The experience of adults with cystic fibrosis using long-term domiciliary non-invasive ventilation

Background: The use of non-invasive ventilation (NIV) in patients with advanced cystic fibrosis (CF) has increased in recent years. Research evidence supports its clinical benefits, but less is known about the patients’ experience of its long-term use in a domiciliary setting.

Objective: To investigate patients’ lived experience of using long-term domiciliary NIV.

Methods: Semi-structured, qualitative interviews were conducted with adults with CF using long-term domiciliary NIV for respiratory failure. The data collected were subject to thematic analysis.

Results: Nine adults (6 female), 5 of whom were awaiting lung transplantation, with a mean age of 39 years and mean FEV1 per cent predicted of 28%, were recruited. Data analysis revealed 2 themes: gratitude, and determination despite challenges. Patients identified some troubling side effects from NIV but were grateful for its symptomatic relief and were determined to continue using it to improve their quality of life.

Conclusions: Participants reported experiences of NIV to be generally positive in terms of symptom relief and quality of life. These findings provide an initial insight into patients’ experience of NIV and have the potential to help guide and improve care.