Cystic Fibrosis - long term results of a treatment package including preventive physical exercise

Sammanfattning: Cystic Fibrosis (CF) is a severe hereditary disorder leading to progressive deterioration of lung function and death. This thesis describes the demography of CF in Sweden, its treatment, with special reference to physiotherapy, and its effects on lung function and peak working capacity (PWC). The study is based on Swedish CF patients and evaluates our current CF treatment package, which differs from those in use in other countries in that it includes preventive physical exercise from the day of diagnosis as an essential part of physiotherapy. Airway clearance therapy based upon physical exercise has been used since the early 1980s, when it replaced the previous airway clearance technique, which was postural drainage with percussion and vibrations. Study I used inhaled radioactive aerosols to compare the effect on mucus mobilisation of three different airway clearance techniques, of which one was based upon physical exercise. There were no significant differences between the techniques. We found increased mucus mobilisation in the dependent lung during postural drainage in the lateral position. Ventilation has a central role in mucus mobilisation and we felt that it was justifiable to continue with physical exercise as the basis for airway clearance therapy. Study II evaluated the demography of the Swedish CF population, its mortality rate and social situation. Survival rate for CF patients in Sweden is good, the median age of the population is high, the age at death is high, and the mortality rate is low. 75% of all patients who have finished school are in full or part time work, and 22% of all adults have at least one child. Studies III and IV investigate the physical condition of patients ≥7 years of age. They include summaries of lung function and PWC and their rates of change in different sub-groups over a 3 year period. Lung function and PWC were good in all age groups with no gender difference and with teenagers at least equally good as other age groups. The rate of decline in lung function and PWC was low. The proportion chronically colonised with Pseudomonas aeruginosa was low, but associated with worse lung function and PWC, and a more rapid rate of decline. These studies show that the Swedish treatment package, which includes preventive physical exercise, is good in an international perspective. It was not however possible to evaluate whether there is a mechanism that make PWC influence Pseudomonas colonisation. Further long term studies are desired.