Causes of death in French cystic fibrosis patients: The need for improvement in transplantation referral strategies!

Abstract

Background: Little data exist on causes of death in cystic fibrosis (CF) patients in the era of lung transplantation.

Methods: Deaths in CF patients in France (2007-2010) were identified using the French CF Registry and causes of deaths were determined based on medical files by a mortality adjudication committee.

Results: Of 256 deaths, half occurred after lung transplantation and were related to early or late complications of transplantation, whereas half occurred in patients who did not receive lung transplantation and were primarily related to respiratory failure or massive hemoptysis. Among patients who did not receive lung transplantation, only 19% died while waiting on a lung transplantation list. Lack of listing for lung transplantation was primarily related to late, or to lack of transplantation referral, rather than to contraindication to transplantation.

Conclusions: These data suggest that improvement in transplantation referral strategies may result in transplantation-related survival benefits.

Keywords: Cystic fibrosis exacerbation; Hemoptysis; Lung transplantation; Respiratory failure.