VWD in the Land of Fire and Ice

Being in the Nordic regions of the world made me think about the origins of von Willebrand disease (VWD), how it was identified. Why?

VWD was named after the doctor who first identified it: Dr. Erik Adolf von Willebrand, a Finnish physician who first described VWD in 1926. He began to study an unusual bleeding disorder in the local communities, called Ålandic hemorrhagic disease. In 1925, Dr. von Willebrand evaluated a five-year-old girl named Hjördis. She lived on the remote island of Föglö, which belongs to Åland, a group of islands in the Baltic Sea between Sweden and Finland.Hjördis was bleeding excessively from her lip following an injury. She had a history of nosebleeds and bleeding following tooth extraction. She was one of 12 children, 10 of whom showed bleeding symptoms. Sadly, four of her siblings died of uncontrollable bleeding between the ages of two and four. Hjördis herself later bled to death during her fourth menstrual period. When Dr. von Willebrand studied the extended family, he discovered that 23 of 66 family members (16 females and 7 males) also had bleeding problems. The history of the original Finnish family has now been traced back to 1750, identifying over 1,000 members, with 125 known to be affected, and 12 who died from bleeding.

According to the website of the Icelandic Hemophilia Society, Iceland has about 127 registered people with VWD. Of these, 60 are mild (Type I) and 67 are severe. We know there are three main types of VWD, and numerous subtypes, so this information isn’t very clear on how many have what type. But we can be assured they are getting excellent care. And we mentioned last week that Icelanders have kept excellent genealogies of their families, meaning we can trace back family lineages for, in some cases, thousands of years, like the Finnish VWD family first recognized as having VWD.

I hope to return to Iceland and meet some of these friendly people who belong to our global family, in the land of fire and ice!

You can read all about VWD in our book, A Guide to Living with von Willebrand Disease, free to patients! Order here.

Got VWD? Get This!

I am very happy to tell everyone that our ground-breaking book, A Guide to Living with von Willebrand Disease, the world’s first book on VWD, is back! We just released the third edition, and it is now available free of charge to those with VWD.

VWD is the most commonly inherited bleeding disorder. I wrote this book together with Reneé Paper, a woman with VWD, emergency room nurse, and powerful advocate. Together we decided on the topics, which include learning to cope with VWD, inheritance, the medical system, treatment, women’s issues, and health insurance. The third edition includes important medical and scientific updates about VWD. This 175-page book offers parents, patients, and healthcare professionals a complete resource guide featuring real-life stories.

As most of you know, Renée Paper dedicated her life to educating people about VWD. We were saddened to lose this incredible advocate in 2007, but I think she would be proud to know her book is in its third edition.
Funding for the development of the book was made possible through a grant provided by CSL Behring, a world leader in developing and manufacturing safe and effective solutions to treat and manage bleeding disorders.
So chapter leaders, program coordinators, HTC staff and home care reps, order online from LA Kelley Communications at https://www.kelleycom.com/books.html or call us at 978-352-7657.
This book is a fantastic resource for any one facing the challenges of VWD. Make Renée proud—read it!
About LA Kelley Communications, Inc. 
Since 1990, LA Kelley Communications has been a world leader of groundbreaking educational materials and programs on hemophilia and related bleeding disorders. With more than a dozen books, numerous publications, and the oldest hemophilia newsletter in America, LA Kelley Communications remains a trusted source of practical information about raising children with bleeding disorders. Laureen Kelley founded the company in response to the need for practical consumer and parenting information on bleeding disorders. For more information about LA Kelley Communications, Inc., please visit https://www.kelleycom.com or call 978-352-7657.
About CSL Behring 
CSL Behring is a leader in the plasma protein therapeutics industry. Committed to saving lives and improving the quality of life for people with rare and serious diseases, the company manufactures and markets a range of plasma-derived and recombinant therapies worldwide. CSL Behring therapies are indicated for the treatment of coagulation disorders including hemophilia and von Willebrand disease, primary immune deficiencies, hereditary angioedema and inherited respiratory disease. The company’s products are also used in cardiac surgery, organ transplantation, and burn treatment, and to prevent hemolytic diseases in newborns. CSL Behring operates one of the world’s largest plasma collection networks, CSL Plasma. CSL Behring is a subsidiary of CSL Limited, a biopharmaceutical company headquartered in Melbourne, Australia. For more information, visit http://www.cslbehring.com.

The Time is NOW!

I’m writing from Phoenix, where a weekend of education and networking for people with von Willebrand disease just wrapped up. The Arizona Hemophilia Association hosted the first annual national von Willebrand conference. Cindy Komar, the very capable executive director of the AHA and her team assembled a great cast of speakers and facilitators, and provided entertainment for the kids for the weekend-long event. It was a tremendous conference and hugely successful.

Saturday morning started with a rousing speech by Jerry Ervin, who shared his hard-luck story and how he overcame many environmental challenges to become educated and a motivational speaker. Then Drs. di Paola and Kouides shared latest developments in VWD treatment. Afterwards came break out sessions, in which I presented the changing insurance climate and how we can protect our access to treatment. Other sessions included dental care, financial planning, parenting and atypical VWD.

25 states were represented at the event, and I saw many friends in the community, from patients and families, and even my dear friend Julie Winton, with whom I climbed Kilimanjaro last August!

This morning we had two great speakers, Jeff Leiken, who spoke about attitude and removing bias from our way of looking at the world, and Pamela Crim, who shared her personal story, from a stroke at age 19 while just a newlywed, to adopting two little orphans from Juarez, Mexico–truly an incredible life story!

Outside the teens were treated to a demonstration by a hot air balloon pilot, Michael Glen. His inspiring talk about ballooning focused not on the fact that he’s a paraplegic (the only one in the world who is also a balloonist) but on how nothing can stop you from achieving your dreams. He was fantastic! I’d highly recommend him for teen workshops (www.rollingpilot.com)

We closed the event by paying our respects to the lady who was the pioneer in VWD advocating and educating, Renee Paper, my co-author and friend. We keenly felt her absence, but we’re pretty sure her spirit was with us.

Thanks to CSL Behring for the funding for making this fantastic event happen, and to the Arizona Hemophilia Association for their time and dedication.

In Memoriam: Renee Paper

It is with great sadness that we’ve learned of the passing of its greatest advocate on von Willebrand Disease. Renee Paper died November 7, at age 49, after an eight week hospital stay following a fall. Renee had been on disability for the past three years, and yet received an achievement award just last Saturday at NHF’s 59th Annual Meeting, in recognition of her outstanding life’s achievement in helping others with bleeding disorders. People around the world knew Renee, as she traveled and lectured frequently, before her disability. As many of you already know, she had had gastric bypass surgery, loss a tremendous amount of weight in a short period of time, seemed to recover and was doing well, but then, health issues compounded. She had VWD herself, and also battled hepatitis C, and various other problems. Many of her friends tried to stay in touch by phone or by visiting her in Las Vegas over the past several years.

Renee was a personal friend, and also my co-author on the book “A Guide to Living With von Willebrand Disease.” She had traveled with me and my family to Puerto Rico and the Dominican Republic on business together, and she and I presented in places as close as Connecticut and far away as Australia. She was brilliant; she was fun-loving. She was could be irreverent yet compassionate. She loved animals, and one of her favorite gifts to her friends was to send a photo of herself each New Year’s with a different animal from different parts of the world: kissing a dolphin in the Caribbean; draping a boa constrictor around her neck in Mexico; nuzzling an alligator in New Orleans; atop a camel in the Canary Islands. Renee knew how to grab life by the horns and tame it, and she wanted others to do that, too, regardless if they had a disorder or disability.

Messages have been pouring in to me from around the world. Helen Campbell, UK, wrote: “Renee has been a great inspiration to me personally (and to other women) in raising awareness and promoting advocacy within, and outside of, the bleeding disorders community regarding the issues surrounding women and their bleeding disorders. Her work has touched the lives of many both directly and indirectly. It is a great loss for the Haemophilia communities and yet must be a greater loss for her family. Thank you, Renee. You made such a difference. From your acorns you saw great oaks grow, now the branches are reaching far and wide.”

Parimal Debnath from the Haemophilia Society of Bangladesh wrote, “It’s really sad news. My condolences for her family. May God rest her soul in eternal peace.”

Dr. Carol Kasper, renowned hematologist, Emerita Professor of Medicine, University of Southern California, Orthopaedic Hospital and colleague and friend of Renee’s, perhaps summed up Renee’s legacy and memory best:

“Renee Paper had a vision for Nevada, its own hemophilia foundation, its own hemophilia treatment center, and she made them happen. It wasn’t easy. Renee was blessed with energy, enthusiasm and perseverance. She had a great ability to organize and to inspire.
“She also knew how to have fun. She was the life of the party! I remember driving with her down a country road in Ireland, in sheep country. Sheep strayed across the road. Renee stopped and shooed them off, and you have no idea how hard it is to shoo a sheep. We cleared a bit of road, drove on around a curve, and, more sheep! We wound up in gales of laughter as we continued to shoo the sheep, and shoo and shoo.

“I am grateful to Dr. Jonathan Bernstein and nurse Becki Berkowitz, also Dr. Heather Allen, all of Las Vegas, who watched over Renee in her last illnesses, whenever Renee’s spirit of independence would allow it.

“I shall remember her achievements, but the images of her exuberance and hilarity are foremost in my mind at this time. Her name will be remembered.”

Good-bye to a tremendous leader, warm and loving human being, educator, visionary, and friend. There was ever only one Renee, and we will miss her. Please post your thoughts, stories and tributes to Renee here.

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