Development of a Cystic Fibrosis Primary Palliative Care Intervention: Qualitative Analysis of Patient and Family Caregiver Preferences

Abstract

To prevent or mitigate chronic illness burden, people with cystic fibrosis (pwCF) and their family caregivers need primary (generalist-level) palliative care from the time of diagnosis forward. We used qualitative methods to explore their preferences about a screening-and-triage model (“ Improving Life with CF”) developed to standardize this care. We purposively sampled and interviewed 14 pwCF and caregivers from 5 Improving Life with CF study sites. Thematic analysis was guided by a priori codes using the National Consensus Project's Guidelines for Quality Palliative Care. Participants included 7 adults and 2 adolescents with CF (3 with advanced disease), 4 parents, 1 partner (7 women; 5 people of color). Few were familiar with palliative care. Illness burden was described in multiple domains, including physical (e.g., dyspnea, pain), psychological (e.g., anxiety), and social (e.g., family well-being; impact on work/school). Most preferred survey-based screening with care coordination by the CF team. Preferences for screening approaches varied. PwCF and caregivers experience illness burden and are receptive to a CF-team delivered primary palliative care screening-and-triage model with flexible processes.

Addressing Symptom Burden and Palliative Care Needs in Cystic Fibrosis: A Narrative Review of the Literature

“…The most prevalent physical symptoms are cough, shortness of breath, fatigue, sinus discharge, difficulty sleeping, and pain [1,5,11,12]. Cough, shortness of breath, sinus discharge, and fatigue often are rated as the most severe or distressing symptoms [1,5,11,25]. Additionally, initial data from one trial in 61 adults with CF found that a significant portion of participants reported cough and fatigue as highly distressing (i.e., "quite a bit" or "very much" distressing) at baseline, as well as at 3-, 6-, and 9-month follow-up assessments [26].…”