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

Clémence Martin; Cécile Hamard; Reem Kanaan; Véronique Boussaud; Dominique Grenet; Michel Abély; Dominique Hubert; Anne Munck; Lydie Lemonnier; Pierre-Régis Burgel

doi:10.1016/j.jcf.2015.09.002

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

J Cyst Fibros. 2016 Mar;15(2):204-12. doi: 10.1016/j.jcf.2015.09.002. Epub 2015 Sep 26.

Authors

Affiliations

¹ Department of Respiratory Medicine, Cochin Hospital, Assistance Publique Hôpitaux de Paris, Paris, France; Université Paris Descartes, Sorbonne Paris Cité, Paris, France. Electronic address: [email protected].
² Department of Respiratory Medicine, Cochin Hospital, Assistance Publique Hôpitaux de Paris, Paris, France; Université Paris Descartes, Sorbonne Paris Cité, Paris, France. Electronic address: [email protected].
³ Department of Respiratory Medicine, Cochin Hospital, Assistance Publique Hôpitaux de Paris, Paris, France; Université Paris Descartes, Sorbonne Paris Cité, Paris, France. Electronic address: [email protected].
⁴ Université Paris Descartes, Sorbonne Paris Cité, Paris, France; Department of Cardiac Surgery, Hôpital Européen Georges Pompidou, Assistance Publique Hôpitaux de Paris, Paris, France. Electronic address: [email protected].
⁵ Department of Respiratory Medicine, Hôpital Foch, Suresnes, France. Electronic address: [email protected].
⁶ Department of Pediatrics A, American Memorial Hospital, Reims, France. Electronic address: [email protected].
⁷ Department of Respiratory Medicine, Cochin Hospital, Assistance Publique Hôpitaux de Paris, Paris, France; Université Paris Descartes, Sorbonne Paris Cité, Paris, France. Electronic address: [email protected].
⁸ Department of Pediatric Gastroenterology and Respiratory Medicine, Hôpital Robert Debré, Assistance Publique Hôpitaux de Paris, University Paris 7, Paris, France. Electronic address: [email protected].
⁹ Vaincre la Mucoviscidose, Paris, France. Electronic address: [email protected].
¹⁰ Department of Respiratory Medicine, Cochin Hospital, Assistance Publique Hôpitaux de Paris, Paris, France; Université Paris Descartes, Sorbonne Paris Cité, Paris, France. Electronic address: [email protected].

PMID: 26391389
DOI: 10.1016/j.jcf.2015.09.002

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.

Publication types

Research Support, Non-U.S. Gov't

MeSH terms

Adult
Cause of Death / trends
Cystic Fibrosis / complications
Cystic Fibrosis / mortality*
Cystic Fibrosis / surgery
Female
Follow-Up Studies
France / epidemiology
Humans
Lung Transplantation*
Male
Prognosis
Referral and Consultation / standards*
Registries*
Respiratory Insufficiency / etiology
Respiratory Insufficiency / mortality*
Respiratory Insufficiency / surgery
Retrospective Studies
Survival Rate / trends
Young Adult