Physician practices for communicating with patients with cystic fibrosis about the use of noninvasive and invasive mechanical ventilation.

Background: Many patients with advanced cystic fibrosis (CF) lung disease receive intensive treatments such as noninvasive and invasive mechanical ventilation for respiratory failure after little or no communication with physicians.

Methods: Using surveys and follow-up interviews, physicians at two major CF care centers reported their practices for discussing intensive treatment preferences with patients with CF and about barriers and facilitators to communication.

Results: Surveys were completed by 30 (88%) and 26 (76%) of 34 eligible CF physicians who provide care for children (60%), adults (23%), or both (17%).

Family caregiver perspectives on symptoms and treatments for patients dying from complications of cystic fibrosis.

Context: 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.

Objective: We aimed to describe symptom prevalence, symptom management, and frequency of use of disease-specific treatments for patients dying from complications of CF.

Caregiver perspectives on discussions about the use of intensive treatments in cystic fibrosis.

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.

Caregivers' perspectives on decision making about lung transplantation in cystic fibrosis.

Context: 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.

Objective: To assess decision making from the perspective of caregivers of patients who faced the transplant decision before dying of cystic fibrosis or transplant complications.