Alglucosidase Alfa

Other Alimentary Tract Preps · Enzyme produced by recombinant DNA technology

Indications

Late (non-infantile) onset Pompe disease (GAA deficiency) in patients 8 yrs and older without evidence of cardiac hypertrophy.

Dosage

IV: Adults: 20 mg/kg body wt every 2 wks as an IV inf. Children > 8 yrs: Same as adults.

Administration

Slowly withdraw the reconstituted solution from each vial. Avoid foaming in the syringe. Remove airspace from the infusion bag to minimize particle formation. Add the reconstituted solution slowly and directly into the NaCl solution. Do not add directly into airspace that may remain within the infusion bag. Avoid foaming in the infusion bag. Gently invert or massage the infusion bag to mix. Do not shake. Administer using an in-line low protein-binding 0.2 mcm filter. Reconstitution: Remove the vials from the refrigerator and allow them to reach room temperature prior to reconstitution (approx 30 mins). Reconstitute vial by slowly injecting 10.3 mL of SWFI to the inside wall of each vial. Each vial will yield 5 mg/mL. The total extractable dose per vial is 50 mg/10 mL. Avoid forceful impact of the WFI on the powder and avoid foaming. This is done by slow drop-wise addition of the WFI…

Adverse Effects

Flushing, tachycardia, cyanosis, facial oedema, chest discomfort, cough, headache, agitation, tremor, irritability, restlessness, dizziness, muscle spasm, sweating, rash, pruritus, urticaria, hypersensitivity reactions, cold extremities, bronchospasm, paraesthesia, pyrexia, antibody formation. Nausea, vomiting, diarrhoea.

Risk Factor (Pregnancy/Lactation)

Precautions: Cardiac and respiratory dysfunction. Monitor patients for the development of systemic immune complex-mediated reactions involving skin and other organs during treatment. Patients should be monitored for IgG antibody formation every 3 months for 2 years and then annually thereafter. Pregnancy, lactation.

Advice to Patient

Injected under the supervision of expert medical practitioner only. If a dose is missed, inform prescriber. Contact prescriber immediately if there is allergic reaction (rash, hives, itching, difficulty in breathing, chest or throat tightness, swelling on face/lips/tongue), back/neck pain, arm/leg pain, restlessness, irritability, blood in urine, blue skin or nails, chest pain/discomfort, less urination, diarrhoea, dizziness, light-headedness, numbness/burning/tingling, slow or irregular heartbeat, feeling cold in arms or legs, fever, changes in hearing, ear pain, increased tears, muscle pain/stiffness/twitching, nosebleeds. Certain lab tests are required during treatment, keep all doctors and lab appointments.

Compiled by Healify Pharmacy from published drug references. How we compile it →

Always consult a pharmacist or doctor.