Background: Advanced cystic fibrosis lung disease (ACFLD) is common, is associated with reduced quality of life, and remains the most frequent cause of death in individuals with cystic fibrosis (CF). These consensus guidelines provide recommendations to the CF community on management of both common and unique issues that arise when individuals reach a state of ACFLD. Methods:The CF Foundation assembled a multidisciplinary expert panel consisting of three workgroups: Pulmonary management; Management of comorbid conditions; Symptom management and psychosocial issues. Topics were excluded if the management considerations did not differ in ACFLD from in the overall CF population or if already addressed in other published guidelines. Recommendations were based on a systematic literature review combined with expert opinion when appropriate. Results:The committee formulated twenty-three recommendation statements specific to ACFLD that address the definition of ACFLD, pulmonary and intensive care unit management, management of selected comorbidities, symptom control, and psychosocial issues.

Background As life expectancy for cystic fibrosis (CF) has increased in recent decades, more individuals with CF are becoming parents. The objectives of this study were to describe the parenting and reproductive health concerns of individuals with CF and to identify the psychosocial and educational needs related to parenthood with CF. Methods Twenty adults with CF, including parents and non‐parents, participated in one‐on‐one, semi‐structured interviews about reproductive health and parenting. Questions pertained to reproductive health knowledge, psychosocial adaptation to CF related to fertility and parenthood, parenting concerns in the context of CF, and psychosocial care needs. We performed thematic content analysis on interview transcripts and descriptive statistical analysis on participant demographics and health variables. Results A majority of participants (ten women and ten men, of whom half were parents) described their health as “stable” and “good/fair”; median FEV 1 was 66% predicted (range, 30‐105). Participants shared a range of experiences related to reproductive health discussions with CF care providers and expressed concerns about pregnancy, infertility, and adoption. Parents and non‐parents expressed concerns about balancing roles as parent and patient, the impact of anticipated health decline and early mortality on children, and communication with children. Participants identified a need for earlier, improved education for potential parents and resources for parents with CF. Conclusions Individuals with CF may not receive sufficient CF‐related reproductive health education, and they have wide‐ranging concerns about the intersecting roles of patient and parent. Results from this study can provide guidance for CF care providers to improve their understanding and response to the needs of individuals and families affected by CF.

ACP 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.

CF-related health promotion efforts may benefit from addressing the impact of friendships on adherence and quality of life.

Context 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. Objectives We aimed to describe caregivers’ perspectives on decision making for the use of intensive treatments for patients with advanced CF lung disease. Methods We conducted semi-structured interviews with 36 caregivers of 36 patients who died of CF about treatment preference discussions and solicited recommendations for improving discussions. Results Twenty (56%) patients received intensive treatments during the last week of life. Twenty-two (61%) caregivers reported ever having discussed intensive treatment preferences with a physician, and 17 (77%) of these discussions were initiated during an acute illness. Only 14 (39%) of all patients participated. Caregivers expressed less certainty about consistency of treatments with patient preferences when patients did not participate. Twenty-nine (81%) caregivers endorsed first discussing treatment preferences during a period of medical stability. Conclusions Discussions about preferences for the use of intensive treatments for patients with CF often take place during episodes of acute illness and may be delayed until patients themselves are too ill to participate. Bereaved caregivers suggest first addressing intensive treatment preferences during a stable period so that patient preferences are understood and unwanted treatments are minimized.