at-a-glance report 2017 - cf-europe · 2019-08-08 · pancreatic enzyme supplements • enzymes...

Cystic Fibrosis in Europe – Facts and Figures 2017

The European Cystic Fibrosis Society Patient Registry (ECFSPR) is happy to present this report with key information about how cystic fibrosis (CF) affects people with CF and their families throughout Europe.

The ECFSPR collects, measures and compares data of people with CF living in Europe and neighbouring countries who agree to be in the registry. The information is important to better understand CF, encourage new European standards of care and treatment, conduct research, and inform public health planning.

If you want to know more about the ECFSPR visit our website.

For more in-depth information see the full report here.

July 2019

At-A-Glance report 2017


https://www.ecfs.eu/ecfspr

http://www.ecfs.eu/sites/default/files/general-content-images/working-groups/ecfs-patient-registry/ECFSPR_Report2017_v1.3.pdf

RU3269

UA181

TR1447

SE686

FR6940

ES2075

PL721

NO254

DE6119

RO167

IT5565

BG155

GR621

LT14

HU507

RS196

AT800

CZ619

IE1284

UK10468

LV41

PT341

HR93

SK294

CH963

MD62

AM33

AL123

MK119

NL1473

DK510

BE1319

IL597

SI112

LU36

At-A-Glance report 2017 2

4MEDIAN AGE ATDIAGNOSIS

months

Proportion children-adults

Map of countriesthat contributed 2017 data to the ECFSPR

• The proportion children-adults varies between the countries.• For some countries only a few individual centres sent data to the ECFSPR.

Genetics

F508del mutation in Europe • F508del is the most common CF-causing mutation in Europe.

• People with CF have two CF-causing mutations, one inherited from the mother and one from the father.

• Homozygous: both mutations are the same.

• Heterozygous: the two mutations are different.


19%41%40% F508del homozygote

F508del heterozygote

Other mutations

48,204registered patients in 35 countries number of patients on map

Children and adults with CF in Europe>=18 Years

<18 Years

Other mutations

F508del heterozygote

F508del homozygote

48.7%Children

51.3%Adults

Alba

nia

Arm

enia

Aust

ria

Belg

ium

Bulg

aria

Croa

tia

Czec

h Re

publ

icDe

nmar

kFr

ance

Ger

man

yG

reec

eH

unga

ryIr

elan

dIs

rael

Ital

yLa

tvia

Lith

uani

aLu

xem

bour

gM

oldo

vaN

ethe

rlan

dsN

orth

Mac

edon

iaN

orw

ayPo

land

Port

ugal

Rom

ania

Russ

ian

Fede

rati

onSe

rbia

Slov

ak R

epub

licSl

oven

iaSp

ain

Swed

enSw

itze

rlan

dTu

rkey

Ukr

aine

Uni

ted

King

dom

Tota

l

0

10

20

30

40

50

60

70

80

90

100

%

Alba

nia

Arm

enia

Aust

ria

Belg

ium

Bulg

aria

Croa

tia

Czec

h Re

publ

icDe

nmar

kFr

ance

Ger

man

yG

reec

eH

unga

ryIr

elan

dIs

rael

Ital

yLa

tvia

Lith

uani

aLu

xem

bour

gM

oldo

vaN

ethe

rlan

dsN

orth

Mac

edon

iaN

orw

ayPo

land

Port

ugal

Rom

ania

Russ

ian

Fede

rati

onSe

rbia

Slov

ak R

epub

licSl

oven

iaSp

ain

Swed

enSw

itze

rlan

dTu

rkey

Ukr

aine

Uni

ted

King

dom

Tota

l

0

10

20

30

40

50

60

70

80

90

100

%

* Lithuania: Coverage is 100% for adults and 0% for children

Median means that50% of the values are above and

50% are below this value.

BMI or body mass index is a measure for nutritional status:

weightheight2

z-score indicates how far a value is from the average (mean) value of the reference population.

BMI

Lung function

Nutrition

• FEV1 is a measure for lung function. It is the maximum amount of air that can be forcefully exhaled in the first second after taking a deep breath.

• FEV1% is a percentage of the average value for healthy people of the same age, gender and height, which is set at 100%.

• In people with CF aged 45 and older there is a higher incidence of mutations causing milder forms of CF. This influences the lung function value for the people with CF in this age group.

• A z-score of 0 means that the BMI is the same as the BMI of healthy children of the same gender and age.

• From the age of 2, children with CF have a lower average BMI than healthy children of a similar age.

• Healthy peers have values for BMI between 18 and 25.

• As people with CF get older BMI increases.

• In adults with CF aged 38 and older there is a higher incidence of mutations causing milder forms of CF. This influences the BMI for the adults in this age group.

Median FEV1%

Median BMI Z-score in children


92%Children

LUNG FUNCTION69%Adults

LUNG FUNCTION92%Children

LUNG FUNCTION69%Adults

LUNG FUNCTION

Pancreatic enzymes

% of people with CF who use pancreatic enzyme supplements

• Enzymes released from the pancreas are vital for the digestion and absorption of fat and vitamins from food. In many people with CF the release of these enzymes is blocked and they must take supplements.


30%

of the people with CF are chronically infected with the Pseudomonas aeruginosa bacteria in their lungs.*

Pseudomonas

30%

83% Pancreatic enzymes 83%

of the people with CF use pancreatic enzyme supplements.*

54

* Missing values are not included nor are data from countries with a high % of missing values.

AlbaniaAustria

BelgiumBulgariaCroatia

Czech RepublicDenmark

FranceGermany

GreeceHungaryIreland

IsraelItaly

LatviaLithuania

LuxembourgMoldova

NetherlandsNorth Macedonia

NorwayPoland

PortugalRomania

Russian FederationSerbia

Slovak RepublicSlovenia

SpainSweden

SwitzerlandTurkey

UkraineUnited Kingdom

Europe

%

0 10 20 30 40 50 60 70 80 90 100

Yes

No

Healthy peers

People with CF

Healthy children

Children with CF

Median BMI in adults Adults with CF

50

60

70

80

90

100

110

45+40-4435-3930-3425-2920-2415-1910-146-9

Age

FEV1

% of

pre

dict

ed

Age

BMI Z

-Sco

re

-0.5

-0.3

-0.1

0.1

0.3

0.5

12-178-112-7

Age

Age

Age

BMI

20.0

20.5

21.0

21.5

22.0

22.5

23.0

38+32-3728-3122-2718-21

Age

Infection

% chronic Pseudomonas aeruginosa in children*

% chronic Pseudomonas aeruginosa in adults*


30%

of the people with CF are chronically infected with the Pseudomonas aeruginosa bacteria in their lungs.*

Pseudomonas

30%

83% Pancreatic enzymes 83%

of the people with CF use pancreatic enzyme supplements.*


6

Inhalation Treatment

Transplantation

% of people with CF who use hypertonic saline*

Number of people with CF living with a lung transplant

% of people with CF who use inhaled antibiotics*


People with CF living with lungtransplantation

FEMALES

5.9%MALES

5.3%

TOTAL

5.6%

People with CF living with livertransplantation

FEMALES

0.4%MALES

0.7%

TOTAL

0.6%

76

AlbaniaAustria

BelgiumBulgariaCroatia

Czech RepublicDenmark

FranceGermany

GreeceHungaryIreland

IsraelItaly

LatviaLuxembourg

MoldovaNetherlands

North MacedoniaNorwayPoland

PortugalRomania



SpainSweden

SwitzerlandTurkey


Europe

%0 10 20 30 40 50 60 70 80 90 100

AlbaniaAustria

BulgariaCroatia

Czech RepublicFrance

GermanyGreece

HungaryIreland

IsraelItaly

LatviaLithuania

LuxembourgMoldova


NorwayPoland

PortugalRomania



SpainSweden

SwitzerlandTurkey


Europe

%0 10 20 30 40 50 60 70 80 90 100

Age

Num

ber

0

25

50

75

100

125

150

175

200

225

250

275

45+40-4435-3930-3425-2920-2415-1910-145-9

Austria

Bulgaria

Croatia

Czech Republic

Denmark

France

Germany

Greece

Hungary

Ireland

Israel

Italy

Latvia

Lithuania

Luxembourg

North Macedonia

Poland

Portugal

Russian Federation

Serbia

Slovak Republic

Slovenia

Spain

Sweden

Switzerland

Turkey

United Kingdom

Europe

%0 10 20 30 40 50 60 70 80 90 100

AlbaniaAustria

BulgariaCroatia

Czech RepublicFrance

GermanyGreece

HungaryIreland

IsraelItaly

LatviaLithuania

LuxembourgMoldova


NorwayPoland

PortugalRomania



SpainSweden

SwitzerlandTurkey


Europe

%0 10 20 30 40 50 60 70 80 90 100

Yes

No

Yes

No

Yes

No

Male

Female

Yes

No

Hypertonic saline is prescribed to clear thick mucus from the lungs.

Inhaled antibiotics are prescribed to treat CF specific bacterial infection, such as chronic Pseudomonas aeruginosa.


Note: Countries with fewer than 5 patients aged 18 years or more are excluded from the graph.


Contact us: [email protected] | www.ecfs.eu/ecfspr

Follow us:Twitter @ECFSRegistry | www.facebook.com/EuropeanCysticFibrosisPatientRegistry/

at-a-glance report 2017 - cf-europe · 2019-08-08 · pancreatic enzyme supplements • enzymes...

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