avalglucosidase alfa

Contraindicated in:

None.

Use Cautiously in:

Acute underlying illness (↑ risk of infusion reactions);
At risk for volume overload, acute underlying respiratory illness, or compromised cardiac or respiratory function (↑ risk for worsening cardiac or respiratory status);
OB: Use during pregnancy only if potential maternal benefit justifies potential fetal risk;
Lactation: Use while breastfeeding only if potential maternal benefit justifies potential risk to infant;
Pedi: Children <1 yr (safety and effectiveness not established).

Adv. Reactions/Side Effects ⬆ ⬇

CV: hypertension, hypotension

Derm: erythema, flushing, pruritus, rash, urticaria

GI: diarrhea, nausea, abdominal pain, vomiting

MS: arthralgia, myalgia

Neuro: dizziness, fatigue, headache, paresthesia

Resp: dyspnea

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

Interactions ⬆ ⬇

Drug-drug:

None reported.

Availability ⬆ ⬇

Lyophilized powder for injection: 100 mg/vial

Route/Dosage ⬆ ⬇

IV (Adults and Children ≥1 yr and ≥30 kg): 20 mg/kg (actual body weight) every 2 wk.
IV (Adults and Children ≥1 yr and <30 kg): 40 mg/kg (actual body weight) every 2 wk.

US Brand Names ⬆ ⬇

Action ⬆ ⬇

Replaces alpha-glucosidase. Without this enzyme, glycogen accumulates in tissues including cardiac and skeletal muscles and hepatic tissues, leading to the development of cardiomyopathy, progressive muscle weakness, and impairment of respiratory function.

Therapeutic effects:

Improved lung function and exercise capacity.

Classifications ⬆ ⬇

Therapeutic Classification: replacement enzyme

Pharmacokinetics ⬆ ⬇

Absorption: IV administration results in complete bioavailability.

Distribution: Not widely distributed to tissues.

Metabolism/Excretion: Metabolized into small peptides by catabolic pathways. Excretion pathway unknown.

Half-Life: 1.6 hr.

Time/Action Profile ⬆ ⬇

(plasma concentrations)

ROUTE	ONSET	PEAK	DURATION
IV	unknown	end of infusion	unknown

Patient/Family Teaching ⬆ ⬇

Explain purpose and side effects of avalglucosidase alfa and need for close monitoring to patient and caregiver.
If one or more doses are missed, restart treatment as soon as possible, maintaining a 2-wk interval between infusions.
Advise patient to notify health care professional immediately if signs and symptoms of hypersensitivity reactions or cardiopulmonary failure occur during or after treatment.
Encourage enrollment in the Pompe Registry to better understand the disease and monitor the effects of avalglucosidase alfa. Direct patient and caregiver towww.registrynxt.com or call 1-800-745-4447, ext. 15500.
Rep: Advise patients who are pregnant or breastfeeding to notify a health care professional and report avalglucosidase alfa exposure by calling 1-800-633-1610, option 1.

section name header

Indications ⬇

Contraind./Precautions ⬆ ⬇