AWMSG Recommends Cinryze®▼ (C1 Inhibitor [Human])for Routine Prevention, Treatment and Pre-Procedural Preventionof Hereditary Angioedema (HAE) Attacks in Adults and Adolescents in Wales

MAIDENHEAD, England, March 25, 2013 /PRNewswire/ --

- Cinryze shown to treat and prevent attacks in rare and life threatening genetic disorder -  

- First and only C1 inhibitor licensed* for routine prevention in patients with severe and recurrent HAE attacks now available for use by NHS Wales -

Adults and adolescents in Wales living with hereditary angioedema (HAE) - a rare and life threatening genetic disorder - now have access to ViroPharma's Cinryze^® (C1 inhibitor [human]), through NHS Wales, following the approval from the All Wales Medicines Strategy Group (AWMSG).^[1] Cinryze is the first and only C1 inhibitor approved in Wales for the treatment and pre-procedure prevention of angioedema attacks in adults and adolescents with hereditary angioedema, and routine prevention of angioedema attacks in adults and adolescents with severe and recurrent attacks of hereditary angioedema who are intolerant to or insufficiently protected by oral prevention treatments or who are inadequately managed with repeated acute treatment.^[1]Cinryze is also recommended for the treatment of acute attacks.^[2]

HAE is a rare, debilitating and potentially life-threatening genetic disorder affecting around 10,000 people across Europe.^[3] Based on an incidence of one in 50,000, it is estimated to affect 50 - 60 people in Wales.^[4] People living with the condition suffer from recurrent and disabling attacks of swelling that can affect the larynx, abdomen, face, extremities and urogenital tract.

"The impact of HAE can cause significant disruption to a patient's life. HAE attacks are usually very unpredictable and can be life threatening if untreated" said Dr. Tariq El-Shanawany, University Hospital of Wales. "The availability of a new product approved in Wales for routine prevention as well as treatment of acute attacks is very much welcomed."

Patients deemed eligible for routine prevention therapy by their physician may also be able to self-administer Cinryze, giving doctors and patients the ability to better manage the condition. To support this, ViroPharma provides eligible patients with the Libertas Plus^™ Home Care Service in Wales. This service provides direct-to-home delivery of the medicine, individualised self-administration training by experienced, specially trained nurses, and ongoing guidance and support to individuals who are receiving therapy.

"HAE UK are delighted that Cinryze has now been included in the list of medications available to patients in Wales," said Ann Price from HAE UK. "The disease profile in HAE varies from patient to patient, and it is important that the specialists and the patients have access to the full range of routine prevention and acute attack medications in order to set up the most effective HAE management program for each individual patient."

"HAE is a disabling and potentially life threatening condition, with the unpredictability of attacks causing many patients significant anxiety. We are excited to offer the only C1 inhibitor approved in Wales with a label that includes routine prevention of attacks, in addition to treating the attacks as they occur," commented Thierry Darcis, ViroPharma General Manager for Europe. "At ViroPharma, we are committed to delivering important solutions that address critical gaps in care for patients living with few, if any, clinical treatment options. We will be working closely with physicians to help and support patients through the prevention and treatment of their HAE attacks."

Clinical Trials

Clinical trials have demonstrated that Cinryze can significantly reduce the duration and frequency of HAE attacks and the time to symptom reliefcompared to placebo.^[5],[6]In a pivotal Phase III study involving patients experiencing acute attacks, Cinryze demonstrated symptom relief in less than half the time compared to placebo, from a median of more than four hours to symptom relief with placebo, to two hours with Cinryze. In the second pivotal study, which examined routine prevention, patients experienced an average reduction from four attacks per month on placebo to two attacks per month on Cinryze. ^[5] Furthermore, in an open label study for routine prevention, Cinryze reduced the frequency of attacks by 93 percent, from a median of three attacks per month at baseline to 0.2 per month. ^[6]

About Cinryze^®(C1 inhibitor [human])

Cinryze is a highly purified, pasteurised and nanofiltered plasma-derived C1 inhibitor product. ^[2]  In the EU, the product is approved by the EMA for the treatment and pre-procedure prevention of angioedema attacks in adults and adolescents with hereditary angioedema (HAE), and routine prevention of angioedema attacks in adults and adolescents with severe and recurrent attacks of HAE, who are intolerant to or insufficiently protected by oral prevention treatments or patients who are inadequately managed with repeated acute treatment. ^[2] Cinryze is for intravenous use only. ^[2]

Hypersensitivity reactions to Cinryze may occur. ^[2] Thrombotic events have occurred in patients receiving off-label high dose C1 inhibitor therapy. ^[2] Patients with known risk factors for thrombotic events (including indwelling catheters) should be monitored closely. ^[2] With any blood or plasma derived product, there may be a risk of transmission of infectious agents, e.g. viruses.  Standard measures to prevent infections resulting from the use of medicinal products prepared from human blood or plasma include selection of donors, screening of individual donations and plasma pools for specific markers of infection and the inclusion of effective manufacturing steps for the inactivation/removal of viruses. ^[2]

The only common adverse reactions in clinical trials associated with Cinryze was rash. ^[2]Uncommon adverse reactions include headache, nausea, erythema, phlebitis and local reactions at the injection site. ^[2] No drug-related serious adverse events (SAEs) were reported in clinical trials.

About Hereditary Angioedema (HAE)

HAE is a rare, severely debilitating, life-threatening genetic disorder caused by a deficiency of C1 inhibitor, a human plasma protein. This condition is the result of a defect in the gene controlling the synthesis of C1 inhibitor. C1 inhibitor maintains the natural regulation of the contact, complement, and fibrinolytic systems, and when left unregulated, can initiate or perpetuate an attack by consuming the already low levels of endogenous C1 inhibitor in HAE patients. Patients with C1 inhibitor deficiency experience recurrent, unpredictable, debilitating, and potentially life threatening attacks of inflammation affecting the larynx, abdomen, face, extremities and urogenital tract. Patients with HAE experience approximately 20 to 100 days of incapacitation per year.^[7] There are estimated to be at least 10,000 people in Europe with HAE,^[3] with approximately 50 - 60 people in Wales living with the disease.

For more information on HAE, please visit HAE UK's website at http://www.haeuk.org/.  

About Libertas Plus™ Home Care Service

The Libertas Plus Home Care Service is a complementary new service that has been specifically devised to help families with HAE increase control of their HAE therapy and gain independence, by providing patients, friends and family training to confidently and successfully self-administer treatment at home. Libertas Plus service provides individualised self-administration training by a specialist nurse and ongoing support to individuals who are receiving routine prevention therapy.

Recommendation from the AWMSG

Cinryze is the first and only C1 inhibitor approved in Wales for the treatment and pre-procedure prevention of angioedema attacks in adults and adolescents with hereditary angioedema, and routine prevention of angioedema attacks in adults and adolescents with severe and recurrent attacks of hereditary angioedema who are intolerant to or insufficiently protected by oral prevention treatments or who are inadequately managed with repeated acute treatment.^[1]

About ViroPharma Incorporated

ViroPharma Incorporated is an international biopharmaceutical company committed to developing and commercializing novel solutions for physician specialists to address unmet medical needs of patients living with diseases that have few if any clinical therapeutic options, including C1 esterase inhibitor deficiency, treatment of seizures in children and adolescents, adrenal insufficiency, and C. difficile infection (CDI).  Our goal is to provide rewarding careers to employees, to create new standards of care in the way serious diseases are treated, and to build international partnerships with the patients, advocates, and health care professionals we serve. ViroPharma's commercial products address diseases including hereditary angioedema (HAE), CDI and treatment of seizures in children and adolescents.

Forward Looking Statements

Certain statements in this press release contain forward-looking statements that involve a number of risks and uncertainties. Forward-looking statements provide our current expectations or forecasts of future events, including our regulatory filings in Europe related to Cinryze, including without limitation statements related to physician and patient acceptance of Cinryze and the estimated number of HAE patients in Wales. There can be no assurance that our commercial launch of Cinryze in the EU, including Wales, will be successful. The commercial success of Cinryze in the EU, including Wales, will depend on a number of factors including, the actual number of HAE patients in the EU and Wales, physician and patient acceptance of Cinryze, the timing and level of pricing approvals obtained in EU member states, including Wales, and the level of manufacturing and supply of Cinryze produced by third party manufacturers. In addition, there can be no assurance that we will be successful in our efforts to continue to deliver important solutions that address critical gaps in care for patients living with few, if any, clinical treatment options in the future.  These factors, and other factors, including, but not limited to those described in our annual report on Form 10-K for the year ended December 31, 2012 and 10-Q filings for the quarters ended March 31, 2012,  June 30, 2012, and September 30, 2012 filed with the Securities and Exchange Commission, could cause future results to differ materially from the expectations expressed in this press release. The forward-looking statements contained in this press release are made as of the date hereof and may become outdated over time. ViroPharma does not assume any responsibility for updating any forward-looking statements. These forward looking statements should not be relied upon as representing our assessments as of any date subsequent to the date of this press release.

*Treatment and pre-procedure prevention of angioedema attacks in adults and adolescents with hereditary angioedema (HAE). Routine prevention of angioedema attacks in adults and adolescents with severe and recurrent attacks of HAE, who are intolerant to or insufficiently protected by oral prevention treatments, or patients who are inadequately managed with repeated acute treatment.

References

1. All Wales Medicines Strategy Group. AWMSG Recommendations. Available at: http://www.wales.nhs.uk/sites3/Documents/371/C1%20inhibitor%20%28Cinryze%29%20FAR.pdf [http://www.wales.nhs.uk/sites3/Documents/371/C1 inhibitor %28Cinryze%29 FAR.pdf ] Last accessed 25 March 2013

2. Cinryze European Summary of Product Characteristics. Available at: http://www.ema.europa.eu/docs/en_GB/document_library/EPAR_-_Product_Information/human/001207/WC500108895.pdf. Last accessed March 2013.

3. Agostoni A, Aygoren-Pursun E, Binkley KE, et al. Hereditary and acquired angioedema: problems and progress: proceedings of the third C1 esterase inhibitor deficiency workshop and beyond. J Allergy Clin Immunol 2004;114(3 Suppl):S51-131.

4. ViroPharma Pricing and Reimbursement Dossier. Cinryze. Data on file.

5. Zuraw BL, et al. Nanofiltered C1 Inhibitor Concentrate for Treatment of Hereditary Angioedema. N Engl J Med 2010; 363:513-522.

6. Zuraw B, Kalfus I, et al. Safety and Efficacy of Prophylactic Nanofiltered C1-inhibitor in Hereditary Angioedema. Am J

Med. 2012; 125(9):938

7. Cicardi M, Agostini A. Hereditary angioedema. N Engl J Med. 1996;334:1666-1667.

ViroPharma Incorporated Contacts:    

Thierry Darcis
General Manager, ViroPharma Europe
Phone +44(0)20-7572-1222

Emma White (Media Enquiries)
PR, Advocacy and Communications Manager, Europe
Phone +44(0)1628-582732