Fabrazyme® (agalsidase beta) safety profile

Summary of safety information

Please refer to the Summary of Product Characteristics before prescribing.

Dosage and administration¹

Treatment with Fabrazyme^® should be supervised by a physician experienced in the management of patients with Fabry disease or other inherited metabolic diseases.

The recommended dose of Fabrazyme^® is 1mg/kg body weight administered once every 2 weeks as an intravenous infusion.

Administration

Intravenous infusion

Initial IV infusion rate: no more than 0.25 mg/min (15 mg/h)

After patient tolerance is well established, the infusion rate may be increased in increments of 0.05 to 0.083 mg/min (3 to 5 mg/hr) with each subsequent infusion. In the event of infusion-associated reactions, the infusion rate may be slowed.

Contraindications¹

Life threatening hypersensitivity to agalsidase beta or to any of the excipients of this medicine (listed in section 6.1 of the Summary of Product Characteristics).

Fabrazyme^® should not be administered with chloroquine, amiodarone, benoquin or gentamycin due to a theoretical risk of inhibition of intra-cellular α-galactosidase A activity.

Precautions and Warnings¹

Immunogenicity

As agalsidase beta is a recombinant protein, the development of IgG antibodies is anticipated in patients with little or no residual enzyme activity. The majority of patients developed IgG antibodies to agalsidase beta, typically within 3 months of the first Fabrazyme^® infusion.

Infusion associated reactions

Patients with antibodies to agalsidase beta have a higher risk of experiencing infusion-associated reactions, defined as any adverse event occurring on the infusion day. These patients should be treated with caution when re-administering agalsidase beta to these patients, and their antibody status should be regularly monitored.

Hypersensitivity

A small number of patients have experienced reactions suggestive of immediate (Type I) hypersensitivity. As with any intravenous protein medicinal product, allergic-type hypersensitivity reactions are possible. If severe allergic or anaphylactic-type reactions occur, immediate discontinuation of the administration of Fabrazyme^® should be considered and appropriate treatment initiated.

Patients with advanced renal disease

The effect of Fabrazyme^® treatment on the kidneys may be limited in patients with advanced renal disease.

Sodium

This medicinal product contains less than 1 mmol sodium per vial, that is to say essentially 'sodium-free'.

Traceability

To enhance traceability, it is recommended to clearly record the name and batch number of the administered product

Adverse reactions¹

Adverse reactions are listed by system organ class and frequency in the list below. Within each frequency grouping, adverse reactions are presented in order of decreasing seriousness.¹

The list presents adverse reactions reported from clinical trials (168 patients [154 males, 14 females] treated with Fabrazyme^® 1 mg/kg EOW for ≥1 infusion up to a maximum of 5 years).¹ Frequencies (very common ≥ 1/10; common ≥ 1/100 to < 1/10 and uncommon ≥ 1/1000 to < 1/100). Adverse reactions reported in post-marketing period are indicated as frequency “not known” (cannot be estimated from data).¹ Adverse reactions were mostly mild or moderate in severity.¹ Adverse reaction terminology is based upon the Medical Dictionary for Regulatory Activities (MedDRA).¹

Did you know?

Fabry disease starts early in the kidney and is a silent threat. Progressive accumulation of globotriaocylceramide (GL-3) leads to cellular changes and histological damage.² Fabry nephropathy shows similarities to other proteinuric nephropathies of metabolic origin.³ Once the mechanisms leading to tissue injury are activated, the progression of Fabry nephropathy becomes irreversible.^3,4

Learn more about Fabrazyme^®

Resources

References

1. Fabrazyme^®. Summary of Product Characteristics 2024.

2. Waldek S and Ferriozi S. BMC Nephrol. 2014;15:72.

3. Ortiz A, et al. Mol Genet Metab. 2018;123(4):416–27.

4. Van der Veen SJ, et al. Mol Genet Metab. 2022;135(2):163–69