Abstract
Background: Cystic fibrosis (CF) is a progressive genetic disease characterized by multisystem symptom burden. Specialist palliative care (PC), as a model of care, has been shown to be effective in improving quality of life and reducing symptom burden in other conditions, but has not been tested in CF. Objectives: To develop and test the feasibility and acceptability of a specialist PC intervention embedded within an outpatient CF clinic. Design: Single-site, equal-allocation randomized pilot study comparing usual care with addition of four protocolized quarterly visits with a PC nurse practitioner. Participants: Adults with CF age ≥18 years with any of the following: FEV(1)% predicted ≤50, ≥2 CF-related hospitalizations in the past 12 months, supplemental oxygen use, or noninvasive mechanical ventilation use, and moderate-or-greater severity of any symptoms on the Edmonton Symptom Assessment Scale. Measurements: Randomization rate, intervention visit completion, data completements, participant ratings of intervention acceptability and benefit, and intervention delivery fidelity. Results: We randomized 50 adults with CF of 65 approached (77% randomization rate) to intervention (n = 25) or usual care (n = 25), mean age 38, baseline mean FEV(1)% predicted 41.8 (usual care), and 41.2 (intervention). No participants withdrew, five were lost to follow-up, and two died (88% retention). In the intervention group, 23 of 25 completed all study visits; 94% stated the intervention was not burdensome, and 97.6% would recommend the intervention to others with CF. More than 90% of study visits addressed topics prescribed by intervention manual. Conclusions: Adding specialist PC to standard clinic visits for adults with CF is feasible and acceptable.