Agalsidase beta

Name: Agalsidase beta

Warnings

Included as part of the PRECAUTIONS section.

What is the most important information I should know about agalsidase beta?

Many people have a severe reaction to agalsidase beta. Tell your caregiver right away if you have a side effect such as: skin rash or hives, fever, chills, headache, muscle pain, dizziness, numbness, swelling, vomiting, diarrhea, chest pain, trouble swallowing or breathing, fast or slow heart rate, or feeling light-headed.

What happens if I miss a dose?

Call your doctor for instructions if you miss an appointment for your agalsidase beta injection.

Agalsidase beta side effects

Get emergency medical help if you have any of these signs of an allergic reaction:

  • skin rash, hives, flushing (warmth, redness, or tingly feeling);

  • trouble swallowing, chest discomfort, difficult breathing, feeling light-headed; or

  • swelling of your face, lips, tongue, or throat.

Many people have a severe reaction to the agalsidase beta infusion (side effects that occur during the injection). Tell your caregiver right away if you have any of these signs of an infusion reaction:

  • fever, headache, chills, stuffy nose, muscle pain, back pain, dizziness, drowsiness, tired feeling;

  • pale skin, feeling hot or cold, itching, numbness or tingly feeling, swelling in your hands or feet;

  • nausea, vomiting, tight feeling in your throat, stomach pain, diarrhea;

  • chest pain, fast or slow heart rate, feeling short of breath; or

  • a light-headed feeling, like you might pass out.

This is not a complete list of side effects and others may occur. Call your doctor for medical advice about side effects. You may report side effects to FDA at 1-800-FDA-1088.

Agalsidase Beta Dosage and Administration

General

  • Administer at a medical facility or under close clinician supervision for >6 months of therapy.5 If infusions are tolerated after that time, some clinicians suggest that agalsidase beta may be administered in a home-care setting.5

  • Administer only under supervision of qualified clinicians and with appropriate medical support available to patients who have developed a positive skin test or IgE antibodies.a (See Immunologic Reactions and Antibody Formation under Cautions.)

  • To minimize the risk of infusion-related reactions, administer antipyretics prior to each infusion. 1

  • If signs or symptoms of an infusion reaction occur, decrease infusion rate or interrupt infusion.1 (See Infusion Reactions under Cautions.)

Administration

IV Administration

For solution and drug compatibility information, see Compatibility under Stability.

Administer by IV infusion.1

May be administered through a low-protein-binding 0.2-mcm filter. 1

Do not infuse in the same IV line with other drugs. 1

Use strict aseptic technique since drug product contains no preservative.a

Reconstitution

Allow vials of lyophilized agalsidase beta and diluent to reach room temperature before reconstitution (approximately 30 minutes). 1

Determine number of 35- and 5-mg vials needed to achieve the proper dosage based on patient’s body weight in kg.1

Reconstitute vial containing 35 or 5 mg of agalsidase beta powder with 7.2 or 1.1 mL, respectively, of sterile water for injection to provide a solution containing 5 mg of agalsidase beta per mL.1 Total extractable amount per 35 or 5 mg vial is 7 or 1 mL, respectively. 1

Direct the diluent slowly toward the side of the vial; gently roll and tilt vial until lyophilized powder dissolves.1 Do not shake or agitate.1

Must be diluted further before IV administration.1

Dilution

Remove the volume of diluent equal to the total required volume of reconstituted agalsidase beta solution (i.e., patient dose divided by 5 mg/mL) from an infusion bag of 0.9% sodium chloride.a

Slowly add reconstituted agalsidase beta solution to the bag to a total volume based on patient weight; inject the agalsidase beta solution directly into sodium chloride solution, not into the airspace within the infusion bag.a (See Table 1.)

Gently invert infusion bag to mix solution; do not agitate or shake vigorously.1

Table 1. Dilution of Agalsidase Beta Solutions

Patient Weight (kg)

Minimum Total Volume (mL)

≤35

50

35.1–70

100

70.1–100

250

>100

500

Rate of Administration

Infuse at an initial rate ≤0.25 mg/minute (15 mg/hr).1

If infusion reactions occur, slow infusion rate.1 (See Infusion Reactions under Cautions.)

Patients weighing ≥30 kg: Once patient tolerance is well established, infusion rate may be increased in increments of 0.05–0.08 mg/minute (3–5 mg/hr) with each subsequent infusion.1 Duration of infusion should be ≥1.5 hours.a

Dosage

Dosage of agalsidase beta is expressed in mg.1 The specific activity of agalsidase beta is approximately 70 units/mg, with 1 unit defined as the amount of activity that results in hydrolysis of 1 mcmol of a synthetic substrate, p-nitrophenyl-α-d-galactopyranoside, per minute under the assay conditions.1

Pediatric Patients

Fabry’s Disease IV

Children weighing <30 kg: 1 mg/kg infused at a rate ≤0.25 mg/minute (15 mg/hr) every 2 weeks.a

Children weighing ≥30 kg: 1 mg/kg every 2 weeks.a Infuse initially at a rate ≤0.25 mg/minute; infusion rate may be increased gradually after patient tolerance is well established.a (See Rate of Administration under Dosage and Administration.)

Rechallenge Protocol in Patients Developing Positive Skin Test or IgE Antibodies IV

Initially, 0.5 mg/kg infused at a rate of 0.01 mg/minute.a Once patient tolerates infusion, gradually titrate dose and infusion rate upward to 1 mg/kg at a maximum rate of 0.25 mg/minute.a

Adults

Fabry’s Disease IV

1 mg/kg every 2 weeks. 1

Rechallenge Protocol in Patients Developing Positive Skin Test or IgE Antibodies IV

Initially, 0.5 mg/kg infused at a rate of 0.01 mg/minute.a Once patient tolerates infusion, gradually titrate dose and infusion rate upward to 1 mg/kg at a maximum rate of 0.25 mg/minute.a

How do I store and/or throw out Agalsidase Beta?

  • If you need to store agalsidase beta at home, talk with your doctor, nurse, or pharmacist about how to store it.

Consumer Information Use and Disclaimer

  • If your symptoms or health problems do not get better or if they become worse, call your doctor.
  • Do not share your drugs with others and do not take anyone else's drugs.
  • Keep a list of all your drugs (prescription, natural products, vitamins, OTC) with you. Give this list to your doctor.
  • Talk with the doctor before starting any new drug, including prescription or OTC, natural products, or vitamins.
  • Keep all drugs in a safe place. Keep all drugs out of the reach of children and pets.
  • Check with your pharmacist about how to throw out unused drugs.
  • Some drugs may have another patient information leaflet. Check with your pharmacist. If you have any questions about this medicine, please talk with your doctor, nurse, pharmacist, or other health care provider.
  • If you think there has been an overdose, call your poison control center or get medical care right away. Be ready to tell or show what was taken, how much, and when it happened.

This information should not be used to decide whether or not to take agalsidase beta or any other medicine. Only the healthcare provider has the knowledge and training to decide which medicines are right for a specific patient. This information does not endorse any medicine as safe, effective, or approved for treating any patient or health condition. This is only a brief summary of general information about this medicine. It does NOT include all information about the possible uses, directions, warnings, precautions, interactions, adverse effects, or risks that may apply to agalsidase beta. This information is not specific medical advice and does not replace information you receive from the healthcare provider. You must talk with the healthcare provider for complete information about the risks and benefits of using this medicine.

Review Date: October 4, 2017

Use Labeled Indications

Fabry disease: For use in patients with Fabry disease. Agalsidase beta reduces globotriaosylceramide (GL-3) deposition in capillary endothelium of the kidney and certain other cell types.

Contraindications

There are no contraindications listed within the manufacturer's labeling.

Dosing Pediatric

Fabry disease: Children ≥8 years and Adolescents: IV: Refer to adult dosing

Storage

Store intact vials between 2°C and 8°C (36°F and 46°F). Reconstituted solutions and solutions diluted in NS are stable for 24 hours at 2°C and 8°C (36°F and 46°F).

Drug Interactions

Amiodarone: May diminish the therapeutic effect of Agalsidase Beta. Avoid combination

Chloroquine: May diminish the therapeutic effect of Agalsidase Beta. Avoid combination

Gentamicin (Systemic): May diminish the therapeutic effect of Agalsidase Beta. Avoid combination

Warnings

Contraindications

Hypersensitivity to product or components

Cautions

Moderate-to-severe HTN, renal impairment, febrile patients, patients with prior sensitivity to agalsidase products

Severe reactions occur may occur despite premedication; decrease infusion rate or temporarily discontinue infusion and/or administer additional antihistamines, antipyretics, and/or steroids to manage infusion reactions; in severe reactions may consider immediate discontinuation of infusion

Development of IgG antibodies reported within 3 months from the onset of therapy; some may also develop IgE antibodies

Premedicate with antipyretics 1 hr before infusion

Use caution in patients with cardiovascular diseases

Fabry Disease Registry has been created to monitor therapeutic responses and adverse effects; www.fabryregistry.com or 1-800-745-4447

Patient Handout

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Usual Pediatric Dose for Fabry Disease

8 years or older: 1 mg/kg IV every 2 weeks

Comments:
-Initial infusion rate should not exceed 0.25 mg/min (15 mg/hr).

Use: For treatment of patients with Fabry disease

Dose Adjustments

Data not available

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