Nexviazyme
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.
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.
Drug facts
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%)
Less Common Side Effects
- Chills
- Cough
- Fever
- Stomach pain
- Blood pressure changes
Nexviazyme (avalglucosidase alfa) 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
The following Nexviazyme (avalglucosidase alfa) 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
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
headache
increased sweating
irregular, fast, slow, or shallow breathing
lightheadedness, dizziness, or fainting
loss of voice
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
shakiness in the legs, arms, hands, or feet
slow or irregular heartbeat
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
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
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
dryness or soreness of the throat
ear discomfort or pain
ear drainage
feeling of constant movement of self or surroundings
general feeling of discomfort or illness
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
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.
Common questions about Nexviazyme (avalglucosidase alfa)
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.
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.
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.
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.
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.
How to save using GoodRx
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
Typical dosage 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
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)?
Nexviazyme (avalglucosidase alfa) images
