AbstractObjectivesTelehealth and home spirometry feasibility for children has been established, but their impact on cystic fibrosis (CF) disease progression remains unassessed. We aimed to evaluate the effects of telehealth and home spirometry on CF disease progression and care.MethodsChildren with CF aged 5–17 years from all Swedish CF centers were provided with home spirometers. A minimum of two in‐person visits were replaced with telemedicine visits and participants were instructed to conduct home spirometry before visits. Linear mixed‐effects models were used to compare annual CF disease trajectories during the intervention period and prepandemic period (1 January 2019 to 28 February 2020). Participants and caregivers completed study questionnaires.ResultsA total of 59 individuals completed the study over a mean (SD) period of 6.8 (1.4) months, made 3.1 (1.0) physical visits and 2.2 (0.6) telehealth visits per patient year during the study period. The mean difference (95% CI) between the intervention and prepandemic period progression rate for FEV1%, lung clearance index and BMI were −0.4 (−1.3 to 0.5, p = 0.39), 0.11 (−0.07 to 0.28, p = 0.25) and −0.02 (−0.13 to 0.08, p = 0.70), respectively. There were no major shifts in the incidence of airway pathogens, sputum cultures, or antibiotics use between the periods (p > 0.05). The intervention did not increase stress. Almost all participants and caregivers expressed a desire to continue with home spirometry and telemedicine.ConclusionCombining telehealth and physical visits with access to home spirometry demonstrated comparable effectiveness as exclusively in‐person care with enhanced flexibility and personalization of CF care.