Effect of Elexacaftor/Tezacaftor/Ivacaftor Treatment on Extrapulmonary Complications of Cystic Fibrosis.

INTRODUCTION

Benefits of elexacaftor/tezacaftor/ivacaftor (ELX/TEZ/IVA) therapy in people with cystic fibrosis (pwCF) may extend beyond the pulmonary system to extrapulmonary complications. This study compared extrapulmonary complication-related healthcare resource utilization (HCRU) prior to and following initiation of ELX/TEZ/IVA.

METHODS

pwCF aged ≥ 6 years newly initiating ELX/TEZ/IVA on or after 10/21/2019 were identified in the Merative™ MarketScan® Commercial and Multi-state Medicaid Databases. pwCF had continuous coverage with medical and pharmacy benefits for ≥ 1 year prior and following their first prescription fill for ELX/TEZ/IVA treatment. The number of pwCF with ≥ 1 medical claim in any site of care or ≥ 1 inpatient claim with an extrapulmonary condition-related diagnosis and/or procedure code was compared pre- to post-treatment; differences in the proportion of pwCF with ≥ 1 extrapulmonary complication were calculated between the pre- and post-treatment periods.

RESULTS

The study included 1612 pwCF. A relative reduction of 14.0% in the proportion of people with ≥ 1 extrapulmonary complication claim in any site of care was observed from the pre- to post-treatment period [1209 (75.0%) to 1040 (64.5%), p < 0.001]. Within the inpatient setting, a relative reduction of 64.2% in the proportion of patients with ≥ 1 extrapulmonary complication-related hospitalization was observed [397 (24.6%) pre-treatment to 142 (8.8%) post-treatment, p < 0.001]. The relative reductions in the proportion of people with ≥ 1 claim in any site of care were significant for four of the seven extrapulmonary systems [electrolyte (41.2%), upper respiratory (32.8%), nutritional (28.1%), and lower gastrointestinal (17.8%), all p < 0.001]; relative reductions in the inpatient setting were significant for six of the seven extrapulmonary systems [nutritional (77.2%), lower gastrointestinal (67.3%), upper gastrointestinal (63.9%), upper respiratory (62.9%), cystic fibrosis-related diabetes (57.1%), and electrolyte (39.7%), all p < 0.01].

CONCLUSIONS

ELX/TEZ/IVA significantly reduced extrapulmonary complication-related HCRU in CF. Findings suggest a multisystemic benefit of ELX/TEZ/IVA treatment in addition to demonstrated transformational benefits on pulmonary function.