From historical roots to the growing community of today: The Åland Island meeting on von Willebrand disease

The 7^th Åland Island meeting on von Willebrand Disease (VWD) took place September 26–28^th, 2024. Set against the beautiful backdrop of the Åland archipelago in the Baltic Sea, the meeting brought together expert clinicians and researchers to discuss the latest developments in diagnosis and management of VWD – the most common bleeding disorder in the world.

This recurring meeting, held since 1998, has always taken place in the same location, serving as a poignant reminder of the history of VWD. The index family afflicted by this devastating disorder lived in Föglö, a municipality in the Åland Islands. Erik von Willebrand made his seminal observations in Helsinki, where the index case was brought for an appointment. Subsequent fieldwork in Föglö was conducted by Dr von Willebrand’s team.

Building on 100 years of research

This year’s meeting held special significance as we commemorated 100 years since Dr von Willebrand first encountered the index case, a 5-year-old girl named Hjördis. Her case, and the 23 other family members who also suffered from severe bleeding symptoms, led him to uncover a hereditary bleeding disorder — what we now know as von Willebrand Disease.

The meeting program was compiled by the organisers and co-chairs, Erik Berntorp (Emeritus Professor at Lund University, Sweden) and Riitta Lassila (Professor of Coagulation Medicine at the University of Helsinki, Finland). As in previous years, Octapharma was a proud sponsor of the event and supported its overall organisation.

The focused meeting brought together a total of 43 clinicians, field experts and researchers from 19 different countries, and allowed a high level of exchange among participants both inside and outside of sessions. Erik Berntorp commented: “One main value of the meeting is to get people from different cultures and with different scientific expertise together so that we can discuss and learn from each other”. Riitta Lassila added: “We learn from historical observations but also from contemporary issues which are fast developing”. Caroline Malcolmson, a participant, summed it up nicely: “It’s an incredibly unique and important opportunity to really connect with all the experts in the field and to get a state-of-the-art overview of this disease and the advances that are going on”.

The meeting was densely packed with 22 presentations across three days, some of the sessions being held on boats to and from the Åland Islands. A wide range of topics were covered, ranging from genetics and cell biology underlying VWD, to clinical manifestations of the disease, diagnostic tools, and the current treatment landscape. Particular attention was given to the unique challenges that VWD poses for women in both management and diagnosis, with enlightening talks on heavy menstrual bleeding and considerations for pregnancy. Several talks also focused on the management of VWD during surgery. Another key focus was prophylaxis, discussed in the context of guidelines, new trial results and strategies for personalised dosing based on pharmacokinetics.

“It’s an incredibly unique and important opportunity to really connect with all the experts in the field and to get a state-of-the-art overview of this disease and the advances that are going on."

Caroline Malcolmson

Tribute to the legacy of VWD

A unique component of the meeting this year was the launch of the newly revised Textbook of von Willebrand Disease, edited by Augusto Federici, Erik Berntorp, David Lillicrap and Robert Montgomery. In line with this legacy, the traditional excursion to the nearby island of Föglö, home to Hjördis and her family, provided a historical touch to the program. Participants had the opportunity to visit the family home and to meet relatives of Hjördis, creating a heartwarming exchange between clinicians, scientists and the family. A visit to the graves of Hjördis and her siblings, many of whom died at a young age due to complications of the disorder, provided a sombre but inspiring context to the scientific discussions. The day was brought to a close with special lectures on the history that led to the development of factor replacement therapy and on the life and work of Dr von Willebrand in the early 1900s.

The visit to Föglö also offered a reminder of how deeply the disorder can impact an affected family, particularly in a time where no understanding of the disease or treatment was available. Veronica Flood reflected: “This is the story of the first family that Erik von Willebrand diagnosed, but there are people all over the world living similar stories”.

“This is the story of the first family that Erik von Willebrand diagnosed, but there are people all over the world living similar stories."

Veronica Flood

Coming together as a community

We have come a long way since the days of Hjördis, with remarkable advances in the diagnosis and treatment of VWD, significantly enhancing both the lifespan and quality of life for patients. However, despite the progress, many unmet needs remain. Public awareness of VWD is still limited, and even within the medical community non-haematologists often lack the knowledge to recognise or diagnose the disorder. Diagnostic methods need improvement, clearer guidelines for prophylactic treatment are urgently required, and significant disparities in diagnosis and access to treatment persist, particularly in middle- and low-income countries. These issues were reflected in the meeting’s discussions.

The Åland Island meeting was well received by the participants, providing them with fresh insights on developments in the field. Moreover, it reminded them of the history that brought us to where we are today, and was an opportunity to exchange thoughts and ideas with other leading figures working on VWD.

Michelle Lavin commented “I think it’s a hugely influential meeting for everyone who gets the opportunity to attend, because not only are we looking at where we have evidence to support our future recommendations or research, but we’re also highlighting the areas and the gaps that we need to address and coming together as a community to try to improve our knowledge in these areas.”

The discussions and the insights gained from the meeting will undoubtedly inspire ongoing efforts to address the gaps in awareness, diagnosis and access to care, ensuring that patients worldwide can benefit from the strides made in this field.