2010 November - EHC

Study results support his view. As demonstrated in Italy and the UK, secondary prophylaxis was
effective in preventing total bleeds and joint bleeds in adults with severe haemophilia . Access to
prophylactic therapy is also one of the European Principles of Haemophilia Care.
Whereas some benefits, for instance “missed work days”, are easily measurable and can be
balanced with costs, others are not. Avoidance of pain and suffering, the pursuit of an education
or career, raising a family, enjoying normal activities, the ability to travel and freedom from
anxiety and restrictions – the list of benefits without a price tag is long. Brian O’Mahony
concluded his presentation by commenting: “We need to work to ensure that governments,
health ministries and health economists focus on the value of haemophilia treatment in addition
to cost.”
Expert Meeting on von Willebrand disease on Åland Islands
Thirty-eight experts from 18 countries gathered on the Åland Island Meeting to discuss the treatment
and science of von Willebrand disease on 22nd- 25th September, 2010, on invitation of Prof. Erik
Berntorp, University of Lund, Sweden with financial support from Octapharma .
One of the first patients described with von Willebrand disease was Hjördis S., a little girl from the
Åland Islands, located halfway between Finland and Sweden in the Baltic Sea. In 1924, she was taken
to the doctor because of a history of serious bleeds. Only one year old, she fell and had a nose bleed
for an “unusually long time”. At age three, she fell again and had a cut in her upper lip. After three
days of heavy bleeding, the child became almost unconscious and recovered only after 10 weeks in
bed. Other family members also suffered from severe bleeds. All but two of her 11 siblings had a
history of bleeding. Three of Hjördis’s siblings died at age two and four from uncontrolled bleeds.
Erik von Willebrand published his observations of Hjördis family and other families on Åland islands
what he called “hereditary pseudohaemophilia”, occurring equally in men and women, and showing
EHC Newsletter| NOVEMBER 2010 14