French Cystic Fibrosis Registry - EURORDIS · Cystic Fibrosis Registry. Eurordis - Berlin May 6th 2006 ... 30 40 50 60 70 80 90 100 Number of patients % of adults % of adults

French Cystic Fibrosis Registry

Patient registries Comparing different systems

Eurordis Meeting - Berlin – May 6th, 20061

Eurordis - Berlin May 6th 2006

Category

Observatoire National de la Mucoviscidose : ONMCystic Fibrosis Registry


Objectives

Improve knowledge on medical and social features of the diseaseEvaluate impact of therapeutic interventionImprove information for patients, health professionals, and health authoritiesPlace this database at researchers and physicians disposalAnticipate future needs of patients


Context of creation

French CF registry created in 1992Proposed by the medical council of AFLM (former name of Vaincre la Mucoviscidose)Decided by the board of directors Like US (1966) and Canadian registries1999: new manager = INED

Institut national d’études démographiques


Collection of information

Paper annual questionnaire 4 pages, 44 questions

Filled in by CF centresAnonymouslyElectronic submission in process


« 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

File structure and observational organization


identity

- patient i- patient j- patient k- patient l

data

- patient i- patient j- patient k-

year n

data

- patient i- patient j- patient k- patient l

year n+1

data

- patient i- patient j-- patient l

year n+2

Data collection and longitudinal follow up


Consent

1999: Agreement from CNIL Commission nationale informatique et libertésInformation sheet and consent form : first year of declarationWhen change to adult care


Choice of database

Access: keyboarding and checking of dataSAS: statistical analysis of data


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)…


Security

Declaration and agreement from CNILRestricted access to data only to authorized staff Reserved and locked premise for annual paper questionnaires and data files


Data collected

IdentityName XXX, First Name XXDOB, Sex, Place of birth

DiagnosticDateMolecular biology (2 mutations)Condition (screening, symptoms,…)

Clinical dataAnthropometricRespiratory functionBacteria in sputum

In and outpatient visitsTreatment

IV antibioticNebulised txOxygen, NIV…

ComplicationsMedicalSurgical

TransplantationClinical trialsSocial data

School, workFamily allowance, employment benefit


QUESTIONNAIRE - PAGE 1



Owner / Users

Promoter: French CF association « Vaincre la Mucoviscidose »Partners:

CF centresINED: data managerResearch units


Quality insurance

Coding of dataKeyboarding by 2nd personChecked by data managerCoherence tests


Results 2004

1739

24422168

25242777

29372811

32203388

3580

39284104

4533

1000

1500

2000

2500

3000

3500

4000

4500

5000

1992

1994

1996

1998

2000

2002

2004

Years

Number of patients

0102030405060708090100

% of adultsNumber of patients

% of adults


Results 2004

Men Women

100 80 60 40 20 0 20 40 60 80 100

0

7

14

21

28

35

42

49

56

63

70

77

Number in each age class

Mean age = 15.8


0

510

1520

2530

3540

45

0 10 20 30 40 50 60 70

Anniversary

1994-19962000-20022001-2003

2004: Life expectancy at different ages


30,4 30,932

34

37,139

3839,1

42,4

28,1 27,8 28,129,6

32,2

35,7 35,6 36,438

25

30

35

40

45

1994

-199

6

1995

-199

7

1996

-199

8

1997

-199

9

1998

-200

0

1999

-200

1

2000

-200

2

2001

-200

3

2002

-200

4

Periods

Age (years)

life expectancy at birth

median survival

2004: Life expectancy, median survival


2004: Prevalence

Absolute numbers Prevalence/100 000 inhabitants


Results 2004Age at diagnosis

0

200

400

600

800

1000

1200

1400bi

rth

1-3

mo

4-6

mo

7-12

mo

1 y

2 y

3 y

4 y

5 y

6 y

7 y

8 y

9 y

10 y

11 y

12 y

13 y

14 y

15 y

16 y

17 y

18 y

19 y

20-2

4 y

25-2

9 y

30-3

4 y

35-3

9 y

40 y

and

ove

r

Ages

# of patients

0

20

40

60

80

100

Cumulated % of patients

# of patients

cumulated % of patients


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 € / yearVaincre la Mucoviscidose: 50%INED: 50%


Staff

Gil Bellis (Ined) 100%Evelyne Le Roux (VLM-Ined) 30%Alain Parant (Ined) 20%Marie-Hélène Cazes (Ined) 20%Annie Nourry (Ined) 100%Temporary contract (Ined) 30%

Documents

French Cystic Fibrosis Registry - EURORDIS · Cystic Fibrosis Registry. Eurordis - Berlin May 6th 2006 ... 30 40 50 60 70 80 90 100 Number of patients % of adults % of adults