Abstract
The recovery process and quality of life with treatment of elexacaftor/tezacaftor/ivacaftor we observed in 32-year-old male cystic fibrosis (CF) patient who showed signs of advanced disease are quite promising. Although our case was receiving 8 L/min oxygen his saturation was still around 80%. Patient was started on triple combination CF transmembrane regulator (CFTR). In the follow-up, the shortness of breath with effort decreased. He became much easier to do his daily work. The patient, who was 51.3 kg at the beginning of the treatment, became 71 kg after 1 year. While forced vital capacity (FVC) was 29%, forced expiratory volume in one second (FEV1) 19% at beginning of treatment, FVC was 62%, FEV1 37% after 1 year of treatment. The SO2 value in room air was 94% after 1 year of treatment. Overcoming the high price, reimbursement problems experienced in all countries will increase using of CFTR modulators by the patients with having compatible genetic characteristics.
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