The goal of this clinical trial is to evaluate the safety and tolerability of TMX in adults patients with cystic fibrosis who do not have mutations currently eligible for therapy with modulator drugs.
The main questions it aims to answer is:
. What medical problems do participants have when taking drug TMX?
Participants will:
* Take drug TMX every day for 6 months * Visit the clinic once every 28 days for checkups and tests
Subjects of both sexes, affected by cystic fibrosis, attending the CF Center in Verona
Patients who do not have mutations currently eligible for therapy with CFTR modulator drugs
Age 18 years or older
Predicted forced expiratory volume in 1 second (ppFEV1) ≥ 40% and ≤ 90% (of the predicted value for people of their age, sex, and height) before bronchodilator administration
Patients on any CFTR modulator therapy
Pulmonary exacerbations within 3 weeks before Day 1 (first dose of study drug)
Changes in therapy for lung disease within 3 weeks before Day 1 (first dose of study drug)
Family and/or personal history of thromboembolism and thromboembolic conditions up to 1st-degree relatives