Agalsidase Beta Dosage

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Usual Adult Dose for:

Usual Pediatric Dose for:

Additional dosage information:

Usual Adult Dose for Fabry Disease

1 mg/kg via IV infusion every 2 weeks

The initial IV infusion rate should not exceed 0.25 mg/min (15 mg/hr) and may be reduced if infusion-related reactions occur. After tolerance to the infusion is well established, the infusion rate may be increased in increments of 0.05 to 0.08 mg/min (3 to 5 mg/hr) with each subsequent infusion. The maximum infusion rate for patients weighing less than 30 kg should remain at 0.25 mg/min (15 mg/hr). The administration duration for patients weighing 30 kg or more should be at least 1.5 hours (based on individual patient tolerability).

Usual Pediatric Dose for Fabry Disease

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

The initial IV infusion rate should not exceed 0.25 mg/min (15 mg/hr) and may be reduced if infusion-related reactions occur. After tolerance to the infusion is well established, the infusion rate may be increased in increments of 0.05 to 0.08 mg/min (3 to 5 mg/hr) with each subsequent infusion. The maximum infusion rate for patients weighing less than 30 kg should remain at 0.25 mg/min (15 mg/hr). The administration duration for patients weighing 30 kg or more should be at least 1.5 hours (based on individual patient tolerability).

Renal Dose Adjustments

Data not available

Liver Dose Adjustments

Data not available

Dose Adjustments

For patients who have had a positive skin test to agalsidase beta or who have tested positive for anti-agalsidase beta IgE antibodies, the initial rechallenge should be 0.5 mg/kg via IV infusion every 2 weeks at an infusion rate of 0.01 mg/min. Once the infusion is tolerated, the dose may be increased to reach the approved dose of 1 mg/kg every 2 weeks and the infusion rate may be slowly titrated upwards (doubled every 30 minutes up to a maximum rate of 0.25 mg/min), as tolerated.

Precautions

Agalsidase beta should be discontinued at once and necessary emergency treatment initiated if anaphylactic or severe allergic reactions occur. Due to the potential for severe allergic reactions, appropriate medical support measures should be readily available during agalsidase beta administration. After an anaphylactic or severe allergic reaction, risks and benefits of readministering agalsidase beta should be considered. If the decision is made to readminister the drug, extreme caution is recommended, with appropriate medical support measures readily available.

Infusion reactions reported during the clinical study period were severe and included dyspnea, fever, rigors, chest tightness, abdominal pain, hypertension, hypotension, myalgia, pruritus, headache, chills, vomiting, and paresthesia. Other infusion reactions included pyrexia, feeling hot or cold, nausea, flushing, fatigue, pain in extremity, chest pain, throat tightness, nasal congestion, diarrhea, dizziness, tachycardia, peripheral edema, urticaria, bradycardia, and somnolence. Infusion reactions occurred in some patients after receiving antipyretics, antihistamines, and oral steroids. Infusion reactions declined in frequency with continued administration of agalsidase beta. However, infusion reactions may still occur despite extended duration of agalsidase beta therapy.

All patients should be given antipyretics prior to infusion. If an infusion reaction occurs, regardless of pretreatment, the following steps may ameliorate the symptoms: decreasing the infusion rate, temporarily stopping the infusion, and/or administration of additional antipyretics, antihistamines, and/or steroids. If severe infusion reactions occur, immediate discontinuation of agalsidase beta should be considered, and appropriate medical treatment should be started. Appropriate medical support measures should be readily available when administering agalsidase beta because of the potential for severe infusion reactions. Patients who have experienced infusion reactions should be treated with caution when readministering agalsidase beta.

Patients with advanced Fabry disease may have compromised cardiac function, which may predispose them to a higher risk of severe complications from infusion events. Patients with compromised cardiac function should be monitored closely.

Physicians should consider testing for IgE antibodies in patients with suspected allergic reactions and consider the benefits and risks of continued treatment in patient with anti-agalsidase beta IgE antibodies. Patients who have had a positive skin test to agalsidase beta or who have tested positive for agalsidase beta-specific IgE antibody have been rechallenged with agalsidase beta using a rechallenge protocol. Rechallenge should only occur under direct supervision of qualified personnel, with appropriate medical support measures readily available.

Clinical trials of agalsidase beta did not include sufficient numbers of patients aged 65 and over to determine whether they respond differently from younger patients.

Safety and effectiveness have not been established in pediatric patients less than 8 years of age.

Dialysis

Data not available

Other Comments

Patients should receive antipyretics prior to infusion.

Agalsidase beta should be administered using an inline low protein binding 0.2 micron filter.

If testing for antibodies against agalsidase beta is needed, contact Genzyme Corporation at 1-800-745-4447 (USA).

A registry has been established to monitor and evaluate the long-term treatment effects of agalsidase beta, and to monitor the effect of the drug on pregnant women and their children. For more information, visit www.fabryregistry.com or call 1-800-745-4447 (USA).

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