pegunigalsidase alfa

Contraindicated in:

None.

Use Cautiously in:

Presence of antidrug antibodies (↑ risk of hypersensitivity and infusion-associated reactions and ↓ effectiveness);
Compromised cardiac function (↑ risk of severe complications from infusion-associated reactions);
OB: Safety not established in pregnancy;
Lactation: Safety not established in breastfeeding;
Pedi: Safety and effectiveness not established in children.

Adv. Reactions/Side Effects ⬆ ⬇

EENT: nasopharyngitis, sinusitis

GI: abdominal pain, diarrhea, nausea

GU: proteinuria, glomerulonephritis, hematuria

Local: extravasation

MS: pain

Neuro: fatigue, headache, peripheral neuropathy

Misc: hypersensitivity reactions (including anaphylaxis), infusion-associated reactions

Interactions ⬆ ⬇

Drug-drug:

None reported.

Availability ⬆ ⬇

Solution for injection: 2 mg/mL

Route/Dosage ⬆ ⬇

IV (Adults ): 1 mg/kg (actual body weight) every 2 wk.

US Brand Names ⬆ ⬇

Action ⬆ ⬇

Replaces lysosomal enzyme alpha-galactosidase A, which is deficient in Fabry disease. Without this enzyme, globotriaosylceramide accumulates in small blood vessels and tissues, leading to the development of heart disease, renal impairment, and cerebrovascular complications.

Therapeutic effects:

Reduction in number of globotriaosylceramide inclusions per kidney interstitial capillary.

Classifications ⬆ ⬇

Therapeutic Classification: replacement enzyme

Pharmacologic Classification: enzymes

Pharmacokinetics ⬆ ⬇

Absorption: IV administration results in complete bioavailability.

Distribution: Widely distributed to tissues.

Metabolism/Excretion: Catabolized into small peptides. Excretion pathway unknown.

Half-Life: 79–121 hr.

Time/Action Profile ⬆ ⬇

(plasma concentrations)

ROUTE	ONSET	PEAK	DURATION
IV	rapid	end of infusion	unknown

Patient/Family Teaching ⬆ ⬇

Explain purpose and side effects of medication to patient. Advise patient to read Patient Information before starting therapy. If ≥one dose is missed, administer dose as soon as possible and restart the 2-wk interval.
Advise patient to notify health care professional of all Rx or OTC medications, vitamins, or herbal products being taken and to consult health care professional before taking other medications.
Advise patient that hypersensitivity reactions (anaphylaxis) and infusion-related reactions can occur during the early course of therapy and after extended duration of therapy. If these reactions occur, seek immediate medical care.
Rep: Advise women of reproductive potential to notify health care professional if pregnancy is suspected or planned or if breastfeeding. Advise patients exposed to pegunigalsidase alfa during pregnancy about the pregnancy safety study for monitoring pregnancy outcomes. Encourage patient to report the pregnancy to Chiesi USA, Inc., at 1-888-661-9260 and https://chiesirarediseases.com/contact-us/medical-information-form.

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Indications ⬇

Contraind./Precautions ⬆ ⬇