At-A-Glance report 14 Cystic Fibrosis in Europe Facts and Figures 14 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. vember 16 At-A-Glance report 14
Map of countries that contributed 14 data to the ECFSPR SW 626 IE 1218 PT 295 UK 1581 ES 1538 FR 6369 NL 1378 BE 1186 DK 488 CH 782 DE 53 CZ 599 IT 4981 AT 712 SI 97 LT 12 LV 38 SK 29 HU 557 RO 44 RS 169 MK 19 GR 517 MD 61 UA 147 RU 2122 IL 666 35,582 registered patients in 26 countries number of patients on map MEDIAN AGE AT DIAGNOSIS 3.6 months Children Adults 48.2 51.8 At-A-Glance report 14 2 2
Proportion children-adults The proportion children-adults varies between the countries. For some countries only a few individual centres sent data to the ECFSPR. Children and adults with CF in Europe Belgium is included only in this graph >=18 Years <18 Years 9 7 6 5 4 3 1 Belgium Greece Hungary Italy Europe Genetics F58del mutation in Europe Other mutations F58del heterozygote F58del homozygote F58del is the most common CF-causing mutation in Europe. 9 7 6 5 People with CF have two CF-causing mutations, one inherited from the mother and one from the father. 4 3 1 Greece Hungary Italy Europe Homozygous: both mutations are the same. Heterozygous: the two mutations are different. 41 F58del homozygote 41 F58del heterozygote 18 Other mutations At-A-Glance report 14 3
FEV1 of predicted 11 9 7 6 5 Lung function Median FEV1* 6-9 1-14 15-19 -24 25-29 3-34 35-39 4-44 45+ Age Nutrition Children 9 LUNG FUNCTION Healthy peers People with CF Adults 68 LUNG FUNCTION 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. 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 represented. * In the 14 report we changed to a new way of calculating lung function; this means that, from now on, ECFSPR results can be easily compared with those of other CF registries around the world. BMI Median means that 5 of the values are above and 5 are below this value. BMI or body mass index is a measure for nutritional status: weight height 2 z-score indicates how far a value is from the average (mean) value of the reference population. Median BMI Z-score in children Median BMI in adults 1 23 BMI Z-Score -1 2-7 8-11 12-17 Age Healthy children Children with CF BMI 22 21 19 18-21 22-27 28-31 Age 32-37 38+ Adults with CF A z-score of 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 represented. At-A-Glance report 14 4 4
Pancreatic enzymes 85 Pancreatic enzymes 85 of the people with CF 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. of people with CF who use pancreatic enzyme supplements* Hungary 1 3 4 5 6 7 9 * Missing values are not included nor are data from countries with a high of missing values. At-A-Glance report 14 5
Infection Pseudomonas 3 3 of the people with CF are chronically infected with the Pseudomonas aeruginosa bacteria in their lungs.* chronic Pseudomonas aeruginosa in children* chronic Pseudomonas aeruginosa in adults* 1 3 4 5 6 7 9 1 3 4 5 6 7 9 * Missing values are not included nor are data from countries with a high of missing values. At-A-Glance report 14 6 6
Inhalation Treatment of people with CF who use hypertonic saline* of people with CF who use inhaled antibiotics* 1 3 4 5 6 7 9 1 3 4 5 6 7 9 Hypertonic saline is prescribed to clear thick mucus from the lungs. Inhaled antibiotics are prescribed to treat CF specific bacteria, 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. Number 1 16 14 1 Transplantation Number of people with CF living with a lung transplant Patients living with lung transplantation FEMALES 5.5 MALES 5.1 TOTAL 5.3 Patients living with liver transplantation FEMALES.4 MALES.6 TOTAL.5 6 4 5-9 1-14 15-19 -24 25-29 3-34 35-39 4-44 45+ Males Females At-A-Glance report 14 7
At-A-Glance report 14