Elfabrio (pegunigalsidase alfa-iwxj)

If a severe hypersensitivity or infusion-associated reaction occurs, immediately discontinue pegunigalsidase alfa administration and initiate appropriate medical treatment.

If a mild to moderate hypersensitivity or infusion-associated reaction occurs, consider temporarily holding the infusion for 15 to 30 minutes or slowing the infusion rate by 25% to 50%. If symptoms persist despite holding or slowing the infusion, stop the infusion and monitor the patient. Consider re-initiating the infusion in 7 to 14 days at 25% to 50% of the rate at which the reaction occurred with appropriate pretreatment. If symptoms subside after holding the infusion, resume infusion at a 25% to 50% reduced rate as tolerated. Alternatively, if symptoms subside after slowing the infusion, complete the infusion at the reduced rate as tolerated. With the next infusion, increase the infusion rate by increments of 25% every third infusion as tolerated until the infusion rate at which the reaction occurred is reached.

Administer pegunigalsidase alfa-iwxj by IV infusion.

Pegunigalsidase alfa-iwxj is available as a 20 mg/10 mL or 5 mg/2.5 mL clear and colorless solution in a single-dose vial. The commercially available injection must be further diluted with 0.9% sodium chloride injection prior to IV infusion.

Store vials of pegunigalsidase alfa-iwxj refrigerated at 2-8degreesC.

Use an in-line, low protein-binding, 0.2 micron filter during administration.

Once the infusion is complete, flush the line with 0.9% sodium chloride injection using the same infusion rate used for the last part of the pegunigalsidase alfa infusion.

Do not infuse pegunigalsidase alfa-iwxj in the same IV line with other medications.

Home infusion of pegunigalsidase alfa may be considered in patients who have reached an infusion duration that is well tolerated; the decision to transition a patient to home treatment should be made after evaluation and recommendation by a healthcare provider. Home treatment should continue under the supervision of a healthcare provider. The infusion duration should remain constant during home administration and should only be changed in a healthcare facility. If a dose is missed or delayed, a healthcare provider should be contacted.

No dosing information is available for this tab.

No formal drug interaction studies with pegunigalsidase alfa have been performed to date.

There are no contraindication interactions for this drug.

There are no severe interactions for this drug.

There are no moderate interactions for this drug.

*None.

There are no contraindication contraindications for this drug.

There are no severe contraindications for this drug.

• No disease contraindications

The most common adverse reactions (>=15%) reported with pegunigalsidase alfa-iwxj in clinical studies are infusion-associated reactions, nasopharyngitis, headache, diarrhea, fatigue, nausea, back pain, pain in extremity, and sinusitis.

Less Frequent

• Extravasation injury
• Hypersensitivity drug reaction

Rare or Very Rare

• Anaphylaxis
• Glomerulonephritis

More Frequent

• Back pain
• Diarrhea
• Fatigue
• Headache disorder
• Nausea
• Pain in extremities
• Pharyngitis
• Sinusitis

Less Frequent

• Acute abdominal pain
• Chest pain
• Chills
• Dizziness
• Dyspnea
• Flushing
• General weakness
• Hematuria
• Nasal congestion
• Neuralgia
• Pain
• Paresthesia
• Peripheral neuropathy
• Proteinuria
• Pruritus of skin
• Sciatica
• Tremor
• Vomiting

No enhanced messaging available.

Safety and efficacy of pegunigalsidase alfa have not been established in pediatric patients.

There are no available data on pegunigalsidase alfa use in pregnant women to evaluate for a drug-associated risk of major birth defects, miscarriage, or adverse maternal or fetal outcomes. However, pegunigalsidase alfa, as an enzyme replacement, is not expected to cause adverse outcomes.

In animal studies, no adverse effects on embryofetal development were observed in pregnant rats who were exposed to 3.6 times the maximum recommended human dose (MRHD). In pregnant rabbits, maternal toxicity was observed at doses that were >=3.2 times the MRHD.

There is a pregnancy safety study for pegunigalsidase alfa. If a patient becomes pregnant while receiving pegunigalsidase alfa, healthcare providers should report pegunigalsidase alfa exposure by calling 1-888-661-9260 or visiting https://chiesirarediseases.com/contact-us/.

It is not known whether pegunigalsidase alfa is distributed into human milk. Effects of pegunigalsidase alfa on breast-fed infants or milk production are not known. Consider the developmental and health benefits of breastfeeding along with the mother's clinical need for pegunigalsidase alfa and any potential adverse effects on the breastfed infant from the drug or from the underlying maternal condition.

The manufacturer makes no recommendations regarding dosage adjustment for patients >=65 years of age.

There were no patients >=65 years of age in clinical trials. It is not known whether geriatric patients >=65 years of age respond differently to pegunigalsidase alfa compared to younger patients.