Efficiency of regular follow-up in patients with cystic fibrosis in the Moscow region

Over the past 40 years, the mean survival age of patients with cystic fibrosis (CF) has increased, driven by advances in diagnosis and treatment. This progress has also been linked to the level of care provided in CF treatment centers.

Aim. To demonstrate the effectiveness of monitoring in a special­ized department/center providing care for patients with CF.

Methods. This study of regular follow-up and microbiological monitoring included 193 patients with cystic fibrosis aged 0 to 17.9 years from the Moscow region. Data for the period from December 2021 to December 2024 were compared. Data from the Russian Federation Cystic Fibrosis Patient Registry for 2021 - 2023 were used in the comparative analysis of the patient health status.

Results. Improved pulmonary function was demonstrated in patients with CF in the Moscow region. Forced expiratory volume (FEV₁) significantly increased from 88.15 ± 20.64%_pred at the end of 2021 to 93.1 ± 18.7%_pred at the end of 2024 (M ± SD; p < 0.00001). Forced Vital Capacity (FVC), increased from 93.23 ± 16.40% to 95.4 ^.± 15.2% in 2024 (M ± SD; p < 0.000^.01). The number of days of intravenous therapy for exacerbations of the bronchopulmonary process decreased from 6 ± 12.25 to 1.3 ± 7.16 (p < 0.00001). Between 2021 and 2024, the proportion of children chron­ically infected with Pseudomonas aeruginosa in the Moscow Region decreased from 18 % to 7.3% against active follow-up care. Similarly, the num­ber of patients with recurrent P. aeruginosa growth decreased from 17.67% to 6.16%. The number of patients with Methicillin-susceptible Staphylococcus aureus (MSSA) growth increased from 76% to 89.2%. Fewer patients tested positive for Methicillin-resistant S. aureus (MRSA) - 8% in 2021 and 3.14% in 2024. The number of patients with Burkholderia cepacia complex (Bcc) growth increased in 2022 compared to 2021, but no Bcc growth was detected in 2024. The number of patients with Achromobacter spp. decreased from 3.03% to 0.52%.

Conclusion.The key disease outcomes vary sig­nificantly in CF patients with different levels of healthcare. CF patients who were followed in specialized centers had better outcomes than patients who were not regularly followed and lacked access to continuous microbiological monitoring. It is necessary to establish cystic fibrosis centers that meet international standards in all regions of the Russian Federation.

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