The General Approach to Cystic Fibrosis Pulmonary Infection in Greece

Summary

Cystic Fibrosis is the second most common autosomal recessive genetic disorder in Greece next to Mediterranean Anaemia (thalassaemia). There are about 50–60 affected neonates expected each year. The carrier frequency is estimated at 5% of the general population. There are 287 CF patients attending the CF Centre which was started in 1966 at the Aghia Sophia Children’s Hospital in Athens. 25% of the patients are older than 16 years and the oldest known patient in Greece is 46 years old. From a group of 211 patients regularly followed at the Centre 101 are free from microbial colonization and 110 have positive sputum culture, in 88 of which P. aeruginosa has been cultured. B. cepacia has never been cultured in this series. Therapeutic approach to chest infection consists of oral or IV antibiotics according to the sensitivity of microorganisms cultured in the sputum; home IV therapy is not applied. Some patients are given antibiotics by inhalation and all have intensive physiotherapy. In the majority of patients clinical progress is good: as evaluated by the Shwachman-Kulczycki score 73% are in very good or excellent condition and only 11.8% are in moderate or poor condition. School-age children are attending regular school with low absence rate and good performance. Some of the adult patients are pursuing higher education and the rest are engaged in various professions. Our experience is that the introduction of a programme of aggressive and intensive therapy has had a positive effect on survival and clinical condition and has reduced hospitalization.