Indication CINRYZE® (C1 esterase inhibitor [human]) is indicated for routine prophylaxis against angioedema attacks in adults, adolescents and pediatric patients (6 years of age and older) with Hereditary Angioedema (HAE).
Routine prophylaxis may help patients prevent and reduce the frequency and severity of hereditary angioedema (HAE) attacks.1,2
Routine prophylaxis can help patients take control
When determining whether prophylactic treatment is appropriate for an HAE patient, frequency of attacks is not the only criterion.2 Disease severity can be different for each patient, so treatment plans should be personalized based on individual needs.1-3 When evaluating your patients, several important factors should be included in consideration of prophylactic therapy, and include the following:
Frequency of attacks1,3
Severity of attacks1,3
Past laryngeal attacks4
Patient experience and preference1
US and international guidelines support routine prophylaxis as an important option2,5
According to guidelines put forth by HAE treatment experts, routine prophylactic therapy is appropriate for patients for whom on-demand acute treatment is inadequate* to minimize the suffering related to the disease.2
Treatment guidelines also reinforce that the decision to use prophylactic therapy cannot be made on rigid criteria but should reflect the needs of the individual patient1-3 and that home plasma-derived C1-INH self-infusion programs should be offered to patients.5 Additionally, guidelines recommend that patients should be evaluated for long-term prophylactic treatment at every visit and that adaptation of a treatment plan may be required to ensure that patient needs are being met.3
* "Inadequate" was tentatively defined as greater than 12 severe attacks per year, or more than 24 days of swelling (even if mild) per year.
Collaborative treatment decisions between physicians and patients are critical1
Patient input is important in determining overall disease burden. When talking with your patients, consider discussing:
Therapy administration options1
Potential risks and physical benefits of prophylaxis1
Zuraw BL, Banerji A, Bernstein JA, et al. US Hereditary Angioedema Association Medical Advisory Board 2013 recommendations for the management of hereditary angioedema due to C1 inhibitor deficiency. J Allergy Clin Immunol Pract. 2013;1(5):458–467.
Cicardi M, Bork K, Cabellero T, et al, on behalf of HAWK (Hereditary Angioedema International Working Group). Evidence-based recommendations for the therapeutic management of angioedema owing to hereditary C1 inhibitor deficiency: consensus report of an International Working Group. Allergy. 2012;67(2):147-157.
Maurer M, Magerl M, Ansotegui I, et al. The international WAO/EAACI guideline for the management of hereditary angioedema – the 2017 revision and update [published online ahead of print January 10, 2018]. Allergy. doi:10.1111/all.13384.
Bowen T, Cicardi M, Bork K, et al. Hereditary angioedema: a current state-of-the-art review, VII: Canadian Hungarian 2007 International Consensus Algorithm for the Diagnosis, Therapy, and Management of Hereditary Angioedema. Ann Allergy Asthma Immunol. 2008;100(1 Suppl 2):S30–S40.
Bowen T, Cicardi M, Farkas H, et al. 2010 International consensus algorithm for the diagnosis, therapy and management of hereditary angioedema. Allergy Asthma Clin Immunol. 2010;6(1):24.
Contraindications: CINRYZE is contraindicated in patients who have manifested life-threatening immediate hypersensitivity reactions, including anaphylaxis, to the product.
Hypersensitivity Reactions: Severe hypersensitivity reactions may occur during or after administration of CINRYZE. Consider treatment methods carefully, because hypersensitivity reactions may have symptoms similar to HAE attacks. In case of hypersensitivity, discontinue CINRYZE infusion and institute appropriate treatment. Have epinephrine immediately available for treatment of an acute severe hypersensitivity reaction.
Thromboembolic Events: Serious arterial and venous thromboembolic (TE) events have been reported at the recommended dose of C1 Esterase Inhibitor (Human) products, including CINRYZE, following administration in patients with HAE. Risk factors may include presence of an indwelling venous catheter/access device, prior history of thrombosis, underlying atherosclerosis, use of oral contraceptives, certain androgens, morbid obesity, and immobility. Benefits of CINRYZE for routine prophylaxis of HAE attacks should be weighed against the risks of TE events in patients with underlying risk factors. Monitor patients with known risk factors for TE events during and after CINRYZE administration.
Transmissible Infectious Agents: Because CINRYZE is made from human blood, it may carry a risk of transmitting infectious agents, e.g. viruses, and, theoretically, the Creutzfeldt-Jakob disease (CJD) agent. ALL infections thought by a physician possibly to have been transmitted by CINRYZE should be reported to Shire Medical Information at 1-800-828-2088.
Adverse Reactions: The only serious adverse reaction observed in clinical studies of CINRYZE was cerebrovascular accident. The most common adverse reactions (≥5%) observed were headache, nausea, rash, vomiting, and fever. Post marketing adverse reactions include local infusion site reactions and hypersensitivity. Post marketing thromboembolic events have been reported, including catheter-related and deep venous thromboses, transient ischemic attack, and stroke.
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To report SUSPECTED ADVERSE REACTIONS, contact Shire Medical Information at 1-800-828-2088 or FDA at 1-800-FDA-1088 or www.fda.gov/medwatch.