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An overview of 40 years Hemophilia in the Netherlands
Iris Plug, Frits Rosendaal & Cees Smit
Department of Clinical Epidemiology
Leiden University Medical Center
BBMRI, Paris, December 15, 2009
The history of Hemophilia in the Netherlands
1972 student project in Leiden
Hemophilia in The Netherlands: results of a survey on the medical,genetic
and social situation of Dutch hemophiliacs. Acta Medica Scandinavica, 1974
Leids Dagblad 1972
Dr J. Veltkamp
Questionnaires
• 3994 questionnaires sent
• 2981 patients participated
• In 2001 192 questions to be answered
• Over 50 publications
• 5 patient reports
Hemofilie in Nederland-5
2001
Landelijk onderzoek onder mensen met hemofilie
Participants
0
200
400
600
800
1000
1200
1972 1978 1985 1992 2001
447
560
935980
1066
Changes in treatment
0
20
40
60
80
100%
1972 1978 1985 1992 2001
prophylaxis
home treatment
(severe hemophilia)
Hospital admissions
5146
2125
18
0
10
20
30
40
50
60%
1972 1978 1985 1992 2001
Number of days in hospital
0
5
10
15
20
25
30aantal nachten
1972 1978 1985 1992 2001
ernstig
matig
Annual number of hemorrhages
0
5
10
15
20
1972 1978 1985 1992 2001
0-16 yr
>25 yr
(severe hemophilia)
66 7361
7459
76
0
20
40
60
80
100j r
1986 1992 2001
severe
mild
NL-man
Life expectancy
Time line
1985
1992
2001
Genetic counselling
HIV
Well being
Mortality in mothers of hemophilia patients
Social functioning
Hepatitis C
Bleeding problems in carriers
mortality
mortality
The use of recombinant products
2005
mortality
Female relatives of men with hemophilia
Possible risk moments during life
• Bleeding independent of clinical interventions
• Bruising, nose-bleeding
• Bleeding after medical interventions
• Tooth extractions, surgery, tonsillectomy
• Bleeding related to delivery
• During pregnancy, postpartum
• * with informed consent, linkage of data of patients with carriers in
their families and with medical data about their carrier status
Bloedingsproblemen bij draagsters van hemofilie A of B
Een HiN-5 deelstudie
Onderzoek onder vrouwen die getest zijn op draagsterschap
van hemofilie A of B
Methods
Clinical implications
• Female relatives of men with hemophilia need to be informed:
• Possibility of being a carrier • 47% not aware of possibility of being a carrier*
• 25% not familiar with the possibility of testing
• Possibility of bleeding problems and the importance of knowing
your factor VIII or IX activity• In 50% of carriers in our study factor VIII of IX unknown
*Data from studentproject Marijke den Uyl, AMC, 2004
Expectations for the future: HiN-6 in 2010/2011
• Stronger link between mail survey and patient data hospitals
• A gradual development to a biobank
• Cooperation between hospitals is a need, it gradually
becomes better
• Financing is a problem
• (Preventiefonds→ ZonMw, not innovative enough)
• Financing haemophilia society + LUMC
• Possibility of a European grant (ageing)
Acknowledgements
University Medical Center UtrechtVan Creveldclinicdr E.P. Mauser-BunschotenDepartment of Medical Geneticsdr J.K. Ploos van Amstel
Leiden University Medical CenterDepartment of Clinical Epidemiologyprof. dr F.R. Rosendaaldr J.G. van der Bomdr,. Iris Plug
Department of Clinical Genetics dr A.H.J.T. Vriendsdr J.E.M. van Diemen-Homandr M. Losekoot
Department of Clinical Epidemiologyprof. dr F.R. Rosendaaldr J.G. van der Bom
Dutch Hemophilia Society (NVHP)drs J. Willemsedr. Cees Smit
Financial supportHaemophilia Foundation