Data show a bleeding episode occurs every three to 15 seconds in people living with hemophilia worldwide

April 17, 2017

On World Hemophilia Day 2017, Shire challenges the community to collectively improve global standards of care for rare bleeding disorders

Zug, Switzerland – April 17, 2017 – Shire plc (LSE: SHP, NASDAQ: SHPG), a leading biotechnology company focused on serving individuals with rare diseases, joins the global bleeding disorders community in honoring the 28th annual World Hemophilia Day (WHD).¹ On this day, Shire highlights initial findings from its estimate of the Global Annual Bleed Rate (GABR), which shows a bleeding episode occurs every three to 15 seconds worldwide, more than three times current global estimates.² These findings build on existing data from the World Federation of Hemophilia (WFH), which show only 25 percent of individuals living with hemophilia worldwide receive proper diagnosis and care, only 8 percent of which receive prophylaxis or regular treatment to prevent bleeds.^3,4 This research reinforces Shire’s ongoing commitment to innovative data collection to improve global standards of care for individuals living with rare bleeding disorders.

“As the global leader in hematology, we are committed to efforts, such as estimating the GABR, that improve our understanding of the global landscape for hemophilia and the true experience of those living with this debilitating bleeding disorder, said Leonard Valentino, M.D., Global Head of Hematology Medical Affairs, Shire.

Shire developed the GABR in collaboration with renowned hemophilia institutions, including WFH. Our shared aim is to identify regions of the world with the greatest need for improved diagnosis and care for hemophilia patients. Together, we created a literature-based model to estimate the GABR at both country and global levels. A full report of the GABR model is expected to be published later in 2017.²

“Shire is proud to support the global hemophilia community in honoring World Hemophilia Day,” continued Valentino. “On this day and every day, Shire is dedicated to supporting hemophilia patients through advancing early diagnosis, prophylaxis, individualized treatment options and innovative research and development efforts.”

This year, Shire is embracing WFH’s theme for World Hemophilia Day of “Hear Their Voices” and joining its partners and local advocacy organizations to raise awareness of those living with bleeding disorders. Throughout the day and across the world, Shire employees are wearing red to show our support for the community and holding celebrations to acknowledge the voices and stories of those living with bleeding disorders, including:

In the United States, Shire is holding a public awareness rally in Chicago and inviting members of the local hemophilia community to join and voice their support for the global bleeding disorders community. To learn more about this event, visit http://www.bleedingdisorders.com/bleeding-disorders-community-support.
In Germany, Shire is engaging media and inviting patients to share their perspective and the impact of hemophilia on their lives.
In Brazil, Shire is partnering with Federacao Nacional de Hemofilia to illuminate public monuments as part of WFH’s “Light it Up Red!” initiative to help raise awareness of hemophilia.
In China, Shire is participating in the “Run for Love” campaign to show support for the bleeding disorders community. Further, throughout the Asia Pacific region, Shire is hosting “Hear Their Voices” sharing sessions for patients and caregivers to share their personal experiences with hemophilia.

About Hemophilia
Hemophilia is a challenging chronic disease that causes longer-than-normal bleeding due to absent or deficient clotting factor in the blood.⁵ Hemophilia A is more common than hemophilia B;⁵ hemophilia A affects about 150,000 people, whereas hemophilia B affects about 30,000 people worldwide.⁴

Treatment regimens typically include on-demand and/or regular prophylactic infusions of factor replacement therapy to control or prevent the risk of bleeding.^6,7 People with hemophilia, working closely with their healthcare professionals, can live healthy lives with proper care and adequate treatment.⁶

Approximately 5-7 percent of people living with hemophilia develop inhibitors.⁸ Inhibitors are a rare but serious complication in patients with hemophilia.⁹ They form when the body's immune system attacks the molecules in factor therapy, causing it to be ineffective.⁹ Bypassing agents help bypass the inhibitor to help the body form a clot and stop bleeding.⁹

References

Hickman RJ. “April 17 is World Hemophilia Day.” Rare Disease Report website. http://www.raredr.com/news/april-17-hemophilia-day. Published April 18, 2016. Accessed March 15, 2017.
Thompson C. et al. “Estimating the Global Annual Bleed Rate in Haemophilia.” European Association for Haemophilia and Allied Disorders. Paris, France. February 1-3, 2017. Available at: http://onlinelibrary.wiley.com/doi/10.1111/hae.13150/full#hae13150-sec-0190.
World Federation of Hemophilia. “What will it take to Close the Gap?” World Federation of Hemophilia website. Last updated January 2014.
World Federation of Hemophilia. Report on the Annual Global Survey 2015. World Federation of Hemophilia website. http://www1.wfh.org/publication/files/pdf-1669.pdf. Accessed April 10, 2017
World Federation of Hemophilia. “What is hemophilia?” World Federation of Hemophilia website. http://www.wfh.org/en/page.aspx?pid=646. Accessed March 6, 2017.
World Federation of Hemophilia. “About Bleeding Disorders: Treatment.” World Federation of Hemophilia website. http://www.wfh.org/en/page.aspx?pid=642. Accessed January 12, 2017.
National Hemophilia Foundation. “Hemophilia A.” National Hemophilia Foundation website. https://www.hemophilia.org/Bleeding-Disorders/Types-of-Bleeding-Disorders/Hemophilia-A. Accessed March 6, 2017.
Wight J. Paisley S. “The Epidemiology of Inhibitors in Haemophilia A: A Systematic Review.” Haemophilia 2003.
World Federation of Hemophilia. “What are inhibitors?” World Federation of Hemophilia website. http://www.wfh.org/en/page.aspx?pid=651. Accessed January 12, 2017.

For further information please contact:

Investor Relations
Ian Karp	[email protected]	+1 781 482 9018
Robert Coates	[email protected]	+44 1256 894874
Media
Molly Poarch	[email protected]	+1 312 965 3413

NOTES TO EDITORS

About Shire

Shire is the leading global biotechnology company focused on serving people with rare diseases and other highly specialized conditions. We strive to develop best-in-class products, many of which are available in more than 100 countries, across core therapeutic areas including Hematology, Immunology, Neuroscience, Ophthalmics, Lysosomal Storage Disorders, Gastrointestinal / Internal Medicine / Endocrine and Hereditary Angioedema; and a growing franchise in Oncology.

Our employees come to work every day with a shared mission: to develop and deliver breakthrough therapies for the hundreds of millions of people in the world affected by rare diseases and other high-need conditions, and who lack effective therapies to live their lives to the fullest.

http://www.shire.com

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