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

Abstract

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.

Methods: We conducted semistructured interviews about end-of-life care in CF with bereaved family caregivers and asked questions about symptoms and treatments.

Results: Twenty-seven caregivers answered questions about symptoms and treatments. Caregivers reported that distressing symptoms were common during the last week of life, including dyspnea (100%), fatigue (96%), anorexia (85%), anxiety (74%), pain (67%), and cough (56%). Most caregivers felt that symptom control was "somewhat good." Many reported that medical providers "did the best they could" to manage symptoms but four (15%) recalled no physician inquiry about symptoms. Caregivers expressed beliefs that symptoms could not be controlled and described concerns about side effects and potential for hastening death with the use of opioids and anxiolytics. Patients received numerous disease-specific treatments, and caregivers described many of them as uncomfortable but necessary.

Conclusions: Distressing symptoms are common in dying CF patients, and disease-specific treatments also cause discomfort. Many family caregivers have low expectations for symptom control. This exploratory research can be used to inform clinical interventions to improve symptom management for patients with advanced CF.