Impact of elexacaftor/tezacaftor/ivacaftor on geographic disparities in health outcomes for adults with cystic fibrosis in British Columbia

RATIONALE Prior research has demonstrated that people with cystic fibrosis (PwCF) in British Columbia (BC) had worse health outcomes if they traveled >360 min to reach their CF clinic compared to those who traveled shorter distances. The status of geographic disparities in CF has not been assessed since the introduction of the transformative drug elexacaftor/tezacaftor/ivacaftor (ETI) to the BC CF population.OBJECTIVE To compare changes in forced expiratory volume in 1 second (FEV1) and body mass index (BMI) of PwCF treated with ETI stratified by travel time from home residence to the Adult CF Clinic at St. Paul’s Hospital in Vancouver.METHODS This was a retrospective chart review of 174 PwCF attending the Adult CF Clinic at St. Paul’s Hospital and started on ETI. PwCF were divided into 4 travel groups: <45, 45-150, 151-360 and >360 min according to travel time by car to reach the CF Clinic. A Jonckheere-Terpstra test was used to assess trends in FEV1 and BMI change following the first year of ETI according to travel time to the CF clinic.MEASUREMENTS AND MAIN RESULTS There was a positive relationship between travel time to the CF clinic and change in BMI following ETI introduction. A similar trend was found regarding FEV1 but the relationship was not statistically significant. The farthest travel group consistently had the lowest median FEV1 prior to and after starting ETI.CONCLUSION PwCF in BC who traveled the farthest distances to reach their CF Clinic experienced the greatest improvements in BMI following ETI treatment reducing the gap in nutritional outcomes.