at-a-glance report 2016 · transplantation % of people with cf who use hypertonic saline* number of...

Cystic Fibrosis in Europe – Facts and Figures 2016

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.

June 2018

At-A-Glance report 2016


www.ecfs.eu/ecfspr

www.ecfs.eu/sites/default/files/general-content-images/working-groups/ecfs-patient-registry/ECFSPR_Report2016_06062018.pdf

RU3108

TR328

UA172

SE649

FR6713

ES1955

DE5738

RO54

IT5384

BG140

LT14

HU507

PT339

RS193

GR611

AT763

CZ603

IE1276

UK10465

LV39

SK263

CH966

MD59

SI108

NL1449

MK119

DK497

IL663

LU32

BE1282

NO230


47.6%Children

52.4%Adults

2

4.1MEDIAN AGE ATDIAGNOSIS

months

Map of countriesthat contributed 2016 data to the ECFSPR

2

44,719registered patients in 31 countries number of patients on map

Proportion children-adults

• 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.

At-A-Glance report 2016 3

18%41%41% F508del homozygote

F508del heterozygote

Other mutations

Children and adults with CF in Europe>=18 Years

<18 Years

Other mutations

F508del heterozygote

F508del homozygote

%

0

10

20

30

40

50

60

70

80

90

100

Aust

ria

Belg

ium

Bulg

aria

Czec

h Re

publ

ic

Denm

ark

Fran

ce

Ger

man

y

Gre

ece

Hun

gary

Irel

and

Isra

el

Ital

y

Latv

ia

Lith

uani

a

Luxe

mbo

urg

Mac

edon

ia

Mol

dova

Net

herl

ands

Nor

way

Port

ugal

Rom

ania

Russ

ian

Fede

rati

on

Serb

ia

Slov

ak R

epub

lic

Slov

enia

Spai

n

Swed

en

Swit

zerl

and

Turk

ey

Ukr

aine

Uni

ted

King

dom

Euro

pe

Aust

ria

Belg

ium

Bulg

aria

Czec

h Re

publ

ic

Denm

ark

Fran

ce

Ger

man

y

Gre

ece

Hun

gary

Irel

and

Isra

el

Ital

y

Latv

ia

Lith

uani

a

Luxe

mbo

urg

Mac

edon

ia

Mol

dova

Net

herl

ands

Nor

way

Port

ugal

Rom

ania

Russ

ian

Fede

rati

on

Serb

ia

Slov

ak R

epub

lic

Slov

enia

Spai

n

Swed

en

Swit

zerl

and

Turk

ey

Ukr

aine

Uni

ted

King

dom

Euro

pe

%

0

10

20

30

40

50

60

70

80

90

100

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 8, 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

At-A-Glance report 2016 4

91%Children

LUNG FUNCTION68%Adults

LUNG FUNCTION91%Children

LUNG FUNCTION68%Adults

LUNG FUNCTION

4

Healthy peers

People with CF

Healthy children

Children with CF

Median BMI in adults Adults with CF

Age

FEV1

% of

pre

dict

ed

50

60

70

80

90

100

110

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

Age

Age

BMI Z

-Sco

re

-0.5

-0.3

-0.1

0.1

0.3

0.5

12-178-112-7

Age Age

BMI

20.0

20.5

21.0

21.5

22.0

22.5

23.0

38+32-3728-3122-2718-21

Age

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.


31%

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

Pseudomonas

31%

83% Pancreatic enzymes 83%

of the people with CF use pancreatic enzyme supplements.*

5

AustriaBelgiumBulgaria

Czech RepublicDenmark

FranceGermany

GreeceHungaryIreland

IsraelItaly

LatviaLithuania

LuxembourgMacedonia

MoldovaNetherlands

NorwayPortugalRomania

Russian FederationSerbia

Slovak RepublicSlovenia

SpainSweden

SwitzerlandTurkey

UkraineUnited Kingdom

Europe

%

0 10 20 30 40 50 60 70 80 90 100

Yes

No

Infection

% chronic Pseudomonas aeruginosa in children*

% chronic Pseudomonas aeruginosa in adults*


31%

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

Pseudomonas

31%

83% Pancreatic enzymes 83%

of the people with CF use pancreatic enzyme supplements.*

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

66


Czech RepublicDenmark

FranceGermany

GreeceHungaryIreland

IsraelItaly

LatviaLuxembourg

MacedoniaMoldova

NetherlandsNorway

PortugalRomania



SpainSweden

SwitzerlandTurkey


Europe

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

Austria

Bulgaria

Czech Republic

Denmark

France

Germany

Hungary

Ireland

Israel

Italy

Latvia

Luxembourg

Macedonia

Netherlands

Portugal

Russian Federation

Serbia

Slovak Republic

Slovenia

Spain

Sweden

Switzerland

Turkey

Ukraine

United Kingdom

Europe

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

Yes

No

Yes

No

Note: Moldova and Romania have less than 5 patients of 18 years or older, and are therefore excluded from the graph.

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

5.7%MALES

5.2%

TOTAL

5.4%

People with CF living with livertransplantation

FEMALES

0.4%MALES

0.7%

TOTAL

0.6%

7


Czech RepublicFrance

GermanyGreece

HungaryIreland

IsraelItaly

LatviaLithuania

LuxembourgMacedonia

MoldovaNetherlands




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

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

AustriaBulgaria

Czech RepublicFrance

GermanyGreece

HungaryIreland

IsraelItaly

LatviaLithuania

LuxembourgMacedonia

MoldovaNetherlands




SpainSweden

SwitzerlandTurkey


Europe

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

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.* In these graphs the missing values are not included nor

are data from countries with a high % of missing values.

at-a-glance report 2016 · transplantation % of people with cf who use hypertonic saline* number of...

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