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Nexviazyme

avalglucosidase alfa-ngpt

Used for Lysosomal Storage Disease

Nexviazyme (avalglucosidase alfa or avalglucosidase alfa-ngpt) is used to treat people with a rare genetic condition called late-onset Pompe disease (LOPD). It's given as an infusion into the vein (intravenous, IV) every 2 weeks. The medication is only available as a brand name, which can be expensive.

Reviewed by:

Robert Chad Hakim, PharmD, BCCCP

Allison Gourley, PharmD

Last reviewed on July 29, 2022

Basics Side effects Pros & cons Pharmacist tips FAQs Warnings Dosage Interactions Alternatives Images Prices Medicare

What is Nexviazyme (avalglucosidase alfa)?

What is Nexviazyme (avalglucosidase alfa) used for?

Late-onset Pompe disease (LOPD)

How Nexviazyme (avalglucosidase alfa) works

Nexviazyme (avalglucosidase alfa) is an enzyme replacement therapy. In Pompe disease, you're missing an enzyme in your body called acid alpha-glucosidase (GAA). Without this enzyme, your body isn't able to break down glycogen into sugar to use as a source of energy.

Nexviazyme (avalglucosidase alfa) serves as a replacement for GAA, which allows your body to convert glycogen into sugar to use for energy.

Are you looking for information on Lumizyme (alglucosidase alfa) instead?

Drug Facts

Common BrandsNexviazyme

Drug ClassEnzyme replacement therapy

Controlled Substance ClassificationNot a controlled medication

Generic StatusNo lower-cost generic available

AvailabilityPrescription only

What are the side effects of Nexviazyme (avalglucosidase alfa)?

The following side effects may get better over time as your body gets used to the medication. Let your healthcare provider know immediately if you continue to experience these symptoms or if they worsen over time.

Common Side Effects

Headache (22%)
Fatigue (18%)
Diarrhea (12%)
Nausea (12%)
Joint pain (10%)
Muscle pain (10%)
Dizziness (10%)
Itchy skin (8%)
Vomiting (8%)
Shortness of breath or trouble breathing (6%)
Numb or tingling sensation (6%)
Skin redness (6%)
Rash (6%)

Other Side Effects

Chills
Cough
Fever
Stomach pain
Blood pressure changes

Serious Side Effects

Contact your healthcare provider immediately if you experience any of the following.

Allergic reactions: hives, rash, blisters, skin reddening, swelling of the lips or tongue, difficulty breathing

Source: DailyMed

The following side effects have also been reported

Along with its needed effects, a medicine may cause some unwanted effects. Although not all of these side effects may occur, if they do occur they may need medical attention.

Check with your doctor or nurse immediately if any of the following side effects occur:

More common

Blue lips, fingernails, or skin

body aches or pain

chest discomfort or pain

chills

cough

difficult or labored breathing

difficulty with swallowing

dizziness

dry, red, hot, or irritated skin

ear congestion

fast, pounding, or irregular heartbeat or pulse

feeling of warmth

fever

headache

hives, itching, or skin rash

increased sweating

irregular, fast, slow, or shallow breathing

lightheadedness, dizziness, or fainting

loss of voice

nasal congestion

pain

pale skin

puffiness or swelling of the eyelids or around the eyes, face, lips, or tongue

rapid shallow breathing

redness of the face, neck, arms, and occasionally, upper chest

redness or pain at the catheter site

runny nose

shakiness in the legs, arms, hands, or feet

slow or irregular heartbeat

sneezing

sore throat

swollen, painful, or tender lymph glands in the neck, armpit, or groin

tightness in the chest

trembling or shaking of the hands or feet

troubled breathing

troubled breathing with exertion

unusual bleeding or bruising

unusual tiredness or weakness

Less common

Blood in the urine

convulsions

decreased urine

dry mouth

increased thirst

loss of appetite

mood changes

muscle pain or cramps

nausea or vomiting

numbness or tingling in the hands, feet, or lips

pain in the groin or genitals

sharp back pain just below the ribs

Incidence not known

Blue-green to black skin discoloration

blurred vision

confusion

dilated neck veins

extreme fatigue

heart stops

inability to speak

no breathing

no pulse or blood pressure

pain, redness, swelling, or sloughing of the skin at the place of injection

seizures

severe or sudden headache

severe pain in the chest

slurred speech

sudden and severe weakness in the arm or leg on one side of the body

sudden onset of severe breathing difficulty

swelling of the face, fingers, feet, or lower legs

temporary blindness

unconscious

weight gain

Some side effects may occur that usually do not need medical attention. These side effects may go away during treatment as your body adjusts to the medicine. Also, your health care professional may be able to tell you about ways to prevent or reduce some of these side effects. Check with your health care professional if any of the following side effects continue or are bothersome or if you have any questions about them:

More common

Abdominal or stomach pain

acid or sour stomach

belching

body aches or pain

change in hearing

congestion

constipation

dryness or soreness of the throat

ear discomfort or pain

ear drainage

earache

feeling of constant movement of self or surroundings

general feeling of discomfort or illness

heartburn

hoarseness

indigestion

muscle or bone pain, stiffness, or tightness

muscle twitching

redness of the skin

redness or swelling in the ear

sensation of spinning

sore mouth or tongue

stomach discomfort, upset, or pain

tender, swollen glands in the neck

upper abdominal or stomach pain

voice changes

welts

white patches in the mouth or on the tongue

Less common

Bloody nose

sleepiness or unusual drowsiness

Incidence not known

Burning, dry, or itching eyes

discharge, excessive tearing

muscle spasm

redness, pain, or swelling of the eye, eyelid, or inner lining of the eyelid

Other side effects not listed may also occur in some patients. If you notice any other effects, check with your healthcare professional.

Call your doctor for medical advice about side effects. You may report side effects to the FDA at 1-800-FDA-1088.

Pros and cons of Nexviazyme (avalglucosidase alfa)

Pros

One of only a few options to treat Pompe disease

Only given every 2 weeks

Safe for use in children 1 year of age and older

Cons

Needs to be given by a healthcare provider

Brand name only, so it can be expensive

Not approved for infantile (or early-onset) Pompe disease

Pharmacist tips for Nexviazyme (avalglucosidase alfa)

Mark your appointments on your calendar for when you need to see your provider and receive Nexviazyme (avalglucosidase alfa). This helps make sure you don't miss any doses. If you accidentally miss an infusion, talk with your provider right away about rescheduling your appointment.

Your provider might give you or tell you to take medications (like acetaminophen (Tylenol) or diphenhydramine (Benadryl)) right before you receive your dose of Nexviazyme (avalglucosidase alfa). This is to help prevent allergic and infusion reactions.

Frequently asked questions about Nexviazyme (avalglucosidase alfa)

What is late-onset Pompe disease?

People with Pompe disease are missing an enzyme (protein) in their body called acid alpha-glucosidase (GAA). Normally, this enzyme breaks down glycogen (the stored form of sugar) to give your muscles energy. But without the GAA enzyme, people with Pompe disease can't break down glycogen in their body. This results in high glycogen levels in the muscles, which can cause muscle weakness. Late-onset Pompe disease (LOPD) is when the condition starts in children at least 1 year old to adulthood or when the condition doesn't affect the heart. Typical symptoms of LOPD include muscle problems (e.g., muscle weakness, pain, cramps), breathing problems, and stomach problems (e.g., trouble swallowing, poor weight gain). Nexviazyme (avalglucosidase alfa) provides your body with GAA so it can use up glycogen. This can help slow disease progression and improve symptoms.

What should I do to prepare for my Nexviazyme (avalglucosidase alfa) infusion?

Nexviazyme (avalglucosidase alfa) is given as an IV infusion over 4 to 7 hours. You'll typically receive this medication at an infusion center or a healthcare provider's office, since it must be given by a trained provider. You might need to get there earlier so your provider can give you medications before your treatment. These medications can help reduce infusion reactions (e.g., headache, nausea, fever, chills) or allergic reactions that happen during or after your infusion. It's also recommended you bring a book, something to work on, or an electronic device to keep yourself occupied while you receive the infusion.

How long do I have to take Nexviazyme (avalglucosidase alfa)?

Since LOPD is a genetic condition, you'll need to take Nexviazyme (avalglucosidase alfa) for your entire life. Continue taking the medication as long as it's helping your condition and you're not having severe side effects.

Can I use Nexviazyme (avalglucosidase alfa) if I'm pregnant?

Nexviazyme (avalglucosidase alfa) hasn't been well studied in human pregnancy, so it's not known whether this medication is safe or works well in pregnant females. In the few studies that looked at how a similar medication called Lumizyme (alglucosidase alfa) affected pregnancy, Lumizyme (alglucosidase) didn't cause harm to unborn babies and their mothers. If you're pregnant or thinking of becoming pregnant, talk with your provider about the risks and benefits of using Nexviazyme (avalglucosidase alfa) during pregnancy. If you're pregnant or breastfeeding and are prescribed Nexviazyme (avalglucosidase alfa), it's recommended to enroll in a pregnancy exposure registry that monitors your and your unborn baby's health by visiting the registry program's website or by calling 1-800-745-4447, extension 15500.

What's the difference between Nexviazyme (avalglucosidase alfa) and Lumizyme (alglucosidase alfa)?

Both Nexviazyme (avalglucosidase alfa) and Lumizyme (alglucosidase alfa) are enzyme replacement therapies that are given as IV infusions and work well to treat Pompe disease. Nexviazyme (avalglucosidase alfa) is specifically used for late-onset Pompe disease in adults and children at least 1 year of age, whereas Lumizyme can be used for Pompe disease in people of all ages.

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What are the risks and warnings for Nexviazyme (avalglucosidase alfa)?

Nexviazyme (avalglucosidase alfa) can cause some serious health issues. This risk may be even higher for certain groups. If this worries you, talk to your doctor or pharmacist about other options.

Allergic or infusion reactions

Allergic or infusion-related reactions to Nexviazyme (avalglucosidase alfa) are possible during and after your treatment. Life-threatening, allergic reactions include facial swelling, swollen tongue, difficulty breathing, and anaphylaxis (closing of the throat). Because of these risks, your provider will start the infusion at a slow speed and then gradually raise the speed as long as you're not having any signs of a reaction. Additionally, your provider might give you an antihistamine, fever medication (e.g., acetaminophen (Tylenol)), or steroid medication before your Nexviazyme (avalglucosidase alfa) dose. These medications help prevent infusion reactions. If you notice dizziness, sweating, nausea, chills, a choking sensation, swelling of the lips or tongue, or difficulty breathing anytime during your infusion or after your appointment, get medical attention right away.

Risk of sudden loss of breathing and heart function in certain people

Risk factors: History of heart or lung problems | Fluid overload

Nexviazyme (avalglucosidase alfa) might raise the risk of serious worsening problems in people who already have poor heart or lung health or are fluid overloaded. This can lead to sudden loss of breathing and heart function. Make sure to tell your provider about all of your medical conditions. Your provider will decide if it's safe for you to receive Nexviazyme (avalglucosidase alfa).

Nexviazyme (avalglucosidase alfa) dosage forms

Typical dosing for Nexviazyme (avalglucosidase alfa)

This medication is given as an infusion into the veins (IV) every 2 weeks.

The typical dose is based on your body weight and will be calculated by your provider. The infusion can take a total of 4 to 7 hours, depending on your dose and how well you tolerate the infusion.

Interactions between Nexviazyme (avalglucosidase alfa) and other drugs

Nexviazyme (avalglucosidase alfa) may interact with certain medications or supplements. Always let your doctor and pharmacist know about any other medications or supplements (including prescribed and over-the-counter medications, vitamins, and dietary or herbal supplements) that you are currently taking. The list below does not include all possible drug interactions with Nexviazyme (avalglucosidase alfa). Please note that only the generic name of each medication is listed below.

Although certain medicines should not be used together at all, in other cases two different medicines may be used together even if an interaction might occur. In these cases, your doctor may want to change the dose, or other precautions may be necessary. Tell your healthcare professional if you are taking any other prescription or nonprescription (over-the-counter [OTC]) medicine.

What are alternatives to Nexviazyme (avalglucosidase alfa)?

There are a number of medications that your doctor can prescribe in place of Nexviazyme (avalglucosidase alfa). Compare a few possible alternatives below.

Nexviazyme (avalglucosidase alfa)

Used for:

Late-onset Pompe disease (LOPD)

View Prices

Lumizyme (alglucosidase alfa)