AbstractBackgroundStudies in cystic fibrosis (CF) report late attention to advance care planning (ACP). The purpose of this study was to examine ACP with patients receiving care at US adult CF care programs.MethodsChart abstraction was used to examine ACP with adults with CF dying from respiratory failure between 2011 and 2013.ResultsWe reviewed 210 deaths among 67 CF care programs. Median age at death was 29years (range 18–73). Median FEV1 in the year preceding death was 33% predicted (range 13–100%); 68% had severe lung disease with FEV1p=pp=0.55). The frequency of ACP varied significantly among the 29 programs contributing data from four or more deaths.ConclusionsACP in CF often occurs late in the disease course. Important decisions default to surrogates when opportunities for ACP are missed. Provision of ACP varies significantly among adult CF care programs. Careful evaluation of opportunities to enhance ACP and implementation of recommended approaches may lead to better practices in this important aspect of CF care
