avalglucosidase alfa

ROUTE	ONSET	PEAK	DURATION
IV	unknown	end of infusion	unknown

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.

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.

Availability ⬆ ⬇

Lyophilized powder for injection: 100 mg/vial

Assessment ⬆ ⬇

Monitor for hypersensitivity reactions, including anaphylaxis and infusion-related reaction (dyspnea, erythema, generalized edema, hyper- or hypotension, hypoxia, rash, urticaria, dysphagia, flushing, dizziness, nausea, ocular hyperemia).If severe reactions occur,discontinue avalglucosidase alfa immediately and initiate appropriate medical treatment. If mild to moderate reactions occur,consider temporarily holding the infusion for 30 min or slowing the infusion rate by 50% and initiating medical treatment as needed. If symptoms persist for >30 min despite holding or slowing rate,stop the infusion. Consider reinitiating infusion on the same day when symptoms subside, at 50% of rate at which reaction occurred and with appropriate pretreatment. If symptoms subside after holding the infusion,resume infusion at 50% of the rate at which the reaction occurred, increasing the rate every 15–30 min by 50% as tolerated. Alternatively, if symptoms subside after slowing the infusion,complete the infusion at the reduced rate. Starting with the next infusion, increase the rate until it reaches the rate at which the reaction occurred. Consider increasing the rate in a stepwise manner until reaching the recommended infusion rate. Monitor patient closely.

Implementation ⬆ ⬇

IV Administration:

Administer antihistamine, antipyretics, and/or corticosteroids prior to infusion, if required, to prevent hypersensitivity reactions.
Administer in a health care setting with appropriate medical monitoring and supportive measures (cardiopulmonary resuscitation equipment) available during administration.
If a severe hypersensitivity reaction (e.g., anaphylaxis) occurs, discontinue immediately and initiate appropriate medical treatment (epinephrine). Consider the risks and benefits of readministering following severe hypersensitivity reactions.
Intermittent Infusion: Determine the number of vials to be reconstituted based on body weight in kg and recommended dose, rounding up to the next whole number. Administer incrementally, as per patient tolerance. On completion of infusion, flush line with D5W.Reconstitution: Allow vials to reach room temperature for 30 min prior to reconstitution if refrigerated. Reconstitute by injecting 10 mL of sterile water for injection to inside wall of each vial. Avoid forceful impact of water on powder to avoid foaming. Tilt and roll each vial gently. Do not invert, swirl, or shake. Allow solution to dissolve. Use immediately after reconstitution or refrigerate up to 24 hr. Do not freeze. Concentration: 10 mg/mL.Diluent: Add the reconstituted avalglucosidase alfa solution to appropriate volume of D5W. Discard any unused diluted solution after 9 hr. Concentration: 0.5–4 mg/mL.
Rate: Administer using an infusion pump and 0.2-micrometer low-protein-binding in-line filter. Recommended dose of 20 mg/kg: Administer initial and subsequent infusions at 1 mg/kg/hr. As tolerated, ↑ rate by 2 mg/kg/hr every 30 min until a max rate of 7 mg/kg/hr is reached. Maintain max rate until infusion is complete, approximately 4–5 hr. Recommended dose of 40 mg/kg: Administer initial infusion at 1 mg/kg/hr. As tolerated, ↑ rate by 2 mg/kg/hr every 30 min until a max rate of 7 mg/kg/hr is reached. Maintain max rate until infusion is complete, approximately 7 hr. For subsequent infusions, the initial process may be used, or the infusion time may be ↓ by using the following 5-hr process. Initiate infusion at 1 mg/kg/hr. As tolerated, ↑ rate every 30 min as follows: 3 mg/kg/hr, 6 mg/kg/hr, 8 mg/kg/hr, then a max of 10 mg/kg/hr. Maintain max rate until infusion is complete.
Y-Site Incompatibility: Do not infuse with other products.

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.

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Pronunciation ⬇

Classifications ⬆ ⬇

Indications ⬆ ⬇

Action ⬆ ⬇

Pharmacokinetics ⬆ ⬇

Time/Action Profile ⬆ ⬇

Contraind./Precautions ⬆ ⬇