The experience of patients with cystic fibrosis during pulmonary exacerbations: a mixed-method study

Abstract

Background: Management of pulmonary exacerbations (PEs) is a pillar of Cystic Fibrosis (CF) care, but little is known about patients’ perspectives. The aim of our study was to explore CF patients’ experience of a PE. Methods: A mixed-method study, using purposive sampling, was undertaken. Quantitative data explored symptom prevalence, controllability and relationships between symptoms, and were analyzed descriptively. Exacerbation experience was explored with semi-structured interviews, and analyzed following Framework Analysis. Results: Eighteen patients (11 males; Median age, 29.5 yrs, range 19-55; Median FEV1 45%, range 23%-105%) participated, with 7 interviewed 3 times. PEs meant being thrust out of normality causing emotional distress. It indicated a period of threat and domination by CF, whilst symptoms and treatment consumed energy, restricted physical activity and daily life roles. Mixed quantitative and qualitative data showed that coughing, breathlessness, lack of energy, pain and anxiety are the ‘core’ symptoms regarding prevalence and distress. Coughing triggered other symptoms. Regarding self-management, the degree of distress, illness beliefs and personal goals influenced help seeking and choice of self-management strategies. Patients managed a broad range of challenges, combining medical and daily demands. In view of outcome, patients aimed at decreasing distress and re-establishing new normality. Conclusions: PEs indicate periods of increased emotional distress and complex self-management. Understanding patients’ experiences during PEs, especially sources of distress and components affecting help seeking, can provide guidance for future self-management support.