Improved lung function using a therapeutic educational multidisciplinary program in a pediatric cystic fibrosis clinic

Background: Pulmonary disease remains the most common cause of morbidity and mortality in cystic fibrosis. The clearing of mucus by chest physiotherapy is a major keystone of treatment. Daily therapy is time consuming, with respiratory therapy, inhalation therapy and a lot of medications to be taken all day long. Treatment burden becomes always a reason for intra-familial conflicts and stress. Adherence to treatment is a main issue, and influences the efficiency of physiotherapy and inhalation Objective: The objective was to implement and evaluate TPE in a pediatric cystic fibrosis consultation. Method: Open interventional study in patient  ≥ 6 years of age. Patients were followed on their regular 3-month basis over 2 years. Interventional education by cystic fibrosis physician and nurse took place at each visit with assessment once yearly. At visit 1, a contract defining the content of the educational program was discussed. Primary endpoint: Evolution of FEV1 and FEF25-75%. Secondary endpoint: Evaluation of disease knowledge and quality of life (CFQR®). At the end of the study we tested the knowledge about nutritional aspects of the disease in the study group. Results: Out of 32 patients from 6 to 16 years, 17 accepted the study. FEV1% predicted increased 5.3% in the intervention group. Conclusion: Using TPE, there is a clear trend to improve lung functions (+2.65% annual rate of change of FEV1% predicted). TPE could be an important strategy to stabilize lung function