Each benefit plan, summary plan description or contract defines which services are covered, which services are excluded, and which services are subject to dollar caps or other limitations, conditions or exclusions. Members and their providers have the responsibility for consulting the member's benefit plan, summary plan description or contract to determine if there are any exclusions or other benefit limitations applicable to this service or supply. If there is a discrepancy between a Medical Policy and a member's benefit plan, summary plan description or contract, the benefit plan, summary plan description or contract will govern.
Blue Cross and Blue Shield of Montana (BCBSMT) may consider CINRYZE® medically necessary for routine prophylaxis against angioedema attacks in adolescent (nine years of age and older) and adult patients diagnosed with hereditary angioedema (HAE) when the diagnosis has been confirmed by C4 and C1 Inhibitor laboratory testing.
BCBSMT considers CINRYZE® experimental, investigational and unproven for all other treatments including but not limited to treatment of an acute HAE attack.
NOTE: Safety and efficacy of CINRYZE have not been established in neonates, infants, or children under the age of nine years old and CINRYZE is contraindicated for patients who have manifested life-threatening immediate hypersensitivity reactions, including anaphylaxis to the product.
NOTE: Serum C4 level is a screening test for C1 INH deficiency, as serum C4 is invariably low in untreated HAE (C4 < 30% of mean normal level). If C4 is normal it is not usually necessary to proceed to C1 INH analysis. If the C4 level is low then C1 INH level and function may be assessed.
NOTE: This MP does not address drugs for the treatment of “acute” HAE attacks.
Prophylaxis against HAE Attacks
In clinical trials, CINRYZE was effective in preventing or decreasing the frequency of attacks in most but not all HAE patients. Adverse reactions reported in the study were considered mild or moderate in severity.
The safety and efficacy of CINRYZE prophylaxis therapy to reduce the incidence, severity, and duration of HAE attacks was demonstrated in a single randomized, double blind, placebo controlled multi-center cross-over study of 24 patients. Patients were screened to confirm a diagnosis of HAE and a history of at least two HAE attacks per month. Twenty-four patients were randomized to one of two treatment groups: either CINRYZE prophylaxis for 12 weeks followed by 12 weeks of placebo prophylaxis; or randomized to placebo prophylaxis for 12 weeks followed by 12 weeks of CINRYZE prophylaxis. Two subjects dropped out (one in each arm); 22 patients crossed over into period two and were included in the efficacy analysis. Patients were given blinded injections (CINRYZE or placebo) every 3 to 4 days, approximately two times per week. Patients recorded all angioedema symptoms daily. An attack was defined as the subject-reported indication of swelling at any location following a report of no swelling on the previous day. The efficacy determination was based on the number of attacks during the 12 week period while receiving CINRYZE as compared to the number of attacks during the placebo treatment period. The effectiveness of C1 inhibitor prophylaxis in reducing the number of HAE attacks was variable among the subjects. Patients treated with CINRYZE had a 66% reduction in days of swelling, and decreased in the average severity and duration of attacks.
Treatment for HAE Attacks
Clinical trials are currently being conducted in the United States to determine the efficacy of CINRYZE® for the treatment of acute attacks of HAE.
A search of peer reviewed literature through April 2011 identified no new clinical trial publications or any additional information that would change the coverage position of this medical policy.
Disclaimer for coding information on Medical Policies
Procedure and diagnosis codes on Medical Policy documents are included only as a general reference tool for each policy. They may not be all-inclusive.
The presence or absence of procedure, service, supply, device or diagnosis codes in a Medical Policy document has no relevance for determination of benefit coverage for members or reimbursement for providers. Only the written coverage position in a medical policy should be used for such determinations.
Benefit coverage determinations based on written Medical Policy coverage positions must include review of the member’s benefit contract or Summary Plan Description (SPD) for defined coverage vs. non-coverage, benefit exclusions, and benefit limitations such as dollar or duration caps.