Abstract
BackgroundCystic Fibrosis (CF) Centers are involved in the decisions regarding the eligibility of CF patients with end-stage lung disease and timing for inclusion on waiting lists (WL) for lung transplantation (LT). There are currently no data on the mortality rates of Italian CF patients on WL and during the first year after LT and we aimed to assess these outcomes by surveying the CF Centers.MethodsA survey was sent to Italian CF Centers which were requested to report the age at which all CF subjects included on the WL between 2010 and 2014 were included on the list, admitted to either standard or urgent LT, or had died either while on the WL or within the first 3 and 12 months after LT. All outcomes were recorded by December 31, 2015.ResultsTwo hundred fifty-nine CF subjects were included on the WL during the 5-year study period. The mortality rate during the WL was 19.3% and was not associated with sex, age at inclusion on the WL or standard or urgent access to LT. 159 (61.4%) subjects underwent LT, 46 (28.9%) with urgent procedure. Deaths within the first 3 and 12 months after LT were significantly more prevalent in individuals who underwent urgent LT compared to those with standard LT (p < 0.01).ConclusionsThe mortality of Italian CF patients, included in our survey, was about twice that reported by the National Transplant Center for all LT indications, including CF, during the same time period and despite the introduction of urgent LT. The latter was associated with an unfavorable early outcome compared to standard LT.
Highlights
Cystic Fibrosis (CF) Centers are involved in the decisions regarding the eligibility of CF patients with end-stage lung disease and timing for inclusion on waiting lists (WL) for lung transplantation (LT)
We considered the number of LTs and age at the surgical procedure recorded by the Italian Cystic Fibrosis Register (ICFR) in Italy during 2010–2014, to compare these data with those of the CF subjects included in our study [11, 13]
In conclusion, despite some limitations, this study is the first attempt to evaluate mortality of Italian CF patients while they are on the waiting list for lung transplantation and within 3 and 12 months after they have received either urgent or standard LTs
Summary
Cystic Fibrosis (CF) Centers are involved in the decisions regarding the eligibility of CF patients with end-stage lung disease and timing for inclusion on waiting lists (WL) for lung transplantation (LT). Lung transplantation (LT) is offered to subjects with cystic fibrosis (CF) with end-stage lung disease as a potential life-extending and life-improving treatment [1, 2]. Different predictive models for mortality have been proposed but the optimal timing for listing of CF individuals for LT is a complex task for the CF specialist [3,4,5]. Italian CF Centers are involved in LT programs, by determining the timing for inclusion on the WL and by caring for patients while they await surgery. Listing criteria were the same as those suggested in the international literature [1, 2]
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