Pegunigalsidase Alfa Dosage

Medically reviewed by Drugs.com. Last updated on Nov 9, 2023.

Usual Adult Dose for Fabry Disease

RECOMMENDED DOSING: 1 mg/kg (based on actual body weight) via IV infusion every 2 weeks

PRE-TREATMENT RECOMMENDATIONS:

• In enzyme replacement therapy (ERT)-experienced patients who pre-treated with antihistamines, antipyretics, and/or corticosteroids during prior ERT, consider a similar pre-treatment strategy before the first several infusions of this drug.
• After 4 to 6 well-tolerated infusions in ERT-experienced patients, consider a stepwise decrease in pre-treatment medication dose(s) and/or discontinuation.
• In ERT-naive patients, consider pre-treating with antihistamines, antipyretics, and/or corticosteroids prior to infusing this drug.

RECOMMENDED INFUSION RATES FOR INITIAL 4 TO 6 INFUSIONS:
ERT-Experienced Patients:

• Actual body weight less than 70 kg: 0.83 mL/min (50 mL/hr); total infusion volume: 150 mL
• Actual body weight 70 to 100 kg: 1.39 mL/min (83 mL/hr); total infusion volume: 250 mL
• Actual body weight greater than 100 kg: 2.78 mL/min (167 mL/hr); total infusion volume: 500 mL
• If infusion rate during prior ERT was greater than 3 hours: Maintain same rate of infusion with this drug.
• Consider decreasing duration of every third infusion by 30 minute decrements after first 4 to 6 doses.
• The minimum maintenance infusion duration is 1.5 hours.

ERT-Naive Patients:

• Actual body weight less than 70 kg: 0.63 mL/min (37.5 mL/hr); total infusion volume: 150 mL
• Actual body weight 70 to 100 kg: 1 mL/min (60 mL/hr); total infusion volume: 250 mL
• Actual body weight greater than 100 kg: 1.38 mL/min (83 mL/hr); total infusion volume: 500 mL

Comments:

• Appropriate medical support measures, including cardiopulmonary resuscitation equipment, should be readily available during administration of this drug.
• Home infusion under the supervision of a healthcare provider may be considered for patients who have reached an infusion duration that is well tolerated and constant.
• Infusion rate decreases should only be made in a healthcare facility setting.

Use: For the treatment of adults with confirmed Fabry disease

Renal Dose Adjustments

Data not available

Liver Dose Adjustments

Data not available

Precautions

US BOXED WARNING: Patients treated with this drug have experienced hypersensitivity reactions, including anaphylaxis. Appropriate medical support measures, including cardiopulmonary resuscitation equipment, should be readily available during administration of this drug. If a severe hypersensitivity reaction (e.g., anaphylaxis) occurs, discontinue this drug immediately and initiate appropriate medical treatment. In patients with severe hypersensitivity reaction, a desensitization procedure to this drug may be considered.

CONTRAINDICATIONS: None

Safety and efficacy have not been established in patients younger than 18 years.

Consult WARNINGS section for additional precautions.

Dialysis

Data not available

Other Comments

Administration advice:

• The manufacturer product information should be consulted prior to product administration.
• This drug should be given by IV infusion only.
• Use the recommended infusion rates based on actual body weight (ABW) for ERT-experienced or ERT-naive patients during the first 4 to 6 doses.
• Do not administer with other products in infusion tubing.
• After 4 to 6 well-tolerated infusions, the duration of every third infusion can be decreased by decrements of 30 minutes as tolerated.
• At infusion end, flush line with 0.9% sodium chloride using the same infusion rate used during the last part drug infusion.

Administration modifications for hypersensitivity or infusion-associated reactions (IARs):
FOR MILD OR MODERATE HYPERSENSITIVITY OR IARs:

• Consider temporarily holding the infusion for 15 to 30 minutes or slowing the infusion rate by 25% to 50%, and initiate appropriate medical treatment as required.
• If symptoms persist, stop infusion and monitor the patient. Consider re-initiating treatment again in 7-14 days at 25% to 50% of the infusion rate at which the reaction occurred; utilize appropriate pre-treatment.
• If symptoms subside after holding infusion, resume infusion at a 25% to 50% reduced rate as tolerated.
• If symptoms subside after slowing the infusion, complete infusion at the reduced rate as tolerated.
• Starting with next infusion, increase the rate by increments of 25% every third infusion as tolerated until the rate at which the original reaction occurred is reached; closely monitor patient.

FOR SEVERE HYPERSENSITIVITY (e.g., ANAPHYLAXIS) OR IARs:

• Discontinue this drug immediately and initiate appropriate medical treatment.
• Carefully consider the risks and benefits of re-administering this drug after severe reactions.
• Patients may be rechallenged using slower infusion rates and/or desensitization measures; once a patient tolerates infusion, rate may be increased to reach to recommended rate.
• Consult Warnings section for additional information on re-administration.

Missed dosing advice:

• If one or more doses are missed, restart treatment with this drug as soon as possible while maintaining the 2 week interval between infusions thereafter.
• Do not double a dose to compensate for a missed dose.
• In the case of a missed or delayed dose during home infusion, contact healthcare provider prior to next administration.

Storage requirements:

• Do not freeze or shake this drug.
• Store single-dose vials under refrigeration at 2C to 8C (36F to 46F).
• Diluted solution can be stored at room temperature between 20C to 25C (38 F to 77F) for up to 8 hours (inclusive of infusion time); discard if not used.
• Diluted solution can be refrigerated between 2C to 8C (36F to 46F) for up to 24 hours.
• Once diluted solution is removed from refrigeration, infuse within 8 hours (inclusive of infusion time); discard if not used.

Preparation techniques:

• The manufacturer product information should be consulted prior to product preparation.
• Allow vials to come to room temperature before use; do not use external heat source.
• Visually inspect vials for discoloration or particulate matter; discard if not clear or colorless.
• This drug must be diluted in 0.9% sodium chloride using a total infusion volume based on ABW as per dosing recommendations.
• Before dilution, remove from infusion bag a volume of 0.9% sodium chloride equal to the dosage volume of this drug.
• After injecting drug volume directly into diluent, gently invert infusion bag to mix; avoid vigorous agitation.

IV compatibility: Do not infuse this drug in the same intravenous line with other products.

Monitoring:

• Closely monitor patients during infusions for signs and symptoms of hypersensitivity or infusion-associated reactions.

Patient advice:

• Advise patients that reactions related to the infusion may occur, including anaphylaxis, or other serious hypersensitivity or infusion-associated reactions.
• Inform patients who are exposed to this drug during pregnancy that there is a Pregnancy Safety Study which monitors pregnancy outcomes.

See also:

Further information

Always consult your healthcare provider to ensure the information displayed on this page applies to your personal circumstances.

Medical Disclaimer