Pulmonary exacerbations of CF are an important cause for the experienced disability of patients, respiratory symptoms, and decreases in lungfunction, which require antibiotic therapy at home or in the hospital. Therefore, prevention of exacerbations in CF is important. The aim of this study was to assess the predictive properties of inflammatory markers in exhaled breath for pulmonary exacerbations in children with CF. In addition the reliability of home monitor assessments of symptoms and lungfunction was investigated.

Inclusion Criteria:

• CF disease is defined as the combination of:

  1. characteristic clinical features (persistent pulmonary symptoms, meconium ileus, failure to thrive, steatorrhoea);
  2. and/or abnormal sweat test (Chloride > 60mM);
  3. and/or two CF mutations.

Exclusion Criteria:

1. cardiac abnormalities;
2. mental retardation;
3. no technical satisfactory performance of measurements;
4. on the waiting list for lung transplantation;
5. non-compliance with the home-assessments;
6. patients with Burkholderia Cepacia;
7. participation in another intervention trial.

Children with Cystic Fibrosis