"The U.S. Food and Drug Administration today approved Vimizim (elosulfase alfa), the first FDA-approved treatment for Mucopolysaccharidosis Type IVA (Morquio A syndrome). Morquio A syndrome is a rare, autosomal recessive lysosomal storage disease "...
Severe hypersensitivity reactions may occur. Epinephrine should be immediately available for treatment of acute severe hypersensitivity reaction (see PATIENT INFORMATION). The signs and symptoms of hypersensitivity reactions may include hives, generalized urticaria, tightness of the chest, wheezing, hypotension, and/or anaphylaxis during or after injection of Berinert.
Because hypersensitivity reactions may have symptoms similar to HAE attacks, treatment methods should be carefully considered. In case of suspected hypersensitivity, immediately discontinue administration of Berinert and institute appropriate treatment.
Thrombotic events have been reported in association with Berinert when used off-label and at higher than labeled doses.1 Animal studies have confirmed the risk of thrombosis from intravenous administration of C1 esterase inhibitor products2 (see OVERDOSAGE and Animal Toxicology and/or Pharmacology).
Transmission of Infectious Agents
Because Berinert is made from human blood, it may contain infectious agents (eg, viruses and, theoretically, the Creutzfeldt-Jakob disease [CJD] agent) that can cause disease. The risk that such products will transmit an infectious agent has been reduced by screening plasma donors for prior exposure to certain viruses, by testing for the presence of certain current virus infections, and by processes demonstrated to inactivate and/or remove certain viruses during manufacturing (see DESCRIPTION and PATIENT INFORMATION). Despite these measures, such products may still potentially transmit disease. There is also the possibility that unknown infectious agents may be present in such products. Since 1979, a few suspected cases of viral transmission have been reported with the use of Berinert outside the US, including cases of acute hepatitis C. From the incomplete information available from these cases, it was not possible to determine with certainty if the infections were or were not related to prior administration of Berinert. With the introduction of the pasteurization step (heat treatment in aqueous solution at 60°C for 10 hours) in 1985, case reports on suspected transmission of viruses have not demonstrated a causal relationship to the administration of Berinert.
The physician should discuss the risks and benefits of this product with the patient before prescribing or administering it to the patient. (See PATIENT INFORMATION). All infections thought by a physician possibly to have been transmitted by Berinert should be reported by lot number, by the physician, or other healthcare provider to the CSL Behring Pharmacovigilance Department at 1-866-915-6958.
Patient Counseling Information
Inform patients to immediately report the following to their physician
- Signs and symptoms of allergic hypersensitivity reactions, such as hives, urticaria, tightness of the chest, wheezing, hypotension and/or anaphylaxis experienced during or after injection of Berinert (see WARNINGS AND PRECAUTIONS/Hypersensitivity)
- Signs and symptoms of thrombosis, such as new onset swelling and pain in the limbs or abdomen, new onset chest pain, shortness of breath, loss of sensation or motor power, or altered consciousness, vision, or speech (see WARNINGS AND PRECAUTIONS/ Thrombotic Events)
Advise female patients to notify their physician if they become pregnant or intend to become pregnant during the treatment of acute abdominal or facial attacks of HAE with Berinert. Advise patients to notify their physician if they are breastfeeding or plan to breastfeed. Advise patients to consult with their healthcare professional prior to travel. Advise patients that, because Berinert is made from human blood, it may carry a risk of transmitting infectious agents, eg, viruses, and, theoretically, the Creutzfeldt-Jakob (CJD) agent (see WARNINGS AND PRECAUTIONS/Transmission of Infectious Agents and DESCRIPTION). Inform patients of the risks and benefits of Berinert before prescribing or administering it to the patient.
Carcinogenesis, Mutagenesis, Impairment of Fertility
No animal studies have been completed to evaluate the effects of Berinert on carcinogenesis, mutagenesis, and impairment of fertility.
Use In Specific Populations
Pregnancy Category C. Animal reproduction studies have not been conducted with Berinert. It is not known whether Berinert can cause fetal harm when administered to a pregnant woman or can affect reproduction capacity. Berinert should be given to a pregnant woman only if clearly needed. In a retrospective case collection study, 20 pregnant women ranging in age from 20 to 35 years received Berinert with repeated doses up to 3,500 units per attack; these women reported no complications during delivery and no harmful effects on their 34 neonates.
Labor and Delivery
The safety and effectiveness of Berinert administration prior to or during labor and delivery have not been established. Use only if clearly needed.
It is not known whether Berinert is excreted in human milk. Because many drugs are excreted in human milk, use only if clearly needed when treating a nursing woman.
Safety and efficacy of Berinert in children (ages 0 through 12) have not been established. The clinical studies included an insufficient number of subjects in this age group to determine whether they respond differently from older subjects. The safety and efficacy of Berinert were evaluated in 5 children (ages 3 through 12) and in 8 adolescent subjects (ages 13 through 16) (see Pharmacokinetics).
Safety and efficacy of Berinert in the geriatric population have not been established. Clinical studies with Berinert included four subjects older than 65 years. The clinical studies included an insufficient number of subjects in this age group to determine whether they respond differently from younger subjects.
Last reviewed on RxList: 9/15/2011
This monograph has been modified to include the generic and brand name in many instances.
Additional Berinert Information
Report Problems to the Food and Drug Administration
You are encouraged to report negative side effects of prescription drugs to the FDA. Visit the FDA MedWatch website or call 1-800-FDA-1088.
Find out what women really need.