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