Abstract

Objectives: Cystic fibrosis (CF) is the most common autosomal recessive life-shortening disorder in the Caucasians. The aim of this study was to analyse clinical data of Estonian CF patients as of the census date 31.12.2011 and to compare it to the corresponding data for 1993 and 2003 and to the data of the European Cystic Fibrosis Registry for 2003–2007.

Methods: A retrospective study was conducted and demographic data (age, sex, age at diagnosis, CFTR genotype) was available for 46 CF patients. Clinical data (weight, height, body mass index (BMI ), lung function, chronic bacterial colonization and CF related conditions) was available for 40 patients who had been under regular follow-up in 2011.

Results: The mean age of the Estonian CF patients increased from 8.16 (1993) to 15.9 (2011). In 1993 there were no adult patients compared with 39% (18/46) in 2011. The median age at diagnosis for the Estonian patients (1 year 6 months) is still higher than it is in the EU countries. The BMI of only 20% of the Estonian CF patients is above the internationally recommended value. We found a significant decline in lung function in malnourished patients (p < 0.05). There was a statistically significant correlation between chronic P. aeruginosacolonization and lower FEV1 (p < 0.005). The patients with chronic P. aeruginosa colonization had lower BMI (p < 0.05) and FEV1 (p < 0.005) and significantly more bronchiectasis (p < 0.0001).

Conclusions: The median age and survival of the Estonian CF patients increased markedly. A steady decrease was observed in mortality. Regular aggressive treatment plans, availability of modern medication, high standard of physiotherapy and multisystemic treatment approach with centralized follow-up at CF centres have proved to be effective for Estonian CF patients. However, compared to traditional EU countries, median age at diagnosis has still remained high.