07.02.2025
CFTR modulators are small molecules that enhance or restore epithelial transport of chloride and bicarbonate ions in people with certain mutations that cause CF. Currently licensed modulators include ivacaftor, lumacaftor, tezacaftor, elexaftor, and combinations thereof.
Patients taking modulators experience relief, with minimal coughing, reduced mucus production, and difficulty obtaining sputum from the lower lung for pathogenic bacteria. This makes it difficult to detect P.aeruginosa and other dangerous pathogens, both in the chronic course of the disease and during exacerbations.
Inhaled antibiotics are known to be the standard of care for chronic P.aeruginosa infection and are suppressive therapy for other infections, such as Achromobacter and Stenotrophomonas, in patients with cystic fibrosis. To maintain lung function and reduce the frequency of acute pulmonary exacerbations, when taking CFTR modulators, courses of inhaled antibiotics such as colistin, levofloxacin and tobramycin (Bramitob, Tobi) are prescribed.
Inhalation therapy can be prescribed continuously or at monthly intervals. When making decisions about treatment with a particular inhaled antibiotic, the severity of the underlying lung disease, the identified pathogenic bacteria and their titer should be taken into account.
Gene modulators significantly alleviate the condition of patients with cystic fibrosis, but in chronic infections it is recommended to continue treatment with inhaled antibiotics, since there is no long-term data that would indicate that stopping such therapy will not worsen the patient’s condition.
Research on this topic is ongoing, and for now doctors prescribe inhaled antibiotic therapy for patients taking CFTR modulators.
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