Long-term Effects of Elexacaftor/Tezacaftor/Ivacaftor (Trikafta) on Exertional Symptoms, Exercise Performance, Ventilatory Responses, and Body Composition in Adults With Cystic Fibrosis
Shortness of breath (dyspnea) during exercise is a major source of distress and is a commonly reported symptom in patients with cystic fibrosis (CF). A recent drug treatment option known as Trikafta, which contains elexacaftor, tezacaftor, and ivacaftor, may be used in patients with CF to help improve lung health. However, the effects of this combination therapy on dyspnea and exercise performance, a known predictor of survival in CF, are not clear. The investigators aim to understand the effects of Trikafta on these symptoms and to gain new insight into the potential health improvements in CF from using this treatment option.
• Confirmed diagnosis of CF and at least one copy of the F508del mutation in the cystic fibrosis transmembrane conductance regulator (CFTR) gene
• Plan to initiate Trikafta by the treating physician within 30 days of the enrolment visit
• Aged 19 years or older
• Stable clinical status based on clinical judgment of the treating physician
• Forced Expiratory Volume in 1 second (FEV1.0) \< 90% predicted
• Body mass index greater than 16 or less than 30 kg/m\^2
• Currently non-smoking or a past smoking history of less than 20 pack-years
• Able to read and understand English
• Fully vaccinated (at least 2 doses) for Covid-19