Names and pronunciation
(mi-gal′a-stat) Galafold
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Drug reference
(mi-gal′a-stat) Galafold
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Func. class.: Metabolic agent
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Chem. class.: Pharmacological chaperone
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ACTION: Stabilizes mutant variants of alpha-galactosidase to increase enzyme trafficking to lysosomes, binds to the active site of the alpha-galactosidase A (alpha-Gal A) protein (encoded by the galactosidase alpha gene, GLA), which is deficient in Fabry disease USES Treatment of a confirmed diagnosis of Fabry disease and an ame- nable galactosidase alpha gene (GLA) variant
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CONTRAINDICATIONS Hypersensitivity, severe renal disease, child
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SIDE EFFECTS CNS: Headache, fever GI: Nausea, vomiting, abdominal pain, diarrhea EENT: Nasopharynigitis GU: UTI MS: Back pain RES: Cough, epistaxis
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INTERACTIONS None known NURSING CONSIDERATIONS
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DOSAGE AND ROUTES Adult: PO 123 mg every other day
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Available forms: Capsules 123 mg
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Administer: • Give every other day at the same time of day • Do not administer on 2 consecutive days • Give on an empty stomach; do not consume food ≥2 hr before or 2 hr after dose • Swallow capsule whole; do not cut, crush, or chew • Store at room temperature in original container and protect from moisture
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Assess: • Assess for nausea, vomiting, diarrhea, fever, headache, UTI • Assess for pain in hands, red spots, hearing loss, inability to sweat baseline and periodically
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Evaluate: • Therapeutic response: Decreasing symptoms of Fabry disease (less pain in hands/red spots/hearing loss)
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Patient and caregiver information: • To report adverse reactions to provider • How to take the product, every other day
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PHARMACOKINETICS Onset and duration unknown, peak 3 hr
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