mg single-dose vials, one per carton (NDC 58468-0426-1), refrigerated at 2°C to 8°C. Vials from actual body weight and dose, rounded up to the next whole number. | FDA label |
| Reconstitute | Let vials sit 30 minutes at room temperature. Add 10mL Sterile Water for Injection down the inside wall; tilt and roll, do not invert, swirl or shake; yields 10mg/mL. Up to 24 hours refrigerated if not diluted at once. | FDA label |
| Dilute | 5% Dextrose Injection to a fixed total volume by weight (Table 1: 100, 200 or 300mL at 40mg/kg; 200 to 700mL at 20mg/kg); final 0.5 to 4mg/mL. Diluted solution up to 24 hours at 2°C to 8°C; infuse within 9 hours of removal. | FDA label |
| Billing code | HCPCS J0219, injection, avalglucosidase alfa-ngpt, 4mg, as listed in the Cigna policy. | Cigna policy |
| Co-Pay Program | Commercial only: 100% of eligible out-of-pocket drug costs up to a program maximum, plus certain infusion-related costs (mixing, administration, supplies). Runs January 1 to December 31; re-enrollment may be needed yearly. Fax 1-800-750-9839; questions 1-855-448-6520. | Co-Pay Program application |
| Co-Pay exclusions | Excludes Medicare (including Part D), Medigap, Medicaid, VA, DoD and TRICARE. Does not cover office visits, nursing or observation, labs, premedications or transport. Infusion-related costs not covered in MA or RI. | Starting Nexviazyme (HCP) |
| Patient Assistance Program | Nexviazyme for patients without insurance or whose insurance has denied coverage; the patient completes a PAP application with the doctor. Sanofi describes PAP as temporary until coverage is secured. | CareConnect for HCPs |
Prior authorization criteria and what to document
One public policy as the example: Cigna, August 15, 2026. Approval 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.
| Element | What the policy asks | In the chart | Source |
|---|---|---|---|
| Age | Patient is 1 year of age or older | Date of birth in the note | Cigna policy |
| Disease form | Late-onset acid alpha-glucosidase deficiency (late-onset Pompe disease) [documentation required] | Diagnosis and onset in the note | Cigna policy |
| Diagnosis, enzyme route | Deficient acid alpha-glucosidase activity in blood, fibroblasts or muscle tissue [documentation required] | Enzyme assay report | Cigna policy |
| Diagnosis, genetic route | Biallelic pathogenic or likely pathogenic GAA variants [documentation required]; either route qualifies | Genetic test report | Cigna policy |
| Prescriber | Geneticist, neurologist, metabolic disorder sub-specialist or lysosomal storage disorder specialist, or in consultation with one | Prescriber specialty or consult note | Cigna policy |
| Dosing | 30kg or more: 20mg/kg IV every 2 weeks. Under 30kg: 40mg/kg IV every 2 weeks. Other doses case by case | Order with current weight | Cigna policy |
| Approval | 1 year; extended approvals if criteria and dosing still met | Updated criteria at each review | Cigna policy |
Nexviazyme dosing and titration
| When | Label | Source |
|---|---|---|
| Dose | 30kg or more: 20mg/kg of actual body weight every 2 weeks IV. Under 30kg: 40mg/kg every 2 weeks. | FDA label |
| Rate, 20mg/kg | 1mg/kg/hour, then every 30 minutes to 3, 5 and 7mg/kg/hour if no reactions; continue 7mg/kg/hour. About 4 to 5 hours. | FDA label |
| Rate, 40mg/kg | Initial infusion: same 4-step process, about 7 hours. Subsequent (optional 5-step): 1, 3, 6, 8, then 10mg/kg/hour, about 5 hours. | FDA label |
| Administration set | In-line, low protein-binding 0.2 micron filter recommended. Flush with 5% Dextrose after; do not infuse in the same line with other products. | FDA label |
| Before infusion | Consider pretreating with antihistamines, antipyretics and/or corticosteroids. CPR equipment and monitoring readily available. | FDA label |
| Mild or moderate reaction | Hold 30 minutes or slow by 50% and treat. If symptoms persist past 30 minutes, stop and monitor; may re-initiate same day at 50% of the reaction rate with pretreatment. | FDA label |
| Resuming | After a hold: 50% of the reaction rate, then up 50% every 15 to 30 minutes. After slowing: finish at the reduced rate; next infusion, step back up to the reaction rate and beyond. | FDA label |
| Severe reaction | Discontinue immediately and treat. Weigh rechallenge at slower rates and a lower dose; desensitization may be considered after severe hypersensitivity. | FDA label |
| Missed dose | Restart as soon as possible, then keep the 2 week interval. | FDA label |
| Switching | Sanofi: no washout between the last alglucosidase alfa dose and the first Nexviazyme dose. | Nexviazyme dosing page (HCP) |
| Pediatric, renal, hepatic | Not established under 1 year; not approved for IOPD. The label has no renal or hepatic impairment section. | FDA label |
Monitoring the Nexviazyme label requires
| When | What | What the label says to do with it | Source |
|---|---|---|---|
| Each infusion | Hypersensitivity, including anaphylaxis: chest discomfort, erythema, generalized edema, hypotension, hypoxia, rash, respiratory distress, tongue edema, urticaria | 48% had hypersensitivity in studies, 4% severe, 2% anaphylaxis. If severe, discontinue and treat immediately | FDA label |
| During and hours after | IARs, more likely at higher rates; 34% across studies | Acute underlying illness raises risk; advanced disease raises risk of severe complications | FDA label |
| Each infusion, at-risk patients | Vital signs in patients prone to fluid overload, or with acute respiratory illness or compromised cardiac or respiratory function | More frequent vitals; some may need prolonged observation | FDA label |
| Ongoing | Anti-drug antibodies | 95% (59/62) of ERT-naive patients developed ADA, median 8 weeks. IARs 69% at peak titer 12,800 or above vs 27% below. Cross-reactive with alglucosidase alfa | FDA label |
| Ongoing | Pompe Registry; pregnancy or lactation exposure | registrynxt.com or 1-800-745-4447, extension 15500; report exposure at 1-800-633-1610, option 1 | FDA label |
Nexviazyme drug interactions and contraindications
| Label category | Agents and what the label says | Source |
|---|---|---|
| Contraindications | None | FDA label |
| Drug interactions | The label has no drug interactions section; do not infuse in the same IV line with other products | FDA label |
| Pregnancy | Case reports insufficient to evaluate risk; alglucosidase alfa data have not identified a drug-associated risk. Individualize continuation; untreated disease may worsen respiratory and musculoskeletal symptoms | FDA label |
| Lactation | No data on milk presence, infant effects or milk production; alglucosidase alfa reported in human milk | FDA label |
| Geriatric | 13 patients 65 to 74 and 4 patients 75 and older in studies; same dosage as younger adults | FDA label |
Why a first fill stalls, and the fix
- 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.
- 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.
- 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.
- 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.
- 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.
- 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.
- 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.
- 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.
- 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.
CareConnectPSS (CareConnect Personalized Support Services)
- 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
Questions prescribers' offices ask about Nexviazyme
What is the Nexviazyme dose for late-onset Pompe disease?
The September 2023 label (DailyMed version 4, June 2025) sets 20mg/kg of actual body weight every 2 weeks IV at 30kg or more. Under 30kg the dose is 40mg/kg every 2 weeks. Indicated from age 1 year; not approved for IOPD. Round vials (100mg) up to the next whole vial.
What are the Nexviazyme infusion rate steps?
The September 2023 label starts every infusion at 1mg/kg/hour, then steps every 30 minutes to 3, 5 and 7mg/kg/hour if no reactions, holding 7mg/kg/hour to completion. That runs about 4 to 5 hours at 20mg/kg and about 7 hours for the first 40mg/kg infusion. Later 40mg/kg infusions may use 1, 3, 6, 8, then 10mg/kg/hour, about 5 hours.
Is premedication required before Nexviazyme infusions?
Not required; section 2.1 of the September 2023 label says to consider pretreating with antihistamines, antipyretics and/or corticosteroids before every infusion. IARs may still occur after pretreatment. CPR equipment and appropriate monitoring should be readily available during every administration.
How should a Nexviazyme infusion reaction be managed?
Section 2.3 of the September 2023 label: for severe hypersensitivity or IAR, stop immediately and treat. For mild to moderate reactions, consider holding 30 minutes or slowing the rate by 50%; if symptoms persist past 30 minutes, stop and monitor. On resolution, resume at 50% of the reaction rate and increase by 50% every 15 to 30 minutes as tolerated.
What are the Nexviazyme prior authorization criteria?
The Cigna policy shown here, IP0279 effective August 15, 2026, approves 1 year for patients 1 year or older with documented late-onset Pompe disease. Diagnosis needs deficient GAA activity in blood, fibroblasts or muscle, or biallelic pathogenic or likely pathogenic GAA variants. It also requires a specialist prescriber and labeled weight-based dosing.
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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, 2025-06-05.
- NEXVIAZYME (avalglucosidase alfa-ngpt) for injection, for intravenous use, prescribing information, Revised: 9/2023, FDA, Drugs@FDA, 2023-09-15.
- NEXVIAZYME (avalglucosidase alfa-ngpt) for injection, for intravenous use, prescribing information, Revised: 8/2021, FDA, Drugs@FDA, 2021-08-06.
- NEXVIAZYME® (avalglucosidase alfa-ngpt) Dosing & Infusion for LOPD Patients, Sanofi, 2025-04.
- Support for LOPD Patients | NEXVIAZYME® (avalglucosidase alfa-ngpt), Sanofi, 2025-04.
- Starting NEXVIAZYME® (avalglucosidase alfa-ngpt), Sanofi, 2026-03.
- Dosing and Administration | NEXVIAZYME® (avalglucosidase alfa-ngpt), Sanofi, 2026-03.
- CareConnect for Healthcare Professionals, Sanofi, 2024-11.
- CareConnect Personalized Support Services Co-Pay Program Application, Enzyme Replacement Therapies (MAT-US-2506611-v1.0-06/2025), Sanofi, 2025-06.
- Pompe Disease – Enzyme Replacement Therapy – Nexviazyme, Drug Coverage Policy IP0279, Cigna, 2026-08-15.
- BLA 761194/S-005 supplement approval letter, additional clinical data and dosage and administration information, FDA, 2023-09-15.
- BLA 761194 approval letter, Nexviazyme (avalglucosidase alfa-ngpt), FDA, 2021-08-06.
A reference drawn from the June 5, 2025 label, the drugmaker's published program and one public insurer policy shown as an example, checked on November 10, 2026. Labels and policies change; the label on DailyMed and the plan's current policy govern. Trial Friend is not a medical provider. It reports what these documents say and does not judge whether a drug is safe or right for any patient; that is the prescriber's decision. Not a substitute for the prescribing information.