Von Willebrand Disease

The Time is NOW!

Laurie Kelley February 27, 2012

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.

Wilate: a New VWD Product

Laurie Kelley December 14, 2009

This is big news for von Willebrand Disease patients: there’s a new product on the market. Wilate® just got approved by the US FDA for “treatment of spontaneous and trauma-induced bleeding episodes in patients with all types of von Willebrand disease (VWD),” according to a press release. Wilate is a high-purity plasma-derived product, which uses a double viral inactivation process in manufacturing: solvent/detergent (S/D) process and a special terminal dry-heating (TDH) system. No albumin is added as a stabilizer. Wilate is exclusively derived from large pools of human plasma collected in U.S. FDA approved plasma donation centers. Wilate will be available in the market in early 2010.

Wilate is produced by Octapharma, a Swiss-based company. This is the first time Octapharma has entered the US bleeding disorders market. Wilate will be in direct competion with Humate-P, manufactured by CLS Behring.

One thing interesting I learned is that this is the first product developed and manufactured specifically for VWD.

Here’s a quote from Octapharma: “Octapharma’s worldwide commitment to coagulation disorders dates back to Octapharma Group’s formation 25 years ago,” said Octapharma USA President Flemming Nielsen.”We are thrilled that U.S. patients will now have access to Wilate following its significant success in Europe as a next generation therapy. Octapharma is committed to providing the U.S. market with life-enhancing therapies.”

VWD patients now have more choices for treatment options. For more information, please visit www.octapharma.com.

Great Book I Just Read
Final Voyage: A Story of Arctic Disaster and One Fateful Whaling Season by Peter Nichols

Massachusetts (my home state) gave birth to the American Revolution and also the global whaling industry. This fascinating book recounts a terrible story of scores of whaling ships trapped in the Arctic region, as the captains tried to score as many whales as possible before the ice closed in for the winter. Nichols deftly parallels that story with the beginning of the whaling industry, which has direct ties to Puritans coming to the new world, religious intolerance in Massachusetts in the 1700s, and the Quakers, who almost single-handedly created this lucrative industry. Whale oil soon was lighting the homes of Massachusetts and then lubricating the machines of the Industrial Age, making some Quakers millionaires, even then. Nichols also details the decline of the industry: the discovery of crude oil in Titusville, Pennsylvania, which soon replaced whale oil; the decimation of so many whales left hunting lean. All these events culminate into a dreadful outcome for the ambitious captains of the whaling ships that last season of hunting, as they searched for the last whales in an early and fearsome storm. This book will enlighten you about many subjects, from religion and commerce, to whales and history. Three stars.

Florence’s Story

Laurie Kelley April 18, 2009

One of the things I find remarkable here in Kenya are the questions patients ask me: Why does my knee hurt? Why do only boys get hemophilia? If I get a shot now, I will be well for six months, right? If my first child has hemophilia, then no other child I have will get it, is that correct?

Maureen Miruka, mother of a child with hemophilia, is determined to provide that education. She is at once shocked by the low level of understanding, and motivated to enlighten. One way to do this is by large family meetings. Another is by personal visits to the homes of families with bleeding disorders. Today we did both.

First, we shouldered past buses, cars and trucks on the congested Nairobi streets to visit Florence Odwar, a mother of five–two with von Willebrand Disease. Some parts of Nairobi are dangerous, and we cruised past slums, where I could not take a photo, because to lower your window might invite a quick snatch of your camera. Florence’s street is in a rambling but safe area. She welcomed our visit.

Her house is urban and small. A room that can only fit a couch and a chair, partitioned by a hung curtain, behind which I assumed was the bedroom, where she, her husband David, and children Moline, James, Nickson, Jovan and Georgina sleep. Seems impossible but they work it out.

David, Moline and Jovan have VWD. Rarely is there treatment. Jovan was diagnosed when his teeth were coming in and bled profusely. Afterwards, the entire family was examined. Florence was shocked. Even more than the physical suffering the three experience, there is financial. VWD infiltrates every aspect of their life.

See all the photos here.

Despite their obvious poverty, they are a hard working family. David is an electrician for a company; Florence is a seamstress, owning her own small shop for 10 years now. They earn about $200 a month. From that they pay rent ($62), school fees ($37), clinic visits ($13); they all take public transport and food costs are high. With each bleed, one parent must take time off from work, which forfeits their pay. Life is a struggle.

I was struck by Florence’s inquisitive nature, willingness to learn more about VWD, and determined to have a better life for her children. The children are ages 21, 20, 19, 9 and 5. All are doing well in school. James and Nickson want to be engineers; Jovan wants to be a pilot. If that fails, I think he wants to be Chuck Norris. I have never in my life been asked so many questions about Chuck Norris, whom Jovan things is real. We had a lengthy discussion about who would win in superhero fights (Mr. Fantastic could beat Chuck Norris, and Bruce Lee would beat Jet Li. Nobody could ever beat Superman)

The home visit was a great opportunity to evaluate Florence’s family for Save One Life and I am happy to say they are our first Kenyan family to enroll! It was also a great time to educate. We discussed many aspects of VWD and treatment, especially for Jovan’s terrible nose bleeds, and Florence asked for my book.

After a photo session which grew to include all the neighborhood children, we traveled on to a family gathering: about 13 families attended the first Family Day for the Jose Memorial Hemophilia Society-Kenya. I find most Kenyans to be soft spoken, polite and deferential. It was hard for them to break the ice, even with one another. The JMHS-K provided delicious food and promoted an atmosphere of community and trust. By the end, everyone was sharing their stories. Another Family Day was promised, with activities and education sessions.

Afterwards, the board members and I sat around, laughing and sharing our own stories about everything. There’s a real bond between parents whose children share the same chronic disorder. Maureen realized I had only been in Kenya for two days. “It seems like a week!” and I agreed. The bonds we made in two days will be unshakable and unbreakable eternally, or as long as hemophilia stalks the earth.

In Memoriam: Renee Paper

Laurie Kelley November 12, 2007

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.

New Product to Treat Von Willebrand Disease

Laurie Kelley February 5, 2007

Great news for von Willebrand patients: The FDA announced last week the approval of Alphanate for the treatment of VWD. The product is approved for patients undergoing surgery or invasive procedures with von Willebrand disease (VWD) in whom the hormone desmopressin is either ineffective or contraindicated. It is not approved for patients with VWD Type 3 who are undergoing major surgery.

Up till now in the US, only Humate-P has been approved for VWD. Of course, other products are used off-label when needed. To have a product legally approved overcomes a big hurdle in treatment, and provides consumers more choice. Alphanate is the first biologic product approved for treatment of surgical and invasive procedures in patients with vWD.

Alphante is manufactured by Grifols Biologicals, Inc., Los Angeles, Calif. (which acquired the former manufacturer Alpha Therapeutics). It’s a plasma derived product, as is Humate-P. Alphanate undergoes two separate steps for viral inactivation to reduce the risk of blood-borne viruses.

It’s nice to report some positive news! This approval has been in the works for years. For more information on which product is best for you, ask your doctor. But also call the manufacturer, and talk to your local hemophilia organization. Thanks to Dennis Penning of the Hemophilia Foundation of Illinois for circulating this to the community, and for always keeping us informed… you rock, Dennis!

And don’t forget you can order our book, the world’s first, A Guide to Living with von Willebrand Disease, through this website. Free to families, HTCs and nonprofits.