French Cystic Fibrosis Registry Patient registries Comparing different systems
Eurordis Meeting - Berlin – May 6th, 2006
1
Category Observatoire National de la
Mucoviscidose : ONM Cystic Fibrosis Registry
Eurordis - Berlin May 6th 2006
Objectives Improve knowledge on medical and
social features of the disease Evaluate impact of therapeutic intervention Improve information for patients, health professionals, and health authorities Place this database at researchers and physicians disposal Anticipate future needs of patients Eurordis - Berlin May 6th 2006
Context of creation French CF registry created in 1992 Proposed by the medical council of
AFLM (former name of Vaincre la Mucoviscidose) Decided by the board of directors Like US (1966) and Canadian registries 1999: new manager = INED Institut
national d’études démographiques
Eurordis - Berlin May 6th 2006
Collection of information Paper annual questionnaire 4
pages, 44 questions
Filled in by CF centres Anonymously Electronic submission in process
Eurordis - Berlin May 6th 2006
File structure and observational organization « identity file » - n° ONM - sex - date of birth - place of birth (zip code)
fonctions : - permanent - duplicate file control
« data file » - n° ONM - n° of centre - sex - Year of birth - Zip code birth - Zip code living place ------------------------ Diagnostic data - Medical data - Therapeutic data ...
fonctions : - proper to each year - statistical analysis Eurordis - Berlin May 6th 2006
Data collection and longitudinal follow up identity - patient i - patient j - patient k - patient l
data - patient i - patient j - patient k -
year n
data
data
- patient i - patient j - patient k - patient l
- patient i - patient j - patient l
year n+1
year n+2
Eurordis - Berlin May 6th 2006
Consent 1999: Agreement from CNIL
Commission nationale informatique et libertés Information sheet and consent form : first year of declaration When change to adult care
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Choice of database Access: keyboarding and checking of
data SAS: statistical analysis of data
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Methods to analyse the data Descriptive studies: structure of the
population, incidence, mortality rate,... Etiologic studies: case-control
studies, (eg: pregnancy, NIV: each patient matched with patient same age, same weight, same centre…) cohort studies,...(eg: patients diagnosed after neonatal screening, patients with diabetes, followed several years) … Eurordis - Berlin May 6th 2006
Security Declaration and agreement from CNIL Restricted access to data only to
authorized staff Reserved and locked premise for annual paper questionnaires and data files
Eurordis - Berlin May 6th 2006
Data collected Identity
Name XXX, First Name XX DOB, Sex, Place of birth
Diagnostic
Date Molecular biology (2 mutations) Condition (screening, symptoms,…)
Clinical data
Anthropometric Respiratory function Bacteria in sputum
In and outpatient visits Treatment
IV antibiotic Nebulised tx Oxygen, NIV…
Complications
Medical Surgical
Transplantation Clinical trials Social data
School, work Family allowance, employment benefit
Eurordis - Berlin May 6th 2006
QUESTIONNAIRE - PAGE 1
Eurordis - Berlin May 6th 2006
Eurordis - Berlin May 6th 2006
Owner / Users Promoter: French CF association
« Vaincre la Mucoviscidose » Partners: CF
centres INED: data manager Research units
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Quality insurance
Coding of data Keyboarding by 2nd person Checked by data manager Coherence tests
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Results 2004 Number of patients 5000
% of adults
Number of patients % of adults
4533
4500
4104 3928
4000
3580 3388 3220
3500
2937 2811 2777 2524
3000
2442 2168
2500 2000
1739
1500
Years Eurordis - Berlin May 6th 2006
2004
2002
2000
1998
1996
1994
1992
1000
100 90 80 70 60 50 40 30 20 10 0
Results 2004 77 Men
Women
70 63
Mean age = 15.8
56 49 42 35 28 21 14 7 0 100
80
60
40
20
0
20
40
Number in each age class
Eurordis - Berlin May 6th 2006
60
80
100
2004: Life expectancy at different ages 45 40 35
1994-1996
30
2000-2002
25
2001-2003
20 15 10 5
Anniversary Eurordis - Berlin May 6th 2006
70
60
50
40
30
20
10
0
0
2004: Life expectancy, median survival Age (years) 45 42,4
40
39 37,1 35,7
35
39,1
38
38
36,4
35,6
34
30
30,4
30,9
28,1
27,8
32,2
32
life expectancy at birth
29,6 28,1
median survival
Periods
Eurordis - Berlin May 6th 2006
2002-2004
2001-2003
2000-2002
1999-2001
1998-2000
1997-1999
1996-1998
1995-1997
1994-1996
25
2004: Prevalence
Absolute numbers
Prevalence/100 000 inhabitants
Eurordis - Berlin May 6th 2006
Results 2004 Age at diagnosis Cumulated % of patients
# of patients
100
1400 1200
80 1000 60
800 600
40 # of patients
400 cumulated % of patients
20
200 0
Eurordis - Berlin May 6th 2006
35-39 y
30-34 y
25-29 y
20-24 y
19 y
18 y
40 y and over
Ages
17 y
16 y
15 y
14 y
13 y
12 y
11 y
10 y
9y
8y
7y
6y
5y
4y
3y
2y
1y
7-12 mo
4-6 mo
1-3 mo
birth
0
Cost and funding sources Staff: 170 000 € Scientists: 1.4 FTE , Technicians: 1 FTE “temporary contracts” 0.3 FTE
Compensation for questionnaires: 80 000 €
15 € / questionnaire
Missions, investment : 20 000 € Total: 270 000 € / year Vaincre la Mucoviscidose: 50% INED: 50%
Eurordis - Berlin May 6th 2006
Staff
Gil Bellis (Ined) Evelyne Le Roux (VLM-Ined) Alain Parant (Ined) Marie-Hélène Cazes (Ined) Annie Nourry (Ined) Temporary contract (Ined)
Eurordis - Berlin May 6th 2006
100% 30% 20% 20% 100% 30%
