[Cystic fibrosis: a new disease in adults]. / Fibrosis quística: una nueva patología del adulto.

OBJECTIVE: Cystic fibrosis (CF) has undergone substantial epidemiological changes. The life expectancy of CF patients and the proportion of affected adults are rising because of advances in diagnosis and treatment. In order to find out more about our current situation, we sent an anonymous questionnaire to all affected members of the Catalonian Association of CF. PATIENTS AND METHODS: The questionnaire was sent to 180 patients and their parents asking for information about demographical aspects, diagnosis, pulmonary function, microbiological data, complications, treatment, number of medical visits to CF centers, personal, social and professional development, and the degree of satisfaction with the medical care received. RESULTS: The questionnaire was correctly completed by 75 patients. Of these, 20% were older than 18 years of age. The mean age at diagnosis was 3.25 years. The initial symptoms were: meconium ileus (8%), growth retardation (24%), respiratory problems (34%) and gastrointestinal problems (36%). Sixty percent of the patients showed positive sputum cultures. The most frequently isolated microorganism was P. aeruginosa. Apart form the usual therapy with antibiotics, enzyme replacement and vitamin supplements, other therapeutic measures employed included: alimentary supplements (54%), mucolitics (27%), bronchodilators (45%) and inhaled anti-inflammatories (20%). Complications were present in 56% of the patients and these included hemoptysis, polyps, rectal prolapse, hepatic disease, diabetes, psychological changes, biliary litiasis, pneumothorax, infertility or pancreatitis. The proportion of positive sputum cultures, the type of germ identified and the complications observed changed according to the patient's age. CONCLUSIONS: National registers can be very useful tools to make public health plans. They are also good instruments to evaluate the progress made in the treatment of diseases such as CF.