Alglucosidase alfa injection

Alglucosidase alfa is injected into a vein through an IV. A healthcare provider will give you this injection. Alglucosidase alfa must be given slowly, and the IV infusion can take up to 4 hours to complete.

This medicine is usually given once every 2 weeks.

Before each injection, tell your doctor if you have recently been sick with a cold, flu, or other illness.

If you need surgery, tell the surgeon ahead of time that you are using alglucosidase alfa.

Alglucosidase alfa can have long lasting effects on your body. Your blood will need to be tested every 3 months for 2 years and then once every year after that.

Usual Adult Dose for Pompe disease:

20 mg/kg via IV infusion every 2 weeks

Comments:
-Total volume of infusion is determined by patient's body weight and should be infused over about 4 hours.

Usual Pediatric Dose for Pompe disease:

20 mg/kg via IV infusion every 2 weeks

Comments:
-Total volume of infusion is determined by body weight and should be infused over about 4 hours.

Other drugs may interact with alglucosidase alfa, including prescription and over-the-counter medicines, vitamins, and herbal products. Tell each of your health care providers about all medicines you use now and any medicine you start or stop using.

Applies to alglucosidase alfa: intravenous powder for injection

General

The most common side effects were hypersensitivity reactions, fever, diarrhea, rash, vomiting, cough, pneumonia, otitis media, upper respiratory tract infection, gastroenteritis, and decreased oxygen saturation. The most serious side effects were anaphylactic reactions, acute cardiorespiratory failure, and cardiac arrest. The most common serious side effects were pneumonia, respiratory failure, respiratory distress, catheter-related infection, respiratory syncytial virus infection, gastroenteritis, and fever.[Ref]

Hypersensitivity

The most common side effects that required intervention in clinical trials in infantile-onset and juvenile-onset Pompe disease were hypersensitivity reactions (51%) and included rash, pyrexia, urticaria, flushing, decreased oxygen saturation, cough, tachypnea, tachycardia, hypertension/increased blood pressure, pallor, rigors, vomiting, cyanosis, agitation, and tremor. These side effects occurred more often with higher infusion rates. Hypersensitivity reactions were reported in some patients who were pretreated with antihistamines, antipyretics, and/or corticosteroids.

Delayed-onset hypersensitivity reactions (defined as side effects occurring 2 to 48 hours after infusion) included hyperhidrosis, fatigue, myalgia, and nausea.

Hypersensitivity reactions in infantile-onset Pompe disease patients included livedo reticularis, irritability, retching, increased lacrimation, ventricular extrasystoles, nodal rhythm, rales, respiratory tract irritation, and cold sweat.

Anaphylaxis and hypersensitivity reactions (included anaphylactic shock, respiratory failure, respiratory arrest, cardiac arrest, hypoxia, dyspnea, wheezing, convulsions, peripheral coldness, restlessness, nervousness, back pain, stridor, pharyngeal edema, abdominal pain, apnea, muscle spasm, conjunctivitis) have been reported during postmarketing experience.[Ref]

Very common (10% or more): Hypersensitivity reactions (included anaphylaxis, headache, nausea, urticaria, dizziness, chest discomfort/pain, vomiting, hyperhidrosis, flushing/feeling hot, paresthesia, pyrexia, local swelling, diarrhea, pruritus, rash, throat tightness, fatigue, myalgia, cough, decreased oxygen saturation, tachycardia, tachypnea, muscle twitching, agitation, cyanosis, erythema, hypertension/increased blood pressure, pallor, rigors, tremor, livedo reticularis, irritability, retching, increased lacrimation, ventricular extrasystoles, nodal rhythm, rales, respiratory tract irritation, cold sweat, anaphylactic shock, respiratory failure, respiratory arrest, cardiac arrest, respiratory distress, hypoxia, dyspnea, bradycardia, bronchospasm, hypotension, angioedema [including tongue or lip swelling, periorbital edema, face edema], wheezing, convulsions, peripheral coldness, restlessness, nervousness, back pain, stridor, pharyngeal edema, abdominal pain, apnea, muscle spasm, conjunctivitis)
Common (1% to 10%): Anaphylaxis (presented as angioedema, throat tightness, chest pain/discomfort)
Postmarketing reports: Systemic and cutaneous immune-mediated reactions (including proteinuria and nephrotic syndrome secondary to membranous glomerulonephritis, ulcerative and necrotizing skin lesions)[Ref]

Cardiovascular

Supraventricular tachycardia was reported in a patient with history of Wolff-Parkinson-White syndrome.

Acute cardiorespiratory failure (possibly associated with fluid overload) was reported in infantile-onset Pompe disease patients with preexisting hypertrophic cardiomyopathy.[Ref]

Very common (10% or more): Tachycardia (up to 23%), flushing (up to 21%), bradycardia (up to 21%)
Common (1% to 10%): Hypertension, increased blood pressure, pallor, cyanosis
Frequency not reported: Supraventricular tachycardia, cardiac arrest, hypotension, vasoconstriction, increased heart rate, decreased blood pressure, decreased heart rate
Postmarketing reports: Acute cardiorespiratory failure[Ref]

Other

The most common side effects that required intervention in clinical trials in infantile-onset Pompe disease were infusion reactions (defined as side effects occurring during or within 2 hours after infusion; 51%) and included rash, fever, urticaria, flushing, decreased oxygen saturation, cough, tachypnea, tachycardia, hypertension, increased blood pressure, irritability, pallor, pruritus, retching, tremor, hypotension, rigors, vomiting, cyanosis, agitation, bronchospasm, erythema, face edema, feeling hot, headache, hyperhidrosis, increased lacrimation, livedo reticularis, nausea, periorbital edema, restlessness, wheezing, cardiac arrest, bradycardia, angioedema, pharyngeal edema, peripheral edema, chest pain, chest discomfort, dyspnea, muscle spasm, fatigue, respiratory distress, throat tightness, and conjunctivitis. Infusion reactions were reported more often in antibody-positive patients, especially those with high antibody titers.

Recurrent reactions consisting of influenza-like illness or a combination of events (e.g., pyrexia, chills, myalgia, arthralgia, pain, fatigue) after infusion generally lasted for 1 to 3 days.

Severe and serious infusion reactions (including cardiac arrest, respiratory arrest, apnea, stridor, pharyngeal edema, peripheral edema, chest pain, chest discomfort, muscle spasm, fatigue, conjunctivitis) have been reported during postmarketing experience.[Ref]

Very common (10% or more): Pyrexia (up to 92%), infusion reactions, otitis media (up to 44%), ear infection (up to 33%), catheter-related infection (up to 28%), postprocedural pain (up to 26%)
Common (1% to 10%): Malaise, rigors, chest discomfort, flushing/feeling hot, peripheral edema, chills, fatigue, increased body temperature
Frequency not reported: Chest pain, face edema, peripheral coldness, enzyme activity inhibited, enzyme uptake inhibited, lethargy, hypothermia
Postmarketing reports: Recurrent reactions (consisting of influenza-like illness or combination of events [e.g., pyrexia, chills, myalgia, arthralgia, pain, fatigue]) after infusion[Ref]

Gastrointestinal

Very common (10% or more): Diarrhea (up to 62%), vomiting (up to 49%), gastroenteritis (up to 41%), oral candidiasis (up to 31%), gastroesophageal reflux disease (up to 26%), constipation (up to 23%), upper abdominal pain (up to 15%)
Common (1% to 10%): Retching, nausea
Frequency not reported: Abdominal pain[Ref]

Dermatologic

Very common (10% or more): Rash (up to 54%), diaper dermatitis (up to 36%), urticaria (up to 21%)
Common (1% to 10%): Erythema, pruritus, hyperhidrosis, papular rash, macular rash, maculopapular rash
Frequency not reported: Erythematous rash, livedo reticularis, palmar erythema
Postmarketing reports: Ulcerative and necrotizing skin lesions[Ref]

Respiratory

Very common (10% or more): Cough (up to 46%), pneumonia (up to 46%), upper respiratory infection (up to 44%), pharyngitis (up to 36%), respiratory distress (up to 33%), respiratory failure (up to 31%), rhinorrhea (up to 28%), tachypnea (up to 23%), bronchiolitis (up to 23%), nasopharyngitis (up to 23%)
Common (1% to 10%): Rhinitis, throat tightness
Frequency not reported: Respiratory arrest, apnea, bronchospasm, wheezing, pharyngeal edema, dyspnea, stridor, respiratory syncytial virus infection, increased respiratory rate, rales[Ref]

Metabolic

Very common (10% or more): Decreased oxygen saturation (up to 41%)
Frequency not reported: Increased blood calcium[Ref]

Immunologic

Very common (10% or more): IgG antibodies to alglucosidase alfa (up to 100%)
Frequency not reported: Development of alglucosidase alfa-specific IgE antibodies, development of neutralizing antibodies[Ref]

Hematologic

Very common (10% or more): Anemia (up to 31%)
Frequency not reported: Decreased hemoglobin, decreased platelet count[Ref]

Musculoskeletal

Common (1% to 10%): Muscle twitching, myalgia, muscle spasms, increased blood creatine phosphokinase MB
Frequency not reported: Arthralgia, increased blood creatine phosphokinase[Ref]

Nervous system

Common (1% to 10%): Headache, tremor, paresthesia, dizziness[Ref]

Psychiatric

Common (1% to 10%): Agitation, insomnia, irritability
Postmarketing reports: Restlessness[Ref]

Local

Common (1% to 10%): Local swelling
Frequency not reported: Infusion site pain, infusion site reaction[Ref]

Renal

Frequency not reported: Increased blood urea
Postmarketing reports: Nephrotic syndrome (secondary to membranous glomerulonephritis)[Ref]

Genitourinary

Postmarketing reports: Proteinuria

Endocrine

Postmarketing reports: Hyperparathyroidism[Ref]

Hepatic

Frequency not reported: Increased ALT, increased AST

Ocular

Frequency not reported: Conjunctivitis, periorbital edema, increased lacrimation

Some side effects of alglucosidase alfa may not be reported. Always consult your doctor or healthcare specialist for medical advice. You may also report side effects to the FDA.

20 mg/kg via IV infusion every 2 weeks

Comments:
-Total volume of infusion is determined by patient's body weight and should be infused over about 4 hours.

Data not available

Data not available

LactMed Record Number

907

Last Revision Date

20130907

Disclaimer

Information presented in this database is not meant as a substitute for professional judgment. You should consult your healthcare provider for breastfeeding advice related to your particular situation. The U.S. government does not warrant or assume any liability or responsibility for the accuracy or completeness of the information on this Site.