Applies to avalglucosidase alfa: intravenous powder for solution.
Warning
Intravenous route (Powder for Solution)
Warning: Severe hypersensitivity reactions, infusion-associated reactions, and risk of acute cardiorespiratory failure in susceptible patientsHypersensitivity Reactions including AnaphylaxisPatients treated with avalglucosidase alfa-ngpt have experienced life-threatening hypersensitivity reactions, including anaphylaxis. Appropriate medical support measures, including cardiopulmonary resuscitation equipment, should be readily available during avalglucosidase alfa-ngpt administration. If a severe hypersensitivity reaction (e.g., anaphylaxis) occurs, avalglucosidase alfa-ngpt should be discontinued immediately and appropriate medical treatment should be initiated. In patients with severe hypersensitivity reaction, a desensitization procedure to avalglucosidase alfa-ngpt may be considered.Infusion-Associated Reactions (IARs)Patients treated with avalglucosidase alfa-ngpt have experienced severe IARs. If severe IARs occur, consider immediate discontinuation of avalglucosidase alfa-ngpt, initiation of appropriate medical treatment, and the benefits and risks of readministering avalglucosidase alfa-ngpt following severe IARs. Patients with an acute underlying illness at the time of avalglucosidase alfa-ngpt infusion may be at greater risk for IARs. Patients with advanced Pompe disease may have compromised cardiac and respiratory function, which may predispose them to a higher risk of severe complications from IARs.Risk of Acute Cardiorespiratory Failure in Susceptible PatientsPatients susceptible to fluid volume overload, or those with acute underlying respiratory illness or compromised cardiac or respiratory function for whom fluid restriction is indicated may be at risk of serious exacerbation of their cardiac or respiratory status during avalglucosidase alfa-ngpt infusion. More frequent monitoring of vitals should be performed during avalglucosidase alfa-ngpt infusion in such patients.
Serious side effects
Along with its needed effects, avalglucosidase alfa 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 while taking avalglucosidase alfa:
More common
- Back pain
- bluish lips or skin
- blurred vision
- chest discomfort or tightness
- chills
- confusion
- cough
- diarrhea
- difficulty swallowing
- dizziness, faintness, or lightheadedness when getting up suddenly from a lying or sitting position
- fast, pounding, or irregular heartbeat or pulse
- feeling of warmth
- fever
- general feeling of discomfort or illness
- headache
- hives, itching, skin rash
- joint pain
- loss of appetite
- muscle aches and pains
- nausea
- nervousness
- pounding in the ears
- puffiness or swelling of the eyelids or around the eyes, face, lips, or tongue
- redness of the face, neck, arms, and occasionally, upper chest
- runny nose
- shakiness in the legs, arms, hands, or feet
- shivering
- slow or fast heartbeat
- sore throat
- sweating
- trembling or shaking of the hands or feet
- trouble breathing
- trouble sleeping
- unusual tiredness or weakness
- vomiting
Other side effects
Some side effects of avalglucosidase alfa 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
- Burning, crawling, itching, numbness, prickling, "pins and needles", or tingling feelings
- difficulty in moving
- swollen joints
For Healthcare Professionals
Applies to avalglucosidase alfa: intravenous injection.
General
The most common adverse reactions were headache, fatigue, diarrhea, nausea, arthralgia, dizziness, myalgia, pruritus, vomiting, dyspnea, erythema, paresthesia, rash, urticaria, and chills.[Ref]
Cardiovascular
Common (1% to 10%): Hypertension, hypotension, increased blood pressure
Uncommon (0.1% to 1%): Tachycardia, ventricular extrasystoles, flushing, increased heart rate[Ref]
Dermatologic
Common (1% to 10%): Rash, pruritus, erythema, urticaria, palmar erythema, contusion
Uncommon (0.1% to 1%): Angioedema, hyperhidrosis, skin discoloration[Ref]
Gastrointestinal
Very common (10% or more): Diarrhea (up to 12%), nausea (up to 12%)
Common (1% to 10%): Vomiting, abdominal pain, upper abdominal pain, lip swelling, swollen tongue, dyspepsia
Uncommon (0.1% to 1%): Oral hypoesthesia, oral paresthesia, dysphagia[Ref]
Genitourinary
Common (1% to 10%): Cystitis[Ref]
Hypersensitivity
Very common (10% or more): Hypersensitivity reactions (up to 48%)
Common (1% to 10%): Anaphylaxis[Ref]
Life-threatening hypersensitivity reactions (including anaphylaxis) have been reported in patients treated with this drug. In clinical studies, 67 (48%) patients treated with this drug reported hypersensitivity reactions, including severe hypersensitivity reactions in 6 (4%) patients and anaphylaxis in 3 (2%) patients.[Ref]
Immunologic
In therapy-naive patients, the incidence of antidrug antibodies (ADA) at baseline was 3.3% and the incidence of ADA after treatment was 95.1%. In therapy-experienced patients, the incidence of ADA at baseline was up to 74% and the incidence of ADA after treatment was up to 55%. The median time to seroconversion was 8 weeks. ADA cross-reactivity studies showed that antibodies to this drug were cross-reactive to alglucosidase alfa.
In therapy-naive patients, the incidences of both neutralizing antibody (NAb) types, inhibition of enzyme activity only, and inhibition of enzyme cellular uptake only were 21.1%, 28%, and 39%, respectively. In therapy-experienced patients, the incidences of both NAb types, inhibition of enzyme activity only, and inhibition of enzyme cellular uptake only were up to 5%, up to 18%, and up to 21%, respectively.[Ref]
Very common (10% or more): Antidrug antibodies positive (up to 95.1%), neutralizing antibodies positive (up to 39%)
Uncommon (0.1% to 1%): Complement factor increased, immune complex level increased[Ref]
Local
Common (1% to 10%): Infusion site pain
Uncommon (0.1% to 1%): Infusion site extravasation, infusion site joint pain, infusion site rash, infusion site reaction, infusion site urticaria[Ref]
Metabolic
Common (1% to 10%): Decreased oxygen saturation[Ref]
Musculoskeletal
Very common (10% or more): Back pain (up to 23.5%), pain in extremity (up to 15.7%)
Common (1% to 10%): Arthralgia, myalgia, muscle spasms[Ref]
Nervous system
Very common (10% or more): Headache (up to 22%)
Common (1% to 10%): Dizziness, paresthesia, tremor
Uncommon (0.1% to 1%): Somnolence[Ref]
Ocular
Common (1% to 10%): Ocular hyperemia
Uncommon (0.1% to 1%): Conjunctivitis, conjunctival hyperemia, eye pruritus, increased lacrimation
Frequency not reported: Eye irritation[Ref]
Other
Very common (10% or more): Infusion-associated reactions (included chills, cough, diarrhea, erythema, fatigue, headache, influenza-like illness, nausea, ocular hyperemia, pain in extremity, pruritus, rash, erythematous rash, tachycardia, urticaria, vomiting, chest discomfort, dizziness, dyspnea, feeling hot, hyperhidrosis, lip swelling, oxygen saturation decreased, pain, palmar erythema, swollen tongue, upper abdominal pain, burning sensation, eyelid edema, feeling cold, flushing, respiratory distress, throat irritation, tremor; up to 34%), fatigue (up to 18%), fall (13.7%)
Common (1% to 10%): Pyrexia, chills, chest discomfort, pain, influenza-like illness, asthenia, noncardiac chest pain, peripheral edema
Uncommon (0.1% to 1%): Facial pain, hyperthermia, localized edema, peripheral swelling, increased body temperature
Frequency not reported: Middle ear effusion[Ref]
In clinical studies, infusion-associated reactions (IARs) occurred at any time during and/or within a few hours after infusion of this drug. IARs were reported in 48 (34%) patients treated with this drug, with 5 (4%) patients reporting severe IARs.[Ref]
Respiratory
Very common (10% or more): Nasopharyngitis (up to 23.5%), influenza (up to 17.6%)
Common (1% to 10%): Cough, dyspnea, upper respiratory tract infection
Uncommon (0.1% to 1%): Tachypnea, laryngeal edema, respiratory distress, throat irritation, abnormal breath sounds
Frequency not reported: Rhinorrhea[Ref]
