Pegunigalsidase Alfa

Pegunigalsidase Alfa is recombinant form of human α-galactosidase-A used for long-term enzyme replacement therapy in aduls with a confirmed diagnosis of Fabry disease.

• Pegunigalsidase Alfa is available under the following different brand names: Elfabrio, Pegunigalsidase Alfa-iwxj

Common side effects of Pegunigalsidase Alfa include:

• diarrhea
• nausea
• vomiting
• headache
• abdominal pain
• muscle pain
• fever
• tiredness
• pain at the injection site
• cold symptoms (such as runny/stuffy nose and cough)
• arthralgia (joint pain)
• Serious side effects of Pegunigalsidase Alfa include:
• infusion-associated reactions
• nasopharyngitis
• headache
• upper respiratory tract congestion
• neuralgia

Rare side effects of Pegunigalsidase Alfa include:

• none

Seek medical care or call 911 at once if you have the following serious side effects:

• Severe headache, confusion, slurred speech, arm or leg weakness, trouble walking, coordination loss, unsteady, very stiff muscles, high fever, profuse sweating, or tremors
• Serious eye symptoms such as sudden vision loss, blurred vision, tunnel vision, eye pain or swelling, or seeing halos around lights
• Serious heart symptoms include fast, irregular, or pounding heartbeats; fluttering in the chest; shortness of breath; sudden dizziness, light-headedness, or passing out

This is not a complete list of side effects and other serious side effects or health problems that may occur because of the use of this drug. Call your doctor for medical advice about serious side effects or adverse reactions. You may report side effects or health problems to FDA at 1-800-FDA-1088.

Adult dosage

Injectable solution

• 2 mg/mL (20 mg/10 mL single-dose vial)

Fabry disease

Adult dosage

• 1 mg/kg IV every 2 weeks
• Use actual body weight to calculate the dose

Pretreatment

Enzyme replacement therapy (ERT)-experienced patients

• If pretreatment with antihistamines, antipyretics, and/or corticosteroids was used before administering ERT, consider similar pretreatment with these medications before the first several Pegunigalsidase infusions
• If tolerated after 4-6 infusions, consider a stepwise decrease in pretreatment medication dose(s) and/or discontinuing
• ERT-naïve patients
  • Consider administering antihistamines, antipyretics, and/or corticosteroids before Pegunigalsidase Alfa infusion

Dosage Considerations – Should be Given as Follows:

• See “Dosages”

If your medical doctor is using this medicine to treat your pain, your doctor or pharmacist may already be aware of any possible drug interactions and may be monitoring you for them. Do not start, stop, or change the dosage of any medicine before checking with your doctor, healthcare provider, or pharmacist first.

• Pegunigalsidase Alfa has no noted severe interactions with any other drugs.
• Pegunigalsidase Alfa has no noted serious interactions with any other drugs.
• Pegunigalsidase Alfa has no noted moderate interactions with any other drugs.
• Pegunigalsidase Alfa has no noted minor interactions with any other drugs.

This information does not contain all possible interactions or adverse effects. Visit the RxList Drug Interaction Checker for any drug interactions. Therefore, before using this product, tell your doctor or pharmacist about all the products you use. Keep a list of all your medications with you and share this information with your doctor and pharmacist. Check with your healthcare professional or doctor for additional medical advice, health questions, or concerns.

Contraindications

• None

Effects of drug abuse

• None

Short-Term Effects

• See “What Are Side Effects Associated with Using Pegunigalsidase Alfa?”

Long-Term Effects

• See “What Are Side Effects Associated with Using Pegunigalsidase Alfa?”

Cautions

• Hypersensitivity
  • Hypersensitivity reactions, including anaphylaxis, were reported
• Consider pretreating with antihistamines, antipyretics, and/or corticosteroids
• Risk for hypersensitivity may be increased in certain patients with pre-existing anti-drug antibodies (ADA) from prior ERT; consider monitoring patients who demonstrate hypersensitivity reactions during treatment for the presence of IgG and IgE ADA
• Also, see Boxed Warning and Dosage Modifications
• Infusion-associated reactions (IARs)
  • IARs reported, including nausea, chills, pruritus, rash, chest pain, dizziness, vomiting, asthenia, pain, sneezing, dyspnea, nasal congestion, throat irritation, abdominal pain, erythema, diarrhea, burning sensation, neuralgia, headache, paresthesia, tremor, agitation, increased body temperature, flushing, bradycardia, myalgia, hypertension, and hypotension
  • Reactions may occur up to 24 hours after completing the infusion
• Consider pretreating with antihistamines, antipyretics, and/or corticosteroids
• Risk for hypersensitivity may be increased in certain patients with pre-existing ADA from prior ERT; consider monitoring
• Patients with advanced Fabry disease may have compromised cardiac function, which may predispose them to a higher risk for severe complications from IARs; closely monitor patients with compromised cardiac function if administered to these patients
• Also, see Dosage Modifications
• Membranoproliferative glomerulonephritis
  • A case of membranoproliferative glomerulonephritis with immune depositions in the kidney was reported during clinical trials
  • This event led to a decline in renal function that slowly improved upon discontinuation but did not return to baseline by the end of the clinical trial
  • Monitor serum creatinine and urinary protein to creatinine ratio
  • If glomerulonephritis is suspected, discontinue Pegunigalsidase Alfa until a diagnostic evaluation can be conducted

Pregnancy and Lactation

• There are no available data regarding use in pregnant women to evaluate a drug-associated risk for major birth defects, miscarriage, or other adverse maternal or fetal outcomes
• As an enzyme replacement, Pegunigalsidase Alfa is not expected to cause adverse outcomes during pregnancy
• Pregnancy safety study
  • There is a pregnancy safety study for Pegunigalsidase Alfa
  • If a patient becomes pregnant during treatment, report exposure by calling 1-888-661-9260 or visiting chiesirarediseases.com/contact-us/medical-information-form
• Lactation
  • Data are unavailable on the presence in either human or animal milk, effects on breastfed infants, or effects on milk production