RECRUITINGOBSERVATIONAL

Trikafta Exercise Study in Cystic Fibrosis

Long-term Effects of Elexacaftor/Tezacaftor/Ivacaftor (Trikafta) on Exertional Symptoms, Exercise Performance, Ventilatory Responses, and Body Composition in Adults With Cystic Fibrosis

About This Trial

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.

Who May Be Eligible (Plain English)

Who May Qualify: - 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 Who Should NOT Join This Trial: - A disease other than CF that could importantly contribute to dyspnea or exercise limitation - Chronic airway infection with Mycobacterium abscessus, Burkholderia cepacia complex, or other organisms with infection control implications based on the treating physicians - Contraindications to clinical exercise testing - Use of supplemental oxygen or desaturation less than 85% with exercise - Diagnosis of pneumothorax in the past 4 weeks - History of organ transplantation Always talk to your doctor about whether this trial is right for you.

Original Eligibility Criteria

View original clinical language

Inclusion Criteria: * 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 Exclusion Criteria: * A disease other than CF that could importantly contribute to dyspnea or exercise limitation * Chronic airway infection with Mycobacterium abscessus, Burkholderia cepacia complex, or other organisms with infection control implications based on the treating physicians * Contraindications to clinical exercise testing * Use of supplemental oxygen or desaturation less than 85% with exercise * Diagnosis of pneumothorax in the past 4 weeks * History of organ transplantation

Treatments Being Tested

DRUG

Trikafta

Elexacaftor/Tezacaftor/Ivacaftor combination therapy

Locations (1)

UBC Centre for Heart Lung Innovation, St. Paul's Hospital

Vancouver, British Columbia, Canada