The use of home Non-Invasive ventilation in Cystic Fibrosis in a large Uk regional paediatric Service.

Background: There are no evidence-based guidelines when to initiate Non-Invasive Ventilation (NIV) in Cystic Fibrosis (CF) in children. Aim: To review the use of NIV in all patients with Cystic Fibrosis, within a large regional paediatric service in UK. Methods: A retrospective case note review of patients within the regional CF service, >300 children (0-18 years,) who have used NIV. For each child DOB, genes, markers of lung health and nutritional status were noted. The date, presence of humidification and mode of initial ventilation was noted. Whether the child had a chest hrCT scan pre NIV setup and any post setup complications were noted. Referral for lung transplant, as well as the current clinical condition on 1/10/2019 was noted. Results: Since 2006, 14 children have used NIV. 1 child9s records were excluded as archived. Of the 13 children with CF, at the time of NIV set up, all had FEV1 Conclusions: Within a large paediatric CF service, NIV has been used infrequently but safely. In our population NIV has been used in those with the most severe disease as a bridge to transplant or for palliation. The role of a CT scan pre-setup needs to be evaluated and the indications for initiation need to be determined.