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Von Willebrand Disease

World’s First Intensive Study of Postpartum Women with von Willebrand Disease Shows Potential Risk of Delayed Hemorrhage, Despite Treatment

CSL King of Prussia, PA –Findings of a first-of-its-kind study of women with von Willebrand disease (VWD) show that current postpartum treatment strategies do not increase levels of von Willebrand factor (VWF) to normal range or even to the levels of women with milder, untreated VWD. VWF is a blood protein important for preventing postpartum hemorrhage. Results of this nationwide study are being presented at the World Federation of Hemophilia 2012 World Congress in Paris. This investigator-initiated study was supported by a $1.2 million unrestricted grant from CSL Behring.

In healthy women, VWF levels fell rapidly after childbirth, approached baseline one week postpartum and reached baseline three weeks postpartum. The pattern of decrease in these levels was consistent among all patient groups, but levels were significantly lower in women with VWD. VWF levels were lowest among treated VWD patients.

"While a rapid decrease in VWF levels after delivery is normal, significant decreases following childbirth can result in an increased risk for postpartum hemorrhage, especially since women continue to bleed for days to weeks after delivery," said Andra James, MD, Professor of Obstetrics and Gynecology, University of Virginia, and principal investigator of the study while she was at Duke University. "Given that current postpartum treatment strategies do not effectively manage VWF levels in the VWD patient, we hope to conduct further study into the benefits of prophylaxis treatment for preventing the potentially serious complication of delayed postpartum hemorrhage."

This prospective, observational cohort study compared changes in blood levels of VWF proteins postpartum between 31 women with and 40 women without VWD. Fourteen of the women with VWD were treated during the postpartum period - 12 with VWF concentrate, one with desmopressin plus VWF concentrate and one with desmopressin. At periodic intervals in the immediate postpartum period, von Willebrand factor: Ristocetin cofactor activity (VWF:RCo), factor VIII (FVIII) and von Willebrand factor antigen (VWF:Ag) levels were measured.

About von Willebrand disease
Von Willebrand disease (VWD) is the most common hereditary bleeding disorder in the United States, and is caused by a deficiency or abnormality of von Willebrand factor, a protein in the blood that is necessary for normal blood clotting. According to the U.S. Centers for Disease Control (CDC), von Willebrand disease affects 1 to 2 percent of Americans - as many as 2.8 million people - more than half of whom are women. Men and women are equally likely to be affected by VWD. Women, though, may suffer severe health consequences, such as life-threatening bleeding following childbirth, if their condition is not correctly diagnosed and managed. Other common symptoms of VWD include easy bruising; frequent or prolonged nosebleeds; heavy, prolonged menstruation; prolonged bleeding following injury or surgery; and prolonged bleeding during dental procedures.

About CSL Behring
CSL Behring is a global leader in the plasma protein biotherapeutics industry. Passionate about improving the quality of patients' lives, CSL Behring manufactures and markets a range of safe and effective plasma-derived and recombinant products and related services. The company's therapies are used in the treatment of immune deficiency disorders, hereditary angioedema, haemophilia, von Willebrand disease, other bleeding disorders and inherited emphysema. Other products are used for the prevention of hemolytic diseases in the newborn, in cardiac surgery, organ transplantation and in the treatment of burns. The company also operates one of the world's largest plasma collection networks, CSL Plasma.

CSL Behring is a subsidiary of CSL Limited, a biopharmaceutical company with headquarters in Melbourne, Australia. For more information, visit www.cslbehring.com.

HealthyWomen Asks: Do You Know the Signs and Symptoms of Von Willebrand Disease (VWD)?

Although von Willebrand disease (VWD) is the most common hereditary bleeding disorder in the United States, affecting up to 1 percent of the population, it is often misdiagnosed or under-diagnosed in women. HealthyWomen, the nation's leading nonprofit health information source for women, has developed new educational materials they hope will shine a light on a disorder that, when undiagnosed and untreated, can severely impact the quality of life for women.

Caused when von Willebrand factor, a protein in the blood that is necessary for clotting, is either missing or not working properly, VWD affects men and women equally. Yet, when undiagnosed and untreated in women, it puts them at a greater risk for life-threatening bleeding following childbirth and for undergoing unnecessary hysterectomies.

"Undiagnosed bleeding disorders, like von Willebrand disease, can have real health consequences for women," said Elizabeth Battaglino Cahill, RN, executive director of HealthyWomen. "The goal of these new materials, available on HealthyWomen.org, is to spark honest conversations among mothers, sisters and daughters about the signs and symptoms of VWD and to hopefully underscore the need for even more education about this condition."

The five signs and symptoms of von Willebrand disease are:

Easy bruising
Frequent of prolonged nosebleeds
Heavy, prolonged menstruation
Prolonged bleeding following injury or surgery
Prolonged bleeding during dental procedures

"Because VWD is a hereditary disorder, many women dismiss tell-tale signs like easy bruising or heavy periods as normal because their mother or other female relatives experienced similar symptoms," said Meera Chitlur, M.D., Director, Hemophilia Treatment Center and Hemostasis Program, Children’s Hospital of Michigan and who also works with The Foundation for Women & Girls with Blood Disorders (FWGBD), a non-profit advocacy and education organization dedicated to advancing physician and healthcare provider knowledge of the unique needs and challenges faced by women and adolescent girls with blood disorders. "Unexplained bleeding or bruising is not normal and is something you should always discuss with your healthcare provider. If you are diagnosed with a bleeding disorder, treatments are available."

The new materials include common questions about VWD answered by an expert, as well as real-life stories of women living with the condition. One of these women, Kristin Prior, 49, recalls her own diagnosis in 1996.

"While I had experienced many of the five signs and symptoms throughout early adulthood it wasn’t until I was 32 that I finally received my diagnosis of von Willebrand disease," said Prior. "My hope is that women who are facing some of the same challenges that I did will read my story on HealthyWomen.org and realize that diagnosis and treatment can make a tremendous difference in their lives."

The von Willebrand disease educational resources were developed through an educational grant from CSL Behring, a world leader in developing and manufacturing safe and effective solutions to treat and manage bleeding disorders.

Full Press Release

Researchers Focus on Rare Bleeding Disorders

VonWillebrandDisease There is more to clotting disorders than severe bleeding, achy joints and high-priced drugs, according to new research on hemophilia and von Willebrand disease (vWD).

Study Demonstrates Relationship Between Subcutaneous IgG (SCIg) Dosage and Clinical Outcomes With Hizentra Treatment in Patients With Primary Immunodeficiencies

KING OF PRUSSIA, Pa., March 21, 2011 /PRNewswire/ -- Data presented by CSL Behring today suggest that treatment with higher dose Hizentra® (IgPro 20) correlates with reduced risk of infection and missed school or work among patients with primary immunodeficiencies (PI).