Use Of Central Venous Access Devices (CVAD) And Associated Outcomes In Hereditary Angioedema (HAE) Patients: Findings From A Large United States (US) Claims Database

RATIONALE: Patients using intravenous C1-esterase-inhibitor (C1-INH) for routine prevention of Hereditary Angioedema (HAE) may have treatment difficulty due to vascular access issues; some patients require long-term CVAD placement for ongoing therapy. This observational study determined the prevalence of CVAD use and associated complications in United States (US) HAE patients using intravenous C1-INH in a real‑world setting.

METHODS: Retrospective cohort study using LifeLink^TM Health Plan database records between January 1, 2006 and December 31, 2014. Health care resource utilization (HCRU) data for patients with HAE and ≥1 claim for a HAE-specific medication were analyzed.

RESULTS: 18/434 (4.1%) patients treated with C1-INH(IV) (Cinryze® and/or Berinert®) had a CVAD placed. 10/18 (55.6%) experienced at least 1 CVAD-related complication, among whom there were 23 separate claims such as infections, mechanical complications, and complications not otherwise specified. 7/10 (70%) experienced ‘other complications’ due to other vascular device, implant, and graft. 5/18 (27.8%) patients with CVADs required hospitalization and 14/18 (77.8%) required an emergency department (ED) visit, compared with 79/521 (15.2%) and 271/521 (52.0%), respectively, among patients without a CVAD. The adjusted rate ratio of combined hospitalization and ED visits with a CVAD was 2.6 (95% CI: 0.17, 39.23; p-value: 0.492) compared with no CVAD.

CONCLUSIONS: The prevalence of CVAD use among US HAE patients receiving intravenous C1-INH therapy was found to be 4.1%. A majority of patients with CVADs had device-related complications. Hence, non-intravenous HAE prophylaxis options should be considered in HAE patients with vascular access failure due to the complication risk of a CVAD placement.