Advance care planning in cystic fibrosis: Current practices, challenges, and opportunities

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

Effects of lung transplantation on inpatient end of life care in cystic fibrosis

The impact of lung transplantation on end of life care in cystic fibrosis (CF) has not been widely investigated

Family Caregiver Perspectives on Symptoms and Treatments for Patients Dying From Complications of Cystic Fibrosis

Most patients with advanced cystic fibrosis (CF) die from respiratory failure and experience distressing symptoms as lung disease progresses. Little has been reported about symptom management and the continuation of disease-specific treatments near the end of life for patients with CF

Caregiver Perspectives on Discussions About the Use of Intensive Treatments in Cystic Fibrosis

Intensive treatments intended to sustain life are often used for patients with advanced cystic fibrosis (CF). There are no guidelines for selecting patients whose survival and quality of life may be enhanced by such treatments or for communication with patients and caregivers about possible treatment outcomes

Caregivers' Perspectives on Decision Making about Lung Transplantation in Cystic Fibrosis

Lung transplantation extends survival for some patients with advanced cystic fibrosis, but it is complicated, has many potential risks, and its outcomes are difficult to predict. No standards exist for informed decision making about transplantation